Chronic Dyspnea Noncardiovascular Origin. MRI Chest There are limited data supporting the use of MRI for the central airways in adult populations [42,43]. Some data supporting the use of MRI in pediatric populations [44,45] may be applicable in adults.


Chronic Dyspnea Noncardiovascular Origin. FDG-PET/CT Skull Base to Mid-Thigh FDG-PET/CT has a supplemental role in the staging of tracheal neoplasms; however, there are no data to support its use in other tracheal conditions.


Chronic Dyspnea Noncardiovascular Origin. US Chest One small study has shown that measurements obtained from US imaging of the extrathoracic trachea correlate well with MRI [46]; however, a transthoracic US technique is limited by its inability to evaluate the entire tracheobronchial tree.


Chronic Dyspnea Noncardiovascular Origin. Chronic Dyspnea-Noncardiovascular Origin Variant 4: Chronic dyspnea. Suspected interstitial lung disease. Initial imaging. Radiography Chest The chest radiograph may be abnormal in diffuse ILD; however, studies documenting the sensitivity of chest radiographs predate the widespread use of CT [47]. A normal chest radiograph in the setting of suspected ILD does not exclude the possibility of clinically important ILD. In subjects with diseases that predispose them to ILD (eg, connective tissue disease), it is reasonable to consider CT rather than radiography as the primary screening modality.


Chronic Dyspnea Noncardiovascular Origin. CT Chest CT is currently the preferred imaging method for evaluating ILD [48]. CT protocols should be tailored to the clinical setting and may include expiratory or prone imaging. Good correlation has been reported between extent of disease on CT and severity of dyspnea [49-53]. CT findings are often sufficient to permit either a limited differential or confident diagnosis; the latter occurs particularly in the diagnosis of usual interstitial pneumonia [54-57], although the diagnostic yield improves with multidisciplinary discussion [58,59]. There is moderate agreement across individuals in the rating of honeycomb change [60]. The presence and extent of honeycomb change and other imaging features of ILD may serve as important prognostic variables [61,62].


Chronic Dyspnea Noncardiovascular Origin. MRI Chest MRI does not currently have an established clinical role in the evaluation of ILD, although small studies have shown good concordance with CT [63-65]. In general, MRI does not yet display the same level of parenchymal detail that is available with CT. A specific advantage of MRI may exist when a single examination is desired for the evaluation of both ILD and its effect on the cardiovascular system [66].


Chronic Dyspnea Noncardiovascular Origin. FDG-PET/CT Skull Base to Mid-Thigh FDG-PET/CT may have a secondary role in ILD evaluation. It can be used as a marker of disease extent and severity in sarcoidosis [67], reveal inflammatory activity before morphological changes are demonstrated on CT [68], and assist in follow-up and the monitoring of treatment response [10]. Some studies show that the degree of FDG activity correlates with severity and prognosis in ILD [69-71].


Chronic Dyspnea Noncardiovascular Origin. US Chest US has been evaluated as a potential ILD screening tool in high-risk populations. In scleroderma, US was concordant with CT in 83% of patients and demonstrated high sensitivity [72]. It is being used in some centers to detect chronic ILD [73,74] and is emerging as a monitoring tool [75].


Chronic Dyspnea Noncardiovascular Origin. Variant 5: Chronic dyspnea. Suspected disease of the pleura or chest wall. Initial imaging. Radiography Chest Pleural effusion is often diagnosed by a chest radiograph, and volume can reasonably be estimated [76]. A radiograph is somewhat limited in its ability to determine the exact location of an abnormality, whether parenchymal, pleural, or extrapleural. A chest radiograph may reveal structural abnormalities of the sternum, ribs, and thoracic spine that may predispose toward dyspnea.


Chronic Dyspnea Noncardiovascular Origin. CT Chest CT is superior to radiographs in detecting and characterizing pleural disease, differentiating it from parenchymal and chest wall disease, and determining the extent of involvement [77]. CT is somewhat limited in its ability to differentiate causes of pleural effusion, although the presence of pleural thickening or enhancement may help document complex exudative and malignant effusions [78,79].


Chronic Dyspnea Noncardiovascular Origin. MRI Chest MRI may provide improved characterization and assessment of the extent of pleural and chest wall abnormalities compared to CT. MRI can help distinguish components of complex fluid collections, including septations [80], and is thought to be slightly better at distinguishing benign from malignant pleural thickening [81]. Improved soft-tissue contrast allows for better demonstration of soft-tissue relationships, which can facilitate the assessment of invasion and neurovascular encasement. Small studies have shown that MRI is capable of providing diagnostic images to guide surgical chest wall reconstruction [82]. There is extensive supportive literature in pediatric populations surrounding the detection and management of pectus excavatum.


Chronic Dyspnea Noncardiovascular Origin. FDG-PET/CT Skull Base to Mid-Thigh FDG-PET/CT remains a secondary test that may be used in the staging of mesothelioma and pleural metastatic disease.


Chronic Dyspnea Noncardiovascular Origin. Chronic Dyspnea-Noncardiovascular Origin US Chest US can complement the imaging evaluation of several abnormalities. US has an established role in pleural effusion, including detection, differentiation from lung disease, characterization, and guidance of intervention. US may be more efficacious than chest radiography and CT at detecting internal septations in complex pleural effusions [83]. Pleural thickening, plaques, and masses may be identifiable on US, although US is not recommended in their workup. US is advantageous in the bedside diagnosis of pneumothorax and is most often used in acute assessment [84].


Chronic Dyspnea Noncardiovascular Origin. Variant 6: Chronic dyspnea. Suspected diaphragm dysfunction. Initial imaging. Radiography Chest A static chest radiograph is useful to assess the relative position of the diaphragm and its effect on lung volumes and can provide clues to diaphragm paralysis compared to a fluoroscopic reference standard [85].


Chronic Dyspnea Noncardiovascular Origin. Fluoroscopy Chest With fluoroscopic visualization, more accurate assessment of diaphragmatic motion can be made [86,87].


Chronic Dyspnea Noncardiovascular Origin. CT Chest While CT can document the position of the diaphragm with multiplanar imaging and in theory can provide dynamic information, there are no data to support its use in the analysis of diaphragmatic dysfunction.


Chronic Dyspnea Noncardiovascular Origin. MRI Chest Although not widely practiced, cine dynamic MRI sequences allow for the direct visualization of diaphragm motion [88]. This can result in comprehensive analysis of both the diaphragm and chest wall muscle movement in neuromuscular diseases [89].


Chronic Dyspnea Noncardiovascular Origin. US Chest US findings have been found to be concordant with fluoroscopic imaging of diaphragm motion, with reproducible results [90]. Diaphragmatic excursion amplitude, thickness, and contraction can be evaluated, and paralysis may be identified as paradoxical movement during respiration [91]. There is high sensitivity and specificity for the diagnosis of neuromuscular disorders of the diaphragm [92-95]. The extent of diaphragm motion in various conditions, including neuromuscular diseases, COPD, and ILD, correlates with respiratory symptoms and lung function [96-100].


Chronic Dyspnea Noncardiovascular Origin. Summary of Recommendations Variant 1: For patients with chronic dyspnea of unclear etiology, it is usually appropriate to initially evaluate with chest radiography, which may reveal a wide variety of abnormalities and guide further imaging decisions.


Chronic Dyspnea Noncardiovascular Origin. Variant 2: The appropriate initial imaging study for patients with chronic dyspnea with suspected COPD is usually a chest radiograph, which can evaluate for comorbidities, complications, and alternative diagnoses. Variant 3: A chest radiograph is usually appropriate for the initial imaging of patients with chronic dyspnea and suspected central airways disease. Alternatively, CT without IV contrast is also usually appropriate, particularly for the detection of airway collapse, stenosis, or tumor. Variant 4: CT without IV contrast is usually appropriate for the initial imaging of patients with chronic dyspnea and suspected ILD, especially if the patient has a disease that predisposes to ILD. Alternatively, initial imaging with chest radiography is usually appropriate, although a normal chest radiograph does not exclude clinically important ILD. Variant 5: For patients with chronic dyspnea and suspected pleural or chest wall disease, initial imaging with chest radiography is usually appropriate. Alternatively, initial imaging by CT without or with IV contrast is usually appropriate; CT is superior in distinguishing, characterizing, and assessing the extent of pleural and chest wall abnormalities. Variant 6: It is usually appropriate to initially image patients with chronic dyspnea and suspected diaphragm dysfunction by chest radiography, which can assess diaphragm position and provide clues to the presence of paralysis. Alternatively, initial imaging by fluoroscopy is usually appropriate and provides a more accurate assessment of diaphragm motion.


Chronic Dyspnea Noncardiovascular Origin. Although there are references that report on studies with design limitations, 32 well-designed or good-quality studies provide good evidence.


Chronic Dyspnea Noncardiovascular Origin. Chronic Dyspnea-Noncardiovascular Origin


Chronic Dyspnea Noncardiovascular Origin. Supporting Documents For additional information on the Appropriateness Criteria methodology and other supporting documents go to www.acr.org/ac.


Congenital or Acquired Heart Disease

Congenital or Acquired Heart Disease. Introduction/Background Congenital and acquired pediatric heart disease is a large and diverse field with an overall prevalence estimated at 6 to 13 per 1,000 live births [1,2]. Congenital heart disease (CHD) can be broadly divided into acyanotic and cyanotic conditions. The 3 most common conditions are all acyanotic; bicuspid aortic valve, ventricular septal defects (VSD), and atrial septal defects. Common acquired cardiovascular conditions in children include aortopathy, cardiomyopathy, myocarditis, and vasculitis. Advanced imaging plays an important complementary role to transthoracic echocardiography (TTE) in the initial evaluation of congenital and acquired cardiovascular disease in children. Advances in treatment [2,3] have resulted in much longer survival of patients with CHD [4]. This longer survival has resulted in postsurgical patients often requiring imaging follow-up to look for secondary complications of corrective procedures like ventricular dysfunction, valvular dysfunction, and secondary vascular compromise. This document covers variants representing some of the most common indications for advanced imaging of childhood onset cardiovascular disease.


Congenital or Acquired Heart Disease

Congenital or Acquired Heart Disease. All elements are essential: 1) timing, 2) reconstructions/reformats, and 3) 3-D renderings. Standard CTs with contrast also include timing issues and reconstructions/reformats. Only in CTA, however, is 3-D rendering a required element. This corresponds to the definitions that the CMS has applied to the Current Procedural Terminology codes.


Congenital or Acquired Heart Disease

Congenital or Acquired Heart Disease. The American College of Radiology seeks and encourages collaboration with other organizations on the development of the ACR Appropriateness Criteria through representation of such organizations on expert panels. Participation on the expert panel does not necessarily imply endorsement of the final document by individual contributors or their respective organization.


Congenital or Acquired Heart Disease

Congenital or Acquired Heart Disease. Reprint requests to: publications@acr.org


Congenital or Acquired Heart Disease

Congenital or Acquired Heart Disease

Congenital or Acquired Heart Disease. Congenital or Acquired Heart Disease


Congenital or Acquired Heart Disease

Congenital or Acquired Heart Disease. Arteriography Coronary with Ventriculography Catheter angiography is a useful modality for the evaluation of the coronary arteries and measurement of atrial and ventricular pressure. It provides direct measurements of RVOT gradients, dynamic assessment of regurgitant flow, and hemodynamic assessment of diastolic dysfunction [9]. Coronary angiography is performed in patients with suspected anomalous coronary artery to delineate the course of the coronary artery before interventions in the RVOT [10,11]. This could be important in the setting of percutaneous intervention, because one concern is coronary artery compression with inflation of a balloon-expandable stent in the RVOT when performing PVR percutaneously.


Congenital or Acquired Heart Disease

Congenital or Acquired Heart Disease. Arteriography Pulmonary Cardiac catheterization is performed in patients only when alternative measures cannot accurately assess the RV and pulmonary artery anatomy or hemodynamics noninvasively. However, angiographic evaluation of PR severity is complicated by the fact that catheter position across the PV may influence the angiographic severity. It allows for simultaneous catheter-guided therapeutic interventions such as pulmonary artery balloon dilatation and stent placement and PVR [12].


Congenital or Acquired Heart Disease

Congenital or Acquired Heart Disease. CT Heart Function and Morphology A common deficiency of TTE imaging in repaired TOF is imaging of the branch pulmonary arteries. Multidetector CT provides accurate assessment of the anatomy of the branch pulmonary arteries, including within stented segments. It provides detailed anatomic evaluation of the heart and RVOT as well as other vascular abnormalities, such as pulmonary artery aneurysms or pseudoaneurysms, associated coronary anomalies, or aortic dilation [13,14]. Performing electrocardiogram (ECG) gating permits dynamic assessment of the RVOT for percutaneous pulmonary valve planning, and measurement of RV and left ventricular (LV) volume and function. Multiplanar reformats allow for precise measurement of the cross-sectional area of the RVOT and valvular annulus, which are essential for selection of the appropriate prosthetic valve, and to screen for conduit calcification and kinking. before repeat surgery to replace the conduit or valve, CT is also helpful to determine safety of sternal re-entry.


Congenital or Acquired Heart Disease

Congenital or Acquired Heart Disease. CTA Chest CTA without ECG gating or with prospective ECG gating may be used in the setting of TOF to avoid the need for sedation for morphological assessment of the branch pulmonary arteries, including within stented segments. It is also helpful to screen for right ventricle to pulmonary artery conduit calcification and stenosis and to determine safety of sternal re-entry before surgery.


Congenital or Acquired Heart Disease

Congenital or Acquired Heart Disease


Congenital or Acquired Heart Disease

Congenital or Acquired Heart Disease. CTA Coronary Arteries CTA technique can be used to study accurate coronary artery anatomy and exclude anomalous coronary artery crossing the RVOT before RVOT intervention or reoperation. Particularly, the relationship of the left or right coronary artery to the pulmonary outflow tract and pulmonary valve is important to assess because this may be compressed during percutaneous valve placement, conduit dilation, or sternal re-entry.


Congenital or Acquired Heart Disease

Congenital or Acquired Heart Disease. FDG-PET/CT Heart There is no relevant literature to support the use of fluorine-18-2-fluoro-2-deoxy-D-glucose (FDG)-PET/CT heart in the evaluation of repaired TOF.


Congenital or Acquired Heart Disease

Congenital or Acquired Heart Disease. MRA Abdomen There is no relevant literature to support the use of MR angiography (MRA) abdomen in the evaluation of repaired TOF.


Congenital or Acquired Heart Disease

Congenital or Acquired Heart Disease. MRA Chest MRA of the chest is routinely performed along with MRI heart for function and morphology for delineation of the extracardiac vasculature, including the branch pulmonary arteries, aorta, and remaining mediastinal vasculature.


Congenital or Acquired Heart Disease

Congenital or Acquired Heart Disease. MRA Neck There is no relevant literature to support the use of MRA neck in the evaluation of repaired TOF.


Congenital or Acquired Heart Disease

Congenital or Acquired Heart Disease. MRI Heart Function with Stress There is no relevant literature to support the use of MR stress perfusion imaging in the setting of repaired TOF.


Congenital or Acquired Heart Disease

Congenital or Acquired Heart Disease. Radiography Chest The chest radiograph in TOF patients with severe PR often demonstrates increased cardiothoracic ratio on frontal view and retrosternal fullness on the lateral view due to cardiomegaly secondary to RV volume overload. The chest radiograph may also demonstrate dilated pulmonary trunk and may demonstrate dilatation of the ascending aorta [9]. In the posttreatment setting, radiography is essential to monitor location and integrity of stents in the pulmonary arteries, screen for RVOT pseudoaneurysm after conduit placement, and screen for asymmetry in the size and branching pattern of the parenchymal pulmonary arteries, which may indicate the presence of proximal vessel hypoplasia or stenosis.


Congenital or Acquired Heart Disease

Congenital or Acquired Heart Disease. Perfusion Scan Lung The use of pulmonary perfusion scintigraphy allows for the assessment of differential pulmonary blood flow in patients with TOF and branch pulmonary artery stenosis or hypoplasia [12].


Congenital or Acquired Heart Disease

Congenital or Acquired Heart Disease. SPECT/CT MPI Rest and Stress Radionuclide angiography may be performed to assess LV function but has been replaced by MRI in this setting. Its use to assess RV function is limited by the confounding effect of counts from other chambers. In addition, its resolution is poor compared with other imaging methods and, therefore, has been of limited use in this setting.


Congenital or Acquired Heart Disease

Congenital or Acquired Heart Disease. Congenital or Acquired Heart Disease US Echocardiography Transesophageal Transesophageal echocardiography (TEE) is considered a useful technique for intraoperative assessment of adequacy of outflow tract repair in terms of ruling out any residual obstruction or valve regurgitation as a consequence of surgery. TEE offers the advantages of permitting visualization of the operative procedure in real time and providing guidance for the surgeon [6,18] . The routine use of TEE during follow-up after surgery for patients with TOF is not established.


Congenital or Acquired Heart Disease

Congenital or Acquired Heart Disease. TEE is also useful in certain patients to guide interventional procedures or evaluate valve anatomy when transthoracic imaging is challenging, or when infectious endocarditis is suspected [12].


Congenital or Acquired Heart Disease

Congenital or Acquired Heart Disease. Variant 2: Child or adult. Transposition of the great arteries after atrial switch. Incomplete or inadequate assessment of cardiovascular morphology and function after transthoracic echocardiography. Next imaging study.


Congenital or Acquired Heart Disease

Congenital or Acquired Heart Disease. Transposition of the great arteries (TGA) is a cardiac congenital defect characterized by discordant ventriculoarterial connections, in which the aorta arises above the morphological RV, and the pulmonary artery arises above the morphological LV. In TGA, the aorta usually is anterior to the PA, but it can be beside or behind the PA.


Congenital or Acquired Heart Disease

Congenital or Acquired Heart Disease. The prevalence of TGA is 4.7 per 10,000 live births [19]. TGA accounts for 3% of all CHD and 20% of cyanotic heart disease. There are 2 major types of surgery performed for D-TGA: the atrial switch procedures (Mustard and Senning procedures) and the arterial switch procedures (Jatene procedure). In the atrial switch procedure, intra- atrial venous baffles redirect the systemic and pulmonary venous blood returns to the appropriate atria restoring circulating blood flow. These baffles are created by either using in situ tissue from the right atrial wall and interatrial septum (Senning procedure) or with autologous or synthetic material (Mustard procedure). Ventriculoseptal defects, if present, are also closed.


Congenital or Acquired Heart Disease

Congenital or Acquired Heart Disease. Patients with Mustard or Senning type repair are now typically adult patients. The most common complications following the atrial switch procedures are intra-atrial venous baffle stenoses and leaks. Baffle stenoses typically occur at the superior limb of the baffle where the superior vena cava meets the right atrium. Baffle obstructions and leaks are estimated to occur in approximately 25% of patients [20]. The most important and relatively common postoperative complication is hypertrophy and decreased function of the systemic RV. Less common complications include pulmonary arterial hypertension, residual VSD, subpulmonary stenosis, and arrhythmias.


Congenital or Acquired Heart Disease

Congenital or Acquired Heart Disease. Arteriography Coronary with Ventriculography Since the coronary arteries are not surgically manipulated during an atrial switch procedure, there is no defined role for coronary arteriography in this setting. In patients with complex coronary anatomy in the setting of TGA, angiography may be helpful to demonstrate dynamic compression and angulation of the coronary arteries in patients with an interarterial course or in those with a muscular bridge [21], but this role has been superseded by CTA in most centers.


Congenital or Acquired Heart Disease

Congenital or Acquired Heart Disease. Arteriography Pulmonary Pulmonary arteriography may be helpful if hemodynamic assessment of pulmonary artery pressure and resistance is required. Pulmonary arteriography is performed during interventions such as branch pulmonary artery balloon dilation and stent placement, to assess for baffle leaks, or narrowing of the systemic or pulmonary venous pathways [21].


Congenital or Acquired Heart Disease

Congenital or Acquired Heart Disease. CT Heart Function and Morphology CT is an alternative imaging modality to provide incremental information to echocardiography. CT functional assessment is a safer option to CMR in patients who are hemodynamically unstable. It is the most useful modality if anatomic restenosis is suspected in the setting of stents.


Congenital or Acquired Heart Disease

Congenital or Acquired Heart Disease. CTA Chest CTA is the most useful modality if anatomic restenosis of pulmonary arteries is suspected in the setting of stents or presence of a metal susceptibility artifact that may hamper the use of MRA for this purpose.


Congenital or Acquired Heart Disease

Congenital or Acquired Heart Disease. CTA Coronary Arteries Coronary CTA would be helpful when combined with cardiac CTA if repeat surgery is being considered given the increased rate of coronary anomalies.


Congenital or Acquired Heart Disease

Congenital or Acquired Heart Disease. Congenital or Acquired Heart Disease FDG-PET/CT Heart There is no relevant literature to support the use of FDG-PET/CT heart in the evaluation of TGA after atrial switch.


Congenital or Acquired Heart Disease

Congenital or Acquired Heart Disease. MRA Abdomen There is no relevant literature to support the use of MRA abdomen in the evaluation of TGA after atrial switch.


Congenital or Acquired Heart Disease

Congenital or Acquired Heart Disease. MRA Chest MRA chest provides important information regarding extracardiac structures such as the branch pulmonary arteries, systemic and pulmonary veins, and superior and inferior limb baffles and is used in conjunction with CMR for morphology and function to provide comprehensive evaluation of the cardiovascular system after treatment for TGA.


Congenital or Acquired Heart Disease

Congenital or Acquired Heart Disease. MRA Neck There is no relevant literature to support the use of MRA neck in the evaluation of TGA after atrial switch.


Congenital or Acquired Heart Disease

Congenital or Acquired Heart Disease. MRI Heart Function and Morphology CMR yields accurate and reproducible data regarding ventricular size and function, especially follow-up of systemic RV status and intracardiac baffles.


Congenital or Acquired Heart Disease

Congenital or Acquired Heart Disease. MRI Heart Function with Stress CMR is a the most useful imaging modality to screen for myocardial ischemia, but this is uncommon after atrial switch for TGA because the coronary arteries are not manipulated during surgery.


Congenital or Acquired Heart Disease

Congenital or Acquired Heart Disease. Radiography Chest Radiography of the chest can be used for preliminary assessment of stent position, pacemaker generator and lead position, and as needed during the course of follow-up.


Congenital or Acquired Heart Disease

Congenital or Acquired Heart Disease. SPECT or SPECT/CT MPI Rest and Stress There is no relevant literature to support the use of single-photon emission CT (SPECT)/CT myocardial perfusion imaging (MPI) rest and stress in the evaluation of TGA after atrial switch.


Congenital or Acquired Heart Disease

Congenital or Acquired Heart Disease. US Echocardiography Transesophageal TEE is useful in patients with poor transthoracic acoustic windows, during intraoperative imaging, and in patients (usually adolescents) who require cardioversion for arrhythmia. It is typically used to assess the adequacy of intraoperative repair TTE and may be more sensitive to detect baffle leaks and baffle obstruction. TEE is also helpful to guide catheter-based treatment of pathway obstruction such as balloon dilation, stent placement, or device closure of baffle leaks.


Congenital or Acquired Heart Disease

Congenital or Acquired Heart Disease. Variant 3: Child or adult. Transposition of the great arteries after arterial switch. Incomplete or inadequate assessment of cardiovascular morphology and function after transthoracic echocardiography. Next imaging study. The arterial switch operation (ASO) has superseded the Mustard and Senning procedures as the most common surgery performed in D-TGA. In the ASO (Jatene procedure), the ascending aorta and the main pulmonary artery are transected above their valve leaflets and moved to their correct circulatory position (Lecompte maneuver). The coronary arteries are then transplanted from the native aortic root to the neoaortic root (which is the native pulmonary trunk). Septal defects, if present, are surgically closed.


Congenital or Acquired Heart Disease

Congenital or Acquired Heart Disease. Well-known sequelae after an ASO include obstruction of the coronary arteries with related myocardial ischemia, LV dysfunction, narrowing of the pulmonary artery side branches, RVOT obstruction, neoaortic valve insufficiency, and dilatation of the aortic root. RVOT and pulmonary artery stenoses may lead to RV dysfunction necessitating corrective procedures. Supravalvular pulmonary stenosis, particularly in the side branches of the pulmonary arteries, occurs early after an ASO. Dilatation and insufficiency of the neoaortic root usually develops over time. Coronary abnormalities can be present in 8% of asymptomatic individuals on 1- to 20-year follow-up and, although uncommon, can cause sudden death [23]. Noninvasive imaging of the postoperative anatomy is integral to the


Congenital or Acquired Heart Disease

Congenital or Acquired Heart Disease. Congenital or Acquired Heart Disease management of patients who have undergone an ASO. A combination of imaging tests and exercise testing are performed to screen for complications and to assess severity and the need for intervention and the type of intervention.


Congenital or Acquired Heart Disease

Congenital or Acquired Heart Disease. Arteriography Coronary with Ventriculography The coronary artery course has a particularly high variability in patients with TGA. After an ASO, many patients require repeated imaging of their reimplanted coronary arteries. Cardiac catheterization and ventriculography after ASO is reserved for only complex cases in whom incremental information is needed following advanced imaging. Angiography is performed to assess for coronary artery stenosis after coronary artery reimplantation or during interventional procedures such as branch pulmonary artery balloon dilation and stent placement. In patients with complex coronary anatomy, angiography is useful in demonstrating dynamic compression and angulation of the coronary arteries in patients with an interarterial course or in those with a muscular bridge [21].


Congenital or Acquired Heart Disease

Congenital or Acquired Heart Disease. Arteriography Pulmonary Pulmonary arteriography is performed during interventions such as branch pulmonary artery balloon dilation and stent placement.


Congenital or Acquired Heart Disease

Congenital or Acquired Heart Disease. CT Heart Function and Morphology CT is an alternative imaging modality to provide incremental information to echocardiography. CT functional assessment is more useful over CMR in patients who are hemodynamically unstable. It is the most useful modality if anatomic restenosis is suspected in the setting of stents.


Congenital or Acquired Heart Disease

Congenital or Acquired Heart Disease. CTA Chest CTA of the chest is useful to study airway compromise, which may happen related to the aortic root or ascending aortic dilatation after ASO. A dynamic CTA may be helpful in this setting to distinguish intrinsic from extrinsic causes of airway obstruction. CTA is the most useful modality if anatomic restenosis of pulmonary arteries is suspected in the setting of stents or presence of a metal susceptibility artifact that may hamper the use of MRA for this purpose.


Congenital or Acquired Heart Disease

Congenital or Acquired Heart Disease. CTA Coronary Arteries Obstructed coronary arteries occur in 8% of survivors and is a common cause of morbidity and mortality after the ASO [23]. ECG-triggered CTA is currently the most useful modality for the primary evaluation of coronary arteries. Its high spatial and temporal resolution allows for the reliable visualization of coronary arteries and screening for potential stenosis, usually without the use of sedation [19,24]. CT coronary angiography may also be performed as an alternative to coronary catheterization when SPECT/CT MPI shows perfusion deficit or CMR stress perfusion imaging shows ischemia or wall motion abnormality in a vascular distribution [25].


Congenital or Acquired Heart Disease

Congenital or Acquired Heart Disease. FDG-PET/CT Heart FDG-PET/CT can be used as the alternative method to SPECT/CT MPI for assessing viability when myocardial ischemia is suspected or to assess blood flow to the branch pulmonary arteries after the ASO. FDG-PET/CT can have confirmatory and/or incremental value to TEE and CTA in diagnosing prosthetic endocarditis after RV-PA conduit placement, particularly when anatomic imaging findings are inconclusive or equivocal.


Congenital or Acquired Heart Disease

Congenital or Acquired Heart Disease. MRA Abdomen There is no relevant literature to support the use of MRA abdomen in the evaluation of TGA after arterial switch.


Congenital or Acquired Heart Disease

Congenital or Acquired Heart Disease. MRA Chest MRA chest provides important information regarding extracardiac structures such as the branch pulmonary arteries, ascending aorta, and the aortic arc, and is used in conjunction with CMR for morphology and function to provide comprehensive evaluation of the cardiovascular system after treatment for TGA.


Congenital or Acquired Heart Disease

Congenital or Acquired Heart Disease. MRA Neck There is no relevant literature to support the use of MRA neck in the evaluation of TGA after arterial switch.


Congenital or Acquired Heart Disease

Congenital or Acquired Heart Disease. MRI Heart Function and Morphology CMR yields accurate and reproducible data regarding both LV and RV size and function, differential pulmonary blood flow, neoaortic root diameter and regurgitant blood flow, coronary artery diameter, myocardial perfusion, and myocardial fibrosis. CMR provides important information regarding myocardial performance and viability, as well as quantitative assessment of valvar function and accurate evaluation of conduits [21]. If the echocardiographic


Congenital or Acquired Heart Disease

Congenital or Acquired Heart Disease. Congenital or Acquired Heart Disease assessment of ventricular parameters or the severity of valve regurgitation is in question, CMR can resolve this uncertainty [26]. The quantitative data on CMR can be validated by comparing the main pulmonary artery flow to the sum of the branch pulmonary artery flows. CMR using 3-D steady-state free precession navigator respiratory gated sequences can noninvasively evaluate the coronary arteries looking for ostial stenoses or proximal kinking and screen for myocardial injury using perfusion and viability sequences [27,28].


Congenital or Acquired Heart Disease

Congenital or Acquired Heart Disease. MRI Heart Function with Stress CMR is the most useful imaging modality to screen for myocardial ischemia [28]. CMR coronary angiography can provide good resolution images of coronary lumen especially in adolescents and larger children, but it is inferior in comparison with CTA in young children [26]. Myocardial stress perfusion MRI can also be done when there is a concern for ischemia following arterial switch procedure [28,29].


Congenital or Acquired Heart Disease

Congenital or Acquired Heart Disease. Radiography Chest Radiography of the chest can be used for the preliminary assessment of stent position, pacemaker and lead position, and as needed during the course of follow-up.


Congenital or Acquired Heart Disease

Congenital or Acquired Heart Disease. SPECT or SPECT/CT MPI Rest and Stress Coronary reimplantation during ASO increases the risk of long-term ischemic complications in the postoperative period. SPECT/CT MPI is used when patient symptoms or ECG findings are concerning for myocardial ischemia [30].


Congenital or Acquired Heart Disease

Congenital or Acquired Heart Disease. US Echocardiography Transesophageal TEE is useful in patients with poor transthoracic acoustic windows, during intraoperative imaging, and in patients (usually adolescents) who require cardioversion for arrhythmia. It is typically used to assess the adequacy of intraoperative repair. If postoperative access to the transthoracic window is limited, TEE can be used to assess the coronary artery origins and flow patterns in the early postoperative period.


Congenital or Acquired Heart Disease

Congenital or Acquired Heart Disease. Arteriography Coronary with Ventriculography Coronary angiography is traditionally reserved for interventional procedures or when noninvasive diagnostic imaging with CT or MRI is inconclusive. It can correctly identify all types of anomalous coronary arteries. However, it lacks optimum 3-D information and the ability to image soft tissue, making it difficult to demonstrate the spatial relationship among the coronary artery course, myocardium, and great vessels.


Congenital or Acquired Heart Disease

Congenital or Acquired Heart Disease. Coronary angiography is essential to clarify the extent of the vulnerable territory of the anomalous vessel (ie, dominant versus nondominant right coronary artery, single coronary, etc).


Congenital or Acquired Heart Disease

Congenital or Acquired Heart Disease. Fractional flow reserve may offer an adjunct to determine the functional significance of AAOCA narrowing or intramyocardial course of the coronary artery, although it is unproven in these settings.


Congenital or Acquired Heart Disease

Congenital or Acquired Heart Disease. Congenital or Acquired Heart Disease In Kawasaki disease, to predict the progress of the disease and determine appropriate treatment and follow-up protocols, it is essential to understand the size and shape of the aneurysms in the early phase. Therefore, traditionally, invasive angiography is performed after the acute stage but, since the advent of CT and MRA invasive coronary angiography, has become second line [33].


Congenital or Acquired Heart Disease

Congenital or Acquired Heart Disease. Intravascular Ultrasound (IVUS): IVUS affords precise sequential cross-sectional imaging of the coronary lumen and coronary wall thickness with a discriminating power, based on superior spatial resolution. It is particularly useful in stenosis of the anomalous vessel in its intramural course.


Congenital or Acquired Heart Disease

Congenital or Acquired Heart Disease. Arteriography Pulmonary There is no relevant literature to support the use of pulmonary arteriography in the evaluation of suspected or confirmed congenital or acquired coronary artery abnormality.


Congenital or Acquired Heart Disease

Congenital or Acquired Heart Disease. CT Heart Function and Morphology Functional assessment with CT may be considered in the setting of Kawasaki disease for assessment of the consequences of coronary artery compromise.


Congenital or Acquired Heart Disease

Congenital or Acquired Heart Disease. CTA Chest CTA of the chest is rarely helpful for assessment of other vascular involvement in the setting of Kawasaki disease, with axillary artery aneurysms being a common manifestation in the chest. MRA of the chest and abdomen is more useful to CTA due to its ability to interrogate a wider vascular territory for screening for aneurysms in the setting of Kawasaki disease [34].


Congenital or Acquired Heart Disease

Congenital or Acquired Heart Disease. CTA Coronary Arteries CTA of coronary arteries is currently the most useful imaging modality for evaluating coronary arteries. Virtual angioscopic view of coronary CTA helps to evaluate anatomic high-risk features, such as the slit-like morphology of the ostium [35]. CT can identify other anatomic high-risk features, such as acute take-off angle, luminal shape, and proximal narrowing of the anomalous coronaries related to the intramural and interarterial course [35]. Coronary CTA, overall, provides a more accurate depiction of the entire anomalous vessel including the distal coronary segments. Coronary CTA is also useful in the setting of Kawasaki disease to screen for the presence and extent of coronary involvement by ectasia, aneurysm, or stenosis and to diagnose and follow-up the presence of mural thrombus in larger aneurysms.


Congenital or Acquired Heart Disease

Congenital or Acquired Heart Disease. FDG-PET/CT Heart There is no relevant literature to support the use of FDG-PET/CT in the evaluation of suspected or confirmed congenital or acquired coronary artery abnormality.


Congenital or Acquired Heart Disease

Congenital or Acquired Heart Disease. MRA Abdomen MRA abdomen is used to screen for extracardiac involvement in Kawasaki disease, with iliac aneurysms being a common manifestation of the disease.


Congenital or Acquired Heart Disease

Congenital or Acquired Heart Disease. MRA Chest MRA chest allows 3-D reconstruction visualization of coronary artery origin and proximal course in AAOCA and the presence of coronary aneurysms in Kawasaki disease, but its spatial resolution is inferior to that of coronary CTA. MRA using 3-D steady-state free precession or 3-D fast gradient echo techniques can be used to evaluate the proximal coronary in AAOCA or the coronary tree in Kawasaki disease. MRA may also be performed with intravenous (IV) contrast to assess the systemic arterial tree for evidence of extracardiac aneurysms in Kawasaki disease [36-38].


Congenital or Acquired Heart Disease

Congenital or Acquired Heart Disease. MRI Heart Function and Morphology CMR offers a wealth of additional relevant information including valvular function, ventricular function, regional contractility, and myocardial viability, all of which could be important considerations during the preoperative evaluation or postoperative follow-up in patients who undergo surgical repair of coronary artery.


Congenital or Acquired Heart Disease

Congenital or Acquired Heart Disease. MRI Heart Function with Stress Pharmacological stress CMR cine and MPI can be performed to assess the functional relevance of a coronary anomaly [36,39].


Congenital or Acquired Heart Disease

Congenital or Acquired Heart Disease. Congenital or Acquired Heart Disease Radiography Chest There is no relevant literature to support the use of chest radiography in the evaluation of suspected or confirmed congenital or acquired coronary artery abnormality.


Congenital or Acquired Heart Disease

Congenital or Acquired Heart Disease. SPECT or SPECT/CT MPI Rest and Stress Nuclear cardiac imaging modalities play an important role in assessing myocardial perfusion, thus allowing for assessment of the functional relevance of any acquired coronary artery. SPECT MPI and PET MPI may unmask ischemia in asymptomatic and symptomatic patients with anomalous coronary artery [40]. The panel disagreed on SPECT or SPECT/CT MPI rest and stress to screen for myocardial ischemia as the next imaging study after TEE, although its use was supported by expert opinion in centers with pediatric experience with coronary ischemia.


Congenital or Acquired Heart Disease

Congenital or Acquired Heart Disease. US Echocardiography Transesophageal TEE may be useful to visualize coronary artery anatomy perioperatively when the thoracic window is limited. However, TEE is not useful for routine imaging of AAOCA or Kawasaki disease.


Congenital or Acquired Heart Disease

Congenital or Acquired Heart Disease. MRA Neck There is no relevant literature to support the use of MRA neck in the evaluation of suspected or confirmed congenital or acquired coronary artery abnormality.


Congenital or Acquired Heart Disease

Congenital or Acquired Heart Disease. Variant 5: Child. Known single ventricle physiology. Preoperative evaluation for stage 2 single ventricle palliation. Incomplete or inadequate assessment of cardiovascular morphology and function after transthoracic echocardiography. Next imaging study. Single ventricle lesions are congenital heart anomalies in which one of the 2 ventricular chambers is either absent or so severely hypoplastic that a biventricular repair is impossible. Typically, the atrioventricular valve associated with the absent or hypoplastic ventricle is also absent or hypoplastic. The arterial outflow valve associated with the absent/hypoplastic ventricle is also frequently affected. Single ventricle congenital heart defects cover a wide spectrum including the following: hypoplastic left heart syndrome, tricuspid atresia, unbalanced atrioventricular, pulmonary atresia with intact ventricular septum, Ebstein anomaly, and double outlet left ventricle.


Congenital or Acquired Heart Disease

Congenital or Acquired Heart Disease. The repair of this entity involves 3-staged reconstruction to establish a circulatory system wherein all the deoxygenated blood flows directly into the lungs, bypassing the heart, while the oxygenated blood goes to the functional single ventricle to be pumped out to the systemic circulation. Stage 1 of this repair involves optimizing the pulmonary arterial blood flow, sometimes with a systemic-pulmonary arterial shunt or pulmonary artery band, and is performed shortly after birth. This procedure allows postoperative recovery and growth until stage 2 palliation can be performed. The stage 2 procedure, otherwise known as superior cavopulmonary connection, involves establishing a connection between the systemic venous return from the upper half of the body and the pulmonary arteries to allow deoxygenated blood flow into the pulmonary circulation. Some procedures in stage 2 are bidirectional Glenn, hemi-Fontan, and Kawashima operations. This procedure is typically performed within 6 months of birth.


Congenital or Acquired Heart Disease

Congenital or Acquired Heart Disease. The primary goals of the imaging evaluation before the stage 2 operation include an assessment for obstruction in the pulmonary arteries and veins, the atrial septal defect, ventricular outflow tract, and aortic arch; for the degree and mechanism of valve regurgitation; and ventricular size and function. Another role of imaging could be to decide if the ventricular volumes and functions still support the patient proceeding down the single ventricle pathway or if the patient is a candidate for a 1.5-ventricle or 2-ventricle repair. A crucial role of imaging in this setting is to demonstrate the pulmonary arterial anatomy, including the distal branches, to evaluate for anatomic obstruction that may impact the Glenn circulation. Knowledge of branch pulmonary artery anatomy adds significantly to the surgical planning process. A complete anatomic assessment of the branch pulmonary arteries to detect stenoses should be performed in all patients undergoing stage 2 repair.


Congenital or Acquired Heart Disease

Congenital or Acquired Heart Disease. Arteriography Coronary with Ventriculography Direct cardiac catheterization and angiography has historically been the standard of care for assessing hemodynamic and anatomic suitability before stage 2 repair. Its routine use is decreasing since the advent of advanced CMR and CT methods. The use of catheter angiography is supported in the literature [41]; for example, in patients with pulmonary atresia and intact ventricular septum, the diagnosis of RV dependent coronary circulation is established by RV and selective coronary angiography and has an important impact upon surgical approach.


Congenital or Acquired Heart Disease

Congenital or Acquired Heart Disease. Congenital or Acquired Heart Disease One of the advantages of cardiac catheterization before bidirectional Glenn operation is that anomalies such as systemic-to-pulmonary collateral vessels and aortic coarctation can be palliated using transcatheter techniques. It particularly pertinent for those who have undergone prior stage 1 palliation with aortic arch reconstruction, which is associated with a 10% to 15% incidence of recurrent coarctation at the distal end of the arch reconstruction [42].


Congenital or Acquired Heart Disease

Congenital or Acquired Heart Disease. Arteriography Pulmonary Cardiac catheterization is useful for the imaging evaluation before stage 2 repair to assess pulmonary arterial anatomy before surgical repair [41]. Routine cardiac catheterization is less frequently performed before undertaking stage 2 repair, but it has a useful role in the assessment of hemodynamic suitability to adapt to this type of circulation by directly measuring pulmonary vascular resistance.


Congenital or Acquired Heart Disease

Congenital or Acquired Heart Disease. CTA Chest CTA chest is a useful modality and provides accurate means of evaluation of pulmonary arterial anatomy and pulmonary and systemic venous status before stage 2 repair. Thrombosis of the systemic-to-pulmonary arterial shunt may occur in 6% to 17% of cases and constitutes a surgical emergency [44]. CTA can be used as the confirmatory test especially if echocardiography is inconclusive.


Congenital or Acquired Heart Disease

Congenital or Acquired Heart Disease. MRA Abdomen There is no relevant literature to support the use of MRA abdomen in the evaluation of known single ventricle physiology.


Congenital or Acquired Heart Disease

Congenital or Acquired Heart Disease. MRA Chest MRA chest is an excellent imaging technique for the evaluation of pulmonary arterial anatomy before stage 2 repair. It has the advantage that it can be easily combined with MRI heart function and morphology for a complete evaluation of the single ventricle patient. MRA has great diagnostic utility compared with echocardiography and is accurate in comparison to catheter angiography for the diagnosis of pulmonary artery stenoses.


Congenital or Acquired Heart Disease

Congenital or Acquired Heart Disease. MRA Neck There is no relevant literature to support the use of MRA neck in the evaluation of known single ventricle physiology.


Congenital or Acquired Heart Disease

Congenital or Acquired Heart Disease. MRI Heart Function and Morphology CMR allows for the quantification of ventricular volume and function, with abnormal ventricular morphology [42]. This can be useful in decisions regarding stage 1, 1.5, or 2 ventricle repair.


Congenital or Acquired Heart Disease

Congenital or Acquired Heart Disease. MRI Heart Function with Stress There is no relevant literature to support the use of MRI heart function with stress in the evaluation of known single ventricle physiology.


Congenital or Acquired Heart Disease

Congenital or Acquired Heart Disease. Radiography Chest Other than evaluation of patent ductus arteriosus or aortopulmonary shunt stent positioning, there is no relevant literature to support the use of radiography chest in the evaluation of known single ventricle physiology.


Congenital or Acquired Heart Disease

Congenital or Acquired Heart Disease. SPECT or SPECT/CT MPI Rest and Stress There is no relevant literature to support the use of SPECT or SPECT/CT MPI rest and stress in the evaluation of known single ventricle physiology.


Congenital or Acquired Heart Disease

Congenital or Acquired Heart Disease. Congenital or Acquired Heart Disease FDG-PET/CT Heart There is no relevant literature to support the use of FDG-PET/CT heart in the evaluation of known single ventricle physiology.


Congenital or Acquired Heart Disease

Congenital or Acquired Heart Disease. US Echocardiography Transesophageal TEE may provide useful information before, during, or after surgery for stage 2 palliation regarding pulmonary arterial or pulmonary venous flow patterns, severity of atrioventricular valve regurgitation, atrial shunt, and cardiac thrombus.


Congenital or Acquired Heart Disease

Congenital or Acquired Heart Disease. Variant 6: Child. Known single ventricle physiology. Preoperative evaluation for stage 3 single ventricle palliation (total cavopulmonary connection). Incomplete or inadequate assessment of cardiovascular morphology and function after transthoracic echocardiography. Next imaging study. The Fontan operation for single ventricle patients is the procedure in which a conduit (the total cavopulmonary connection) is placed to channel the remaining systemic venous return (including the hepatic veins) into the pulmonary arteries. The aim of the diagnostic evaluation before Fontan is to identify those few patients in whom the Fontan operation should not be performed and those who require additional intervention before or at the time of Fontan. The main consideration before Fontan procedure is elevated pulmonary artery pressure, which determines the prognosis during the postoperative course. However, with the modern stage 2 repair techniques, pulmonary resistance is seldom an important issue at the pre-Fontan stage.


Congenital or Acquired Heart Disease

Congenital or Acquired Heart Disease. Arteriography Coronary with Ventriculography Cardiac catheterizations are done when elevated pulmonary artery pressure or elevated ventricular filling pressure is suspected. Cardiac catheterization also provides an opportunity for interventional procedures before the Fontan operation consisted of embolization of aortopulmonary collateral vessels [45].


Congenital or Acquired Heart Disease

Congenital or Acquired Heart Disease. Other important indications for cardiac catheterization include severe atrioventricular valve regurgitation semiquantitatively evaluated by means of echo color Doppler, suspected pulmonary vein stenosis at echo or CMR, suspected pulmonary pathway or Glenn anastomosis obstruction, or suspected significant venovenous collaterals at MRA [46].


Congenital or Acquired Heart Disease

Congenital or Acquired Heart Disease. Arteriography Pulmonary Pulmonary arteriography is performed after echocardiography when there is a suspicion of branch pulmonary artery stenosis because smaller pulmonary arteries are not optimally seen on echocardiography.


Congenital or Acquired Heart Disease

Congenital or Acquired Heart Disease. CTA Chest Pulmonary embolism and thrombosis are known complications in patients undergoing single ventricle palliation. CTA is an effective method for diagnosis of pulmonary embolism in patients presenting with symptoms suspicious for pulmonary embolism [48].


Congenital or Acquired Heart Disease

Congenital or Acquired Heart Disease. CTA Coronary Arteries There is no relevant literature to support the use of CTA coronary arteries in the evaluation of known single ventricle physiology.


Congenital or Acquired Heart Disease

Congenital or Acquired Heart Disease. MRI Heart Function and Morphology Routine preoperative imaging before Fontan procedure is obtained using CMR for anatomy and to determine the current hemodynamics and physiology [49,50]. CMR is a comprehensive modality for accurate assessment of ventricular function, pulmonary artery, pulmonary venous return, cavopulmonary anastomosis, aortic arch, valves, and any valvular regurgitation.


Congenital or Acquired Heart Disease

Congenital or Acquired Heart Disease. Congenital or Acquired Heart Disease MRA Chest MRA chest provides good assessment of branch pulmonary arteries, their caliber, and presence of thrombus within but often fails to visualize very small aortopulmonary collaterals that may require preoperative embolization.


Congenital or Acquired Heart Disease


Congenital or Acquired Heart Disease

Congenital or Acquired Heart Disease. Radiography Chest Other than evaluation of device or stent positioning, there is no relevant literature to support the use of radiography chest in the evaluation of known single ventricle physiology.


Congenital or Acquired Heart Disease

Congenital or Acquired Heart Disease. FDG-PET/CT Heart There is no relevant literature to support the use of FDG-PET/CT heart in the evaluation of known single ventricle physiology.


Congenital or Acquired Heart Disease

Congenital or Acquired Heart Disease. US Echocardiography Transesophageal TEE may provide useful information before, during, or after surgery for stage 3 palliation regarding pulmonary arterial or pulmonary venous flow patterns, severity of atrioventricular valve regurgitation, atrial shunt, and cardiac thrombus.


Congenital or Acquired Heart Disease

Congenital or Acquired Heart Disease. Variant 7: Child or adult. Known single ventricle physiology. Postoperative evaluation after stage 3 single inadequate assessment of ventricle palliation (total cavopulmonary connection). Incomplete or cardiovascular morphology and function after transthoracic echocardiography. Next imaging study. Stage 3 repair for single ventricle palliation involves baffling of all systemic venous drainage directly to the pulmonary arteries, known as total cavopulmonary connection or Fontan operation. This is typically performed between 2 to 4 years of age and most commonly consists of either a lateral tunnel intracardiac baffle or an extracardiac conduit, with or without a fenestration. The Fontan operation also ensures that hepatic blood flow is directed into the lungs via the pulmonary arteries, thus preventing the formation of pulmonary arteriovenous malformations. Today, the estimates of 20-year survival for survivors of the Fontan procedure vary between 61% and 85% [51].


Congenital or Acquired Heart Disease

Congenital or Acquired Heart Disease. Early diagnosis and management of complications associated with Fontan physiology hold the most promise for improving longevity. Imaging after stage 3 repair can assess anatomy, ventricular and valve function, blood flow, and myocardial fibrosis. Anatomic considerations include the presence of thrombus in the circuit, the patency of the extracardiac conduit, stenoses within the caval veins and pulmonary arteries, and the size and location of aortopulmonary collaterals and venovenous collaterals. Considerations for assessment of flow include quantification of the degree of aortopulmonary and venovenous collateralization, the relative flow within the 2 branch pulmonary arteries, and valvular regurgitation. Considerations for assessment of ventricular function and morphology include screening for systolic and diastolic dysfunction, ventricular type and shape, and myocardial characterization.


Congenital or Acquired Heart Disease

Congenital or Acquired Heart Disease. Arteriography Coronary with Ventriculography Clinical indications for this examination include investigating symptoms associated with decreased ventricular function such as unexplained volume retention, fatigue, exercise limitation, and cyanosis. Cardiac catheterization with coronary angiography can evaluate coronary artery anatomy, and ventriculography can be used to estimate the function of the single ventricle and valvular flow. Cardiac catheterization is also helpful to assess the presence and severity of aortopulmonary, systemic-pulmonary venous, and systemic venovenous collaterals and also provides access to perform embolization of clinically significant collaterals. Cardiac catheterization is also suggested in the


Congenital or Acquired Heart Disease

Congenital or Acquired Heart Disease. Congenital or Acquired Heart Disease assessment of patients with failing Fontan physiology and features of Fontan-associated liver disease. Imaging of a patent fenestration is seen well with direct angiography on cardiac catheterization [52].


Congenital or Acquired Heart Disease

Congenital or Acquired Heart Disease. Arteriography Pulmonary Cardiac catheterization for pulmonary angiography can be performed when pulmonary artery stenosis is detected on echocardiography or MRI and pulmonary artery angioplasty is indicated [52].


Congenital or Acquired Heart Disease

Congenital or Acquired Heart Disease. CT Heart Function and Morphology CT heart function and morphology is indicated when MRI is limited by the presence of significant metallic susceptibility artifact obscuring the heart or due to the presence of MR-incompatible pacemakers/devices.


Congenital or Acquired Heart Disease

Congenital or Acquired Heart Disease. CTA Chest CTA is useful for assessment of anatomy after stage 3 repair. Anatomic considerations include the presence of thrombus in the circuit, the patency of the extracardiac conduit, stenoses within the caval veins and pulmonary arteries, and the size and location of aortopulmonary collaterals and venovenous collaterals [52,53]. After Fontan surgery, imaging of coronaries, systemic arteries, and Fontan circuit require different timing and dose considerations, and dedicated contrast injection and scanning protocols designed for the Fontan circulation are needed to optimize the study [48].


Congenital or Acquired Heart Disease

Congenital or Acquired Heart Disease. CTA Coronary Arteries CTA of the coronary arteries is useful and allows for high spatial resolution evaluation of coronary artery anatomy in patients after stage 3 repair [52].


Congenital or Acquired Heart Disease

Congenital or Acquired Heart Disease. FDG-PET/CT Heart There is no role for routine performance of FDG-PET imaging after stage 3 palliation in single ventricle. FDG- PET/CT of the heart can be performed in the setting of changes of clinical status that might indicate underlying cardiac dysfunction.


Congenital or Acquired Heart Disease

Congenital or Acquired Heart Disease. MRA Abdomen There is no relevant literature to support the use of MRA abdomen after stage 3 repair.


Congenital or Acquired Heart Disease

Congenital or Acquired Heart Disease. MRA Chest MRA chest can provide accurate anatomic characterization of stage 3 repair. Anatomic considerations include the presence of thrombus in the circuit, the patency of the extracardiac conduit, stenoses within the caval veins and pulmonary arteries, and the size and location of aortopulmonary collaterals and venovenous collaterals [52,53]. Gadolinium-enhanced CMR angiography has been demonstrated to be more accurate in the diagnosis of a collateral vessels in comparison to catheter angiography and offers promise to plan transcatheter interventions in those who prove to have clinically significant collateral vessels. Of note, MRA chest has lower spatial resolution compared with CTA chest for small vessels such as the coronary arteries [52].


Congenital or Acquired Heart Disease

Congenital or Acquired Heart Disease. MRA Neck There is no relevant literature to support the use of MRA neck after stage 3 repair.


Congenital or Acquired Heart Disease

Congenital or Acquired Heart Disease. MRI Heart Function and Morphology CMR is used regularly in addition to echocardiography for long-term monitoring of Fontan patients. MRI for heart function and morphology is helpful in assessment of anatomy, ventricular and valve function, flows, and myocardial fibrosis. It allows for estimating ventricular function, quantifying valvular regurgitation, quantifying pulmonary and systemic blood flow, and aortopulmonary collaterals [54]. Late gadolinium enhancement CMR is used to detect myocardial fibrosis and infarction [55].


Congenital or Acquired Heart Disease

Congenital or Acquired Heart Disease. Phase-contrast MRA flow measurements in the branch pulmonary arteries are useful for the quantification of differential pulmonary blood flow in the Fontan patient compared with nuclear scintigraphy, due to venous streaming effects on caval flows [53]. Four-dimensional flow, with or without the use of blood pool contrast agents, has been used to simplify, accelerate, and abbreviate the MR scanning protocol in these patients [56].


Congenital or Acquired Heart Disease

Congenital or Acquired Heart Disease. MRI Heart Function with Stress There is no role for routine performance of vasodilator stress perfusion after stage 3 palliation in single ventricle. There is some evidence that vasodilator stress perfusion MRI can be used to assess latent diastolic dysfunction after stage 3 repair [57,58].


Congenital or Acquired Heart Disease

Congenital or Acquired Heart Disease. Congenital or Acquired Heart Disease Radiography Chest There is no relevant literature to support the routine use of chest radiography after stage 3 palliation in single ventricle. However, chest radiography may be used to screen for the location of devices and metallic implants that are placed in this patient population.


Congenital or Acquired Heart Disease

Congenital or Acquired Heart Disease. SPECT or SPECT/CT MPI Rest and Stress There is no role for routine performance of vasodilator stress perfusion after stage 3 palliation in single ventricle. SPECT or SPECT/CT MPI rest and stress tests are used in the setting of changes of clinical status that might indicate underlying cardiac dysfunction.


Congenital or Acquired Heart Disease

Congenital or Acquired Heart Disease. US Echocardiography Transesophageal TEE can be used for comprehensive evaluation of the heart and central vascular anatomy, ventricular and valvular function, and flows in areas that are difficult to assess by TTE. One area in which it is better than TTE is in the detection of intracardiac thrombus.


Congenital or Acquired Heart Disease

Congenital or Acquired Heart Disease. Variant 8: Child or adult. Known or suspected anomalous pulmonary venous return with inadequate evaluation after transthoracic echocardiography. Next imaging study. Total anomalous pulmonary venous connection (TAPVC) refers to the condition in which all of the pulmonary veins drain into the right atrium, either directly or indirectly through other systemic pathways. TAPVC is rare, with prevalence estimated at approximately 0.01% [59]. The contexts of imaging in TAPVC management are to define the surgical anatomy, when one or more pulmonary veins are not visualized by TTE, a TTE showing right-sided cardiac chamber dilation with unclear delineation of the pulmonary venous anatomy, when obstruction is suspected, or when there is an associated abnormality like a varix that is not characterized adequately by TTE. There is also an important role for imaging in the postoperative period after TAPVC repair to screen for recurrent obstruction.


Congenital or Acquired Heart Disease

Congenital or Acquired Heart Disease. Partial anomalous pulmonary venous connection (PAPVC) is where one or more pulmonary veins drains into the right atrium, either directly or indirectly. PAPVC is more common with a prevalence at approximately 1% [59]. PAPVC has a few common variants. The most common form is where the left upper pulmonary vein connects to the left brachiocephalic vein into the superior vena cava. Another type is where the right upper pulmonary veins connect directly into the superior vena cava, which is commonly associated with other left to right shunts, including atrial septal and superior sinus venosus defects. Another unique variant of PAPVC is Scimitar syndrome, in which part or all of the right pulmonary veins connect to the inferior vena cava through the diaphragm. The right lung is often hypoplastic and/or has anomalous bronchial or arterial anatomy in addition to the PAPVC. The ultimate management of PAPVC is usually surgical correction, but unlike TAPVC, this does not always happen shortly after birth due to delayed diagnosis or mild symptoms. Advanced imaging in diagnosis of PAPVC is usually requested in the setting in which a TTE shows right-sided cardiac chamber dilation with unclear delineation of the pulmonary venous anatomy. The role of imaging in PAPVC management is to confirm the diagnosis, define the surgical anatomy, and quantify the shunt to help decide on need for and timing of intervention.


Congenital or Acquired Heart Disease

Congenital or Acquired Heart Disease. Arteriography Coronary with Ventriculography Cardiac catheterization was traditionally the imaging modality used for the diagnosis of TAPVC and PAPVC; however, it has mostly been replaced by less invasive modalities [60]. Cardiac catheterization can be used to demonstrate the anatomy and quantify the degree of shunting using oximetry. However, cardiac catheterization has been shown by some studies to be less accurate in the setting of PAPVC [61]. Occasionally, simultaneous depiction of systemic and pulmonary vascular systems may be difficult in catheter angiography due to overlapping views of adjacent vascular structures. Moreover, it carries high risk of mortality in obstructive TAPVC. The main role for a cardiac catheterization is now in the rare patient with TAPVC who requires interventional cardiac palliation before complete repair, such as atrial septostomy or stent placement [62,63].


Congenital or Acquired Heart Disease

Congenital or Acquired Heart Disease. Arteriography Pulmonary There is no relevant literature to support the use of pulmonary arteriography in the evaluation of known or suspected anomalous pulmonary venous return with inadequate evaluation after TTE.


Congenital or Acquired Heart Disease

Congenital or Acquired Heart Disease. CT Heart Function and Morphology CT heart function and morphology is indicated when MRI is limited by the presence of significant metallic susceptibility artifact obscuring the heart or due to the presence of MR-incompatible pacemakers/devices.


Congenital or Acquired Heart Disease

Congenital or Acquired Heart Disease. Congenital or Acquired Heart Disease CTA Chest CTA chest is useful for the anatomical definition of anomalous pulmonary venous drainage anatomy. CTA chest can also be used in the critical setting of possible obstructed TAPVC to define the level and degree of obstruction, especially in children with infracardiac and mixed TAPVC [64,65]. In obstructed infradiaphragmatic TAPVC, CTA chest can correctly depict the drainage site of the common pulmonary vein, stenosis of the vertical vein, and the course of the atypical vessel into the systemic vein, especially outside the usual echocardiographic windows [66].


Congenital or Acquired Heart Disease

Congenital or Acquired Heart Disease. In other types of TAPVC and PAPVC, CTA chest has excellent spatial resolution and can help define the complete anomalous drainage pathways of the pulmonary veins. CTA chest has the added advantage of being an excellent modality for evaluating the pulmonary parenchyma to look for associated pulmonary and bronchovascular abnormalities (eg, pulmonary sequestration, hypoplastic lung, abnormal bronchial branching) [64,67,68].


Congenital or Acquired Heart Disease

Congenital or Acquired Heart Disease. CTA Coronary Arteries There is no relevant literature to support the use of CTA coronary arteries in the evaluation of known or suspected anomalous pulmonary venous return with inadequate evaluation after TTE.


Congenital or Acquired Heart Disease

Congenital or Acquired Heart Disease. MRA Abdomen Vascular imaging of the abdomen may be helpful in the cases of infracardiac TAPVC or PAPVC to demonstrate the drainage of pulmonary vein into hepatic vein or portal vein. It may be needed to assess the resulting complications such as portal venous system aneurysm formation and collaterals.


Congenital or Acquired Heart Disease

Congenital or Acquired Heart Disease. MRA Chest MRA chest is excellent at identifying the anatomy, specifically the anomalous course and connections of the pulmonary veins [68,69]. A small case study showed that MRA chest was more accurate than both echocardiography and cardiac catheterization in accurately identifying the anomalous veins in 7 patients with TAPVC [70].


Congenital or Acquired Heart Disease

Congenital or Acquired Heart Disease. MRI Heart Function and Morphology MRI heart function and morphology is complementary to MRA chest and can be performed during the same imaging session. MRI heart function and morphology is useful in cases of PAPVC, because it is the ideal noninvasive modality to quantify the shunt amount by means flow rate assessment, ventricular muscle masses, and ventricular volumes and function [68,71]. The shunt quantification is usually performed using phase-contrast imaging through the aorta and pulmonary vein.


Congenital or Acquired Heart Disease

Congenital or Acquired Heart Disease. MRI Heart Function with Stress There is no relevant literature to support the use of MRI heart function with stress in the evaluation of known or suspected anomalous pulmonary venous return with inadequate evaluation after TTE.


Congenital or Acquired Heart Disease

Congenital or Acquired Heart Disease. MRA Neck There is no relevant literature to support the use of MRA neck in the evaluation of known or suspected anomalous pulmonary venous return with inadequate evaluation after TTE.


Congenital or Acquired Heart Disease

Congenital or Acquired Heart Disease. Radiography Chest Chest radiography may demonstrate additional findings such as associated dextrocardia, signs of vascular congestion, and pulmonary edema, which may point towards obstructed TAPVC [65].


Congenital or Acquired Heart Disease

Congenital or Acquired Heart Disease. FDG-PET/CT Heart There is no relevant literature to support the use of FDG-PET/CT heart in the evaluation of known or suspected anomalous pulmonary venous return with inadequate evaluation after TTE.


Congenital or Acquired Heart Disease

Congenital or Acquired Heart Disease. SPECT or SPECT/CT MPI Rest and Stress There is no relevant literature to support the use of SPECT or SPECT/CT MPI rest and stress in the evaluation of known or suspected anomalous pulmonary venous return with inadequate evaluation after TTE.


Congenital or Acquired Heart Disease

Congenital or Acquired Heart Disease. US Echocardiography Transesophageal TEE can be used in cases of PAPVC in which TTE does not completely delineate the partial veins and cardiac chamber enlargement [72,73]. However, the procedure is more invasive than CT or MRI, and it can suffer from


Congenital or Acquired Heart Disease

Congenital or Acquired Heart Disease. Congenital or Acquired Heart Disease airways obscuring some of the acoustic windows, especially for anomalous venous drainage that occurs superior to the right atrium. TEE is also less accurate than MRI for some pulmonary vein anatomy and shunt quantification [72].


Congenital or Acquired Heart Disease

Congenital or Acquired Heart Disease. TEE has just recently been shown to be safe in patients with TAPVC, but only after median sternotomy [74]. There is no published role for TEE as the most useful imaging modality after an insufficient TTE with suspicion for TAPVC.


Congenital or Acquired Heart Disease

Congenital or Acquired Heart Disease. Variant 9: Child or adult. Suspected aortic coarctation with inadequate evaluation after transthoracic echocardiography. Next imaging study. Coarctation of aorta (CoA) refers to discrete or diffuse narrowing in the aorta causing obstruction to the flow of blood. It accounts for 6% to 8% of all CHDs [75]. Aortic coarctation can be more commonly associated with other cardiac lesions such as bicuspid aortic valve, aortic arch hypoplasia, aberrant aortic branch vessels, and other intracardiac and conotruncal defects. This variant will focus on imaging recommendations for aortic coarctation and associated aortic arch pathology. If a patient has associated intracardiac or conotruncal abnormalities, please refer to those variants for recommendations on how to image those concurrent diseases. Aortic coarctation is managed by surgical treatment, transcatheter balloon angioplasty, and transcatheter stent placement.


Congenital or Acquired Heart Disease

Congenital or Acquired Heart Disease. Arteriography Coronary with Ventriculography Before advances in echocardiography, cardiac catheterization was the mainstay for making the diagnosis of CoA. The pressure gradient across the coarctation segment and the collateral vascularity can be accurately assessed with direct angiography. In the current era, it is primarily used when as catheter-based investigation such as balloon angioplasty or stent insertion are being considered [76].


Congenital or Acquired Heart Disease

Congenital or Acquired Heart Disease. Arteriography Pulmonary There is no relevant literature to support the use of pulmonary arteriography in the evaluation of known or suspected aortic coarctation.


Congenital or Acquired Heart Disease

Congenital or Acquired Heart Disease. CT Heart Function and Morphology There is no relevant literature to support the use of CT heart function and morphology in the evaluation of suspected aortic coarctation, although its use was supported in adults by expert opinion in situations in which TEE windows are inadequate. The panel agreed that CT for function is not useful for a child in this clinical scenario.


Congenital or Acquired Heart Disease

Congenital or Acquired Heart Disease. CTA Chest CTA chest is a complementary imaging modality in patients with suspected aortic coarctation after echocardiography. In patients with suboptimal arch imaging with echocardiography, CTA chest works as a great tool to assist with surgical planning [77-79]. Aortic coarctation is often associated with a hypoplastic aortic arch, and CTA chest is helpful in identifying the severity of arch hypoplasia, the length of the hypoplasia, and the relative origins of the aortic arch branch vessels in relation to the coarctation and arch hypoplasia [79].


Congenital or Acquired Heart Disease

Congenital or Acquired Heart Disease. CTA Coronary Arteries There is no relevant literature to support the use of CTA coronary arteries in the evaluation of suspected aortic coarctation.


Congenital or Acquired Heart Disease

Congenital or Acquired Heart Disease. MRA Abdomen There is no relevant literature to support the use of MRA abdomen in the evaluation of suspected aortic coarctation.


Congenital or Acquired Heart Disease

Congenital or Acquired Heart Disease. MRA Chest MRA chest can be used as the second line of imaging after echocardiography in young children who do not require sedation and adolescents [79,80]. One advantage of MRA chest is that it can be combined with MRI heart function and morphology to quantify the degree of collaterals around the coarctation, more accurately estimate pressure gradients across the coarctation, and assess associated cardiac and conotruncal abnormalities [81,82].


Congenital or Acquired Heart Disease

Congenital or Acquired Heart Disease. MRI Heart Function and Morphology MRI heart function and morphology can quantify the degree of collaterals around the coarctation. It can also be easily combined with an MRA chest to provide more comprehensive evaluation in patients with aortic coractation and associated cardiac and conotruncal abnormalities [75].


Congenital or Acquired Heart Disease

Congenital or Acquired Heart Disease

Congenital or Acquired Heart Disease. Congenital or Acquired Heart Disease


Congenital or Acquired Heart Disease

Congenital or Acquired Heart Disease. MRI Heart Function with Stress There is no relevant literature to support the additional use of MRI heart function with stress in the evaluation of suspected aortic coarctation.


Congenital or Acquired Heart Disease

Congenital or Acquired Heart Disease. MRA Neck There is no relevant literature to support the use of MRA neck in the evaluation of suspected aortic coarctation.


Congenital or Acquired Heart Disease

Congenital or Acquired Heart Disease. Radiography Chest Radiography chest has limited clinical utility in diagnosis. Conventional signs on the chest radiograph may be subtle and not always easy to appreciate but may provide clues about the presence of an aortic coarctation.


Congenital or Acquired Heart Disease

Congenital or Acquired Heart Disease. SPECT or SPECT/CT MPI Rest and Stress There is no relevant literature to support the use of SPECT or SPECT/CT MPI rest and stress in the evaluation of suspected aortic coarctation.


Congenital or Acquired Heart Disease

Congenital or Acquired Heart Disease. FDG-PET/CT Heart There is no relevant literature to support the use of FDG-PET/CT heart in the evaluation of suspected aortic coarctation.


Congenital or Acquired Heart Disease

Congenital or Acquired Heart Disease. US Echocardiography Transesophageal TEE is used for intraoperative imaging when other cardiac abnormalities are present in conjunction with CoA. The role of TEE for Doppler examination is limited because the ultrasound beam is almost perpendicular to the line of blood flow, leading to inaccuracies in velocity estimation.


Congenital or Acquired Heart Disease

Congenital or Acquired Heart Disease. Common manifestation of Marfan syndrome include annuloaortic ectasia with or without aortic valve insufficiency, aortic dissection, aortic aneurysm, pulmonary artery dilatation, and mitral valve prolapse. Aortic root aneurysms are present in up to 98% of patients with Loeys-Dietz syndrome, with thoracic aortic dissection being the leading cause of death (67%), followed by abdominal aortic dissection. Arterial tortuosity is a distinguishing feature of Loeys-Dietz syndrome, and there is a propensity for involvement of the extracranial carotid arteries and vertebral arteries [83]. Other cardiovascular abnormalities described in Loeys-Dietz syndrome include coronary artery aneurysms, pulmonary artery aneurysms, and aneurysms of the ductus arteriosus.


Congenital or Acquired Heart Disease

Congenital or Acquired Heart Disease. Ehlers-Danlos syndrome also predisposes to aortic dissections. Nearly 25% of people with bicuspid aortic valve may experience severe aortic valve dysfunction, ascending aortic aneurysm, cardiac death, hospital admission for heart failure, and aortic dissection or rupture [84].


Congenital or Acquired Heart Disease

Congenital or Acquired Heart Disease. Children with aortopathy require regular imaging surveillance to measure the aortic diameter, status of the aortic valve, and cardiac functional status. Current surgical mandate is based on aortic diameter cutoffs to decide the time of surgical intervention [85,86]. The guidelines from the American Heart Association provide thresholds for prophylactic replacement of the aorta, based primarily on aortic diameter or rapid aortic enlargement [87,88].


Congenital or Acquired Heart Disease

Congenital or Acquired Heart Disease. Arteriography Coronary with Ventriculography Coronary artery ectasia can be an associated finding in many connective tissue diseases like bicuspid aortic valve [89], Marfan syndrome, and systemic lupus erythematosus. Coronary arteriography has been useful for imaging, but in current practice, it is used only if coronary CTA or coronary MRA provide insufficient information.


Congenital or Acquired Heart Disease

Congenital or Acquired Heart Disease. Arteriography Pulmonary Pulmonary arteriography is seldom required for diagnosis or surveillance of pulmonary artery dilatation which can occur in Marfan syndrome.


Congenital or Acquired Heart Disease

Congenital or Acquired Heart Disease. CTA Chest CTA chest may be used for imaging surveillance to measure the aortic diameter, and to screen for involvement of the arterial tree in the chest. CTA is especially useful in the emergent setting. ECG gating is critical for accurate assessment of the aortic root.


Congenital or Acquired Heart Disease

Congenital or Acquired Heart Disease. CTA Coronary Arteries CTA is the most useful modality for the evaluation of coronary ectasia or aneurysms, which are often seen in the setting of connective tissue diseases. The diagnostic utility of CTA is very useful compared to coronary MRA in terms of spatial resolution and shorter scan duration.


Congenital or Acquired Heart Disease

Congenital or Acquired Heart Disease. FDG-PET/CT Heart There is no relevant literature to support the use of FDG-PET/CT heart in the evaluation of suspected aortopathy or connective tissue disorder.


Congenital or Acquired Heart Disease

Congenital or Acquired Heart Disease. MRA Chest MRA is the most useful modality for aortic assessment when TTE is suboptimal for assessment of aortopathy in pediatric patients due to its ability to screen multiple territories with the same contrast injection, including the head, neck, chest, and abdomen. To avoid multiple administrations of gadolinium-based contrast agents, follow-up imaging of the aortic root may be performed with noncontrast MRA techniques like cine gradient echo and navigator respiratory gated 3-D steady-state free precession. MRA may offer further valuable information, when complemented with phase-contrast flow data in bicuspid aortic valve, about the possibility of associated valvular regurgitation and alterations in shear stress that may predispose to progressive dilation [91-93].


Congenital or Acquired Heart Disease

Congenital or Acquired Heart Disease. MRA Neck MRA neck is frequently performed at the same setting as MRA chest to screen for extracardiac vascular involvement in connective tissue disease and in all cases of aortopathy to measure vertebral artery tortuosity, which is used as a prognostic marker to determine risk of aortic dissection [94].


Congenital or Acquired Heart Disease

Congenital or Acquired Heart Disease. MRA Abdomen MRA abdomen is frequently performed at the same setting as MRA chest at initial assessment to screen for extracardiac vascular involvement in connective tissue disease. MRA abdomen is also useful for regular follow-up of vessel involvement from connective tissue diseases [86].


Congenital or Acquired Heart Disease

Congenital or Acquired Heart Disease. MRI Heart Function with Stress There is no relevant literature to support the use of MR heart function with stress in the evaluation of aortopathy.


Congenital or Acquired Heart Disease

Congenital or Acquired Heart Disease. Radiography Chest Chest radiography can be used to evaluate for emphysematous bullae and resultant pneumothoraces in the setting of connective tissue disorders [98]. However, chest radiography has no role in aortic surveillance.


Congenital or Acquired Heart Disease

Congenital or Acquired Heart Disease. SPECT or SPECT/CT MPI Rest and Stress There is no relevant literature to support the use of SPECT or SPECT/CT MPI rest and stress in the evaluation of pediatric patients with aortopathy.


Congenital or Acquired Heart Disease

Congenital or Acquired Heart Disease. US Echocardiography Transesophageal TEE can be supplementary modality for assessment of distal ascending aorta especially when TTE is suboptimal, but it is less useful than MRI or CT.


Congenital or Acquired Heart Disease

Congenital or Acquired Heart Disease. Congenital or Acquired Heart Disease Supporting Documents The evidence table, literature search, and appendix for this topic are available at https://acsearch.acr.org/list. The appendix includes the strength of evidence assessment and the final rating round tabulations for each recommendation.


Congenital or Acquired Heart Disease

Congenital or Acquired Heart Disease. For additional information on the Appropriateness Criteria methodology and other supporting documents go to www.acr.org/ac.


Hemoptysis

Hemoptysis. Initial Imaging Definition Imaging is considered at the beginning of the care episode for the medical condition defined by the variant. More than one procedure can be considered usually appropriate in the initial imaging evaluation when:


Hemoptysis

Hemoptysis. aRadiology Imaging Associates, Englewood, Colorado. bResearch Author, Keck School of Medicine of USC, Los Angeles, California. cPanel Chair, Vanderbilt University Medical Center, Nashville, Tennessee. dPanel Vice-Chair, University of California San Francisco, San Francisco, California. eStanford University Medical Center, Stanford, California; The Society of Thoracic Surgeons. fSchmidt College of Medicine, Florida Atlantic University, Boca Raton, Florida. gUniversity of Southern California, Los Angeles, California. hBeaumont Health System, Royal Oak, Michigan: American College of Emergency Physicians. iNaval Medical Center Portsmouth, Portsmouth, Virginia. jUniversity of Iowa Hospitals and Clinics, Iowa City, Iowa. kVanderbilt University Medical Center, Nashville, Tennessee; American College of Chest Physicians. lUniversity of Wisconsin, Madison, Wisconsin. mMallinckrodt Institute of Radiology, Saint Louis, Missouri. nJohn H. Stroger, Jr. Hospital of Cook County, Chicago, Illinois; American College of Physicians. oDuke University School of Medicine, Durham, North Carolina; The Society of Thoracic Surgeons. pThe University of Texas MD Anderson Cancer Center, Houston, Texas. qSpecialty Chair, University of Wisconsin School of Medicine and Public Health, Madison, Wisconsin.


Hemoptysis

Hemoptysis. The American College of Radiology seeks and encourages collaboration with other organizations on the development of the ACR Appropriateness Criteria through society representation on expert panels. Participation by representatives from collaborating societies on the expert panel does not necessarily imply individual or society endorsement of the final document.


Hemoptysis

Hemoptysis. Reprint requests to: publications@acr.org


Hemoptysis

Hemoptysis. All elements are essential: 1) timing, 2) reconstructions/reformats, and 3) 3-D renderings. Standard CTs with contrast also include timing issues and recons/reformats. Only in CTA, however, is 3-D rendering a required element. This corresponds to the definitions that CMS has applied to the Current Procedural Terminology codes.


Hemoptysis

Hemoptysis. Massive hemoptysis due to an unknown cause (ie, cryptogenic hemoptysis) has similar BAE outcomes compared with hemoptysis from a known cause. An early study treating cryptogenic hemoptysis with BAE was published in 2010 [22]. In this study, 39 patients with cryptogenic hemoptysis who presented with varying severity of hemorrhage were all treated medically. Hemoptysis remained uncontrolled in 21 patients, who subsequently underwent successful BAE; 2 patients had recurrent hemoptysis. A second retrospective review compared BAE outcomes of 26 patients with cryptogenic hemoptysis to 152 patients with a known cause of hemoptysis over the same interval. Both groups showed a 100% immediate success rate following BAE, and both groups demonstrated similar recurrence rates (12% versus 25%, respectively, which was not statistically different) [8].


Hemoptysis

Hemoptysis. Over 90% of massive hemoptysis is due to a systemic arterial supply, and therefore conventional pulmonary arteriography is rarely performed. An early study documenting this was authored by Sbano et al [23], reporting a pulmonary arterial bleeding origin in 8 of 76 patients with massive hemoptysis. Although all of these patients were initially treated with BAE, 7 out of 8 patients required additional pulmonary artery embolization. Shin et al [24] identified 10 patients with pulmonary artery pseudoaneurysms (PAPs) out of 286 patients presenting with massive hemoptysis undergoing BAE. These authors reported a pulmonary artery embolization success rate above 90%. Khalil et al [25] retrospectively reviewed 272 patients, 13 of whom had bleeding from a pulmonary artery origin. Of these 13 patients, 11 underwent pulmonary artery embolization, all of which were successful. The largest study to date isolating patients with massive hemoptysis due to a pulmonary arterial source identified 24 patients from a cohort of 712 patients presenting with massive hemoptysis [26]. This study reported an 88% pulmonary artery embolization success rate.


Hemoptysis

Hemoptysis. Overall, BAE is now universally accepted as a safe and effective intervention for the treatment of massive hemoptysis.


Hemoptysis

Hemoptysis. CT Chest with IV Contrast The utility of CT in determining the cause for hemoptysis was first established in the late 1990s. Naidich et al [27] reported CT-bronchoscopic correlations of 58 patients with hemoptysis (severity not specified), showing CT was superior to bronchoscopy in both the diagnosis of lung cancer (17 CT positive versus 15 bronchoscopy positive) and definitive staging of lung cancer (48% versus 14%, respectively). They also established that CT was superior to radiography. Of the 23 normal chest radiographs, subsequent CT provided a definitive cause in 9 patients. Revel et al [28] published data from 80 patients with massive hemoptysis demonstrating CT was more efficient than the previous reference standard of bronchoscopy in identifying the etiology of hemoptysis (77% versus 8%, respectively; P < .001).


Hemoptysis

Hemoptysis. Based on these studies, CT can facilitate BAE planning by potentially identifying a specific lesion or isolating the bleeding artery based on increased arterial diameter and wall irregularity in a high percentage of cases.


Hemoptysis

Hemoptysis. CT Chest without IV Contrast Several early studies have established the use of CT in the diagnosis of hemoptysis used high-resolution CT (HRCT). For example, Tsoumakidou et al [32] followed 184 patients with varying degrees of hemoptysis, demonstrating that HRCT identified a cause in 41% of patients with a normal chest radiograph. In 2008, Khalil et al [25] reported on the utility of performing HRCT in the emergent management of hemoptysis in the intensive care unit.


Hemoptysis

Hemoptysis. With the advancement of technology, the vast majority of CT examinations can be reformatted to the resolution of the previously ordered HRCT, and there is rarely an added benefit of HRCT to a routine CT. Khalil et al [33] subsequently published a study retrospectively evaluating patient outcomes comparing a cohort of patients who had a CT without IV contrast with a cohort of patients who underwent CTA. There was a statistically significant difference in the number of emergent surgical resections following embolization in patients who did not have a CTA preceding the BAE (10% CT without cohort versus 4.5% CTA cohort). IV contrast is also well established as an agent that significantly improves the visualization of mediastinal structures. As discussed above, IV contrast shows an added benefit to preprocedural embolization planning.


Hemoptysis

Hemoptysis. Therefore, CT chest without IV contrast is only warranted in the diagnosis of massive hemoptysis in patients with poor renal function or life-threatening contrast allergy.


Hemoptysis

Hemoptysis. CT Chest without and with IV Contrast Although early studies used CT chest protocols without IV contrast followed by with IV contrast, there are no data to support any added value of a CT chest without IV contrast prior to administering contrast in the diagnosis of hemoptysis or in preprocedural planning for BAE.


Hemoptysis

Hemoptysis. CTA Chest CTA has also proven to be beneficial in detecting bronchial and nonbronchial arteries in preprocedural planning. Remy-Jardin et al [34] documented the utility of CTA for BAE preprocedural planning, demonstrating an 86% concordance rate between the 58 abnormal arteries identified on CTA compared with the gold standard of conventional arteriography. Hartmann et al [35] retrospectively reviewed 251 patients (with varying severity of hemoptysis) who were imaged by CTA. Of these 251 patients, 214 had CTAs that were of diagnostic quality without confounding central mediastinal pathology. The purpose of this study was to analyze the frequency of aberrant and ectopic locations of bleeding arteries. Of the 24 patients who required BAE, 36% had aberrant bronchial origins, and the authors asserted that CTA information guided successful and rapid catheterization in 22 patients and precluded repeated arteriograms. CTA information changed treatment strategy in 2 of the 24 patients with aberrant arteries, resulting in initial treatment with surgical ligation rather than BAE because of an anticipated high risk of


Hemoptysis

Hemoptysis. Hemoptysis embolization based on vascular location. Mori et al [36] were also early in advocating CTA for preprocedural planning, identifying bronchial artery diameter as an important diagnostic clue on CTA in determining the bleeding artery requiring embolization. Jiang et al [37] reported results from 818 patients obtaining a CTA for preprocedural BAE planning, which isolated 6 aberrant arteries that would not have been detected by standard angiographic procedures. Lin et al [38] also reported a high concordance rate between CTA and conventional arteriography, reporting that 107 of the 110 arteries embolized (97%) were prospectively identified on CTA. This article noted that CTA was useful in both identifying the number of vessels involved in hemoptysis as well as identifying collateral vessels and shunts that increase the risk of complications during arterial embolization.


Hemoptysis

Hemoptysis. As discussed in the section above, approximately 10% of massive hemoptysis is due to a pulmonary arterial source, which can be occult on bronchial arteriography. Khalil et al [25] retrospectively reviewed 272 patients, 13 of whom had bleeding from a pulmonary artery origin. Of these 13 patients, 8 were initially and successfully treated with pulmonary artery embolization based on the CTA findings, and 3 more patients were subsequently imaged and successfully treated with pulmonary artery embolization after BAE failed to treat the hemoptysis. This study highlighted CTA guiding embolization therapy. A study by Shin et al [24] also documented the importance of detecting a pulmonary artery source of hemoptysis prior to embolization. In his study of 286 patients presenting with massive hemoptysis, they used CTA to identify 10 patients for a total of 11 PAPs as the source of the massive hemoptysis by CTA prior to undergoing BAE. Of these 11 PAPs, 6 PAPs detected on CTA could not be detected prospectively on conventional pulmonary arteriography, but CTA guided subselection pulmonary arteriography and resulted in successful embolization of the bleeding artery. The authors reported a pulmonary artery embolization success rate of over 90%. A subsequent study by Shin et al [26] also identified patients presenting with massive hemoptysis due to a pulmonary arterial source. Out of 712 patients with massive hemoptysis, 24 patients demonstrated PAPs on their preprocedural CTA examination. Fifteen of these PAPs identified on CTA were also visualized on pulmonary arteriography, all of which were successfully embolized. The remaining 9 PAPs identified on CTA were not detectable on conventional pulmonary arteriography and were subsequently treated with bronchial and systemic nonbronchial embolization, resulting in a 33% rate of hemoptysis cessation. Persistently symptomatic PAPs were subsequently treated by percutaneous or surgical interventions based on CTA findings.


Hemoptysis

Hemoptysis. There are no recent data comparing the diagnostic advantages between routine CT with IV contrast with CTA. However, CTA may offer a slight advantage over routine CT with IV contrast when the intention is to treat massive hemoptysis with BAE because CTA typically provides slightly better opacification of vessels, possibly improving detection of abnormal arteries potentially causing hemoptysis. At present, the vast majority of publications reporting BAE outcomes obtain CTA chest CTs prior to BAE for preprocedural planning.


Hemoptysis

Hemoptysis. Radiography Chest Chest radiography has long been established as the initial imaging examination of choice given its portability, rapid acquisition, and interpretation time. Fartoukh et al [12], in a large retrospective study of 1,087 patients, correlated morbidity and mortality to findings on chest radiographs. However, there is discrepancy in the literature regarding the usefulness of radiographs in determining the etiology of hemoptysis.