When signs of volume overload are indicative of significant left-to-right shunt, closing the PDA is likely to prevent further left atrial or LV enlarge- ment, progression or development of PAH, and pulmonary hypertension secondary to left HF and will possibly provide symptom relief if symp- toms are present. Closure is typically performed percutaneously with good success and minimal Pulmonary blood flow and thus Qp:Qs can be difficult to calculate accurately because of differences in right/left PA blood flow caused by the flow from the PDA. Invasive he- modynamics including pulmonary vascular resist- ance are generally relied on for decision-making. Surgical closure can be performed but is poten- tially hazardous in adults because of calcification and tissue fragility. 4. Even with elevated pulmonary pressure and el- evated pulmonary vascular resistance, closure of a PDA may improve clinical status in some patients with persistent left-to-right shunting and prevent further progression of PAH.S4.1.5-3,S4.1.5-4 Consultation with ACHD and pulmonary hyper- tension providers is important given the low fre- quency of this circumstance and the complexity of decision-making. 5. Morbidity and mortality are high when closure of a shunt is attempted in patients with Eisenmenger physiology with elevated pulmonary pressure and net right-to-left shunting.S4.1.5-5 Table 16. PDA: Routine Follow-Up and Testing Intervals Frequency of Routine Follow-Up and Testing Physiological Stage A* (mo) Physiological Stage B* (mo) Physiological Stage C* (mo) Physiological Stage D* (mo) Pulse oximetry As needed As needed Each visit Each visit Exercise test As needed As needed 12 24 6 12 *See Tables 3 and 4 for details on the ACHD AP classification system. Upper and lower extremity. 6-minute walk test or CPET, depending on the clinical indication. ACHD indicates adult congenital heart disease; ECG, electrocardiogram; CPET, cardiopulmonary exercise test; PDA, patent ductus arteriosus; and TTE, transthoracic echocardiogram. CLINICAL STATEMENTS AND GUIDELINES 4.2. Left-Sided Obstructive Lesions 4.2.1. Cor Triatriatum Recommendations for Cor Triatriatum Referenced studies that support recommendations are summarized in Online Data Supplement 31. COR LOE Recommendations Diagnostic I B-NR Adults presenting with cor triatriatum sinister should be evaluated for other congenital abnormalities, particularly ASD, VSD, and anomalous pulmonary venous connection.S4.2.1-1 IIa B-NR In adults with prior repair of cor triatriatum sinister and recurrent symptoms, it is reasonable to evaluate for pulmonary vein Therapeutic I B-NR Surgical repair is indicated for adults with cor triatriatum sinister for symptoms attributable to the obstruction or a substantial gradient across the membraneS4.2.1-3 Synopsis Cor triatriatum occurs when a membrane divides either the left atrium (sinister), or right atrium (dexter). Cor tria- triatum sinister is usually associated with other congen- ital malformations, specifically ASD, VSD, or anomalous pulmonary venous connection (partial or total).S4.2.1-1 The left atrial appendage is invariably in the same chamber as the mitral valve, separated from the pulmo- nary veins by the membrane. Supravalvular mitral steno- sis is typically caused by a fibrous ring on the atrial side of the mitral valve, separating the mitral valve from both the left atrial appendage and the pulmonary veins. The finding will have similar physiology to cor triatriatum and similar indications for intervention. It can be associated with an abnormal mitral valve that may also require in- tervention. Supravalvular mitral stenosis often comprises one part of a more complex sequence of serial left-sided inflow and outflow obstructions (ie, Shone complex). See Section 3.3 for recommendations on who should perform surgeries, cardiac catheterization, and other procedures in these patients; and Section 3.4 for recommendations on diagnostic evaluation. Recommendation-Specific Supportive Text 1. Cor triatriatum sinister is a membrane spanning the left atrium. Surgery has been largely success- ful with relatively few early or late deaths, which are usually attributable to associated congenital The gradient across the de- fect at the time of surgery was at least 8 mm Hg (mean 17 mm Hg; range 8 to 40 mm Hg).S4.2.1-3 After repair, recurrence of stenosis is not ex- pected. Although pulmonary vein stenosis has been demonstrated before and after surgery,S4.2.1-2 it is not usually progressive over time and has not been associated with PAH. 2. Pulmonary venous stenosis has been demon- strated before and after surgery, but it is not usually progressive over time and has not been associated with PAH. 3. Although risks of isolated cor triatriatum sinister surgery is low, it should be performed when there is evidence of a substantial gradient. In 1 series, the mean gradient at the time of surgical repair was at least 8 mm Hg.S4.2.1-3 It is conceivable that on occasion, clinical circumstances (ie, symp- toms, arrhythmia) would warrant intervention in patients with lower gradients. 4.2.2. Congenital Mitral Stenosis Recommendation for Congenital Mitral Stenosis Referenced studies that support the recommendation are summarized in Online Data Supplement 32. COR LOE Recommendation I B-NR Adults with congenital mitral stenosis or a parachute mitral valve should be evaluated for other left-sided obstructive Synopsis Congenital mitral valve disease may be anatomically complex and is often accompanied by other lesions. Indications for intervention in mitral stenosis are de- scribed in the 2014 VHD guidelineS4.2.2-3 and apply to those patients with congenital mitral stenosis. Balloon mitral valvuloplasty is rarely, if ever, indicated or effec- tive in congenital mitral stenosis. See Section 3.3 for recommendations on who should perform surgeries, cardiac catheterization, and other procedures in these patients; Section 3.4 for rec- ommendations on diagnostic evaluation; and Table 17 for routine testing and follow-up intervals. Recommendation-Specific Supportive Text 1. Parachute mitral valve is most commonly found in the presence of other congenital abnormali- ties such as the components of Shone complex. Recurrence and progression of the various asso- ciated lesions are expected, subsequent surger- ies are common, and mortality may be associated with other defects.S4.2.2-2 Therefore, these patients require follow-up at a center where such abnor- malities can be followed and future interventions considered. Choices and techniques for valve re- pair or replacement are based on consideration of coexisting abnormalities including the likelihood of future surgery. CLINICAL STATEMENTS AND GUIDELINES 4.2.3. Subaortic Stenosis Recommendations for Subaortic Stenosis Referenced studies that support recommendations are summarized in Online Data Supplement 33. COR LOE Recommendations Diagnostic IIb C-LD Stress testing for adults with LVOT obstruction to determine exercise capacity, symptoms, electrocardiographic changes, or arrhythmias may be reasonable in the presence of otherwise equivocal indications Therapeutic I C-EO Surgical intervention is recommended for adults with subAS, a maximum gradient 50 mm Hg or more and symptoms attributable to the subAS. I C-LD Surgical intervention is recommended for adults with subAS and less than 50 mm Hg maximum gradient and HF or ischemic symptoms, and/or LV systolic dysfunction attributable to subAS.S4.2.3-3 IIb C-LD To prevent the progression of AR, surgical intervention may be considered for asymptomatic adults with subAS and at least mild AR and a maximum gradient of 50 mm Hg or more.S4.2.3-4 S4.2.3-6 Synopsis SubAS may occur as a discrete membrane below the aortic valve in the LVOT, as a longer tunnel-like obstruc- tion, as a consequence of chordal attachments in pa- tients with abnormalities such as AVSD, or because of surgical repairs involving VSD baffled to a transposed aorta, such as seen in the Rastelli operation. SubAS may occur in isolation or as part of a suite of abnormalities. In adults with Shone complex or its variants, subAS may be one of several LV obstructive lesions, including vari- ants of congenital mitral stenosis, supravalvular mitral stenosis, valvular aortic stenosis, supravalvular aortic stenosis, and CoA.S4.2.3-7 SubAS tends to recur, particularly when initial resec- tion is needed in childhood. Surgical repair for subAS carries a 10% to 15% risk of complete heart block.S4.2.3-6 SubAS may be first diagnosed in adulthood and may be confused with hypertrophic obstructive cardiomyopa- thy when LV hypertrophy of sufficient severity has de- veloped such that the subaortic membrane is less evi- dent on imaging. The recommendations in this guideline apply to subAS caused by a discrete membrane or tunnel-like obstruction. Similar principles may apply to more com- plex causes of subAS, but insufficient data exist to sup- port recommendations for more complex lesions, and extrapolation needs to take the additional anatomic complexity into account. Turbulent flow created distal to the subaortic ob- struction may cause barotrauma to the adjacent aortic valve leaflets and result in progressive AR, which may itself become clinically significant. Resection of the sub- aortic obstruction ideally delays or prevents the even- tual need for aortic valve replacement, and concomi- tant aortic valve repair could also help delay the need for aortic valve replacement in these cases. See Section 3.3 for recommendations on who should perform surgeries, cardiac catheterization, and other procedures in these patients; Section 3.4 for rec- ommendations on diagnostic evaluation; and Table 18 for routine testing and follow-up intervals. Recommendation-Specific Supportive Text 1. Exercise stress testing may be reasonable in the assessment of exercise capacity, stress-induced arrhythmias, and ischemia in patients with subAS and may be considered as an adjunct to echocar- diographic imaging. 2. Patients with symptomatic subAS should attain symptomatic improvement from surgical relief of the obstruction. In some cases, concomitant AVR may be needed, if indicated according to GDMT. 3. Patients with depressed LV systolic function and severe subAS may not manifest a resting gradient of 50 mm Hg. In this population, evaluation and decisions regarding surgical relief of LVOT obstruc- tion can be extrapolated from the existing aortic stenosis data and should be considered as per the Additionally, patients Table 17. Congenital Mitral Stenosis: Routine Follow-Up and Testing Intervals Frequency of Routine Follow-Up and Testing Physiological Stage A* (mo) Physiological Stage B* (mo) Physiological Stage C* (mo) Physiological Stage D* (mo) *See Tables 3 and 4 for details on the ACHD AP classification system. 6-minute walk test or CPET, depending on the clinical indication. ACHD indicates adult congenital heart disease; CPET, cardiopulmonary exercise test; ECG, electrocardiogram; and TTE, transthoracic echocardiogram. CLINICAL STATEMENTS AND GUIDELINES with preserved LV systolic function but poor LV compliance may present with signs or symptoms of HF and a resting maximum gradient <50 mm Hg. These patients may benefit from surgical re- lief of LVOT obstruction. Patients with evidence of resting or stress-induced ischemia in the absence of obstructive coronary artery disease and in the presence of moderate subAS (maximum gradient >30 mm Hg and <50 mm Hg) may benefit from surgical relief of subAS.S4.2.3-9 4. Discrete subAS tends to be progressive with age, and patients with a resting maximum gradient 50 mm Hg are more likely to have progressive subAS and concomitant moderate or severe aortic Therefore, surgical inter- vention may be considered in the asymptomatic patient with severe subAS. Tunnel-type subAS, which is often associated with a small aortic valve annulus, is associated with worse long-term out- comes and a higher risk of recurrence after sur- gical resection compared with subAS caused by a discrete membrane.S4.2.3-5 Surgical intervention on patients with asymptomatic subAS (maximum gradient 50 mm Hg) with preserved LV ejection fraction may delay progression of, or improve the degree of, aortic valve regurgitation. SubAS in adults may progress more slowly than in children, and although mild AR is common, it may not be progressive in medium-term follow-up.S4.2.3-10 4.2.4. Congenital Valvular Aortic Stenosis Recommendations for Congenital Valvular Aortic Stenosis Referenced studies that support recommendations are summarized in Online Data Supplement 34. COR LOE Recommendations Diagnostic I B-NR Adults with bicuspid aortic valve should be evaluated for coarctation of the aorta by clinical examination and imaging IIa B-NR It is reasonable to screen first-degree relatives of patients with bicuspid aortic valve or unicuspid aortic valve with echocardiography for valve disease and Therapeutic IIb B-NR In adults with bicuspid aortic valve stenosis and a noncalcified valve with no more than mild AR meeting indications for intervention it may be reasonable to treat with balloon valvuloplasty.S4.2.4-6 Synopsis Indications for aortic valve replacement according to generally apply. Recom- mendations above deal with issues specific to congen- ital aortic valve disease, which includes BAV, as well as unicuspid aortic valve and aortic stenosis caused by hy- poplastic aortic annulus. The underlying anatomy must be taken into account in patients with congenital aortic stenosis, as intervention may need to include annular enlarging procedures and other surgical techniques not commonly used in valvular aortic stenosis. These pa- tients are often young adults, for whom lifestyle consid- erations such as athletic endeavors, employment, and childbearing may influence the type of intervention. See Section 3.4 for recommendations on diagnos- tic evaluation; and Table 19 for routine testing and fol- low-up intervals. Recommendation-Specific Supportive Text 1. CoA has a male-to-female ratio of 1.5:1.S4.2.4-7 S4.2.4-13 A BAV is present in 50% to 70% of cases of CoA. Table 18. Subaortic Stenosis: Routine Follow-Up and Testing Intervals Frequency of Routine Follow-Up and Testing Physiological Stage A* (mo) Physiological Stage B* (mo) Physiological Stage C* (mo) Physiological Stage D* (mo) *See Tables 3 and 4 for details on the ACHD AP classification system. 6-minute walk test or CPET, depending on the clinical indication. ACHD indicates adult congenital heart disease; CPET, cardiopulmonary exercise test; ECG, electrocardiogram; SubAS, subaortic stenosis; and TTE, transthoracic echocardiogram. Recommendations for Congenital Valvular Aortic Stenosis (Continued) COR LOE Recommendations Table 19. Congenital Aortic Stenosis: Routine Follow-Up and Testing Intervals* Stage Frequency of Echocardiogram Progressive (Stage B) Every 3 5 y (mild severity, Vmax 2.0 2.9 m/s) Every 1 2 y (moderate severity, Vmax 3.0 3.9 m/s) Severe (Stage C) Every 6 12 mo (Vmax 4.0 m/s) Aortic dilation >4.5 cm Every 12 mo (echocardiogram, MRI or CT) *Modified from existing GDMT for valvular heart disease.S4.2.4-5 CT indicates computed tomography; GDMT, guideline-directed management and therapy; MRI, magnetic resonance imaging; and Vmax, maximum velocity. CLINICAL STATEMENTS AND GUIDELINES Given the association of these abnormalities, eval- uation of patients with BAV for CoA is warranted. 2. BAV is the most prevalent congenital cardiac abnormality with an estimated prevalence of 4.6 per 1000 live births, and is 1.5 times more prevalent in males than females.S4.2.4-7 S4.2.4-13 Most cases are spontaneous; however, familial inheritance may occur in an autosomal domi- nant pattern with variable penetrance. On ech- ocardiographic screening, 1 study reports the prevalence of asymptomatic BAV in first-degree relatives of patients is 9%, and 32% of first-de- gree relatives without a BAV will have an ab- 3. Calcification of the aortic valve in adults neces- sitates that most patients who require therapy for aortic stenosis will require aortic valve re- placement per GDMT.S4.2.4-5 However, young patients with congenitally abnormal valves and relatively little calcification may be candidates for balloon valvuloplasty. Balloon valvuloplasty may improve the degree of stenosis and symp- toms in patients with mobile noncalcified BAV stenosis. In general, the valves that would be amenable to successful balloon valvuloplasty are found in young patients, who are often <25 years of age. Restenosis will occur over time and in a relatively short time in some patients. Balloon valvuloplasty of calcified BAV is associ- ated with decreased efficacy and an increased risk of AR.S4.2.4-14,S4.2.4-15 Although transcatheter interventions for aortic stenosis are increasingly commonly performed in older adults and, thus, there are increasing numbers of interventional cardiologists technically skilled at balloon aortic valvuloplasty and transcatheter aortic valve re- placement, the differences in anatomy and pa- tient population necessitate collaboration with an ACHD cardiologist for younger patients. 4.2.4.1. Turner Syndrome Recommendations for Turner Syndrome Referenced studies that support recommendations are summarized in Online Data Supplement 35. COR LOE Recommendations Diagnostic I B-NR Women with Turner syndrome should be evaluated for bicuspid aortic valve, coarctation of the aorta, and enlargement of the ascending aorta.S4.2.4.1-1 Therapeutic IIa B-NR Prophylactic replacement of the aortic root or ascending aorta in adults with Turner syndrome is reasonable when the aortic Synopsis The management of valve dysfunction is generally as directed by the 2014 VHD guideline.S4.2.4.1-3 Aortopathy is a commonly associated condition, and frequently in- volves the mid-ascending aorta, which may not be re- liably seen on TTE. Measurement of aortic dimensions with magnetic resonance angiography and CCT has not been standardized, and clinicians should be wary of comparisons of reported diameters between modali- ties. Side-by-side comparisons are more reliable for de- tecting changes over time. Baseline and routine serial measurements of the aortic size are useful, with im- aging interval determined by the indexed size and rate of progression. Pregnancy in Turner syndrome, which often requires assisted reproductive technology, is asso- ciated with an increased risk of aortic dissection, espe- cially if there is a preexisting abnormality of the aortic valve or aorta.S4.2.4.1-4 Recommendation-Specific Supportive Text 1. Women with Turner syndrome are at substantial risk of BAV, CoA, and aortic enlargement, which can result in morbidity and mortality if left un- treated. Therefore, evaluation is necessary to help decide what interventions may be necessary and provide accurate risk assessment for exercise, pregnancy, or other considerations that could be influenced by aortic pathology. 2. Because of case series reporting dissection at smaller aortic diameters than in non-Turner aor- topathy, prophylactic surgery is reasonable at lower diameters, particularly if rapid dilation is present. Measurements must take into account the patient's stature either by indexing to body surface area utilizing Turner-specific norma- tive data or by using ratio of aortic area to body Several CHD subtypes and/or repairs are associated with enlargement of the aorta. The management of these varies by condition, as some are perceived to have a stronger association with aortic dissection or rupture than others, although the true natural history of most is unknown. There is wide heterogeneity of timing of surgical referral, which makes interpretation of longitu- dinal studies problematic. BAV is the most common CHD, is associated with aortopathy, and is of high concern for aortic complica- tions, as discussed in other guideline statements.S4.2.4.2-1 Although in many published series of aortic dis- section, BAV patients account for a higher proportion of dissections than expected from prevalence of BAV CLINICAL STATEMENTS AND GUIDELINES in the general population alone, the risk of dissection or rupture amongst all BAV patients is less clear. The largest population study reported a 0.5% risk of aortic rupture or dissection after a mean of 16 years of follow- although 11% underwent elective aortic sur- gery. Risk factors for aortic complications were age and an enlarged aorta at baseline. Frequency of dissection in BAV disease is higher in adults with Turner syndrome. A dilated neoaortic root after a Ross procedure is not uncommon, although only a single dissection has been Because of this, it is generally believed that prophylactic root replacement strategies based on sinus of Valsalva diameters can be less aggressive after a Ross procedure than in a native BAV patient, but practice patterns vary. Most patients with a Ross repair had under- lying congenitally abnormal aortic valves (BAV or unicus- pid aortic valve) and, therefore, are at risk of the ascend- ing aortic dilation typical of those abnormalities. Thus, in addition to the dilation at the sinuses of Valsalva associ- ated with the Ross repair, dilation of the native ascending aorta above the sinotubular junction can also occur. Although patients with conotruncal abnormalities (TOF, dextrotransposition of the great arteries [d-TGA] after arte- pulmonary atresia with VSD, trun- cus arteriosus) commonly have aortic diameters of 40 mm to 50 mm, aortic complications are extremely rare (only Therefore, there is no strong justification for empiric prophylactic surgery in such patients based solely on aortic diameter. Watchful observation has often been recommended unless surgery is being undertaken for other indications.S4.2.4.2-15 However, there are rare patients who develop substantially greater aortic enlargement and for whom prophylactic surgery may have more of a role. Risk factor management such as control of hypertension is important. There are no RCTs evaluating the efficacy of medical therapy to reduce the rate of progression of aortic dilation or incidence of aortic dissection in this population. See Online Data Supplement 36 for referenced studies. 4.2.5. Supravalvular Aortic Stenosis Recommendations for Supravalvular Aortic Stenosis Referenced studies that support recommendations are summarized in Online Data Supplement 37. COR LOE Recommendations Diagnostic I C-LD Aortic imaging using TTE, TEE, CMR, or CTA is recommended in adults with Williams syndrome or patients suspected of having supravalvular aortic stenosis.S4.2.5-1 I C-LD Coronary imaging is recommended in patients with Williams syndrome and supravalvular aortic stenosis presenting with symptoms of coronary ischemia.S4.2.5-2 S4.2.5-4 Therapeutic I B-NR Surgical repair is recommended for adults with supravalvular aortic stenosis (discrete or diffuse) and symptoms or decreased LV systolic function deemed secondary to aortic I C-LD Coronary artery revascularization is recommended in symptomatic adults with supravalvular aortic stenosis and coronary Synopsis Supravalvular aortic stenosis is a relatively rare condition o- verall but is seen commonly in patients with Williams syn- drome or homozygous familial hypercholesterolemia. The stenotic ridge tends to occur distal to the coronary artery orifices at the sinotubular junction. In addition to pressure load physiology similar to other causes of LVOT obstruc- tion, coronary abnormalities can occur, including signifi- cant coronary ostial stenosis resulting in risk of SCD and Unlike subAS or valvular aortic stenosis, the coronary arteries are exposed to the higher pressure generated by the supravalvular obstruction. See Section 3.3 for recommendations on who should perform surgeries, cardiac catheterization, and other procedures in these patients; Section 3.4 for rec- ommendations on diagnostic evaluation; and Table 20 for routine testing and follow-up intervals. Table 20. Supravalvular Aortic Stenosis: Routine Follow-Up and Testing Intervals Frequency of Routine Follow-Up and Testing Physiological Stage A* (mo) Physiological Stage B* (mo) Physiological Stage C* (mo) Physiological Stage D* (mo) *See Tables 3 and 4 for details on the ACHD AP classification system. Routine TTE may be unnecessary in a year when CMR imaging is performed unless clinical indications warrant otherwise. CMR may be indicated for assessment of aortic anatomy. Baseline study is recommended with periodic follow-up CMR, with frequency of repeat imaging determined by anatomic and physiological findings. If CCT is used instead of CMR imaging, the frequency should be weighed against radiation exposure. 6-minute walk test or CPET, depending on the clinical indication. ACHD indicates adult congenital heart disease; CCT, cardiac computed tomography; CMR, cardiovascular magnetic resonance; CPET, cardiopulmonary exercise test; ECG, electrocardiogram; and TTE, transthoracic echocardiogram. CLINICAL STATEMENTS AND GUIDELINES Recommendation-Specific Supportive Text 1. TTE with Doppler imaging is useful in deriving peak and mean pressure gradients across the area of supravalvular aortic stenosis from apical, supra- sternal, and right parasternal views; however, vis- ualization of the full extent of supravalvular aortic stenosis with TTE is limited. TEE is superior in this regard, and 3D TEE allows excellent visualization of the narrowed ascending aorta. CMR and CTA provide comprehensive and detailed images of supravalvular aortic stenosis and are used with echocardiography in the assessment of patients before and after repair.S4.2.5-15 2. Impaired coronary perfusion may occur because of varying degrees of aortic valve leaflet adhe- sion to the narrowed sinotubular junction or because of fibrotic thickening in the area im- mediately surrounding the coronary ostia. This causes ostial stenosis with restriction in diastolic filling of the coronary arteries; the left coronary is most frequently involved. TEE with Doppler can be used in the assessment of proximal coronary patency and to search for flow turbulence. CMR can also be used in assessing the coronary ostia. Electrocardiographic-gated CT coronary angiog- raphy or invasive selective coronary angiography provides excellent visualization of the coronary ar- terial anatomy. 3. Supravalvular aortic stenosis is usually a progres- sive problem with a progressive increase in LV systolic pressure resulting in exertional symptoms and, if the stenosis is severe, eventual decreases in LV systolic function. 4. Impaired coronary perfusion may occur because of varying degrees of aortic valve leaflet adhesion to the narrowed sinotubular junction with restric- tion in diastolic filling of the coronary arteries; the left coronary is most frequently involved. Surgical coronary revascularization is recommended for patients with symptoms of coronary ischemia. 4.2.6. Coarctation of the Aorta Recommendations for Coarctation of the Aorta Referenced studies that support recommendations are summarized in Online Data Supplement 38. COR LOE Recommendations Diagnostic I B-NR Initial and follow-up aortic imaging using CMR or CTA is recommended in adults with coarctation of the aorta, including those who have had surgical or catheter I C-EO Resting blood pressure should be measured in upper and lower extremities in all adults with coarctation of the aorta. Diagnostic (Continued) IIa C-LD Ambulatory blood pressure monitoring in adults with coarctation of the aorta can be useful for diagnosis and management of IIb B-NR Screening for intracranial aneurysms by magnetic resonance angiography or CTA may be reasonable in adults with coarctation of the aorta.S4.2.6-5,S4.2.6-6 IIb C-LD Exercise testing to evaluate for exercise- induced hypertension may be reasonable in adults with coarctation of the aorta who Therapeutic I B-NR Surgical repair or catheter-based stenting is recommended for adults with hypertension and significant native or recurrent coarctation of the aorta.S4.2.6-1,S4.2.6-2,S4.2.6-8 S4.2.6-12 I C-EO GDMT is recommended for treatment of hypertension in patients with coarctation of IIb B-NR Balloon angioplasty for adults with native and recurrent coarctation of the aorta may be considered if stent placement is not feasible and surgical intervention is not an option.S4.2.6-14 Synopsis CoA typically occurs near the ductal remnant and left subclavian artery. Hypertension is the most common se- quela of CoA, whether repaired or unrepaired. BAV is commonly associated with CoA and is present in more than half of CoA patients.S4.2.6-15 S4.2.6-21 Intracranial aneu- rysms may occur. Ascending aortic aneurysms are often found in those with BAV, and aneurysms are seen at the site of coarctation repair in the descending thoracic aorta or arch. Dissection can occur, presumably more likely in the setting of poorly controlled hypertension. Even with excellent repair, hypertension remains common and pre- disposes to later myocardial infarction, stroke, and HF. See Section 3.3 for recommendations on who should perform surgeries, cardiac catheterization, and other procedures in these patients; Section 3.4 for rec- ommendations on diagnostic evaluation; and Table 21 for routine testing and follow-up intervals. Recommendation-Specific Supportive Text 1. Complications of CoA repair include recoarcta- tion, aneurysm, pseudoaneurysm, and dissection. Long-term follow-up after successful surgical in- tervention for CoA reveals that 11% of patients may require reintervention for restenosis, visual- ized by CMR or CTA (if CMR is contraindicated or there is a history of stent therapy) and supported by physical examination findings.S4.2.6-1 Although Recommendations for Coarctation of the Aorta (Continued) COR LOE Recommendations CLINICAL STATEMENTS AND GUIDELINES evidence of recoarctation can be found on clinical examination and echocardiography, aneurysms near the site of repair may not be well seen by echocardiography. Patients who have undergone surgical patch repair are at an increased risk of developing aneurysms that can be evaluated by CMR or CTA. After successful transcatheter in- tervention with stenting or balloon angioplasty, follow-up CMR or CTA imaging is recommended to evaluate for long-term complications (eg, aneurysm formation, stent fracture, or stent The same CMR or CTA study will also evaluate the ascending aorta, which may be- come aneurysmal over the years of follow-up. 2. Unoperated adults with CoA almost invari- ably present with systemic arterial hypertension measured in the upper extremities. Brachial and femoral pulse timing and amplitude evaluation on physical examination reveals a delay or de- crease in amplitude of the femoral pulse. Upper and lower extremity noninvasive blood pressure measurement is recommended in all patients with unoperated or operated/intervened CoA. 3. Upper body systemic hypertension is prevalent in patients with unoperated coarctation and may be present in up to one third of patients who have undergone operative or transcatheter Systemic hypertension may not consistently be identifiable at rest; therefore, am- bulatory blood pressure monitoring can be use- ful in identifying and appropriately managing patients with ambulatory hypertension. 4. Multiple studies have demonstrated an increased frequency of intracranial aneurysm in adults with CoA. Approximately 10% of patients with CoA have intracranial aneurysms identified on mag- netic resonance angiography or CTA. Increasing age has been identified as a risk factor. Many such identified aneurysms are small; however, the expected outcome and ideal management of such aneurysms are not clear. Providers and patients should be aware of management uncer- tainties when considering routine screening for Additionally, there are some data suggesting that intracranial aneurysms are not commonly found in children and teenagers reinforcing the possibility that coarctation alone may not be sufficient for devel- opment of intracranial aneurysm, and other fac- tors, such as hypertension and/or age, play a role in development and progression of aneurysms. 5. Despite successful surgical repair or transcatheter intervention, hypertension can persist and may not be identified during resting blood pressure meas- urement. Up to 80% of patients with prior CoA in- tervention manifest an abnormally elevated upper extremity exercise blood pressure response, and peak blood pressure is correlated with increased LV Moreover, restenosis of the previously repaired or stented region may be identified by increased peak blood pressure response, increased upper to lower extremity blood pressure gradient with exercise, and increased Doppler velocity across the coarctation site during exercise TTE. 6. Significant native or recurrent aortic coarctation has been defined as follows: upper extremity/ lower extremity resting peak-to-peak gradient >20 mm Hg or mean Doppler systolic gradient >20 mm Hg; upper extremity/lower extremity gradient >10 mm Hg or mean Doppler gradient >10 mm Hg plus either decreased LV systolic function or AR; upper extremity/lower extremity gradient >10 mm Hg or mean Doppler gradient >10 mm Hg with collateral flow.S4.2.6-2,S4.2.6-8,S4.2.6-12 This should be coupled with anatomic evidence for CoA, typ- ically defined by advanced imaging (CMR, CTA). The best evidence to proceed with intervention for CoA includes systemic hypertension, upper Table 21. CoA: Routine Follow-Up and Testing Intervals Frequency of Routine Follow-Up and Testing Physiological Stage A* (mo) Physiological Stage B* (mo) Physiological Stage C* (mo) Physiological Stage D* (mo) *See Tables 3 and 4 for details on the ACHD AP classification system. Routine TTE may be unnecessary in a year when CMR imaging is performed unless clinical indications warrant otherwise. CMR may be indicated for assessment of aortic size and aortic arch/coarctation repair site anatomy. Baseline study is recommended with periodic follow-up CMR, with frequency of repeat imaging determined by anatomic and physiological findings. CCT may be used if CMR is not feasible and to evaluate cross-sectional imaging status post-stent therapy for coarctation of the aorta; the frequency should be weighed against radiation exposure. 6-minute walk test or CPET, depending on the clinical indication. ACHD indicates adult congenital heart disease; CCT, cardiac computed tomography; CMR, cardiovascular magnetic resonance imaging; CoA, coarctation of the aorta; CPET, cardiopulmonary exercise; ECG, electrocardiogram; and TTE, transthoracic echocardiogram. CLINICAL STATEMENTS AND GUIDELINES extremity/lower extremity blood pressure gra- dient and echocardiography Doppler gradient as defined above, and anatomic evidence of CoA. Multiple factors help determine whether surgery or stenting is optimal, including anatomic features such as proximity of native coarctation to head and neck vessels or concomitant aneurysm, and, if stenting, whether a covered stent is needed. 7. The long-term complications of CoA are gener- ally related to chronic upper body systemic hyper- tension, therefore, systemic hypertension should be identified by resting, ambulatory, or exercise blood pressure assessment and medical treatment should follow GDMTS4.2.6-13,S4.2.6-25 8. Balloon angioplasty alone is associated with a higher rate of intimal tears and aneurysm forma- tion compared with stent placement. 4.3. Right-Sided Lesions 4.3.1. Valvular Pulmonary Stenosis Recommendations for Valvular Pulmonary Stenosis Referenced studies that support recommendations are summarized in Online Data Supplement 39. COR LOE Recommendations I B-NR In adults with moderate or severe valvular pulmonary stenosis and otherwise unexplained symptoms of HF, cyanosis from interatrial right-to-left communication, and/ or exercise intolerance, balloon valvuloplasty I B-NR In adults with moderate or severe valvular pulmonary stenosis and otherwise unexplained symptoms of HF, cyanosis, and/ or exercise intolerance who are ineligible for or who failed balloon valvuloplasty, surgical repair is recommendedS4.3.1-1,S4.3.1-5 S4.3.1-8 IIa C-EO In asymptomatic adults with severe valvular pulmonary stenosis, intervention is reasonable. Synopsis Valvular PS is one of the most common congeni- tal heart defects, estimated to occur in up to 7% of children born with CHD.S4.3.1-9 S4.3.1-11 Some common findings associated with isolated valvular PS include a dilated main PA and dysplastic valve cusps. Surgical or catheter-based intervention depends on degree of obstruction, RV pressure and function, and associated symptoms. Patients with isolated pulmonary valve ste- nosis (native or recurrent after an intervention) require ongoing cardiac follow-up and monitoring for evi- dence of progressive valve stenosis or regurgitation, RV hypertrophy, HF, and arrhythmias.S4.3.1-12 Patients with mild native pulmonary valve stenosis (Table 22) have a reassuring natural history, and intervention is not usually necessary. Patients with severe PS (Table 22) usually require intervention in childhood with a good prognosis into adulthood.S4.3.1-6 Patients with moderate stenosis (Table 22) have more variable histories, with some having received surgical or catheter intervention in childhood or adulthood and some not. Patients with moderate PS, whether native or postintervention, have a good long-term outcome, although some will go on to require an intervention in adulthood because of pro- gressive PS or, commonly, significant PR as a sequela of earlier intervention. Pulmonary atresia with intact ventricular septum is a rare congenital heart lesion that is associated with vary- ing degrees of RV hypoplasia and tricuspid valve hy- poplasia in addition to pulmonary valve atresia. Adults with pulmonary atresia with intact ventricular septum followed various surgical pathways in childhood, ei- ther biventricular repair, 1 1/2 ventricular repair, Fontan procedure, transplant, or shunt palliation.S4.3.1-13 Adults with history of pulmonary atresia with intact ventricu- lar septum have a high incidence of need for reinter- vention and management of atrial arrhythmias.S4.3.1-14, Restrictive RV physiology is common in adults with history of pulmonary atresia with intact ventric- ular septum and may be associated with substantial and RV-dependent coronary circulation. See Section 3.3 for recommendations on who should perform surgeries, cardiac catheterization, and other procedures in these patients; Section 3.4 for rec- ommendations on diagnostic evaluation; Table 23 for routine testing and follow-up intervals; and Figure 3 for a diagnostic and treatment algorithm for isolated PR after repair of PS. Recommendation-Specific Supportive Text 1. In patients with moderate or severe isolated pul- monary valve stenosis, pulmonary balloon val- vuloplasty is safe and effective in reducing the pulmonary valve gradient and improving symp- toms in most patients 2. Surgical valvotomy is usually sufficient, particularly when the pulmonary annulus is not hypoplastic. Pulmonary valve replacement may be necessary Table 22. Severity of RVOT Obstruction Mild Peak gradient <36 mm Hg (peak velocity <3 m/s) Moderate Peak gradient 36 64 mm Hg (peak velocity 3 4 m/s) Severe Peak gradient 64 mm Hg (peak velocity >4 m/s); mean gradient >35 mm Hg Estimations of RV systolic pressure by TR velocity is part of the echocardiographic assessment of RV obstruction, as Doppler measurements across the RV obstruction itself may be unreliable. RV indicates right ventricular; RVOT, right ventricular outflow tract; and TR, tricuspid regurgitation. CLINICAL STATEMENTS AND GUIDELINES Table 23. Valvular PS: Routine Follow-Up and Testing Intervals Frequency of Routine Follow-Up and Testing Physiological Stage A* (mo) Physiological Stage B* (mo) Physiological Stage C* (mo) Physiological Stage D* (mo) *See Tables 3 and 4 for details on the ACHD AP classification system. 6-minute walk test or CPET, depending on clinical indication. ACHD indicates adult congenital heart disease; CPET, cardiopulmonary exercise test; ECG, electrocardiogram; PS, pulmonary stenosis; and TTE, transthoracic echocardiogram. Figure 3. Isolated PR after repair of PS. *Significant PR causes RV dilation. If a patient has moderate or greater PR and normal RV size, most likely the estimation of PR severity is inaccurate or there may be restrictive RV physiology, which would warrant further investigation. Symptoms may include dyspnea, chest pain, and/or exercise intolerance referable to PR or otherwise unexplained. ACHD indicates adult congenital heart disease; CPET, cardiopulmonary exercise test; PR, pulmonary regurgitation; PS, pulmonary stenosis; and RV, right ventricular. CLINICAL STATEMENTS AND GUIDELINES when there is marked dysplasia of the pulmonary valve or significant hypoplasia of the annulus. 3. Relief of a severely stenotic pulmonary valve in an asymptomatic patient will reduce the RV pres- sure and the possibility of potential sequelae. As in symptomatic patients, the procedure can be per- formed by surgery or interventional catheterization with low morbidity and mortality. If intervention is deferred, careful follow-up to evaluate for symp- toms, decline in exercise capacity, worsening RV function, or development of cyanosis is important and may prompt reconsideration of intervention. 4.3.1.1. Isolated PR After Repair of PS Recommendations for Isolated PR After Repair of Pulmonary Stenosis COR LOE Recommendations I C-EO In symptomatic patients with moderate or greater PR resulting from treated isolated pulmonary stenosis, with RV dilation or RV dysfunction, pulmonary valve replacement is recommended. I C-EO For asymptomatic patients with residual PR resulting from treatment of isolated pulmonary stenosis with a dilated right ventricle, serial follow-up is recommended. IIb C-EO In asymptomatic patients with moderate or greater PR resulting from treatment of isolated pulmonary stenosis with progressive RV dilation and/or RV dysfunction, pulmonary valve replacement may be reasonable. Synopsis Although many patients with valvular PS do not require intervention, some have PS that is severe enough to warrant intervention, often in infancy or childhood. PS can be alleviated either by surgical valvotomy or with balloon valvuloplasty. Either surgical or catheter inter- vention may result in hemodynamically important PR that can result in symptoms, RV enlargement, and/or dysfunction requiring pulmonary valve replacement. Recommendation-Specific Supportive Text 1. Patients with isolated PS who have previously undergone an intervention on the pulmonary valve require ongoing clinical follow-up and mon- itoring of PR, RV size and function, and functional capacity. This may include echocardiography, CPET, and advanced imaging. The right ventricle in patients with PR after intervention for PS may be smaller than in patients with TOF; however, patients with PR may have evidence of decreased RV ejection fraction or decreased exercise ca- pacity. Pulmonary valve replacement can improve symptoms for patients with symptoms that are attributable to moderate or greater PR, and can improve RV size and/or RV function if there is RV dilation or decreased RV ejection fraction. 2. PR resulting from treatment of isolated PS may have progressive impact on RV size and function, and may result in symptoms, such that pulmonary valve replacement would be considered. Serial follow-up for clinical evaluation, CPET, and imaging to eval- uate for symptoms, exercise intolerance attribut- able to PR, and/or RV dilation or RV dysfunction will allow appropriate timing of intervention if needed. 3. There are no data to suggest appropriate timing for pulmonary valve replacement in the presence of RV dilation, but it is likely inappropriate to di- rectly extrapolate the data applicable to patients However, RV dilation or dysfunc- tion should improve, or at least not progress fur- ther, if the volume overload from PR is alleviated by pulmonary valve replacement. Thus, although specific RV size criteria are not available for these patients to determine timing of pulmonary valve replacement, patients with progressively worsen- ing RV size or function presumably represent a subset of patients for whom valve replacement could be beneficial. 4.3.2. Branch and Peripheral Pulmonary Stenosis Recommendations for Branch and Peripheral PS Referenced studies that support recommendations are summarized in Online Data Supplement 40. COR LOE Recommendations Diagnostic I B-NR For adults with peripheral or branch PS, ongoing surveillance is Therapeutic IIa B-NR In adults with peripheral or branch PA stenosis, PA dilation and stenting can be Synopsis Pulmonary branch and peripheral PS can be isolated, occur as part of a constellation of right ventricular out- flow tract (RVOT) obstruction, or be found in associa- tion with a syndrome (eg, Noonan, Alagille, Williams, maternal rubella exposure). Intervention decisions are typically based on symptoms, distribution of pulmonary blood flow, RV function, and RV systolic pressure. TTE is a good modality to obtain RV pressure and function but does not adequately image the peripheral pulmonary arteries. Alternative imaging (eg, CMR, CCT) can vis- ualize anatomic obstructions and branch PA anatomy. In addition, CMR and pulmonary perfusion testing can quantify relative pulmonary blood flow. CLINICAL STATEMENTS AND GUIDELINES See Section 3.3 for recommendations on who should perform surgeries, cardiac catheterization, and other procedures in these patients; Section 3.4 for rec- ommendations on diagnostic evaluation; and Table 24 for routine testing and follow-up intervals. Recommendation-Specific Supportive Text 1. Cardiac follow-up and imaging may include evaluation of RV pressure; quantifying relative pulmonary blood flow and imaging for evidence of residual lesions or PA obstruction or aneu- rysm at sites of prior intervention; and in-stent stenosis and/or stent fracture (the latter often best seen by fluoroscopy). Stenting of branch PA stenosis is effective in reducing the pressure gradients, but patients often require further In-stent stenosis with a re- duction in the ipsilateral pulmonary blood flow is seen in approximately 25% of patients after percutaneous PA angioplasty and stent place- ment, more common in patients with abnormal pulmonary arteries, such as those with TOF or Regular surveillance and imaging, with intervention as required, may prevent the development of RV hypertension and its sequelae.S4.3.2-1 2. Balloon angioplasty or stenting of a peripheral PA is effective in reducing pressure gradients and improving pulmonary blood flow. Indications for pulmonary angioplasty or stenting include symp- toms attributed to the decreased pulmonary blood flow, focal narrowing, abnormal differen- tial perfusion, and/or elevated RV pressure. The decision for intervention with PA angioplasty or stenting includes assessment of clinical symp- toms, imaging, and discussion with an ACHD in- terventional cardiologist. 4.3.3. Double-Chambered Right Ventricle Recommendations for Double-Chambered Right Ventricle Referenced studies that support recommendations are summarized in Online Data Supplement 41. COR LOE Recommendations I C-LD Surgical repair for adults with double- chambered right ventricle and moderate or greater outflow obstruction is recommended in patients with otherwise unexplained symptoms of HF, cyanosis, or exercise (Table 22). IIb C-LD Surgical repair for adults with double- chambered right ventricle with a severe gradient may be considered in asymptomatic (Table 22). Synopsis Double-chambered right ventricle is uncommon in adults. Hypertrophied muscle bundles develop in the RV cavity, creating RVOT obstruction.S4.3.3-5,S4.3.3-6 It is com- monly associated with a VSD. Double-chambered right ventricle can be missed on TTE if not sought specifically, and alternative imaging or cardiac catheterization is of- ten required to confirm the diagnosis and establish the hemodynamic impact. See Section 3.3 for recommendations on who should perform surgeries, cardiac catheterization, and other procedures in these patients; Section 3.4 for rec- ommendations on diagnostic evaluation; and Table 25 for routine testing and follow-up intervals. Recommendation-Specific Supportive Text 1. Surgery typically involves transatrial or transven- tricular resection of obstructing muscle bundles and VSD closure if present. Occasionally, patch enlargement of RVOT may be necessary to ade- quately relieve obstruction. Table 24. Branch and Peripheral PS: Routine Follow-Up and Testing Intervals Frequency of Routine Follow-Up and Testing Physiological Stage A* (mo) Physiological Stage B* (mo) Physiological Stage C* (mo) Physiological Stage D* (mo) *See Tables 3 and 4 for details on the ACHD AP classification system. Routine TTE may be unnecessary in a year when CMR imaging is performed unless clinical indications warrant otherwise. CMR may be indicated for assessment of branch PS. Baseline study is recommended with periodic follow-up CMR, with frequency of repeat imaging determined by anatomic and physiological findings. CCT may be used if CMR is not feasible and to evaluate cross-sectional imaging status and post-stent therapy for peripheral PS; the frequency should be weighed against radiation exposure. 6-minute walk test or CPET, depending on clinical indication. ACHD indicates adult congenital heart disease; CCT, cardiac computed tomography; CMR, cardiovascular magnetic resonance imaging; CPET, cardiopulmonary exercise test; ECG, electrocardiogram; PS, pulmonary stenosis; and TTE, transthoracic echocardiogram. CLINICAL STATEMENTS AND GUIDELINES 2. VSD is often present and may communicate with the higher or lower pressure chamber in the right ventricle, with resulting differences in shunt di- rection and flow characteristics. In patients with a severe gradient through the right ventricle, the VSD may be associated with right-to-left shunting if proximal to the obstruction, or associated with left-to-right shunting if distal. Exercise testing performed in a subjectively asymptomatic patient will often be abnormal. Patients may benefit from repair of both the VSD and outflow obstruction, especially if exercise capacity is decreased. 4.3.4. Ebstein Anomaly Recommendations for Ebstein Anomaly Referenced studies that support recommendations are summarized in Online Data Supplement 42. COR LOE Recommendations Diagnostic IIa B-NR In adults with Ebstein anomaly, CMR can be useful to determine anatomy, RV dimensions, and systolic function.S4.3.4-1,S4.3.4-2 IIa B-NR In adults with Ebstein anomaly, TEE can be useful for surgical planning if TTE images are inadequate to evaluate tricuspid valve morphology and function.S4.3.4-1 IIa B-NR Electrophysiological study with or without catheter ablation can be useful in the diagnostic evaluation of adults with Ebstein anomaly and ventricular preexcitation but without supraventricular IIa B-NR In adults with Ebstein anomaly, electrophysiological study (and catheter ablation, if needed) is reasonable before surgical intervention on the tricuspid valve even in the absence of preexcitation or Therapeutic I B-NR Surgical repair or reoperation for adults with Ebstein anomaly and significant TR is recommended when one or more of the following are present: HF symptoms, objective evidence of worsening exercise capacity, progressive RV systolic dysfunction by echocardiography or Therapeutic (Continued) I C-LD Catheter ablation is recommended for adults with Ebstein anomaly and high-risk pathway conduction or multiple accessory IIa B-NR Surgical repair or reoperation for adults with Ebstein anomaly and significant TR can be beneficial in the presence of progressive RV enlargement, systemic desaturation from right-to-left atrial shunt, paradoxical embolism, and/or atrial IIb B-NR Bidirectional superior cavopulmonary (Glenn) anastomosis at time of Ebstein anomaly repair may be considered for adults when severe RV dilation or severe RV systolic dysfunction is present, LV function is preserved, and left atrial pressure and LV end diastolic pressure are not Synopsis Ebstein anomaly is an uncommon congenital heart defect occurring in about 0.005% of live births.S4.3.4-16 It is a malformation of the tricuspid valve and the right ventricle and varies in severity, including babies who do not survive infancy, asymptomatic adults diagnosed incidentally in the sixth and seventh decades of life, and many variations in severity between those extremes. Ebstein anomaly can occur with other defects including ASD, VSD, and PS. A patent foramen ovale, otherwise usually considered normal, may have signif- icant impact in Ebstein anomaly. Accessory pathways and arrhythmias are relatively common. Patient sur- veillance and management varies depending on age, severity of the lesion, and associated abnormalities including HF, cyanosis, and arrhythmias. Surveillance includes echocardiographic and other advanced imag- ing to assess RV size and function, rhythm assessment, pulse oximetry, and stress testing. Treatments include medical and surgical therapy for patients with manifest symptoms as well as catheter-based structural and elec- trophysiological interventions when indicated. Table 25. Double-Chambered Right Ventricle: Routine Follow-Up and Testing Intervals Frequency of Routine Follow-Up and Testing Physiological Stage A* (mo) Physiological Stage B* (mo) Physiological Stage C* (mo) Physiological Stage D* (mo) *See Tables 3 and 4 for details on the ACHD AP classification system. 6-minute walk test or CPET, depending on clinical indication. ACHD indicates adult congenital heart disease; CPET, cardiopulmonary exercise test; ECG, electrocardiogram; and TTE, transthoracic echocardiogram. Recommendations for Ebstein Anomaly (Continued) COR LOE Recommendations CLINICAL STATEMENTS AND GUIDELINES See Section 3.3 for recommendations on who should perform surgeries, cardiac catheterization, and other procedures in these patients; Section 3.4 for rec- ommendations on diagnostic evaluation; and Table 26 for routine testing and follow-up intervals. Recommendation-Specific Supportive Text 1. Deciphering the anatomy and size of the right atrium and right ventricle in Ebstein anomaly is often difficult using echocardiography alone, par- ticularly in adults. Data obtained from CMR can inform clinical care and surgical planning or de- cision-making, because CMR data correlate well with intraoperative findings. 2. Two-dimensional and 3D TEE can better define the anatomy and function of the tricuspid valve before surgery and provide valuable information in planning surgical repair. 3. Approximately one third of adults with Ebstein anomaly and ventricular preexcitation have multiple accessory pathways, associated with a high risk of SCD. Adults with Ebstein anomaly also have a high prevalence of atrial tachyarrhythmia.S4.3.4-3,S4.3.4-4 In the setting of ventricular preexcitation, atrial tachyarrhythmia may expose the patient to a higher risk of lethal ventricular arrhythmia. In patients with clinical supraventricular tachycardia, management is according to existing GDMT.S4.3.4-19 A Pediatric & Congenital Electrophysiology Society (PACES)/HRS expert consensus document provides additional information on the manage- ment of arrhythmias.S4.3.4-20 4. Concealed accessory pathways are common in Ebstein anomaly and may coexist with manifest accessory pathways. In addition, preexcitation may be present but difficult to appreciate on the surface ECG. Tricuspid valve surgery can hinder transcatheter access to right-sided accessory pathways and the slow pathway in AV node reen- try, such that it may be reasonable to assess for arrhythmia substrates and proceed with catheter ablation if identified, before surgery. 5. Data demonstrate that delay of surgery until HF or RV systolic dysfunction ensues is associated with poorer outcomes; surgery before either of those develops is recommended.S4.3.4-6,S4.3.4-7,S4.3.4-10 Ebstein anomaly is understood as not just valve di- sease but also a myopathic process. Consequently, threshold for operation may be different than in other RV volume-loading lesions, because there is more concern regarding the capacity of the myo- pathic Ebstein right ventricle to tolerate a volume load. Also, there are cohort series of Ebstein patients to inform decisions.S4.3.4-6,S4.3.4-7,S4.3.4-10 Surgical repair generally consists of tricuspid valve repair (preferred when feasible) or replacement, selective plication of atrialized right ventricle, re- duction atrioplasty, arrhythmia surgery, and/or closure of atrial level shunt. Surgery may result in improvement of symptoms and functional ability, and prevent or delay worsening symptoms. 6. Adults with Ebstein anomaly and ventricular pre- excitation often have multiple accessory path- ways, which are associated with a higher risk of SCD. Surgical interruption of accessory pathways is largely reserved for patients who have failed attempts at catheter ablation. High-risk path- ways are those with an increased risk of SCD, largely related to VF resulting from rapidly con- ducting AF. Definition and discussion of high-risk Table 26. Ebstein Anomaly: Routine and Follow-Up and Testing Intervals Frequency of Routine Follow-Up and Testing Physiological Stage A* (mo) Physiological Stage B* (mo) Physiological Stage C* (mo) Physiological Stage D* (mo) CXR As needed As needed 12 24 12 24 Pulse oximetry 24 12 Each visit Each visit Holter monitor As needed As needed 24 12 24 *See Tables 3 and 4 for details on the ACHD AP classification system. Routine TTE may be unnecessary in a year when CMR imaging is performed unless clinical indications warrant otherwise. CMR may be indicated for assessment of right ventricular size and function. Baseline study is recommended with periodic follow-up CMR, with frequency of repeat imaging determined by anatomic and physiological findings. CCT may be used if CMR is not feasible; the frequency should be weighed against radiation exposure. 6-minute walk test or CPET, depending on clinical indication. ACHD indicates adult congenital heart disease; CCT, cardiac computed tomography; CMR, cardiovascular magnetic resonance imaging; CPET, cardiopulmonary exercise test; CXR, chest x-ray; ECG, electrocardiogram; and TTE, transthoracic echocardiogram. CLINICAL STATEMENTS AND GUIDELINES pathways is beyond the scope of these guide- lines but can be found elsewhere, such as the PACES/HRS Expert Consensus Statement on the Recognition and Management of Arrhythmias in 7. Systemic desaturation and arrhythmias are fre- quently signs of worsening hemodynamics, pro- gressive TR, or worsening RV function. Surgery for the tricuspid valve as well as closure of the ASD or stretched patent foramen ovale and ar- rhythmia surgery can be beneficial. When ar- rhythmia surgery is required, it typically involves a modified right atrial maze procedure. In the pres- ence of AF, the addition of a left atrial Cox Maze III procedure can be beneficial to reduce the risk of recurrent AF. 8. The use of the bidirectional cavopulmonary shunt is much more common in children than in adults. When it is applied in the adult, it is usu- ally reserved for patients with severe RV dysfunc- tion with concern that the right ventricle will not tolerate supporting the entirety of stroke Preoperative catheterization to determine hemodynamics and feasibility of applying the bidirectional cavopulmonary shunt becomes progressively more important in older patients, particularly those with longstanding hy- pertension with LV hypertrophy, which can lead to diastolic dysfunction and elevated pulmonary pressures. 4.3.5. Tetralogy of Fallot Recommendations for TOF Referenced studies that support recommendations are summarized in Online Data Supplement 43. (See Section 4.3.6. for recommendations regarding evaluation and management of right ventricle to-PA conduits.) COR LOE Recommendations Diagnostic I B-NR CMR is useful to quantify ventricular size and function, pulmonary valve function, pulmonary artery anatomy, and left heart abnormalities in patients with repaired I B-NR Coronary artery compression testing is indicated before right ventricle to-PA conduit stenting or transcatheter valve placement in repaired TOF.S4.3.5-2 IIa B-NR Programmed ventricular stimulation can be useful to risk-stratify adults with TOF and additional risk factors for SCD.S4.3.5-3 S4.3.5-8 IIa C-EO In patients with repaired TOF, cardiac catheterization with angiography, if indicated, is reasonable to assess hemodynamics when adequate data cannot be obtained noninvasively in the setting of an arrhythmia, HF, unexplained ventricular dysfunction, suspected pulmonary hypertension or cyanosis. Therapeutic I B-NR Pulmonary valve replacement (surgical or percutaneous) for relief of symptoms is recommended for patients with repaired TOF and moderate or greater PR with cardiovascular symptoms not otherwise IIa B-NR Pulmonary valve replacement (surgical or percutaneous) is reasonable for preservation of ventricular size and function in asymptomatic patients with repaired TOF and ventricular enlargement or dysfunction and moderate or greater IIa B-NR Primary prevention ICD therapy is reasonable in adults with TOF and multiple risk factors IIb C-EO Surgical pulmonary valve replacement may be reasonable for adults with repaired TOF and moderate or greater PR with other lesions requiring surgical interventions. IIb C-EO Pulmonary valve replacement, in addition to arrhythmia management, may be considered for adults with repaired TOF and moderate or greater PR and ventricular tachyarrhythmia. Synopsis Long-term survival after surgery for TOF continues to improve. However, residual hemodynamic and electro- physiological abnormalities are common in adulthood. Adults with repaired TOF face an increased risk of ar- rhythmias, exercise intolerance, HF, and death begin- ning in early adulthood.S4.3.5-1,S4.3.5-18 S4.3.5-20 Surgical re- pair of TOF has evolved over time, with relief of the RVOT obstruction usually involving infundibulotomy, resection of obstructive muscle bundles, and the use of a patch to enlarge the pathway from the right ventricle to the pulmonary arteries. These procedures result in scar tissue and create a dyskinetic and often aneurys- mal area in the RVOT. Residual RVOT stenosis, branch PA stenosis, residual ASD or VSD, TR, RV dilation and dysfunction, aortic dilation, AR, and LV dysfunction are some of the anatomic and functional abnormalities en- countered in patients with repaired TOF. The most com- mon hemodynamic sequela of TOF repair is PR. Cur- rent evidence confirms that adults with repaired TOF are at risk of severe PR, RV dilation and dysfunction, LV dysfunction and electromechanical dyssynchrony, all of which contribute to adverse clinical outcomes late after TOF repair.S4.3.5-1,S4.3.5-20 S4.3.5-24 Despite intense in- terest and numerous publications on pulmonary valve replacement in adults with repaired TOF, optimal tim- ing for this intervention remains uncertain, and most studies have focused on preoperative RV volumes that would result in normalization of postoperative RV In adults with repaired Recommendations for TOF (Continued) COR LOE Recommendations CLINICAL STATEMENTS AND GUIDELINES TOF, prevalence rates for atrial and ventricular arrhyth- mias have been estimated to be 20% and 15%, respec- tively, with steep increases after 45 years of age.S4.3.5-28 The incidence of SCD after surgical repair of TOF is Currently, factors associated with SCD in patients with TOF have largely been identified from observa- tional, predominantly retrospective studies. Despite nu- merous studies that identified factors associated with malignant ventricular arrhythmias and SCD, risk stratifi- cation remains imperfect. Primary prevention ICDs should generally be consid- ered in patients who otherwise meet standard qualify- ing criteria (ie, LV ejection fraction 35% with NYHA class II or III symptoms).S4.3.5-31 S4.3.5-33 There may be a role for primary prevention ICDs in selected adults with TOF who have additional risk factors for SCD but would not meet standard criteria otherwise. See Section 3.3 for recommendations on who should perform surgeries, cardiac catheterization, and other procedures in these patients; Section 3.4 for rec- ommendations on diagnostic evaluation; Figure 4 for Figure 4. Pulmonary valve replacement in patients with TOF repair and PR. *Symptoms may include dyspnea, chest pain, and/or exercise intolerance referable to PR or otherwise unexplained. See recommendation 6 supporting text. ACHD indicates adult congenital heart disease; HF, heart failure; LV, left ventricular; LVEDV, left ventricular end diastolic volume; PR, pulmonary regurgitation; RV, right ventricular; RVEDV, right ventricular end diastolic volume; RVEDVI, right ventricular end diastolic volume index; RVESVI, right ventricular end systolic volume index; RVOT, right ventricular outflow tract; RVSP, right ventricular systolic pressure; and TOF, tetralogy of Fallot. CLINICAL STATEMENTS AND GUIDELINES a diagnostic and treatment algorithm for repaired TOF with residual PR; and Table 27 for routine testing and follow-up intervals. Recommendation-Specific Supportive Text 1. Cardiac magnetic resonance imaging is the gold standard imaging modality for quantification of right ventricular size and function in patients with repaired TOF. It also allows for quantification of valve regurgitation and pulmonary and sys- temic flows as well as delineating pulmonary ar- tery anatomy and detection of scar tissue in the ventricular myocardium. Serial cardiac magnetic resonance imaging examinations allows for longi- tudinal follow-up of patients with repaired TOF and provides useful information that aids in the timing of pulmonary valve replacement.S4.3.5-1,S4.3.5-34 S4.3.5-37 2. Before any surgical or percutaneous intervention in patients with TOF, the origins and proximal courses of the coronary arteries should be delin- eated. Patients with repaired TOF and abnormal coronary artery anatomy have a substantial risk of coronary artery compression during percuta- neous pulmonary valve replacement or direct in- jury to the coronary during surgical pulmonary valve replacement. During cardiac catheteriza- tion, the coronary pattern may be demonstrated by performing simultaneous RVOT angiography and coronary angiography.S4.3.5-2 Coronary com- pression testing generally involves simultaneous coronary angiography or aortography and bal- loon dilation of the RVOT to ascertain whether a balloon expanded stent will compress the cor- onary artery. 3. Additional risk factors for SCD include:S4.3.5-24,S4.3.5-38, a. LV systolic or diastolic dysfunction b. Nonsustained VT, QRS duration 180 ms c. Extensive RV fibrosis by CMR In adults with TOF, inducible sustained VT has been associated with an increased risk of clinical VT or SCD, beyond standard ECG, hemodynamic, and clinical factors.S4.3.5-5 Programmed ventricular stimulation is most useful in risk stratifying pa- tients at moderate risk of SCD rather than as a rou- tine surveillance tool in low-risk populations.S4.3.5-7 4. Cardiac catheterization is the only method that can accurately and reliably determine PA pressure and pulmonary vascular resistance. 5. Symptomatic patients (with dyspnea, chest pain, and/or exercise intolerance otherwise unex- plained) with repaired TOF and severe PR who undergo pulmonary valve replacement often re- port improved functional class after intervention. Improvement in symptoms often correlates with a reduction in RV size and relief of PR.S4.3.5-9 S4.3.5-11 Symptom improvement is more likely in patients with underlying PS and PR than in patients with PR alone. For patients with significant LV or RV dysfunction, pulmonary valve replacement may not be tolerated or sufficient; therefore, evalua- tion by ACHD cardiologists and HF cardiologists is appropriate to decide appropriate course of action, particularly in deciding if a patient may be appropriate for mechanical circulatory support or heart transplant. Table 27. TOF: Routine Follow-Up and Testing Intervals Frequency of Routine Follow-Up and Testing Physiological Stage A* (mo) Physiological Stage B* (mo) Physiological Stage C* (mo) Physiological Stage D* (mo) Pulse oximetry As needed As needed Each visit Each visit Holter monitor As needed As needed 12 24 12 24 *See Tables 3 and 4 for details on the ACHD AP classification system. Routine TTE may be unnecessary in a year when CMR imaging is performed unless clinical indications warrant otherwise. CMR may be indicated for assessment of right ventricular size and function, pulmonary valve function, pulmonary artery anatomy and left heart abnormalities. Baseline study is recommended with periodic follow-up CMR, with frequency of repeat imaging determined by anatomic and physiological findings. 