WRITING COMMITTEE MEMBERS* Karen K. Stout, MD, FACC, Chair Curt J. Daniels, MD, Vice Chair* Jamil A. Aboulhosn, MD, FACC, FSCAI* Biykem Bozkurt, MD, PhD, FACC, FAHA Craig S. Broberg, MD, FACC* Jack M. Colman, MD, FACC Stephen R. Crumb, DNP, AACC Joseph A. Dearani, MD, FACC Stephanie Fuller, MD, MS, FACC# Michelle Gurvitz, MD, FACC** Paul Khairy, MD, PhD* Michael J. Landzberg, MD, FACC* Arwa Saidi, MB, BCH, FACC* Anne Marie Valente, MD, FACC, FAHA, FASE George F. Van Hare, MD, FACC 2018 by the American Heart Association, Inc., and the American College of Cardiology Foundation. AHA/ACC GUIDELINE 2018 AHA/ACC Guideline for the Management of Adults With Congenital Heart Disease A Report of the American College of Cardiology/American Heart Association Task Force on Clinical Practice Guidelines Circulation https://www.ahajournals.org/journal/circ *Writing committee members are required to recuse themselves from voting on sections to which their specific rela- tionships with industry may apply; see Appendix 1 for recusal information. ACC/AHA Representative. International Society for Adult Congenital Heart Disease Representative. Society for Cardiovascular Angiography and Interventions Representative. ACC/AHA Task Force on Clinical Practice Guidelines Liaison. Society of Thoracic Surgeons Represen- tative. #American Association for Thoracic Surgery Representative. **ACC/AHA Task Force on Performance Measures Liaison. American Society of Echocardiography Representative. Heart Rhythm Society Representative. Developed in Collaboration With the American Association for Tho- racic Surgery, American Society of Echocardiography, Heart Rhythm Society, International Society for Adult Congenital Heart Disease, Society for Cardiovascular Angi- ography and Interventions, and Society of Thoracic Surgeons ACC/AHA Task Force Members, see page e772 The American Heart Association requests that this document be cited as follows: Stout KK, Daniels CJ, Aboulhosn JA, Bozkurt B, Broberg CS, Colman JM, Crumb SR, Dearani JA, Fuller S, Gurvitz M, Khairy P, Landzberg MJ, Saidi A, Valente AM, Van Hare GF. 2018 AHA/ACC guideline for the management of adults with congenital heart disease: a report of the American College of Cardiology/American Heart Association Task Force on Clinical Practice Guidelines. Key Words: AHA Scientific Statements arrhythmias cardiac catheterization cardiac defects congenital heart disease congenital heart surgery unoperated/repaired heart defect CLINICAL STATEMENTS AND GUIDELINES TABLE OF CONTENTS Preamble e700 1. Introduction e701 1.1. Methodology and Evidence Review e701 1.2. Organization of the Writing Committee e702 1.3. Document Review and Approval e703 1.4. Scope of the Guideline e703 1.5. Abbreviations e705 2. Background and Pathophysiology e705 2.1. Anatomic and Physiological Terms e705 2.2. Severity of ACHD e705 2.3. The ACHD Anatomic and Physiological Classification e705 3. General Principles e709 3.1. ACHD Program e709 3.2. Access to Care e709 3.3. Delivery of Care e710 3.4. Evaluation of Suspected and Known CHD e710 3.4.1. Electrocardiogram e713 3.4.2. Ionizing Radiation Principles e713 3.4.3. Echocardiography e713 3.4.4. CMR Imaging e714 3.4.5. Cardiac Computed Tomography e714 3.4.6. Cardiac Catheterization e715 3.4.7. Exercise Testing e715 3.5. Transition Education e716 3.6. Exercise and Sports e716 3.7. Mental Health and Neurodevelopmental Issues e717 3.8. Endocarditis Prevention e717 3.9. Concomitant Syndromes e717 3.10. Acquired Cardiovascular Disease e718 3.11. Noncardiac Medical Issues e719 3.12. Noncardiac Surgery e719 3.13. Pregnancy, Reproduction, and Sexual Health e720 3.13.1. Pregnancy e720 3.13.2. Contraception e722 3.13.3. Infertility Treatment e722 3.13.4. Sexual Function e722 3.14. Heart Failure and Transplant e723 3.14.1. Heart Failure e723 3.14.2. Heart Transplant e723 3.14.3. Multiorgan Transplant e723 3.15. Palliative Care e724 3.16. Cyanosis e724 3.17. Pharmacological Therapy for ACHD e725 4. Specific Lesions e726 4.1. Shunt Lesions e726 4.1.1. Atrial Septal Defect e726 4.1.2. Anomalous Pulmonary Venous Connections e729 4.1.3. Ventricular Septal Defect e730 4.1.4. Atrioventricular Septal Defect e732 4.1.5. Patent Ductus Arteriosus e734 4.2. Left-Sided Obstructive Lesions e736 4.2.1. Cor Triatriatum e736 4.2.2. Congenital Mitral Stenosis e736 4.2.3. Subaortic Stenosis e737 4.2.4. Congenital Valvular Aortic Stenosis e738 4.2.4.1. Turner Syndrome e739 4.2.4.2. Aortopathies e739 4.2.5. Supravalvular Aortic Stenosis e740 4.2.6. Coarctation of the Aorta e741 4.3. Right-Sided Lesions e743 4.3.1. Valvular Pulmonary Stenosis e743 Isolated PR After Repair of PS e745 4.3.2. Branch and Peripheral Pulmonary Stenosis e745 4.3.3. Double-Chambered Right Ventricle e746 4.3.4. Ebstein Anomaly e747 4.3.5. Tetralogy of Fallot e749 4.3.6. Right Ventricle to Pulmonary Artery Conduit e752 4.4. Complex Lesions e754 4.4.1. Transposition of the Great Arteries e754 Transposition of the Great Arteries With Atrial Switch e754 Transposition of the Great Arteries With Arterial Switch e756 Transposition of the Great Arteries With Rastelli Type Repair e758 4.4.1.4. Congenitally Corrected Transposition of the Great Arteries e758 4.4.2. Fontan Palliation of Single Ventricle Physiology (Including Tricuspid Atresia and Double Inlet Left Ventricle) e759 4.4.3. Hypoplastic Left Heart Syndrome/Norwood Repair e763 4.4.4. Truncus Arteriosus e763 4.4.5. Double Outlet Right Ventricle e763 4.4.6. Severe PAH and Eisenmenger Syndrome e763 4.4.6.1. Severe PAH e763 4.4.6.2. Eisenmenger Syndrome e765 4.4.7. Coronary Anomalies e767 Anomalous Coronary Artery Evaluation e768 Anomalous Aortic Origin of Coronary Artery e769 Anomalous Coronary Artery Arising From the PA e769 4.4.8. Coronary Artery Fistula e770 5. Evidence Gaps and Future Directions e772 References e773 Appendix 1 Author Relationships With Industry and Other Entities (Relevant) e796 Appendix 2 Reviewer Relationships With Industry and Other Entities (Comprehensive) e798 CLINICAL STATEMENTS AND GUIDELINES PREAMBLE Since 1980, the American College of Cardiology (ACC) and American Heart Association (AHA) have translat- ed scientific evidence into clinical practice guidelines (guidelines) with recommendations to improve cardio- vascular health. These guidelines, which are based on systematic methods to evaluate and classify evidence, provide a cornerstone for quality cardiovascular care. The ACC and AHA sponsor the development and pub- lication of guidelines without commercial support, and members of each organization volunteer their time to the writing and review efforts. Guidelines are official policy of the ACC and AHA. Intended Use Practice guidelines provide recommendations applica- ble to patients with or at risk of developing cardiovas- cular disease. The focus is on medical practice in the United States, but guidelines developed in collabora- tion with other organizations can have a global impact. Although guidelines may be used to inform regulatory or payer decisions, they are intended to improve pa- tients quality of care and align with patients interests. Guidelines are intended to define practices meeting the needs of patients in most, but not all, circumstances and should not replace clinical judgment. Clinical Implementation Management in accordance with guideline recommenda- tions is effective only when followed by both practitioners and patients. Adherence to recommendations can be enhanced by shared decision-making between clinicians and patients, with patient engagement in selecting in- terventions on the basis of individual values, preferences, and associated conditions and comorbidities. Methodology and Modernization The ACC/AHA Task Force on Clinical Practice Guidelines (Task Force) continuously reviews, updates, and modi- fies guideline methodology on the basis of published standards from organizations, including the Institute of and on the basis of internal reevaluation. Similarly, the presentation and delivery of guidelines are reevaluated and modified on the basis of evolving tech- nologies and other factors to facilitate optimal dissemi- nation of information to healthcare professionals at the point of care. Toward this goal, this guideline continues the intro- duction of an evolved format of presenting guideline recommendations and associated text called the mod- ular knowledge chunk format. Each modular chunk includes a table of related recommendations, a brief synopsis, recommendation-specific supportive text, and when appropriate, flow diagrams or additional tables. References are provided at the end of the document in their respective sections. Additionally, this format will facilitate seamless updating of guidelines with fo- cused updates as new evidence is published, as well as content tagging for rapid electronic retrieval of related recommendations on a topic of interest. This evolved approach format was instituted when this guideline was near completion; therefore, the present document represents a transitional format that best suits the text as written. Future guidelines will fully implement this format, including provisions for limiting the amount of text in a guideline. Recognizing the importance of cost value consid- erations in certain guidelines, when appropriate and feasible, an analysis of the value of a drug, device, or intervention may be performed in accordance with the To ensure that guideline recommendations remain current, new data are reviewed on an ongoing basis, with full guideline revisions commissioned in approx- imately 6-year cycles. Publication of new, potentially practice-changing study results that are relevant to an existing or new drug, device, or management strategy will prompt evaluation by the Task Force, in consul- tation with the relevant guideline writing committee, to determine whether a focused update should be commissioned. For additional information and policies regarding guideline development, we encourage read- ers to consult the ACC/AHA guideline methodology and other methodology articles.P-5 P-8 Selection of Writing Committee Members The Task Force strives to avoid bias by selecting experts from a broad array of backgrounds. Writing commit- tee members represent different geographic regions, sexes, ethnicities, races, intellectual perspectives/biases, and scopes of clinical practice. The Task Force may also invite organizations and professional societies with re- lated interests and expertise to participate as partners, collaborators, or endorsers. Relationships With Industry and Other Entities The ACC and AHA have rigorous policies and meth- ods to ensure that guidelines are developed without bias or improper influence. The complete relationships with industry and other entities (RWI) policy can be found online. Appendix 1 of the present document lists writing committee members relevant RWI. For the purposes of full transparency, writing committee mem- bers comprehensive disclosure information is available online. Comprehensive disclosure information for the Task Force is also available online. CLINICAL STATEMENTS AND GUIDELINES Evidence Review and Evidence Review Committees In developing recommendations, the writing commit- tee uses evidence-based methodologies that are based on all available data.P-4 P-7 Literature searches focus on randomized controlled trials (RCTs) but also include registries, nonrandomized comparative and descriptive studies, case series, cohort studies, systematic reviews, and expert opinion. Only key references are cited. An independent evidence review committee (ERC) is commissioned when there are 1 or more questions deemed of utmost clinical importance that merit formal systematic review. The systematic review will determine which patients are most likely to benefit from a drug, device, or treatment strategy and to what degree. Cri- teria for commissioning an ERC and formal systematic review include: a) the absence of a current authoritative systematic review, b) the feasibility of defining the ben- efit and risk in a time frame consistent with the writing of a guideline, c) the relevance to a substantial number of patients, and d) the likelihood that the findings can be translated into actionable recommendations. ERC members may include methodologists, epidemiologists, healthcare providers, and biostatisticians. The recom- mendations developed by the writing committee on the basis of the systematic review are marked with SR. Guideline-Directed Management and Therapy The term guideline-directed management and therapy (GDMT) encompasses clinical evaluation, diagnostic testing, and pharmacological and procedural treat- ments. For these and all recommended drug treat- ment regimens, the reader should confirm the dosage by reviewing product insert material and evaluate the treatment regimen for contraindications and interac- tions. The recommendations are limited to drugs, de- vices, and treatments approved for clinical use in the United States. Class of Recommendation and Level of Evidence The Class of Recommendation (COR) indicates the strength of the recommendation, encompassing the es- timated magnitude and certainty of benefit in propor- tion to risk. The Level of Evidence (LOE) rates the quality of scientific evidence that supports the intervention on the basis of the type, quantity, and consistency of data from clinical trials and other sources (Table 1).P-4 P-6 Glenn N. Levine, MD, FACC, FAHA Chair, ACC/AHA Task Force on Clinical Practice Guidelines 1.1. Methodology and Evidence Review The recommendations listed in this guideline are, whenever possible, evidence-based. An initial exten- sive evidence review, which included literature de- rived from research involving human subjects, pub- lished in English, and indexed in MEDLINE (through PubMed), EMBASE, the Cochrane Library, the Agency for Healthcare Research and Quality, and other select- ed databases relevant to this guideline, was conduct- ed from April 2014 to November 2014. Key search words included but were not limited to the follow- ing: adult congenital heart disease, anesthesia, aortic aneurysm, aortic stenosis, atrial septal defect, arterial switch operation, bradycardia, bicuspid aortic valve, cardiac catheterization, cardiac imaging, cardiovas- cular magnetic resonance, cardiac reoperation, car- diovascular surgery, chest x-ray, cirrhosis, coarctation of the aorta, congenital heart defects, congenitally corrected transposition of the great arteries, con- traception, coronary artery abnormalities, cyanotic congenital heart disease, dextro-transposition of the great arteries, double inlet left ventricle, Ebstein a- nomaly, echocardiography, Eisenmenger syndrome, electrocardiogram, endocarditis, exercise test, Fon- tan, heart catheterization, heart defect, heart failure, infertility, l-transposition of the great arteries, medical therapy, myocardial infarction, noncardiac surgery, patent ductus arteriosus, perioperative care, physical activity, postoperative complications, pregnancy, pre- operative assessment, psychosocial, pulmonary arte- rial hypertension, hypoplastic left heart syndrome, pulmonary regurgitation, pulmonary stenosis, pul- monary valve replacement, right heart obstruction, right ventricle to pulmonary artery conduit, single ventricle, supravalvular pulmonary stenosis, surgical therapy, tachyarrhythmia, tachycardia, tetralogy of Fallot, transplantation, tricuspid atresia, Turner syn- drome, and ventricular septal defect. Additional rele- vant studies published through January 2018, during the guideline writing process, were also considered by the writing committee, and added to the evidence tables when appropriate. The final evidence tables, included in the Online Data Supplement, summarize the evidence used by the writing committee to for- mulate recommendations. References selected and published in this document are representative and not all-inclusive. As noted in the preamble, an independent ERC was commissioned to perform a formal systematic review of critical clinical questions related to adult congen- ital heart disease (ACHD), the results of which were considered by the writing committee for incorporation CLINICAL STATEMENTS AND GUIDELINES into this guideline. Concurrent with this process, writ- ing committee members evaluated study data relevant to the rest of the guideline. The findings of the ERC and the writing committee members were formally presented and discussed, and then recommendations were developed. The systematic review reports on Medical Therapy for Systemic Right Ventricles: A Sys- tematic Review (Part 1) for the 2018 AHA/ACC Guide- line for the Management of Adults With Congenital and Interventional Therapy Versus Medical Therapy for Secundum Atrial Septal Defect: A Systematic Review (Part 2) for the 2018 AHA/ACC Guideline for the Management of Adults With Con- genital Heart Disease S1.1-2 are published in conjunction with this guideline. 1.2. Organization of the Writing Committee The writing committee consisted of pediatric and adult congenital cardiologists, interventional cardiologists, electrophysiologists, surgeons, and an advance prac- Table 1. Applying Class of Recommendation and Level of Evidence to Clinical Strategies, Interventions, Treatments, or Diagnostic Testing in Patient Care* (Updated August 2015) CLINICAL STATEMENTS AND GUIDELINES tice nurse. The writing committee included represent- atives from the ACC, AHA, and American Association for Thoracic Surgery (AATS), American Society of Ech- ocardiography (ASE), Heart Rhythm Society (HRS), In- ternational Society for Adult Congenital Heart Disease (ISACHD), Society for Cardiovascular Angiography and Interventions (SCAI), and the Society of Thoracic Sur- geons (STS). 1.3. Document Review and Approval This document was reviewed by 3 official reviewers each nominated by the ACC and AHA, and 1 to 2 reviewers each from the AATS, ASE, HRS, ISACHD, SCAI, STS; and 32 individual content reviewers. Re- viewers RWI information was distributed to the writ- ing committee and is published in this document (Appendix 2). This document was approved for publication by the governing bodies of the ACC and the AHA and endorsed by the AATS, ASE, HRS, ISACHD, SCAI, and STS. 1.4. Scope of the Guideline The 2018 ACHD guideline is a full revision of the 2008 ACC/AHA Guidelines for the Management of Adults with Congenital Heart Disease, S1.4-1 which was the first US guideline to be published on the topic. This revision uses the 2008 ACHD guideline as a framework and incorporates new data and growing ACHD expertise to develop recommendations. Con- genital heart disease (CHD) encompasses a range of structural cardiac abnormalities present before birth attributable to abnormal fetal cardiac develop- ment but does not include inherited disorders that may have cardiac manifestations such as Marfan syn- drome or hypertrophic cardiomyopathy. Also not in- cluded are anatomic variants such as patent foramen ovale. Valvular heart disease (VHD) may be congen- ital, so management overlaps with the 2014 AHA/ ACC Guidelines for the Management of Patients With Valvular Heart Disease, S1.4-2 particularly for bicuspid aortic valve (BAV) disease. Where overlap exists, this document focuses on the diagnosis and treatment of congenital valve disease when it differs from acquired valve disease, whether because of anatomic differ- ences, presence of concomitant lesions, or differences to consider given the relatively young age of patients with ACHD. This guideline is not intended to apply to children (<18 years of age) with CHD or adults with acquired VHD, heart failure (HF), or other cardiovas- cular diseases. The prevalence of ACHD is growing because of the success of pediatric cardiology and congenital cardiac surgery in diagnosing and treating congen- ital heart defects in children. Improved survival to adulthood is most striking for those with the most severe disease, with survival to age 18 years now ex- pected for 90% of children diagnosed with severe Patients with ACHD are a heteroge- neous population, both in underlying anatomy and physiology, as well as surgical repair or palliation. Consequently, although the prevalence of ACHD is in- creasing, the population of patients with a given con- genital abnormality or specific repair may be relatively Patients with CHD are not cured of their disease after successful treatment in childhood. Almost all patients with ACHD will have sequelae of either their native CHD or its surgical repair or palliation, although these sequelae can take decades to man- ifest. The heterogeneity of the population and the long, symptom-free intervals constrain the ability to generate data applicable across the population of ACHD or to adults with specific lesions or repairs. Despite the difficulty in studying ACHD populations, there is a growing body of high-quality data in these patients to guide the care of this relatively new population and, whenever feasible, these data were used to develop recommendations. Recommenda- tions are made based on the available data; however, when important clinical issues lacked data, first prin- ciples, extrapolation from data in other populations, and expert consensus are used to guide care. Patients with ACHD may have concomitant disease to which other existing guidelines apply, such as coronary ar- tery disease, HF, and arrhythmias. The data from ac- quired heart disease populations may apply to some patients with ACHD, and those circumstances are acknowledged in these recommendations and refer- enced accordingly. Patients with ACHD who are cared for in ACHD cen- ters have better outcomes than those cared for in cen- ters without ACHD expertise,S1.4-9 and this need for spe- cialized care is noted throughout the guideline. These recommendations are intended to provide guidance to a wide variety of providers caring for patients with ACHD, including general, pediatric, and ACHD cardiol- ogists, as well as surgeons, primary care providers, and other healthcare providers. In developing the 2018 ACHD guideline, the writing committee reviewed previously published guidelines and related scientific statements. Table 2 contains a list of publications and scientific statements deemed per- tinent to this writing effort; it is intended for use as a CLINICAL STATEMENTS AND GUIDELINES Table 2. Associated Guidelines and Statements Title Organization Publication Year (Reference) Guidelines Syncope ACC/AHA/HRS 2017S1.4-10 Supraventricular tachycardia ACC/AHA/HRS 2015S1.4-11 Cardiopulmonary resuscitation and emergency cardiovascular care Part 8: post cardiac arrest care AHA 2015S1.4-12 Non ST-elevation acute coronary syndromes AHA/ACC 2014S1.4-13 Perioperative cardiovascular evaluation and noncardiac surgery ACC/AHA 2014S1.4-14 Atrial fibrillation AHA/ACC/HRS 2014S1.4-15 Stable ischemic heart disease ACC/AHA/ACP/AATS/PCNA/SCAI/STS 2014,S1.4-16 Assessment of cardiovascular risk ACC/AHA 2014S1.4-18 Blood cholesterol to reduce atherosclerotic cardiovascular risk in adults ACC/AHA 2014S1.4-19 Overweight and obesity in adults AHA/ACC/TOS 2014S1.4-20 Lifestyle management to reduce cardiovascular risk AHA/ACC 2014S1.4-21 Valvular heart disease AHA/ACC 2017S1.4-22 High blood pressure in adults ACC/AHA/AAPA/ABC/ACPM/AGS/ APhA/ASH/ASPC/NMA/PCNA Aortic valve and ascending aorta STS 2013S1.4-24 ST-elevation myocardial infarction ACC/AHA 2013S1.4-25 Heart failure ACC/AHA/HFSA 2017S1.4-26 Device-based therapy for cardiac rhythm abnormalities ACC/AHA/HRS 2012S1.4-27 Coronary artery bypass graft surgery ACC/AHA 2011S1.4-28 Percutaneous coronary intervention ACC/AHA/SCAI 2011S1.4-29 Secondary prevention and risk reduction therapy AHA/ACC 2011S1.4-30 Cardiovascular disease in women AHA/ACC 2011S1.4-31 Grown-up congenital heart disease ESC 2010S1.4-32 Thoracic aortic disease ACC/AHA/AATS/ACR/ASA/SCA/ SCAI/SIR/STS/SVM Adult congenital heart disease CCS 2010S1.4-34 Infective endocarditis ESC 2009S1.4-35 Scientific statements Imaging for patients with transposition of the great arteries ASE 2016S1.4-36 Cardiac chamber quantification by echocardiography ASE 2015S1.4-37 Consensus on arrhythmia management in ACHD PACES/HRS 2014S1.4-38 Imaging for patients with repaired tetralogy of Fallot ASE 2014S1.4-39 Thoracic aortic disease CCS 2014S1.4-40 Promotion of physical activity for children and adults with CHD AHA 2013S1.4-41 Neurodevelopmental outcomes in children with CHD AHA 2012S1.4-42 Pregnancy in women with heart disease ESC 2011S1.4-43 Transition to adulthood for adolescents with CHD AHA 2011S1.4-44 Pulmonary hypertension ACC/AHA 2009S1.4-45 Prevention of infective endocarditis AHA 2007S1.4-46 AATS indicates American Association for Thoracic Surgery; ABC, Association of Black Cardiologists; ACC, American College of Cardiology; ACHD, adult congenital heart disease; ACP, American College of Physicians; ACPM, American College of Preventive Medicine; ACR, American College of Radiology; AGS, American Geriatrics Society; AHA, American Heart Association; APhA, American Pharmacists Association; ASA, American Stroke Association; ASE, American Society of Echocardiography; ASH, American Society of Hypertension; ASPC, American Society of Preventive Cardiology; CCS, Canadian Cardiovascular Society; CHD, congenital heart disease; ESC, European Society of Cardiology; HFSA, Heart Failure Society of America; HRS, Heart Rhythm Society; NMA, National Medical Association; PACES, Pediatric and Congenital Electrophysiology Society; PCNA, Preventive Cardiovascular Nurses Association; SCA, Society of Cardiovascular Anesthesiologists; SCAI, Society for Cardiovascular Angiography and Interventions; SIR, Society of Interventional Radiology; STS, Society of Thoracic Surgeons; SVM, Society for Vascular Medicine; and TOS, The Obesity Society. CLINICAL STATEMENTS AND GUIDELINES resource and does not repeat existing guideline recom- mendations. Abbreviation Meaning/Phrase AAOCA anomalous aortic origin of the coronary artery ACHD adult congenital heart disease AP anatomic and physiological AR aortic regurgitation ASD atrial septal defect AVSD atrioventricular septal defect BAV bicuspid aortic valve CCT cardiac computed tomography CCTGA congenitally corrected transposition of the great arteries CHD congenital heart disease CMR cardiovascular magnetic resonance CoA coarctation of the aorta CPET cardiopulmonary exercise test CT computed tomography CTA computed tomography angiography d-TGA dextro-transposition of the great arteries ECG electrocardiogram ERC evidence review committee GDMT guideline-directed management and therapy HF heart failure ICD implantable cardioverter-defibrillator IE infective endocarditis LV left ventricular LVOT left ventricular outflow tract PA pulmonary artery PAH pulmonary arterial hypertension PDA patent ductus arteriosus PR pulmonary regurgitation PS pulmonary stenosis QoL quality of life Qp:Qs pulmonary systemic blood flow ratio RCT randomized controlled trial RV right ventricular RVOT right ventricular outflow tract SCD sudden cardiac death SubAS subaortic stenosis TEE transesophageal echocardiography TGA transposition of the great arteries TOF tetralogy of Fallot TR tricuspid regurgitation TTE transthoracic echocardiography VHD valvular heart disease VSD ventricular septal defect VT ventricular tachycardia 2. BACKGROUND AND PATHOPHYSIOLOGY 2.1. Anatomic and Physiological Terms The International Society for Nomenclature of Pediatric and Congenital Heart Disease (also known as the No- menclature Working Group) defined, codified, mapped, and archived examples of nomenclatures and devel- oped standards for terminology.S2.1-1 S2.1-5 The Interna- tional Paediatric and Congenital Cardiac Code (IPCCC) nomenclature for anatomic lesions and repairs is used in this guideline (http://ipccc.net).S2.1-6 2.2. Severity of ACHD In a patient with CHD, severity of disease is deter- mined by native anatomy, surgical repair, and current physiology. Prior documents, including the 2008 ACHD relied primarily on anatomic classifica- tions to rank severity of disease. However, patients with the same underlying anatomy may have very different repairs and experienced variable physiological conse- quences of those repairs. For example, a patient with tetralogy of Fallot (TOF) after a valve-sparing primary re- pair may have excellent biventricular function with nor- mal exercise capacity and no arrhythmias, whereas an- other patient of the same age with TOF may have had palliative shunting followed by a transannular patch repair resulting in severe pulmonary regurgitation (PR) with right ventricular (RV) enlargement, biventricular dysfunction, and ventricular tachycardia (VT). To cate- gorize disease severity in CHD in a more comprehensive way, the writing committee developed an ACHD Ana- tomic and Physiological (AP) classification system (Ta- bles 3 and 4) that incorporates the previously described CHD anatomic variables as well as physiological vari- ables, many of which have prognostic value in patients with ACHD. 2.3. The ACHD AP Classification The ACHD AP classification (Tables 3 and 4), newly elab- orated in this guideline, is intended to capture the com- plexity of ACHD anatomy and physiology, which are not always correlated. Certain anatomic abnormalities of clinical importance are shared across diagnoses (eg, aortic enlargement), which may be found in patients with BAV, coarctation of the aorta (CoA), transposition (Continued) Abbreviation Meaning/Phrase CLINICAL STATEMENTS AND GUIDELINES Table 3. Physiological Variables as Used in ACHD AP Classification Variable Description Aortopathy Aortic enlargement is common in some types of CHD and after some repairs. Aortic enlargement may be progressive over a lifetime. There is no universally accepted threshold for repair, nor is the role of indexing to body size clearly defined in adults, as is done in pediatric populations. For purposes of categorization and timing of follow-up imagingS2.2-2 S2.2-4 : Mild aortic enlargement is defined as maximum diameter 3.5 3.9 cm Moderate aortic enlargement is defined as maximum diameter 4.0 4.9 cm Severe aortic enlargement is defined as maximum diameter 5.0 cm Arrhythmia Arrhythmias are very common in patients with ACHD and may be both the cause and consequence of deteriorating hemodynamics, valvular dysfunction, or ventricular dysfunction. Arrhythmias are associated with symptoms, outcomes, and prognosis,S2.2-5 S2.2-8 thus are categorized based on presence and response to treatment. No arrhythmia: No documented clinically relevant atrial or ventricular tachyarrhythmias Arrhythmia not requiring treatment: Bradyarrhythmia, atrial or ventricular tachyarrhythmia not requiring antiarrhythmic therapy, cardioversion, or ablation Arrhythmia controlled with therapy: Bradyarrhythmia requiring pacemaker implantation Atrial or ventricular tachyarrhythmia requiring antiarrhythmic therapy, cardioversion, or ablation AF and controlled ventricular response Patients with an ICD Refractory arrhythmias: Atrial or ventricular tachyarrhythmia currently unresponsive to or refractory to antiarrhythmic therapy or ablation Concomitant VHD Severity defined according to the 2014 VHD guideline.S2.2-2 Mild VHD Moderate VHD Severe VHD End-organ dysfunction Clinical and/or laboratory evidence of end-organ dysfunctionS2.2-9 S2.2-11 including: Renal (kidney) Hepatic (liver) Pulmonary (lung) Exercise capacity Patients with ACHD are often asymptomatic notwithstanding exercise limitations demonstrated as diminished exercise capacity when evaluated objectively.S2.2-12 S2.2-14 Thus, assessment of both subjective and objective exercise capacity is important (see NYHA classification system below). Exercise capacity is associated with prognosis.S2.2-15 S2.2-17 Abnormal objective cardiac limitation to exercise is defined as an exercise maximum ventilatory equivalent of oxygen below the range expected for the specific CHD anatomic diagnosis.S2.2-18 Expected norms for CPET values should take into account age, sex, and underlying congenital diagnosis. Published studies with institution-specific norms can be used as guides, bearing in mind variability among institutional norms and ranges. Hypoxemia/hypoxia/ cyanosis See Section 3.16. for detailed definition of cyanosis. Hypoxemia is defined as oxygen saturation measured by pulse oximetry at rest 90%. Severe hypoxemia is defined as oxygen saturation at rest <85%. In patients with normal or high hemoglobin concentrations, severe hypoxemia will be associated with visible cyanosis (which requires 5 g/L desaturated hemoglobin to be appreciated). The terms cyanosis and hypoxemia (or hypoxia) are sometimes used interchangeably. Such interchangeability would not apply; however, in the presence of anemia, severe hypoxemia can be present without visible cyanosis. NYHA functional classification Class Functional Capacity I Patients with cardiac disease but resulting in no limitation of physical activity. Ordinary physical activity does not cause undue fatigue, palpitation, dyspnea, or anginal pain. II Patients with cardiac disease resulting in slight limitation of physical activity. They are comfortable at rest. Ordinary physical activity results in fatigue, palpitation, dyspnea, or anginal pain. III Patients with cardiac disease resulting in marked limitation of physical activity. They are comfortable at rest. Less than ordinary activity causes fatigue, palpitation, dyspnea, or anginal pain. IV Patients with cardiac disease resulting in inability to carry on any physical activity without discomfort. Symptoms of HF or the anginal syndrome may be present even at rest. If any physical activity is undertaken, discomfort increases. (Continued) CLINICAL STATEMENTS AND GUIDELINES of the great arteries, and TOF, amongst others. In every patient, anatomic and physiological variables should be considered. In using Tables 3 and 4, a patient should be classified based on the highest relevant anatomic or physiological feature. For example, a normotensive pa- tient with repaired CoA, normal exercise capacity, and normal end-organ function would be ACHD AP classifi- cation IIA, whereas an otherwise similar patient with as- cending aortic diameter of 4.0 cm would be ACHD AP classification IIB, and if moderate aortic stenosis were also present, the ACHD AP classification would be IIC. Patients with ACHD may have baseline exercise limitations, cyanosis, end-organ dysfunction, or other clinically important comorbidities related to their CHD. They are also at risk of HF, arrhythmias, sudden car- diac death (SCD), and development or progression of cardiac symptoms such as dyspnea, chest pain, and exercise intolerance. Concomitant valvular disease or aortic pathology may be present. There are growing data regarding the prognostic implications of these variables in patients with ACHD, but not the abun- dance of data available for patients with acquired The variables forming part of the ACHD AP classifi- cation (Table 3) were selected because data exist sug- gesting their importance in prognosis, management, or quality of life (QoL). As new data become availa- ble, we expect changes in the relative weights attrib- uted to the components of the ACHD AP classification and perhaps new components, resulting in a scheme that ever more precisely tracks overall severity of di- sease and need for more or less intensive follow-up and management. Similar to the New York Heart Association (NYHA) classification of functional status, patients may move from one ACHD AP classification to another over time. If clinical status worsens, the classification will change to a higher severity group, but improvement in status, for example after an intervention such as valve replace- ment or control of arrhythmia, can result in change to a lower severity classification. Such movement among classes is unlike the AHA HF A to D classification,S2.3-17 in which patients move in only one direction. This ACHD AP classification is used throughout this document, par- ticularly when considering follow-up visits and need for testing. As the ACHD AP classification worsens because of changes in physiology (eg, development of arrhyth- mias, HF, end-organ disease), the nature and frequency of recommended follow-up visits and testing will also change, adapting to the patient s changing circum- stance instead of depending solely on a description of anatomic disease, which may not adequately discrimi- nate physiological changes that alter severity over time. Some patients with ACHD may have substantial ac- quired comorbidities unrelated to CHD and, as a conse- quence, their follow-up strategies might be more appro- priately based on other existing guidelines for acquired heart disease. For example, an 80-year-old patient who Pulmonary hypertension Pulmonary hypertension is a broad term that encompasses pulmonary arterial hypertension, which is pulmonary hypertension with increased pulmonary vascular resistance. This document defines PH and PAH as they are used in the field of pulmonary hypertension. Pulmonary hypertension is defined as: Mean PA pressure by right heart catheterization 25 mmHg. PAH is defined as: Mean PA pressure by right heart catheterization 25 mmHg and a pulmonary capillary wedge pressure 15 mmHg and pulmonary vascular resistance 3 Wood unitsS2.2-20 Shunt (hemodynamically significant shunt) An intracardiac shunt is hemodynamically significant if: There is evidence of chamber enlargement distal to the shunt And/or evidence of sustained Qp:Qs 1.5:1 An intracardiac shunt not meeting these criteria would be described as small or trivial Venous and arterial stenosis Aortic recoarctation after CoA repair Supravalvular aortic obstruction Venous baffle obstruction Supravalvular pulmonary stenosis Branch PA stenosis Stenosis of cavopulmonary connection Pulmonary vein stenosis ACHD indicates adult congenital heart disease; AF, atrial fibrillation; AP, anatomic and physiological; CHD, congenital heart disease; CoA, coarctation of the aorta; CPET, cardiopulmonary exercise test; HF, heart failure; ICD, implantable cardioverter-defibrillator; NYHA, New York Heart Association; PA, pulmonary artery; PAH, pulmonary arterial hypertension; Qp:Qs, pulmonary systemic blood flow ratio; and VHD, valvular heart disease. Table 3. Continued Variable Description CLINICAL STATEMENTS AND GUIDELINES Table 4. ACHD AP Classification (CHD Anatomy + Physiological Stage = ACHD AP Classification) CHD Anatomy* I: Simple Native disease Isolated small ASD Isolated small VSD Mild isolated pulmonic stenosis Repaired conditions Previously ligated or occluded ductus arteriosus Repaired secundum ASD or sinus venosus defect without significant residual shunt or chamber enlargement Repaired VSD without significant residual shunt or chamber enlargement II: Moderate Complexity Repaired or unrepaired conditions Aorto-left ventricular fistula Anomalous pulmonary venous connection, partial or total Anomalous coronary artery arising from the pulmonary artery Anomalous aortic origin of a coronary artery from the opposite sinus AVSD (partial or complete, including primum ASD) Congenital aortic valve disease Congenital mitral valve disease Coarctation of the aorta Ebstein anomaly (disease spectrum includes mild, moderate, and severe variations) Infundibular right ventricular outflow obstruction Ostium primum ASD Moderate and large unrepaired secundum ASD Moderate and large persistently patent ductus arteriosus Pulmonary valve regurgitation (moderate or greater) Pulmonary valve stenosis (moderate or greater) Peripheral pulmonary stenosis Sinus of Valsalva fistula/aneurysm Sinus venosus defect Subvalvar aortic stenosis (excluding HCM; HCM not addressed in these guidelines) Supravalvar aortic stenosis Straddling atrioventricular valve Repaired tetralogy of Fallot VSD with associated abnormality and/or moderate or greater shunt III: Great Complexity (or Complex) Cyanotic congenital heart defect (unrepaired or palliated, all forms) Double-outlet ventricle Fontan procedure Interrupted aortic arch Mitral atresia Single ventricle (including double inlet left ventricle, tricuspid atresia, hypoplastic left heart, any other anatomic abnormality with a functionally single ventricle) (Continued) III: Great Complexity (or Complex) (Continued) Pulmonary atresia (all forms) TGA (classic or d-TGA; CCTGA or l-TGA) Truncus arteriosus Other abnormalities of atrioventricular and ventriculoarterial connection (ie, crisscross heart, isomerism, heterotaxy syndromes, ventricular inversion) Physiological Stage A NYHA FC I symptoms No hemodynamic or anatomic sequelae No arrhythmias Normal exercise capacity Normal renal/hepatic/pulmonary function B NYHA FC II symptoms Mild hemodynamic sequelae (mild aortic enlargement, mild ventricular enlargement, mild ventricular dysfunction) Mild valvular disease Trivial or small shunt (not hemodynamically significant) Arrhythmia not requiring treatment Abnormal objective cardiac limitation to exercise C NYHA FC III symptoms Significant (moderate or greater) valvular disease; moderate or greater ventricular dysfunction (systemic, pulmonic, or both) Moderate aortic enlargement Venous or arterial stenosis Mild or moderate hypoxemia/cyanosis Hemodynamically significant shunt Arrhythmias controlled with treatment Pulmonary hypertension (less than severe) End-organ dysfunction responsive to therapy D NYHA FC IV symptoms Severe aortic enlargement Arrhythmias refractory to treatment Severe hypoxemia (almost always associated with cyanosis) Severe pulmonary hypertension Eisenmenger syndrome Refractory end-organ dysfunction *This list is not meant to be comprehensive; other conditions may be important in individual patients. ACHD indicates adult congenital heart disease; AP, anatomic and physiological; ASD, atrial septal defect; AVSD, atrioventricular septal defect; CCTGA, congenitally corrected transposition of the great arteries; CHD, congenital heart disease; d-TGA, dextro-transposition of the great arteries; FC, functional class; HCM, hypertrophic cardiomyopathy; l-TGA, levo-transposition of the great arteries; NYHA, New York Heart Association; TGA, transposition of the great arteries; and VSD, ventricular septal defect. Table 4. Continued CHD Anatomy* (Continued) CLINICAL STATEMENTS AND GUIDELINES has a small atrial septal defect (ASD), but whose symp- toms are related to diastolic HF, chronic kidney disease caused by hypertension and diabetes mellitus, and mod- erate aortic stenosis is well-suited to be followed ac- cording to existing guidelines for those diseases, rather than according to the ACHD AP classification for the ASD. Nevertheless, the added hemodynamic complexity brought by the ASD must be kept in mind. Throughout this document, the ACHD AP classifica- tion is used to help guide resource utilization, including ACHD consultation and routine diagnostic studies. 3. GENERAL PRINCIPLES See Online Data Supplements 1 and 2 for additional data supporting this section. 3.1. ACHD Program Patients with complex CHD have generally better out- comes when cared for in an integrated, collaborative, and multidisciplinary program.S3.1-1 Many medical is- sues in patients with ACHD involve cardiac sequelae, and the diagnosis and management may require car- diac anesthesiologists, electrophysiologists, and in- terventional cardiologists; imaging services such as cardiovascular magnetic resonance (CMR)/cardiac computed tomography (CCT); and pulmonary hy- pertension services with expertise in ACHD (Table 5). Appropriate specialty care must be available to address pregnancy, acquired cardiovascular disease, and acute noncardiac illness complicating CHD, man- agement of which is frequently more complicated in patients with ACHD. Although individual providers may be communi- ty-based affiliates, ACHD programs are inpatient, outpatient, and hospital-based with staffing and ex- pertise available on-site or accessible when needed (Table 5). 3.2. Access to Care Recommendation for Access to Care Referenced studies that support the recommendation are summarized in Online Data Supplement 3. COR LOE Recommendation I B-NR Physicians caring for patients with ACHD should support access to care by a) assuring smooth transitions for adolescents and young adults from pediatric to adult (Level of Evidence: B-NR); and b) promoting awareness of the need for lifelong specialized care through outreach and educational programs (Level of Evidence: C-EO). C-EO Synopsis As patients with ACHD grow beyond the pediatric age group, continued access to specialized cardiovascular care presents several challenges: Lack of guided transfer from pediatric to adult care; Insufficient availability of ACHD programs; Inadequate insurance coverage; Deficient education of patients and caregivers regarding ACHD; Inadequate resources for patients with cognitive or psychosocial impairment; Lack of comprehensive case management; and Table 5. Key Personnel and Services Recommended for ACHD Programs Personnel ACHD board-eligible/board-certified cardiologists Congenital cardiac surgeons Nurses/physician assistants/nurse practitioners Cardiac anesthesiologists with CHD training/expertise Multidisciplinary teams: High-risk obstetrics Pulmonary hypertension HF/transplant Genetics Hepatology Cardiac pathology Rehabilitation services Social services Psychological services Financial counselors Services Echocardiography, including TEE and intraoperative TEE* CHD diagnostic and interventional catheterization* CHD electrophysiology/pacing/ICD implantation*: Exercise testing Echocardiographic Radionuclide Cardiopulmonary Cardiac imaging/radiology*: CMR CCT Nuclear medicine Information technology: Data collection Database support Quality assessment review/protocols *These modalities must be supervised/performed and interpreted by clinicians with expertise and/or training in CHD. ACHD indicates adult congenital heart disease; CCT, cardiac computed tomography; CHD, congenital heart disease; CMR, cardiovascular magnetic resonance; HF, heart failure; ICD, implantable cardioverter-defibrillator; and TEE, transesophageal echocardiography. CLINICAL STATEMENTS AND GUIDELINES Different needs for evaluation and management compared with adults with acquired cardiovascular disease. Recommendation-Specific Supportive Text 1. Many patients with CHD face gaps in care during and after adolescence.S3.2-2 Common reasons include lack of knowledge regarding need for follow-up, in- ability to find specialized providers, insurance issues, and feeling well.S3.2-1 Patients with gaps in care are more likely to develop medical problems requir- ing intervention than those receiving continuous Canadian patients with CHD in special- ized care programs had lower mortality than those in centers without ACHD expertise.S3.2-5 Improving transition programs and recognizing the importance of long-term care will hopefully improve access to specialty care. Insurance barriers and lack of spe- cialty providers for the large number of patients are issues; thus, relationships with regulatory agencies to address these challenges are important. 3.3. Delivery of Care Recommendations for Delivery of Care Referenced studies that support recommendations are summarized in Online Data Supplements 3, 4, and 5. COR LOE Recommendations I B-NR Patients with ACHD AP classification IB-D, IIA-D, and IIIA-D* should be managed in collaboration with an ACHD cardiologist.S3.3-1 I C-LD Cardiac surgery, catheter-based interventional cardiac procedures, and electrophysiological procedures involving congenital heart lesions in patients with ACHD should be performed by operators with expertise in CHD procedures and in collaboration with an ACHD *See Tables 3 and 4 for details on the ACHD Anatomic and Physiological classification system. Recommendation-Specific Supportive Text 1. Patients with ACHD, particularly those with more severe CHD, cared for in specialized centers have lower mortality than those managed without spe- Although clinical practice guide- lines can be helpful, many management decisions for patients with ACHD must be based on insuf- ficient data or care guidelines and require clinical experience often involving multiple members of an ACHD team. Patients with complex anatomic and physiological forms of ACHD may need approaches to evaluation and treatment that differ from those applicable to adults without ACHD who have valve disease, HF, or arrhythmias. From a practical perspective, it may be difficult to identify clinicians with expertise in ACHD, and expertise in ACHD varies across medical and sur- gical specialties. Some specialties, such as cardi- ology and congenital heart surgery, have defined ACHD fellowship training and board certification, whereas for others, ACHD expertise is gained by focused experience during training and practice. In 2012, the American Board of Medical Spe- cialties approved ACHD as a subspecialty of in- ternal medicine ( adult ) cardiology and pedi- atric cardiology. Therefore, for cardiologists, one marker of ACHD expertise is board eligibility/board certification in ACHD. There are expert ACHD cli- nicians who are not board-certified, including those whose expertise was acquired before the development of formal certification programs and those trained outside the United States who may also have different pathways to achieve ACHD ex- pertise. Expertise in the surgical management of patients with ACHD may be identified through board eligibility/board certification in congenital heart surgery. There are expert ACHD surgeons who are not board-certified, including those sur- geons trained in other countries who are not eligi- ble for certification in the United States. Specific ACHD training options are not gen- erally available for cardiac anesthesiologists, but many of them develop expertise through training in pediatric anesthesiology, cardiac anesthesiology, mentoring, and practice experience. Other provid- ers involved in the care of patients with ACHD (eg, obstetricians, pulmonologists, radiologists, nurse practitioners, physician assistants) derive expertise from training and/or practice. Individual provid- ers may gain ACHD expertise in a specific area or discipline, such as intraoperative transesophageal echocardiography (TEE) or interpretation of CMR. 2. Patients with ACHD who are undergoing in- vasive cardiovascular procedures in specialized ACHD centers generally have better outcomes, including survival, than those managed in other Special attention is required to ensure appropriate periprocedural care, including identification of procedure-related risk factors and availability of ancillary imaging.S3.3-3 S3.3-10 Table 6 addresses delivery of care where circumstances of ACHD expertise may improve patient outcomes. 3.4. Evaluation of Suspected and Known CHD Tools commonly used in the evaluation of adults with suspected or known acquired cardiovascular disease CLINICAL STATEMENTS AND GUIDELINES Table 6. Delivery of Care: Circumstances Where ACHD Expertise May Improve Outcomes Circumstance Possible Solution Rationale Example Care of patients in the lowest ACHD AP classification (IA)* Face-to-face consultation with an ACHD cardiologist. Patients in ACHD AP classification IA* are likely to be asymptomatic and not require frequent routine congenital cardiac care. Patients with small VSDs are thought to have excellent long-term survival, although complications (double-chamber RV, IE, aortic valve prolapse and aortic regurgitation) may manifest in adulthood; consequently, patients with small VSDs warrant lifelong Collaborative care planning between an ACHD patient s general cardiologist or primary care provider and an ACHD cardiologist. The very long-term outcomes of patients with ACHD AP classification IA* lesions have not been well described, although available data suggest that patients with simple CHD have higher cardiac mortality in long-term follow-up than age-matched controls.S3.3-11 Consultation with an ACHD cardiologist should help to accurately assess the patient s ACHD AP class, provide information regarding potential long-term outcomes, and reinforce signs and symptoms that should prompt further evaluation. Cardiac imaging of patients with ACHD Imaging studies should be performed and interpreted by individuals with expertise in CHD imaging. The complexity and variability of lesions, repairs, and sequelae in CHD constrain the use of standard protocols and sequences and often require modification of plans during acquisition of images, as well as specialized skills in interpretation. Thus, CHD expertise is helpful for optimal quality and interpretation of cardiac imaging studies. Although imaging of a patient with TOF may seem straightforward because many have familiar chamber and great vessel relationships, there are nuances to echocardiographic imaging of RV size and function, PR severity, and/or location of right ventricular outflow tract obstruction that affect clinical care and are thus best carried out by sonographers and echocardiographers with appropriate expertise. Similarly, expertise in congenital CMR is important in evaluating patients with TOF, as RV volumes and function are key components in evaluation for timing of pulmonary valve replacement.S3.3-15,S3.3-16 Use of a multimodality cardiac imaging approach can be used for patients with ACHD, accounting for patient-specific considerations, strengths and weaknesses of each modality, institutional resources, and expertise. ACHD programs need a dedicated CMR service, and CMR expertise is integral to an ACHD program, as is expertise in ACHD Electrophysiological care of patients with ACHD Perform procedures in electrophysiology laboratories equipped for 3D mapping and ablation and involve specialists experienced in the management of arrhythmias in patients with ACHD. Examples of diagnostic questions best answered by electrophysiological study: a) evaluation of the conduction system in cases of suspected postsurgical conduction abnormalities b) evaluation of syncope c) diagnosis of the mechanism of supraventricular tachycardia or wide complex tachycardia d) programmed ventricular stimulation particularly in patients following repair of TOF and its variants (Section 4.4.1.) as well as preoperative assessment of arrhythmia substrates that may be amenable to operative intervention, such as an atrial maze procedure for atrial arrhythmias. The latter procedure is commonly used at the time of conversion of atriopulmonary connection Fontan, and may also be useful in other forms of repaired CHD with postoperative atrial arrhythmias such as TOF. Bradyarrhythmia and tachyarrhythmias are common in TGA with atrial switch patients and may seem straightforward, but the altered anatomy adds complexity to the procedures and emphasizes the need for specialized equipment and expertise to ensure the best chance for procedural success. For example: 1) pacemaker placement in a patient with TGA with atrial switch can be challenging because of the altered atrial anatomy and interatrial baffle that will necessitate placement of an atrial lead in the anatomic left atrium, often scarred such that tissue amenable to pacing is difficult to find; and 2) atrial flutter is a common arrhythmia in TGA with atrial switch, but the flutter circuit may be on the systemic side of the interatrial baffle and thus may require baffle puncture or retrograde approach to effectively ablate the circuit. Diagnostic and interventional cardiac procedures, including electrophysiology procedures Perform procedure in a hospital with cardiologists, anesthesiologists, surgeons, and other providers with expertise in the management of patients with ACHD. Patients with ACHD often have complex underlying cardiac anatomy and physiology. In patients with CHD, the presence of anatomic and physiological complexity from the specific defect or surgical palliation, may change the overall care plan and procedural decision-making. Procedures that may seem straightforward, such as pacemaker implantation or ASD closure, may be more complex when accounting for the nuances imparted by CHD. The data obtained and the interventions performed during ACHD cardiac procedures are difficult to sort out without specialized knowledge of the CHD. Consultation with providers with ACHD expertise may be substituted if the procedure is urgent such that timely transfer is not feasible. An ACHD program has additional resources such as cardiac anesthesia, congenital cardiac surgery, and specialty cardiac imaging, should the need for those services arise during or after the procedure. (Continued) CLINICAL STATEMENTS AND GUIDELINES are also valuable in the evaluation of patients with ACHD. Some tools (eg, echocardiography) are reg- ularly used in the serial evaluation of patients with ACHD, whereas other tools (eg, CMR and CCT) may have more utility in the evaluation and management of patients with ACHD than in patients with acquired cardiovascular disease (Tables 7 and 8). Cost and risk to patients can be minimized by ensuring studies are acquired and interpreted by centers and providers with CHD expertise. Administration of anesthesia for invasive procedures in patients with ACHD AP classification IB-D, IIA-D, and IIIA-D* Performed by, or in collaboration with, an anesthesiologist with expertise in the management of patients with ACHD. ACHD-specific issues need to be addressed when considering anesthesia, including underlying cardiac physiology and hemodynamics, and the effects of anesthetic medications and ventilation strategies. The application of anesthesia for laparoscopic procedures can be especially challenging in Fontan patients. Significant cardiovascular and respiratory alterations may occur as a result of increased intra-abdominal pressure and decreased venous return. Abdominal insufflation may lead to lower preload and hypotension, while at the same time elevating systemic vascular resistance and compromising cardiac output. Elevations in pulmonary vascular resistance attributable to hypercarbia can be caused by either direct carbon dioxide absorption or hypoventilation.S3.3-19 If clinical urgency precludes transfer, consultation with an anesthesiologist with ACHD expertise would be of benefit to on-site providers who do not have ACHD expertise. Many patients with ACHD have had surgeries in the past, which may have created or identified airway or vascular access concerns. Patients with ACHD can also have underlying restrictive and/or obstructive lung disease that should be Patients with ACHD and pulmonary hypertension Consultation with experts in pulmonary hypertension and ACHD to assist in the interpretation of diagnostic and invasive studies and determine the best course of management. PAH imparts a poor prognosis compared with CHD without PAH. Because of the complexity of PAH in the setting of CHD, patients with ACHD benefit from the expertise of both ACHD providers and pulmonary hypertension Management of PAH in patients with shunts can be difficult. For example, in patients for whom PAH treatment is expected to allow subsequent closure of a shunt, cohort series demonstrate progression of pulmonary vascular resistance or late mortality if defects with associated pulmonary vascular resistance elevation beyond 2.5 Wood units (>4 Wood ) or Qp:Qs >3 were closed.S3.3- The utility of acute administration of pulmonary vasodilator therapy as a marker of reversibility of pulmonary vascular resistance remains uncertain. Treat-to-repair strategies involving use of PAH therapies to bring pulmonary vascular resistance into a range where repair can be considered have been applied, but the utility of such strategies also remains uncertain. *See Tables 3 and 4 for details on the ACHD AP classification system. 3D indicates 3-dimensional; ACHD, adult congenital heart disease; AP, anatomic and physiological; ASD, atrial septal defect; CCT, cardiac computed tomography; CHD, congenital heart disease; CMR, cardiovascular magnetic resonance; IE, infective endocarditis; PAH, pulmonary arterial hypertension; PR, pulmonary regurgitation; Qp:Qs, pulmonary systemic blood flow ratio; RV, right ventricle; TGA, transposition of the great arteries; TOF, tetralogy of Fallot; and VSD, ventricular septal defect. Table 6. Continued Circumstance Possible Solution Rationale Example Table 7. Use of ECGs in ACHD Evaluation Identification of sinus bradycardia or junctional rhythm in patients at risk of sinus node dysfunction (especially after the Mustard, Senning, Glenn, or Fontan procedure) Identification of clinically inapparent intra-atrial re-entry tachycardia in patients who have had atriotomy Identification of atrioventricular block in patients at risk for progression of atrioventricular conduction system disease (especially CCTGA) Evaluation of rhythm in patients with pacemakers Measurement of QRS duration in patients after repair of TOF and as part of CRT evaluation Preoperatively to compare with postoperative ECGs in patients undergoing heart surgery and noncardiac surgery Postoperatively to identify arrhythmias (eg, atrial ectopic tachycardia, atrial flutter, AF, junctional ectopic tachycardia, atrioventricular block) Diagnosis of Wolff-Parkinson-White Syndrome in patients with Ebstein anomaly Initial evaluation of suspected acute coronary syndromes ACHD indicates adult congenital heart disease; AF, atrial fibrillation; CCTGA, congenitally corrected transposition of the great arteries; CRT, cardiac resynchronization therapy; ECG, electrocardiogram; and TOF, tetralogy of Fallot. Table 8. Circumstances Where CMR, CCT, TEE, and/or Cardiac Catheterization May be Superior to TTE Assessment of RV size and function in repaired TOF, systemic right ventricles, and other conditions associated with RV volume and pressure Identification of anomalous pulmonary venous connectionsS3.4.4-16 Serial assessment of thoracic aortic aneurysms, especially when the dilation might extend beyond the echocardiographic windowsS3.4.4-7 Accurate assessment of PA pressure and pulmonary vascular resistance Assessment for recoarctation of the aorta Sinus venosus defects Vascular rings Evaluation of coronary anomalies Quantification of valvular regurgitation CCT indicates cardiac computed tomography; CMR, cardiovascular magnetic resonance; PA, pulmonary artery; RV, right ventricular; TEE, transesophageal echocardiography; and TOF, tetralogy of Fallot. CLINICAL STATEMENTS AND GUIDELINES Recommendations for Electrocardiogram COR LOE Recommendations I C-EO A standard 12-lead electrocardiogram (ECG) is recommended in adults with CHD with serial assessment depending on the specific ACHD AP classification or when symptoms develop or worsen. I C-EO Ambulatory electrocardiographic monitoring should be performed in patients with CHD who are at risk of tachyarrhythmia, bradyarrhythmia or heart block, or when symptoms possibly of arrhythmic origin develop. Recommendation-Specific Supportive Text 1. The ECG is an essential part of a complete car- diovascular evaluation of a patient with ACHD, similar to elements of the physical examination. Regardless of anatomic diagnosis, it is important to obtain an ECG at baseline for comparison to any subsequently obtained ECG, because an ab- normal baseline ECG is expected in many forms of CHD, particularly those who have undergone surgical repair. A follow-up ECG is recommended in specific lesions and in the setting of new or worsening congestion or low cardiac output syn- drome (Table 7). 2. Asymptomatic arrhythmias seen in patients with ACHD may be associated with development of symptoms and increased risk of death, and are more common in particular lesions or repairs. Bradyarrhythmias or tachyarrhythmias may occur, with some requiring treatment even when asymp- tomatic. For example, sinus node dysfunction is common in patients with atrial switch repairs of transposition of the great arteries (TGA), whereas complete heart block is seen in patients with con- genitally corrected transposition of the great arter- ies (CCTGA) or late after atrioventricular septal defect (AVSD) repair, especially in those patients with transient postoperative heart block.S3.4.1-1 S3.4.1-3 Some of these events have occurred as late as 15 years after surgery. The atrioventricular node is typically displaced inferiorly in AVSD, which is associated with relative hypoplasia of the left Atrial tachyarrhythmias are common in atrial switch repairs of TGA, Fontan repairs, and Ebstein anomaly.S3.4.1-5 S3.4.1-7 Thus, baseline and periodic screening for asymp- tomatic arrhythmias with ambulatory electrocar- diographic monitoring is advised to ensure that asymptomatic arrhythmias that would warrant a change in therapy are not present,S3.4.1-8 acknowl- edging the limitations of monitoring over short periods of time. Any symptoms of arrhythmia should prompt investigation to establish an accu- rate diagnosis and direct subsequent therapy. 3.4.2. Ionizing Radiation Principles Recommendation for Ionizing Radiation Principles Referenced studies that support the recommendation are summarized in Online Data Supplement 6. COR LOE Recommendation I B-NR Strategies to limit and monitor radiation exposure are recommended during imaging of patients with ACHD, with studies not involving ionizing radiation chosen whenever Recommendation-Specific Supportive Text 1. Low-dose ionizing radiation is a known carcin- ogen, and certain levels of exposure similar to medical exposure have been associated with later Patients with ACHD have multiple potential exposures to low-dose ionizing radiation throughout their lifetimes from cardiac catheterizations, computed tomographic (CT) scans, nuclear perfusion scans, stress tests, and chest x-rays. It remains unclear whether there is an increased risk of malignancy among patients with ACHD, but the exposure levels from multiple pro- cedures are in the range of concern. Every effort should be made to use tests without radiation whenever possible or to select protocols with the lowest possible doses of radiation compatible with securing the needed clinical information. Recommendations for Echocardiography Referenced studies that support recommendations are summarized in Online Data Supplement 7. COR LOE Recommendations I B-NR Intraoperative TEE is recommended to guide surgical repair of CHD in adults.S3.4.3-1 I C-EO Patients with ACHD should undergo transthoracic echocardiography (TTE) for initial assessment, with timing of serial assessment based on anatomic and physiological severity and clinical status. Recommendation-Specific Supportive Text 1. A large retrospective study has shown that the routine use of intraoperative TEE has a substantial impact on patient care, leading to alteration of planned procedure or revision of the initial repair in 14% of cases and was also determined to be CLINICAL STATEMENTS AND GUIDELINES 2. For patients with ACHD in whom abnormalities and changes that may be identified on echocar- diography (eg, valvular or ventricular function or pulmonary pressures) commonly influence man- agement decisions; echocardiography is an indis- pensable tool in the initial and serial follow-up evaluation. TTE is also valuable in the initial and serial evaluation of patients without symptoms or changes in examination (Table 8). 3.4.4. CMR Imaging Recommendations for CMR Imaging Referenced studies that support recommendations are summarized in Online Data Supplement 8. COR LOE Recommendations I B-NR In patients with ACHD who have or who are at risk of developing RV enlargement and dysfunction, serial CMR is recommended for quantitative assessment of RV size and IIa C-LD CMR can be useful in the initial evaluation and serial assessment of selected patients with CHD based on anatomic complexity and clinical status.S3.4.4-1,S3.4.4-2,S3.4.4-4 S3.4.4-10 Recommendation-Specific Supportive Text 1. CMR plays a valuable role in assessment of RV size and function, because it provides data that are re- producible and more reliable than data obtained with alternative imaging techniques.S3.4.4-1 S3.4.4-4 Real-time 3-dimensional (3D) echocardiography is an emerging technique that shows some promise for replacing CCT and even CMR for serial studies, especially when focusing on ventricular volumes and intracardiac structures only, and if reasonably complete data sets can be obtained.S3.4.4-11 2. CMR has unique value in the assessment and serial follow-up of patients with ACHD, because it offers unrestricted access to the heart and great vessels noninvasively and without ionizing radi- ation. The complexity and variability of lesions, repairs, and sequelae in CHD constrain the use of standard protocols and sequences, and often require modification of plans during acquisi- tion of images, as well as specialized skills in Thus,adedicatedCMR service is integral to an ACHD program.S3.4.4-4,S3.4.4-5 CMR can provide exquisite anatomic detail and unique physiological information in many forms of CHD. It has a particularly important role in the assessment of extracardiac cardiovascular defects (eg, CoA, aortic aneurysm, and abnor- malities of the thoracic arterial and venous anatomy and connections).S3.4.4-6,S3.4.4-7 The eluci- dation of uncommon, complex forms and vari- ations of CHD is routinely facilitated by a CMR Contraindications to CMR are com- mon in patients with ACHD, so they should be sought and confirmed. However, the high value of serial CMR has encouraged modification of newer pacemakers, leads, and other devices and imaging protocols to facilitate imaging in an expanding subset of patients with ACHD who have had previous instrumentation. If a contraindication is confirmed, alternative forms of imaging, especially CCT, can obtain much of the information otherwise obtained from CMR and some unique information not provided by However, CCT has the disadvan- tage of substantial patient exposure to ionizing radiation, especially when serial studies are con- templated over a lifetime.S3.4.4-9 Real-time 3D ech- ocardiography shows promise for replacing CCT and even CMR for serial studies, especially when focusing on ventricular volumes and intracardiac structures only, and if reasonably complete data sets can be obtained (Tables 8 and 9).S3.4.4-11,S3.4.4-15 3.4.5. Cardiac Computed Tomography Recommendation for Cardiac Computed Tomography Referenced studies that support the recommendation are summarized in Online Data Supplement 9. COR LOE Recommendation IIa C-LD CCT imaging can be useful in patients with ACHD when information that cannot be obtained by other diagnostic modalities is important enough to justify the exposure to Table 9. Comparison of Imaging Modalities Useful in ACHD Evaluation Radiation Exposure Relative Cost Ventricular Volumes/ Function Valvular Structure/ Function Coronary Anatomy and Course Extracardiac Vascular Anatomy Echocardiography No $ ++ +++ +/- +/- CMR No $$ +++ ++ ++* +++ CCT Yes $$ +* + +++ +++ Cardiac catheterization Yes $$ + ++ +++ ++ $ indicates less expensive; $$, more expensive; +/-, possible value; +, good; ++, very good; and +++, excellent. *In specific gated imaging protocols. ACHD indicates adult congenital heart disease; CCT, cardiac computed tomography; and CMR, cardiovascular magnetic resonance. CLINICAL STATEMENTS AND GUIDELINES Recommendation-Specific Supportive Text 1. The most important disadvantage of CCT (in- cluding CT angiography) as an imaging technique is the associated exposure to ionizing radiation. 