CLINICAL STATEMENTS AND GUIDELINES being wrong for a young patient with AAOCA may be perceived to be greater than for many other con- ditions. Consequently, there is often a clinical urge to seek a reason to do something like surgical repair, because the available data do not identify clinical fea- tures that provide reassurance that a patient is at low risk of cardiovascular events. Unfortunately, evidence demonstrating that surgical repair ameliorates SCD risk, derived from large enough cohorts followed over a sufficient period of time, is not available. 4.4.7.1. Anomalous Coronary Artery Evaluation Recommendations for Anomalous Coronary Artery Evaluation Referenced studies that support recommendations are summarized in Online Data Supplement 51. COR LOE Recommendations Diagnostic I C-LD Coronary angiography, using catheterization, CT, or CMR, is recommended for evaluation of anomalous I C-LD Anatomic and physiological evaluation should be performed in patients with anomalous aortic origin of the left coronary from the right sinus and/or right coronary from the left sinus.S4.4.7.1-4 S4.4.7.1-9 Recommendation-Specific Supportive Text 1. CTA, CMR, and catheterization can all delineate the proximal course of the coronary artery and relationship to other structures. CTA is generally preferred because it has superior spatial and tem- poral resolution, although CMR may also pro- vide adequate delineation of the relationship of the coronary artery to the aorta, PA and other structures, including whether the proximal course appears to be intramural. Coronary angiography by catheterization can be helpful when there is concern about stenosis in the coronary artery or when concomitant hemodynamic evaluation for shunt assessment or intravascular ultrasonog- raphy/flow evaluation is needed. 2. Assessment of AAOCA is enhanced when the precise anatomy and physiological impact of the coronary artery anomaly are understood.


Assessment of AAOCA is enhanced when the precise anatomy and physiological impact of the coronary artery anomaly are understood. As described in Table 34, the specific anomalous or- igin, anatomy of the orifice and proximal vessel and presence of ischemia may all influence the clinical course and thus the management options. Understanding these issues as precisely as pos- sible will better inform clinical decisions. Figure 5. Anomalous aortic origin of the coronary artery. *Surgical intervention to involve unroofing or coronary revascularization for patients with concomitant fixed obstruction. CLINICAL STATEMENTS AND GUIDELINES 4.4.7.2. Anomalous Aortic Origin of Coronary Artery Recommendations for Anomalous Aortic Origin of Coronary Artery Referenced studies that support recommendations are summarized in Online Data Supplement 51. COR LOE Recommendations Therapeutic I B-NR Surgery is recommended for AAOCA from the left sinus or AAOCA from the right sinus for symptoms or diagnostic evidence consistent with coronary ischemia attributable to the anomalous coronary IIa C-LD Surgery is reasonable for anomalous aortic origin of the left coronary artery from the right sinus in the absence of symptoms or IIa C-EO Surgery for AAOCA is reasonable in the setting of ventricular arrhythmias. IIb B-NR Surgery or continued observation may be reasonable for asymptomatic patients with an anomalous left coronary artery arising from the right sinus or right coronary artery arising from the left sinus without ischemia or anatomic or physiological evaluation suggesting potential for compromise of coronary perfusion (eg, intramural course, fish-mouth-shaped orifice, acute Recommendation-Specific Supportive Text 1. In patients with symptoms related to AAOCA, re- pair of the anomaly should alleviate symptoms. In autopsy and surgical series, cardiac symptoms are more common in patients with a left coronary ar- tery arising from the right coronary cusp.


In autopsy and surgical series, cardiac symptoms are more common in patients with a left coronary ar- tery arising from the right coronary cusp. In au- topsy studies of patients who died because of an anomalous coronary artery, fibrosis is a common finding, suggesting that ischemia preceded the ter- minal event. However, there are patients in whom a SCD event occurred despite normal stress ECG, and consequently absence of ischemia is not reas- suring. Autopsy series show that many patients whose death is attributed to anomalous coronary arteries are young, thus management of patients should take age into account, with heightened concern about the risk of sudden death in younger 2. Anomalous left coronary from the right sinus is less common than anomalous right coronary from the but anomalous left coronary artery from the right is more commonly found in autopsy series of athletes and military recruits who had non- traumatic death than right coronary from the left The overrepresentation of the anomalous left coronary from the right sinus suggests a higher risk of SCD, particular at extremes of exertion and in patients <35 years of age. There are some anatomic features that are thought to be associated with increased risk of com- promise of coronary flow and/or SCD, including a fish-mouth-shaped or slit-like orifice, or intramural although the slit-like orifice is more commonly encountered in a right coronary arising from the left cusp. It is difficult to quantitate the ab- solute risk of SCD associated with anomalous aortic origin of the left coronary from the right sinus, and data demonstrating that surgery ameliorates the SCD risk have not been published.


It is difficult to quantitate the ab- solute risk of SCD associated with anomalous aortic origin of the left coronary from the right sinus, and data demonstrating that surgery ameliorates the SCD risk have not been published. Until studies suggest otherwise, limited data and expert con- sensus suggest that it is reasonable that adults with this malformation should undergo surgical unroof- ing unless there are extenuating circumstances that would make surgery high risk. 3. In patients with ventricular arrhythmias presumed related to ischemia caused by anomalous origin of a coronary artery, repair is an option to alleviate the ischemia and presumably mitigate the recurrence of ventricular arrhythmias. However, care should be individualized, as there may be other factors (eg, CAD, cardiomyopathy, residual ischemia) contrib- uting to ventricular arrhythmias that warrant con- tinued vigilance and additional therapy. 4. Anomalous aortic origin of the right coronary from the left sinus is more common than anomalous aortic origin of the left coronary from the right sinus. The risk of SCD with the former malformation is difficult to quantitate. There is some physiological rationale to believe that asymptomatic patients without ev- idence of compromised blood flow would benefit from unroofing, but there are not data to demon- strate that surgical interventions alter the risk of SCD. Thus, watchful waiting may be an appropriate course as well, particularly for a patient with an anomalous right coronary arising from the left sinus. 4.4.7.3. Anomalous Coronary Artery Arising From the PA Recommendations for Anomalous Coronary Artery Arising From the PA Referenced studies that support recommendations are summarized in Online Data Supplement 51.


Anomalous Coronary Artery Arising From the PA Recommendations for Anomalous Coronary Artery Arising From the PA Referenced studies that support recommendations are summarized in Online Data Supplement 51. COR LOE Recommendations Therapeutic I B-NR Surgery is recommended for anomalous left coronary artery from the PA.S4.4.7.3-1 S4.4.7.3-7 I C-EO In a symptomatic adult with anomalous right coronary artery from the PA with symptoms attributed to the anomalous coronary, surgery is recommended. IIa C-EO Surgery for anomalous right coronary artery from the PA is reasonable in an asymptomatic adult with ventricular dysfunction or with myocardial ischemia attributed to anomalous right coronary artery from the PA. CLINICAL STATEMENTS AND GUIDELINES Recommendation-Specific Supportive Text 1. Surgery can include reimplantation of the left coronary artery directly into the aorta with or without an interposition graft. Ligation or clo- sure of the left coronary artery at the level of the PA with coronary artery bypass grafting can also be performed, usually using the left internal mammary artery anastomosed to the left anterior descending. 2. Surgery can include reimplantation of the right coronary artery directly into the aorta with or without an interposition graft. Ligation or clo- sure of the right coronary artery at the level of the PA with coronary artery bypass grafting can also be performed, usually using the right internal mammary artery anastomosed to the right coro- nary or posterior descending coronary artery. 3. Surgery to alleviate ischemia or ventricular dys- function is reasonable if the anomalous coronary artery is thought to be the cause. Surgery can in- clude reimplantation of the right coronary artery directly into the aorta with or without an interpo- sition graft.


Surgery can in- clude reimplantation of the right coronary artery directly into the aorta with or without an interpo- sition graft. Ligation or closure of the right cor- onary artery at the level of the PA with coronary artery bypass grafting can also be performed, usually using the right internal mammary artery anastomosed to the right coronary or posterior descending coronary artery. 4.4.8. Coronary Artery Fistula Coronary artery fistula is an abnormal communication between a coronary artery and another cardiovascular Table 35. High-Impact Research Questions in ACHD General Pathophysiology What are the mechanisms of heart failure that can be prevented, reversed, or treated? Why does the systemic right ventricle fail? Will all patients with Fontan physiology develop clinically important cirrhosis, and how can we prevent this? Who is at risk of aortic rupture and dilation? Are patients with manipulated coronary arteries (eg, ASO, Ross repair) at risk of premature coronary artery disease? What is the impact of radiation exposure on long-term health? Can we predict who will develop pulmonary hypertension/pulmonary vascular disease? Medical and surgical treatment How can we modify current CHD surgical procedures to prevent or reduce later development of heart failure and/or arrhythmias? Which patients with ACHD can use direct oral anticoagulants instead of warfarin? What is the best algorithm for contraception choices? Beyond those with severe PAH, which patients will benefit from PAH therapies? Do patients with ACHD with systemic right ventricles and HF benefit from standard therapies (beta blockers, ACE inhibitors/ARBs, aldosterone antagonist)? Which one(s)? What medical therapies benefit patients with failing Fontan physiology? Do asymptomatic patients with ACHD with PAH benefit from PAH-specific therapy? Who will benefit from ventricular assist devices? What should be the threshold(s) for aortic aneurysm surgery? What pacing and resynchronization strategies are of most benefit, and when should they be used?


What pacing and resynchronization strategies are of most benefit, and when should they be used? Outcomes/risk assessment What criteria should determine transplantation eligibility? Which patients benefit from primary prevention ICDs? How can we risk stratify for SCD in patients with systemic right ventricles? What operative risk score predicts outcomes in ACHD reoperations? What HF risk score predicts outcomes in patients with ACHD? Is there a level of exercise where risk exceeds benefit? What is the rate and/or risk of endocarditis? Assessment Who is at high risk of neurodevelopmental abnormalities and would benefit from neuropsychiatric evaluation and treatment? Who should be screened for anxiety and depression, what treatment is most effective, and are there differences compared with non- patients with ACHD? What is the standard protocol for assessing right ventricular size and function by CMR imaging? Which biomarkers are predictive of mortality and morbidity? (Continued) CLINICAL STATEMENTS AND GUIDELINES structure, which may include a cardiac chamber, coro- nary sinus, superior vena cava, or PA. The incidence of coronary artery fistula is 0.1% to 0.2% in all patients undergoing coronary angiography.S4.4.8-1,S4.4.8-2 Fistulous communications may be congenital or acquired. Spe- cific management strategies, which can include surgical repair or catheter embolization, have been controver- sial. In a series of 46 patients treated with surgery, pre- dominant preoperative symptoms included angina and Importantly, postoperative myocardial infarc- tion occurred in 11% because of low flow in the dilated coronary artery proximal to fistula closure. Late survival was also significantly reduced compared with an age- matched population. The presence of coronary artery fistula(s) requires review by a knowledgeable team that may include congenital or noncongenital cardiologists and surgeons to determine the role of medical therapy and/or percutaneous or surgical closure.S4.4.8-3 Disease-specific Coarctation of the aorta Which measure of hypertension resting, exercise, or ambulatory best predicts outcomes?


The presence of coronary artery fistula(s) requires review by a knowledgeable team that may include congenital or noncongenital cardiologists and surgeons to determine the role of medical therapy and/or percutaneous or surgical closure.S4.4.8-3 Disease-specific Coarctation of the aorta Which measure of hypertension resting, exercise, or ambulatory best predicts outcomes? Is there an optimal antihypertensive regimen? What should blood pressure goals be? How often should patients be screened for thoracic aneurysm? Should exercise-induced hypertension be treated? What criteria warrant reintervention in recoarctation? Is long-term outcome better with medical therapy or catheter intervention for less than severe recoarctation? Should patients be screened for intracranial aneurysm, and if so, how often? Ebstein anomaly What is the indication for surgery in the asymptomatic patient? Who should have a Glenn shunt at the time of tricuspid valve surgery? Should surgeons attempt tricuspid valve repair or routinely perform replacement in all patients? TOF What is the optimal timing for pulmonary valve replacement in asymptomatic patients with TOF? Do pulmonary valve replacement and ventricular tachycardia ablation decrease the risk of SCD? Who needs a primary prevention ICD, and does this strategy reduce mortality? Is there a role for PAH therapies in TOF? Why does left ventricular dysfunction develop? TGA/systemic right ventricle Who benefits from ACE inhibitors/ARBs/beta blockers/spironolactone? Who needs a primary prevention ICD, and does this strategy prevent mortality? What imaging findings predict mortality/morbidity? In CCTGA with VSD/PS, does the double switch have better long-term outcomes than VSD closure and left ventricle to-PA conduit? When should tricuspid valve replacement be performed? What is the role of cardiac resynchronization therapy in patients with systemic right ventricle? ASO What are the long-term outcomes after ASO? How should the possibility of asymptomatic coronary disease (ostial, compression) and ischemia be assessed?


How should the possibility of asymptomatic coronary disease (ostial, compression) and ischemia be assessed? Single ventricle/ Fontan Is warfarin or aspirin beneficial in patients with a Fontan? Are PAH therapies beneficial? Is exercise capacity predictive of mortality? What liver screening is appropriate and at what intervals? How is protein-losing enteropathy best medically treated? Why do some patients fail with preserved ejection fraction, whereas other have decreased ejection fraction? What are the long-term outcomes of hypoplastic left heart syndrome? What is ideal timing for heart transplantation in single ventricle Fontan patients, and should liver issues prompt earlier transplantation than might be felt necessary from a cardiac perspective? Which has better long-term outcomes, the Fontan operation or bidirectional Glenn alone? Coronary anomalies Does surgical intervention in anomalous aortic origin of coronary arteries improve survival? ACE indicates angiotensin-converting enzyme; ACHD, adult congenital heart disease; ARB, angiotensin-receptor blocker; ASO, arterial switch operation; CCTGA, congenitally corrected transposition of the great arteries; CHD, congenital heart disease; CMR, cardiac magnetic resonance; ICD, implantable cardioverter- defibrillator; HF, heart failure; PA, pulmonary artery; PAH, pulmonary artery hypertension; PS, pulmonary stenosis; SCD, sudden cardiac death; TGA, transposition of the great arteries; TOF, tetralogy of Fallot; and VSD, ventricular septal defect. Table 35. Continued CLINICAL STATEMENTS AND GUIDELINES 5. EVIDENCE GAPS AND FUTURE DIRECTIONS There are multiple challenges to developing evi- denced-based care for patients with ACHD. The heterogeneity of conditions leads to small numbers of specific ACHD populations from which to derive guidelines. Additionally, lack of infrastructure to track prevalence, fragmented care systems, loss to follow-up, and changes in treatment strategies over time all contribute to the challenges of developing Comprehensive multicenter and pop- ulation registries and databases are needed to have adequate numbers of patients to address clinical questions.


Additionally, lack of infrastructure to track prevalence, fragmented care systems, loss to follow-up, and changes in treatment strategies over time all contribute to the challenges of developing Comprehensive multicenter and pop- ulation registries and databases are needed to have adequate numbers of patients to address clinical questions. Novel study methodologies are needed to ascertain effectiveness of diagnostic and therapeutic options when each disease is sufficiently rare and e- vents occur over sufficiently long periods that RCTs are impractical. Although there are data that patients with complex CHD have improved survival when cared for at an ACHD center, how can networks of care be developed that ensure patients get the ex- pert care needed when there are inadequate number of ACHD cardiologists and ACHD centers? How do we ensure that patients are not lost to care as they transition from pediatric to adult cardiology? How do we ensure that patients with ACHD who would ben- efit from heart transplantation receive accurate listing priority? See Table 35 for a collection of high-impact research questions in ACHD.


See Table 35 for a collection of high-impact research questions in ACHD. ACC/AHA TASK FORCE MEMBERS Glenn N. Levine, MD, FACC, FAHA, Chair; Patrick T. O Gara, MD, MACC, FAHA, Chair-Elect; Jonathan L. Halperin, MD, FACC, FAHA, Immediate Past Chair; Nancy M. Albert, PhD, RN, FAHA*; Sana M. Al-Khatib, MD, MHS, FACC, FAHA; Joshua A. Beckman, MD, MS, FAHA; Kim K. Birtcher, PharmD, MS, AACC; Biykem Bozkurt, MD, PhD, FACC, FAHA*; Ralph G. Brindis, MD, MPH, MACC*; Joaquin E. Cigarroa, MD, FACC; Lesley H. Curtis, PhD, FAHA*; Anita Deswal, MD, MPH, FACC, FAHA; Lee A. Fleisher, MD, FACC, FAHA; Federico Gentile, MD, FACC; Samuel S. Gidding, MD, FAHA*; Zachary D. Goldberger, MD, MS, FACC, FAHA; Mark A. Hlatky, MD, FACC; John Ikonomidis, MD, PhD, FAHA; Jos Joglar, MD, FACC, FAHA; Richard J. Kovacs, MD, FACC, FAHA*; Laura Mauri, MD, MSc, FAHA; E. Magnus Ohman, MD, FACC*; Mariann R. Piano, RN, PhD, FAHA, FAAN; Susan J. Pressler, PhD, RN, FAHA*; Barbara Riegel, PhD, RN, FAHA; Frank W. Sellke, MD, FACC, FAHA*; Win-Kuang Shen, MD, FACC, FAHA*; Duminda N. Wijeysundera, MD, PhD PRESIDENTS AND STAFF American College of Cardiology C. Michael Valentine, MD, FACC, President Cathleen C. Gates, Interim Chief Executive Officer and Chief Operating Officer William J. Oetgen, MD, MBA, FACC, Executive Vice Presi- dent, Science, Education, Quality, and Publishing MaryAnne Elma, MPH, Senior Director, Science, Educa- tion, Quality, and Publishing Amelia Scholtz, PhD, Publications Manager, Science, Education, Quality, and Publishing American College of Cardiology/ American Heart Association Katherine A. Sheehan, PhD, Director, Guideline Strategy and Operations Abdul R. Abdullah, MD, Senior Manager, Guideline Science American Heart Association Ivor Benjamin, MD, FAHA, President Nancy Brown, Chief Executive Officer Rose Marie Robertson, MD, FAHA, Chief Science and Medical Officer Gayle R. Whitman, PhD, RN, FAHA, FAAN, Senior Vice President, Office of Science Operations Prashant Nedungadi, PhD, Science and Medicine Advi- sor, Office of Science Operations Jody Hundley, Production and Operations Manager, Sci- entific Publications, Office of Science Operations ARTICLE INFORMATION This document was approved by the American College of Cardiology Clinical Policy Approval Committee in May 2018, the American Heart Association Sci- ence Advisory and Coordinating Committee in June 2018, and the American Heart Association Executive Committee in July 2018.


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