Perceptions of exercise behavior and well-being in anomalous aortic origin of coronary arteries.

Anomalous aortic origin of a coronary artery (AAOCA) is associated with sudden death in the young. Risk stratification and management decision-making remain challenging. Data addressing post-diagnosis perceptions of exercise behavior and safety are lacking. We aimed to determine how AAOCA affects exercise behaviors, safety perceptions, and emotional well-being of patients/parents. Qualitative and quantitative analysis of AAOCA patient-/parent-specific survey was conducted to examine exercise frequency/restrictions, perceived safety of competitive/recreational exercise, and psychosocial well-being. Subgroups stratified by AAOCA subtype, surgical intervention, and physician-driven restrictions were compared using chi-squared and Fisher's exact tests. Cohen's kappa determined agreement in parent/child responses. AAOCA subtypes included 13 (24%) left AAOCA, 36 (67%) right AAOCA, and 5 (9%) other/unknown. Of 54 parents and 41 paired child responses, 22% of patients were physician-restricted from exercise. Parents imposed restrictions on competitive/recreational exercise 34%/26% of the time, respectively. Children without physician restrictions still self-restricted exercise 35% of the time. Parents reported feeling their child was unsafe exercising 61% competitively and 33% recreationally. Twenty-two percent of children reported feeling unsafe exercising, with good agreement to parental perceptions of competitive exercise safety (kappa = 0.779, p < 0.001). One-third of parents and children reported feeling sad, angry, or lonely, and about half reported feeling different. Importantly, 47% of children desired to exercise more. No difference was seen across restriction status, AAOCA subtype, or surgical management strategy. CONCLUSION: There are different perceptions of exercise behavior and safety following AAOCA evaluation, regardless of risk category or management strategy, impacting their well-being. These unmet needs should be at the forefront of care. WHAT IS KNOWN: • AAOCA is one of the leading causes of sudden cardiac death in the young. • Exercise restriction varies according to AAOCA subtype and its perceived risk of inducing myocardial ischemia. WHAT IS NEW: • There are different perceptions of exercise behavior and safety in patients and parents following a diagnosis of AAOCA, impacting their well-being. • Risk category or management strategy has no effect in patients' and parents' perception of exercise safety. • These unmet needs in this population should be at the forefront of care.

Transection and reimplantation of anomalous right coronary artery from single left coronary artery in a collegiate athlete.

Coronary artery anomalies may induce myocardial ischaemia and sudden cardiac death when associated with areas of compression or stenosis. We present a case of transection and reimplantation of an anomalous interarterial right coronary artery arising from a single left main coronary artery. The 18-year-old collegiate athlete had exertional chest pain and haemodynamically significant compromise to coronary blood flow.

Evolution of surgical repair of intraseptal anomalous left coronary artery with myocardial ischaemia.

BACKGROUND: Anomalous aortic origin of the left coronary artery with intraseptal course is a rare coronary anomaly associated with an increased risk of myocardial ischaemia. The role and techniques for surgical intervention are evolving, with numerous novel surgical techniques for this challenging anatomy reported in the last 5 years. We report our single-centre experience with surgical repair of intraseptal anomalous left coronary artery in the paediatric population, including clinical presentation, evaluation, and short- to mid-term outcomes. METHODS: All patients with coronary anomalies presenting to our institution undergo standardised clinical evaluation. Five patients aged 4 to 17 years underwent surgical intervention for intraseptal anomalous aortic origin of the left coronary artery between 2012 and 2022. Surgical techniques included coronary artery bypass grafting (n = 1), direct reimplantation with limited supra-arterial myotomy via right ventriculotomy (n = 1), and transconal supra-arterial myotomy with right ventricular outflow tract patch reconstruction (n = 3). RESULTS: All patients had evidence of haemodynamically significant coronary compression, and three had evidence of inducible myocardial ischaemia pre-operatively. There were no deaths or major complications. Median follow-up was 6.1 months (range 3.1-33.4 months). Patients who underwent supra-arterial myotomy (with or without reimplantation) had improved coronary flow and perfusion based on stress imaging and catheterisation data. CONCLUSIONS: Surgical approaches to intraseptal anomalous left coronary artery with evidence of myocardial ischaemia continue to evolve, with new techniques demonstrating promising improvement in coronary perfusion. Further studies are warranted to determine long-term outcomes and refine indications for repair.

Wall motion assessment by feature tracking in pediatric patients with coronary anomalies undergoing dobutamine stress CMR.

Background: Left ventricular (LV) wall motion assessment is an important adjunct in addition to perfusion defects in assessing ischemic changes. This study aims to investigate the feasibility and utility of performing feature tracking (FT) in pediatric patients with coronary anomalies undergoing dobutamine stress CMR to assess wall motion abnormalities (WMA) and perfusion defects. Method: This is a retrospective study where 10 patients with an inducible first-pass perfusion (FPP) defect and 10 without were selected. Global LV circumferential strain/strain rate (GCS/GCSR) was measured at rest and at peak stress (systole and diastole) using a commercially available feature tracking software. Peak GCS and GCSR were compared to indexed wall motion score (WMSI) between groups with and without FPP defect and in subjects with and without WMA. Results: The median age of patients was 13.5 years (Q1, 11 years; Q3, 15 years). Five subjects had qualitatively WMA at peak stress. A moderate correlation of GCS with WMSI at peak stress (0.48, p = 0.026) and a significant difference between GCS at rest and stress in patients with no inducible WMA (p = 0.007) were seen. No significant difference was noted in GCS between rest and stress in patients with WMA (p = 0.13). There was a larger absolute GCS/GCSR at peak stress in subjects with no inducible FPP defect or WMA. Conclusion: Smaller absolute GCS and a lack of significant change in GCS at peak stress in those with inducible WMA or perfusion defect are suggestive of compromised LV deformation in subjects with inducible WMA. Given these findings, GCS derived from CMR-FT may be used to objectively assess WMA in pediatric patients undergoing stress CMR.

High-Risk Anomalous Aortic Origin of the Left Coronary Artery: Consecutive Admissions Presenting With Sudden Cardiac Arrest.

Anomalous aortic origin of the left coronary artery (AAOLCA) confers high risk for sudden cardiac arrest (SCA). This series aims to describe consecutive admissions with interarterial AAOLCA presenting with SCA and distinct clinical trajectories. An eight-year-old boy collapsed at school and received 10-min of cardiopulmonary resuscitation (CPR) and defibrillation prior to return of spontaneous circulation. He had no end-organ dysfunction and underwent uneventful coronary unroofing. In contrast, a 14-year-old boy presented with collapse while jogging. He received 40-min of CPR prior to extracorporeal membranous oxygenation cannulation with multisystem dysfunction and persistent severely depressed left ventricular function. He is now rehabilitating following uneventful orthotropic heart transplantation. These cases illustrate the diverse outcomes of AAOLCA with SCA following exertional syncope.

Myocardial bridges in a pediatric population: Outcomes following a standardized approach.

OBJECTIVE: To describe clinical, functional, surgical, and outcomes data in pediatric patients with a myocardial bridge (MB) evaluated and managed following a standardized approach. METHODS: Prospective observational study included patients evaluated in the Coronary Artery Anomalies Program. Anatomy was determined by computed tomography angiography, myocardial perfusion by stress perfusion imaging, and coronary hemodynamic assessment by cardiac catheterization. RESULTS: In total, 39 of 42 patients with a complete evaluation for MB were included (December 2012 to June 2022) at a median age of 14.1 years (interquartile range, 12.2-16.4). Sudden cardiac arrest occurred in 3 of 39 (8%), exertional symptoms in 14 (36%), and no/nonspecific symptoms in 7 (18%) patients. Exercise stress test was abnormal in 3 of 34 (9%), stress perfusion imaging in 8 of 34 (24%), and resting instantaneous wave-free ratio ≤0.89 or diastolic dobutamine fractional flow reserve ≤0.80 in 11 of 21 (52%) patients. As a result, 15 of 39 (38%) patients were determined to have hemodynamically significant MB, 1 of 15 patients started beta-blocker, and 14 of 15 were referred for surgery. Myotomy (n = 11) and coronary bypass (n = 1) were performed successfully, resulting in improved symptoms and stress testing results. One patient required pericardiocentesis postoperatively, and all were discharged without other complications. At median follow-up time of 2.9 (1.8-5.8) years, all (except 2 pending surgery) were doing well without exercise restriction. CONCLUSIONS: Pediatric patients with MB can present with myocardial ischemia and sudden cardiac arrest. Provocative stress test and intracoronary hemodynamic tests helped risk-stratify symptomatic patients with MB and concern for ischemia. Surgical repair was safe and effective in mitigating exertional symptoms and stress test results, allowing patients to return to exercise without restriction.

Invasive Coronary Assessment in a 9-Year-Old With Anomalous Aortic Origin of the Left Coronary Artery.

A previously healthy nine-year-old boy with anomalous aortic origin of the left coronary artery (AAOLCA) with high-risk anatomy demonstrated negative stress on magnetic resonance imaging. Invasive cardiac catheterization for intracoronary flow measurement was performed and demonstrated compromised coronary flow during pharmacologic stress and significant stenosis on angiography. The patient underwent surgical intervention with normalization of coronary flow upon postoperative evaluation. Invasive intracoronary flow determination with angiography under provocative stress is emerging as a critical data point for risk stratification and management decision-making in high-risk AAOLCA patients with negative noninvasive perfusion studies.

Is Exercise Stress Testing Useful for Risk Stratification in Anomalous Aortic Origin of a Coronary Artery?

Data on maximal exercise-stress-testing (m-EST) in anomalous-aortic-origin-of-coronary-arteries (AAOCA) is limited and correlation with stress perfusion imaging has not been demonstrated. AAOCA patients ≤20 years were prospectively enrolled from 6/2014-01/2020. A m-EST was defined as heart rate >85%ile on ECG-EST and respiratory-exchange-ratio ≥1.05 on cardiopulmonary-exercise-testing (CPET). Abnormal m-EST included significant ST-changes or high-grade arrhythmia, V̇O2max and/or O2 pulse <85% predicted, or abnormal O2 pulse curve. A (+) dobutamine-stress cardiac-magnetic-resonance-imaging (+DS-CMR) had findings of inducible-ischemia. Outcomes: (1) Differences in m-EST based on AAOCA-type; (2) Assuming DS-CMR as gold-standard for detection of inducible ischemia, determine agreement between m-EST and DS-CMR. A total of 155 AAOCA (right, AAORCA = 126; left, AAOLCA = 29) patients with a median (IQR) age of 13 (11-15) years were included; 63% were males and a m-EST was completed in 138 (89%). AAORCA and AAOLCA had similar demographic and m-EST characteristics, although AAOLCA had more frequently evidence of inducible ischemia on m-EST (P = 0.006) and DS-CMR (P = 0.007). Abnormal O2 pulse was significantly associated with +DS-CMR (OR 5.3, 95% CI 1.6-18,P = 0.005). Sensitivity was increased with addition of CPET to ECG-EST (to 58% from 19%). There was no agreement between m-EST and DS-CMR for detection of inducible ischemia. A m-EST has very low sensitivity for detection of inducible ischemia in AAOCA, and sensitivity is increased with addition of CPET. Stress perfusion abnormalities on DS-CMR were notconcordant with m-EST findings and adjunctive testing should be considered for clinical decision making in AAOCA.

Anomalous Aortic Origin of Coronary Arteries in Children: Postoperative High-risk Anatomic Features.

BACKGROUND: This study aimed to assess postoperative presumed high-risk anatomic features (HRAFs) by using computed tomographic angiography (CTA) in patients with anomalous aortic origin of a coronary artery (AAOCA) after surgical unroofing vs transection and reimplantation (TAR) if unroofing was thought to provide unsatisfactory results. METHODS: The study included 62 children with postoperative CTA performed at a median of 3 months (interquartile range, 3-4 months) after unroofing (n = 45) and TAR (n = 17). HRAFs included slitlike ostium, intramural course, acute angle takeoff (<45o), interarterial course, proximal stenosis >50%, or course through a thickened intercoronary pillar. RESULTS: Median age at surgery was 13.8 years (interquartile range, 10.5-15.8 years). None of the patients had a slitlike ostium or an intramural course on postoperative CTA. Acute takeoff was seen in 100% after unroofing and in 2 of 17 (12%) after TAR (P < .001). After unroofing, the interarterial course improved to 35 of 45 (78%) from 43 of 45 (96%) (P = .003), and a thickened intercoronary pillar improved to 10 of 45 (22%) from 22 of 45 (49%) (P = .0001), compared with none seen after TAR. Preoperative intramural length <5 mm was associated with a postoperative thickened intercoronary pillar in right AAOCA after unroofing (P = .0004). Severe coronary stenosis occurred in 2 of 17 (12%) after TAR, and both patients needed urgent revision procedures. All patients except 2 (97%) returned to exercise activities at a median follow-up of 4.9 years (range, 0.6-9.2 years). CONCLUSIONS: The slitlike ostium and intramural course resolved in all patients. Residual acute angle takeoff, an interarterial course, and mild coronary narrowing related to a thickened intercoronary pillar were common after unroofing. TAR allows resolution of all HRAFs, although severe narrowing requiring surgical revision happened only in TAR. Long-term studies are needed to understand the clinical significance of these residual presumed HRAFs.

Ischemia in Anomalous Aortic Origin of a Right Coronary Artery: Large Pediatric Cohort Medium-Term Outcomes.

BACKGROUND: Anomalous aortic origin of a right coronary artery may cause myocardial ischemia and sudden death in the young. Data on myocardial ischemia or longitudinal outcomes are sparse in pediatric anomalous aortic origin of a right coronary artery population. METHODS: Patients <21 years with anomalous aortic origin of a right coronary artery were prospectively enrolled. Computerized tomography angiography defined morphology. Exercise stress test and stress perfusion imaging (sPI) were performed if >7 years or younger with concern for ischemia. High-risk features included intramural length, slit-like/hypoplastic ostium, exertional symptoms, or evidence of ischemia. RESULTS: A total of 220 patients (60% males) were enrolled December 2012 to April 2020 at a median age 11.4 years (interquartile range, 6.1-14.5), including 168 (76%) with no/nonexertional symptoms (group 1) and 52 (24%) with exertional chest pain/syncope (group 2). Computerized tomography angiography was available in 189/220 (86%), exercise stress test in 164/220 (75%), and sPI in 169/220 (77%). Exercise stress test was positive in 2/164 (1.2%) patients in group 1, both had positive sPI. Inducible ischemia (sPI) was detected in 11/120 (9%) in group 1 and 9/49 (18%) in group 2 (P=0.09). Intramural length was similar in patients with/without ischemia (5 [interquartile range, 4-7] versus 5 [interquartile range, 4-7] mm; P=0.65). Surgery was recommended in 56/220 (26%) patients with high-risk features. In 52 surgical patients (38 unroofing, 14 reimplantation), all subjects were alive and have returned to exercise at last median follow-up of 4.6 (interquartile range, 2.3-6.5) years. CONCLUSIONS: Anomalous aortic origin of a right coronary artery patients can present with inducible ischemia on sPI despite symptoms or intramural length. Exercise stress test is a poor predictor of ischemia and caution should be given to determine low-risk based solely on this assessment. All patients are alive at medium-term follow-up.

Clinical Presentation and Medium-Term Outcomes of Children With Anomalous Aortic Origin of the Left Coronary Artery: High-Risk Features Beyond Interarterial Course.

BACKGROUND: Anomalous aortic origin of the left coronary artery (AAOLCA) confers a rare, but significant, risk of sudden cardiac death in children. Surgery is recommended for interarterial AAOLCA, and other subtypes considered benign. We aimed to determine the clinical characteristics and outcomes of 3 AAOLCA subtypes. METHODS: All patients with AAOLCA <21 years old were prospectively enrolled (December 2012-November 2020), including group 1: AAOLCA from the right aortic sinus with interarterial course, group 2: AAOLCA from the right aortic sinus with intraseptal course, and group 3: AAOLCA with a juxtacommissural origin between the left and noncoronary aortic sinus. Anatomic details were assessed using computed tomography angiography. Provocative stress testing (exercise stress testing and stress perfusion imaging) was performed in patients >8 years old or younger if concerning symptoms. Surgery was recommended for group 1 and in select cases in group 2 and group 3. RESULTS: We enrolled 56 patients (64% males) with AAOLCA (group 1, 27; group 2, 20; group 3, 9) at median age of 12 years (interquartile range, 6-15). Intramural course was common in group 1 (93%) compared with group 3 (56%) and group 2 (10%). Seven (13%) presented with aborted sudden cardiac death (group 1, 6/27; group 3, 1/9); 1 (group 3) with cardiogenic shock. Fourteen/42 (33%) had inducible ischemia on provocative testing (group 1, 32%; group 2, 38%; group 3, 29%). Surgery was recommended in 31/56 (56%) patients (group 1, 93%; group 2, 10%; and group 3, 44%). Surgery was performed in 25 patients at a median age 12 (interquartile range, 7-15) years; all have been asymptomatic and free from exercise restrictions at median follow-up of 4 (interquartile range, 1.4-6.3) years. CONCLUSIONS: Inducible ischemia was noted in all 3 AAOLCA subtypes while most aborted sudden cardiac deaths occurred in interarterial AAOLCA (group 1). Aborted sudden cardiac death and cardiogenic shock may occur in AAOLCA with left/nonjuxtacommissural origin and intramural course, thus also deemed high-risk. A systematic approach is essential to adequately risk stratify this population.

Pattern, behavior, and clinical implications of electrocardiographic changes in patients undergoing repair of anomalous aortic origin of coronary arteries.

OBJECTIVES: Surgical repair in anomalous aortic origin of a coronary artery aims at mitigating the risk of sudden cardiac death in a subset of patients. The pattern and behavior of electrocardiogram changes in a large cohort of these patients are lacking. We aim to describe postoperative electrocardiogram changes in this population and its clinical implications on follow-up. METHODS: All patients aged less than 21 years who underwent surgical repair for anomalous aortic origin of a coronary artery between December 2012 and June 2020 at our institution were considered for inclusion. Electrocardiograms were reviewed at 5 defined time intervals, from preoperative to 90-day follow-up, with attention to significant findings of ST-segment changes, abnormal T waves, and pathologic Q waves. The electrocardiogram changes were analyzed for correlation with surgical reintervention and medium-term outcomes. RESULTS: Sixty-two patients met inclusion criteria (median age 13.7 years, 61% male). ST-segment changes in the initial postoperative period were seen in 52 patients (84%), all resolving over time. Abnormal T waves were seen in 19 patients (31%), occurred commonly at the predischarge period, and mostly resolved over time. Pathologic Q waves were observed in only 1 patient and associated with reintervention due to coronary artery stenosis. There was no association between postoperative electrocardiogram changes and inducible myocardial ischemia, ventricular dysfunction, or restriction from exercise at follow-up. CONCLUSIONS: ST-segment changes and T-wave abnormalities are commonly seen in the postoperative period after anomalous aortic origin of a coronary artery repair, tend to resolve over time, and are not associated with adverse medium-term outcomes. Pathologic Q waves were associated with the need for early coronary reintervention.

Beta-Blockade in Intraseptal Anomalous Coronary Artery With Reversible Myocardial Ischemia.

Anomalous aortic origin of a left coronary artery (L-AAOCA) with an intraseptal course is a rare anomaly and can be associated with myocardial ischemia and sudden cardiac death. No surgical or medical intervention is known to improve patient outcomes. A 7-year-old boy with intraseptal L-AAOCA presented with nonexertional chest pain, syncope, and had reversible myocardial ischemia on provocative testing. The patient was started on ß-blockade, following which his symptoms improved and resolved over a period of six years. A follow-up dobutamine stress magnetic resonance imaging no longer showed reversible ischemia, and cardiac catheterization with fractional flow reserve did not show coronary flow compromise.

Dobutamine stress cardiac MRI is safe and feasible in pediatric patients with anomalous aortic origin of a coronary artery (AAOCA).

BACKGROUND: Risk stratification in anomalous aortic origin of a coronary artery (AAOCA) is challenged by the lack of a reliable method to detect myocardial ischemia. We prospectively studied the safety and feasibility of Dobutamine stress-cardiac magnetic resonance (DSCMR), a test with excellent performance in adults, in pediatric patients with AAOCA. METHODS: Consecutive DSCMR from 06/2014-12/2019 in patients≤20 years old with AAOCA were included. Hemodynamic response and major/minor events were recorded. Image quality and spatial/temporal resolution were evaluated. Rest and stress first-pass perfusion and wall motion abnormalities (WMA) were assessed. Inter-observer agreement was assessed using kappa coefficient. RESULTS: A total of 224 DSCMR were performed in 182 patients with AAOCA at a median age of 14 years (IQR 12, 16) and median weight of 58.0 kg (IQR 43.3, 73.0). Examinations were completed in 221/224 (98.9%), all studies were diagnostic. Heart rate and blood pressure increased significantly from baseline (p < 0.001). No patient had major events and 28 (12.5%) had minor events. Inducible hypoperfusion was noted in 31/221 (14%), associated with WMA in 13/31 (42%). Inter-observer agreement for inducible hypoperfusion was very good (Κ = 0.87). Asymptomatic patients with inducible hypoperfusion are considered high-risk and those with a negative test are of standard risk. CONCLUSIONS: DSCMR is feasible in pediatric patients with AAOCA to assess for inducible hypoperfusion and WMA. It can be performed safely with low incidence of major/minor events. Thus, DSCMR is potentially a valuable test for detection of myocardial ischemia and helpful in the management of this patient population.

Outcomes in anomalous aortic origin of a coronary artery after surgical reimplantation.

OBJECTIVE: Anomalous aortic origin of a coronary artery (AAOCA) can be associated with myocardial ischemia and sudden cardiac arrest. We compared outcomes data of patients who underwent transection and reimplantation (TAR) and patients who underwent an unroofing. METHODS: Patients who presented to the Coronary Artery Anomalies Program were evaluated and managed following a standardized approach. Anatomy was determined using computed tomography angiography, myocardial perfusion using advanced stress imaging, and surgical intervention according to anatomic features. RESULTS: Sixty-one patients underwent surgical repair of AAOCA between 2012 and 2019: 16 (26%) patients underwent TAR of the anomalous coronary without an aortic button and 45 (74%) patients underwent coronary unroofing. Compared with patients who underwent an unroofing, patients who underwent TAR had similar intramural length (5 mm with interquartile range of 4-7.7 vs 6 mm with interquartile range of 5-7; P = .6). One patient with an anomalous right coronary underwent coronary artery bypass grafting after TAR because of persistent postoperative ischemic changes. One patient with unroofing of an anomalous left coronary artery presented with recurrent aborted sudden cardiac death and underwent subsequent TAR, without further events. At last follow-up, 15 of 16 patients (94%) who underwent TAR and 42 of 45 (93%) patients who underwent an unroofing were released to unrestricted exercise activities. CONCLUSIONS: Coronary artery TAR is a useful surgical alternative for AAOCA when there is a course below the commissure, when unroofing does not relocate the ostium to the appropriate sinus, or when unroofing results in compression by the intercoronary pillar.

Myocardial Ischemia in Children With Anomalous Aortic Origin of a Coronary Artery With Intraseptal Course.

BACKGROUND: Intraseptal anomalous aortic origin of a coronary artery is considered a benign condition. However, there have been case reports of patients with myocardial ischemia, arrhythmia, and sudden cardiac death. The purpose of this study was to determine the clinical presentation, myocardial perfusion on provocative stress testing, and management of children with anomalous aortic origin of a coronary artery with an intraseptal course in a prospective cohort. METHODS: Patients with anomalous aortic origin of a coronary artery and intraseptal course were prospectively enrolled from December 2012 to May 2019, evaluated, and managed following a standardized algorithm. Myocardial perfusion was assessed using stress imaging. Fractional flow reserve was performed in patients with myocardial hypoperfusion on noninvasive testing. Exercise restriction, ß-blockers, and surgical intervention were discussed with the families. RESULTS: Eighteen patients (female 6, 33.3%), who presented with no symptoms (10, 55.6%), nonexertional (4, 22.2%), and exertional symptoms (4, 22.2%), were enrolled at a median age of 12.4 years (0.3-15.9). Perfusion imaging was performed in 14/18 (77.8%) and was abnormal in 7/14 (50%); fractional flow reserve was positive in 5/8 (62.5%). All 4 patients with exertional symptoms and 3/10 (30%) with no or nonexertional symptoms had myocardial hypoperfusion. Coronary artery bypass grafting was performed in a 4-year-old patient; ß-blocker and exercise restriction were recommended in 4 patients not suitable for surgery. One patient had nonexertional chest pain and 17 were symptom-free at median follow-up of 2.5 years (0.2-7.1). CONCLUSIONS: Up to 50% of patients with intraseptal anomalous aortic origin of a coronary artery had inducible myocardial hypoperfusion during noninvasive provocative testing. Long-term follow-up is necessary to understand the natural history of this rare anomaly.