Labyrinthine cor triatriatum sinister in fetal hypoplastic left heart syndrome is associated with poor outcomes.

OBJECTIVES: A subset of hypoplastic-left-heart-syndrome (HLHS) fetuses have a complex cor-triatriatum sinister that we named "labyrinthine-cor (L-cor)". We sought to determine the prevalence of L-cor in HLHS fetuses and hypothesized that it is associated with increased mortality. METHODS: This single-center retrospective cohort study included all HLHS fetuses from January 2010-December 2020. Fetuses with other hypoplastic-left-heart variants, inadequate images, lack of follow-up and fetal atrial-septal interventions were excluded. RAS was defined as the ratio of pulmonary-vein forward-to-reverse velocity-time-integral (VTI) ≤ 5 and severe-RAS defined as VTI-ratio <3. Kaplan-Meier survival-analysis was performed for the primary outcome of transplant-free survival for 62 weeks after gestational-age of 30 weeks (∼1 year). RESULTS: Of the 156 consecutive fetuses with HLHS, 11 (7.7%) had L-cor and 8/11 (72.7%) of these had RAS. When compared to HLHS-RAS without L-cor, fetuses with HLHS-RAS and L-cor were less likely to survive to 28 days (87% vs. 62.5%, p = 0.017) and to 1 year (69.6% vs. 25%, p = 0.029). When comparing by survival analysis, fetuses with severe-RAS with L-cor had lower survival compared severe-RAS without L-cor (p = 0.020). CONCLUSION: L-cor in fetal HLHS is associated with increased mortality. Recognition of this finding is important for prognostication and atrial-septal-intervention planning.

Creating a flow restrictor in ductal stenting: a novel technique.

Ductal stenting has transformed the care of neonates with ductal-dependent critical CHD, especially in low-income countries. In small infants, a 3.5- or 4-mm stent may lead to too much pulmonary blood flow resulting in pulmonary oedema. We herein presented a novel technique to restrict ductal stent flow in a premature neonate with pulmonary atresia and intact ventricular septum following radiofrequency perforation of the pulmonary valve.

Abnormal Left Ventricular Strain Correlates with Left Ventricular Dysfunction but not Aortic Pathology in Marfan Syndrome in Children.

Cardiomyopathy is a complication in adults with Marfan syndrome (MFS). Early recognition of MFS patients at high risk of cardiomyopathy could impact monitoring and treatment. Abnormal ventricular strain has been associated with impaired ventricular function among adults with MFS but remains understudied in children. We retrospectively analyzed a cohort of patients with MFS undergoing cardiac magnetic resonance imaging (CMR) performed in 2003-2018 at age < 19 years. Correlations were evaluated between initial global circumferential strain (GCS) and global longitudinal strain (GLS) and the outcomes of left ventricular ejection fraction (LVEF), aortic root z-score, and vertebral artery tortuosity index corrected for height (VTI-h), all measured from CMR, using Spearman correlation. In those with serial CMR, the ability of ventricular strain to predict development of abnormal LVEF within a 5-year period was assessed. A total of 31 subjects were included (median age at initial CMR 13.5 years, Q1Q3 10.7-16.2 years), with 48% (n = 15) having LVEF < 55%. Worse GCS and worse GLS were associated with lower LVEF (ρ = - 0.629, p < 0.001 and ρ = - 0.411, p = 0.030, respectively). A clinical cutoff of GCS = - 34% predicted LVEF < 55% with sensitivity = 80% and specificity = 50%. Neither GCS nor GLS was associated with aortic root z-score (GCS: p = 0.524; GLS: p = 0.624) nor VTI-h (GCS: p = 0.949; GLS: p = 0.593). Of those with LVEF ≥ 55%, initial GCS and GLS did not differ between those with later normal versus abnormal LVEF (GCS: p = 0.505; GLS: p = 0.232). In this cohort, abnormal LV strain was associated with abnormal LVEF, but not with aortic dilation or low LVEF within the 5 years post-CMR.

Perforated posterior mitral valve leaflet in mitral annular disjunction and infective endocarditis.

We herein presented a 17-year-old female with history of mild mitral valve prolapse who was admitted for methicillin-sensitive Staphylococcus aureus endocarditis and diagnosed with mitral annular disjunction and perforated posterior mitral valve leaflet on two-dimensional and three-dimensional echocardiography (P1-P2). A perforation in the posterior leaflet was confirmed and repaired during surgical intervention. This is a rare presentation of leaflet perforation in the area of mitral annular disjunction.

Intramural course of an anomalous left coronary artery is not always associated with a slit-like ostium.

Anomalous aortic origin of the left coronary artery from an incorrect aortic sinus has been reported as the second most common causes of sudden cardiac arrest in young athletes. Intramural course of the proximal left coronary artery is considered a high-risk morphology. It is associated with a slit-like ostium and elliptical shape of the proximal artery. In this case, all pre-operative cardiac images demonstrated a round ostium and round luminal shape of the left coronary artery, which suggested no evidence of intramural course. On intraoperative inspection, although the ostium and proximal left coronary artery appeared round, the patient had a long intramural course of the left coronary artery which our surgeons performed a successful unroofing procedure. The post-operative images showed a patent new ostium from the correct aortic sinus. These findings are very unusual and proved that we should not exclude intramural course even in the absence of a slit-like ostium and elliptical shape of the proximal left coronary artery.

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.

Right Ventricular Global Longitudinal Strain in Fetuses with Hypoplastic Left Heart Syndrome Does Not Differ Between Those With and Without Genetic Conditions.

The presence of a genetic condition is a risk factor for increased mortality in hypoplastic left heart syndrome (HLHS). Speckle tracking strain analysis in interstage echocardiograms have shown promise in identifying patients with HLHS at increased risk of mortality. We hypothesized that fetuses with a genetic condition and HLHS have impaired right ventricular global longitudinal strain compared with fetuses with HLHS and no evident genetic condition. We performed a retrospective analysis of 60 patients diagnosed in fetal life with HLHS from 11/2015 to 11/2019. We evaluated presenting echocardiograms and calculated right ventricular global longitudinal strain (RV GLS) and fractional area of change (FAC) using post-processing software. We first compared RV GLS and FAC between those with genetic conditions to those without. We examined the secondary outcome of mortality among those with and without genetic conditions and among HLHS subgroups. Of the 60 patients with available genetic testing, 11 (18%) had an identified genetic condition. Neither RV GLS nor FAC was significantly different between patients with and without genetic conditions. There was no difference in RV GLS or FAC among HLHS phenotype or those who died or survived as infants. However, patients with a genetic syndrome had increased neonatal and overall mortality. In this cohort, RV GLS did not differ between those with and without a genetic diagnosis, among HLHS phenotypes, or between those surviving and dying as infants. Further analysis of strain throughout gestation and after birth could provide insight into the developing heart in fetuses with HLHS.

Two Cases of a Prenatally Diagnosed Double Aortic Arch with Postnatal Obliteration of the Distal Left Aortic Arch.

We report two cases of prenatally diagnosed double aortic arch with dominant right arch and a left-sided ductus arteriosus, consistent with a complete vascular ring. Postnatal transthoracic echocardiogram and cardiac magnetic resonance imaging demonstrated a spontaneous closure of the ductus arteriosus and obliteration of the left aortic arch distal to the origin of the left subclavian artery in both cases. Spontaneous closure of the ductus arteriosus involving extended ductal tissue in the left aortic arch likely led to obliteration of the distal left arch after birth. One patient presented with recurrent symptoms suggestive of dysphagia and underwent a successful surgical repair of the vascular ring with resolution of symptoms. The other patient has been asymptomatic and is 4 years old at the time of this report.

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.

Evidence of cardiomyopathy associated with Marfan syndrome in children.

OBJECTIVE: Marfan syndrome (MFS)-associated cardiomyopathy, defined as ventricular dilation and dysfunction unexplained by volume loading, is not well defined in children. This study evaluated ventricular size and function in paediatric MFS using cardiac MRI (cMRI). METHODS: This retrospective cohort study examined patients with MFS <19 years old at first cMRI. Left ventricular (LV) ejection fraction (EF) <55% was considered abnormal, as were z-scores >2. Combined mitral and aortic regurgitation indexed to LV stroke volume <20% defined absent/mild volume load. Biventricular volumes and EF on serial cMRI studies were compared with normative paediatric cMRI values, with measures converted to z-scores as appropriate. Longitudinal changes in volumes and EF were evaluated by mixed linear regression. Associations between ventricular, aortic and mitral characteristics were evaluated. RESULTS: 58 patients (60% male) were evaluated. Median age at initial cMRI was 13.6 years (IQR 10.0-15.8 years). Among patients with absent/mild LV volume load at initial cMRI (n=44, 76%), indexed LV end-diastolic volume (EDV) was significantly increased above normative values (median z-score 1.8, IQR 0.6-3.5, p<0.0001) and LVEF was abnormal in 48% (21/44). In the absence of volume loading, mitral valve prolapse (MVP) was associated with larger ventricular volumes and lower LVEF. Among those with serial cMRIs, LVEF and EDV z-scores did not significantly change over a mean follow-up time between cMRI studies of 1.5 years. CONCLUSION: Ventricular dilation and reduced EF are common in children with MFS and occur with no/mild LV volume load, suggesting intrinsic cardiomyopathy. MVP may be associated with cardiomyopathy.

Long-Term Outcomes of Ascending Sliding Arch Aortoplasty.

Background: Coarctation of the aorta can be associated with significant hypoplasia of the aortic arch. In contrast to patch aortoplasty, ascending sliding arch aortoplasty uses viable autologous tissue for potential growth in children. We reviewed the mid- to long-term outcomes of this technique. Methods: Between 2002 and 2023, 28 patients underwent ascending sliding arch aortoplasty for the patients with coarctation of the aorta (n = 22) and interrupted aortic arch (n = 2). Four patients underwent previous surgical coarctation repair at other institutions. The median patient age and body weight were 28.5 months (3 weeks to 15.6 years) and 13.4 kg (3.7-70 kg), respectively. Results: Although one patient had a recurrent nerve injury postoperatively, there were no other major morbidities or mortalities. The last follow-up echocardiography demonstrated that the mean peak velocity improved from 3.9 ± 0.6 to 0.9 ± 0.8 m/s, and the pressure gradient improved from 63.6 ± 21.5 to 7.1 ± 7.7 mm Hg. The postoperative diameters of the ascending aorta, proximal arch, distal arch, and isthmus all increased significantly. The mean postoperative length of stay was 5.9 ± 2.1 days, and the median follow-up time was 7.3 years (10 days to 20.5 years). No reoperation or catheterization-based intervention was performed for residual coarctation. Conclusions: Ascending sliding arch aortoplasty is safe and effective for treating coarctation of the aorta with aortic arch hypoplasia. This technique is applicable for children ranging in size from neonates to older children (or adolescents), recurrent coarctation cases, and provides complete relief of narrowing by utilizing viable native aortic tissue.

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.

Managing Bilateral Discontinuous Pulmonary Arteries of Ductal Origin in Single-Ventricle Anatomy.

BACKGROUND: We describe outcomes and management strategies for single-ventricle and bilaterally discontinuous pulmonary arteries (PAs) originating from bilateral ductus arteriosus. METHODS: We reviewed 22 patients with aforementioned anatomy and PA centralization from 1995 to 2023, excluding those with biventricular repair. RESULTS: Median age at centralization was 9 days (minimum-maximum, 0 days-2 years). Centralization was performed with systemic-to-pulmonary shunt (n =20 [91%]; 2 after bilateral ductal stents) or bidirectional cavopulmonary connection (n = 2 [9%]) using pericardial roll (n = 14 [64%]), patch-augmented direct anastomosis (n = 7 [32%]), and interposition graft (n = 1 [5%]) techniques. Concurrent total anomalous pulmonary venous connection (TAPVC, n = 11 [50%]) was associated with significantly inferior survival (P = .01). Five patients (23%) died at a median of 59 days (minimum-maximum, 6-257 days) after centralization, all with noncardiac TAPVC. At the latest follow-up for 17 survivors (median, 13.5 years; minimum-maximum, 0.5-25.1 years after centralization), 12 completed Fontan, 4 completed second-stage palliation, and 1 received a transplant before second-stage palliation. PA reintervention was required in 14 patients (64%), including 3 with reoperations independent of staged palliation. Echocardiography from baseline to before the second stage demonstrated branch PA growth with significantly increased diameters (left, P = .0006; right, P = .0002); z-scores significantly increased for right (P = .004) but not left (P = .11). CONCLUSIONS: Successful single-ventricle palliation is possible, although high risk, for patients with bilateral discontinuous ductal PAs. Early postcentralization mortality remains substantial, particularly with associated noncardiac TAPVC. Many require reintervention to maintain PA growth, typically concurrently with staged palliation.

Mitral Annular Disjunction and Its Progression during Childhood in Marfan Syndrome.

BACKGROUND: Data on mitral annular disjunction (MAD) in children with Marfan syndrome (MFS) are sparse. OBJECTIVES: To investigate the diagnostic yield of MAD by echocardiography and cardiac magnetic resonance imaging (CMR), its prevalence and progression during childhood. METHODS: We included patients <21 years old with MFS, defined by 2010 Ghent criteria and a pathogenic FBN1 variant or ectopia lentis. Two readers measured systolic separation between the mitral valve (MV) posterior hinge point and left ventricular (LV) myocardium on initial and subsequent imaging. MAD was defined as MV-LV separation ≥2 mm, MV prolapse (MVP) as atrial displacement ≥2 mm. Kappa coefficients evaluated echocardiogram-CMR agreement. Bland-Altman and intraclass correlation coefficients (ICC) assessed interrater and intermodality reliability. Univariable mixed-effects linear regression was used to evaluate longitudinal changes of MAD. RESULTS: MAD was detected in 60% (110/185) eligible patients. MVP was present in 48% (53/110) of MAD and MAD in 90% (53/59) of MVP. MAD detection by CMR and echocardiography had 96% overall agreement (Kappa = 0.89, p < 0.001) and a 0.32-mm estimate bias (95%CI 0.00, 0.65). ICC by echocardiography, CMR, and between modalities were 0.97 (95%CI 0.93, 0.98), 0.92 (95%CI 0.79, 0.97), and 0.91 (95%CI 0.85, 0.94), respectively. MAD was associated with aortic root dilation (p < 0.001). MAD was found in children of all ages, increased +0.18 mm/year (95%CI +0.14, + 0.22) during a median duration of 5.5 years (IQR 3.1, 7.5 years). MAD indexed by height yielded a constant value +0.0002 mm/m/year (95%CI -0.0002, + 0.0005 mm/m/year). CONCLUSIONS: MAD was common in pediatric MFS and was associated with aortic root dilation. MAD detection by echocardiography and CMR was highly reliable, suggesting that routine assessment in MFS is feasible. MAD was present in neonates and progressed over time but remained constant when indexing by height. Further studies are needed to evaluate MAD as a biomarker for clinical outcomes in pediatric MFS.

Imaging and guiding intervention for tricuspid valve disorders using 3-dimensional transesophageal echocardiography in pediatric and congenital heart disease.

In the pediatric and congenital heart disease (CHD) population, tricuspid valve (TV) disorders are complex due to the variable TV morphology, its sophisticated interaction with the right ventricle as well as associated congenital and acquired lesions. While surgery is the standard of care for TV dysfunction in this patient population, transcatheter treatment for bioprosthetic TV dysfunction has been performed successfully. Detailed and accurate anatomic assessment of the abnormal TV is essential in the preoperative/preprocedural planning. Three-dimensional transthoracic and 3D transesophageal echocardiography (3DTEE) provides added value to 2-dimensional imaging in the characterization of the TV to guide therapy and 3DTEE serves as an excellent tool for intraoperative assessment and procedural guidance of transcatheter treatment. Notwithstanding advances in imaging and therapy, the timing and indication for intervention for TV disorders in this population are not well defined. In this manuscript, we aim to review the available literature, provide our institutional experience with 3DTEE, and briefly discuss the perceived challenges and future directions in the assessment, surgical planning, and procedural guidance of (1) congenital TV malformations, (2) acquired TV dysfunction from transvenous pacing leads, or following cardiac surgeries, and (3) bioprosthetic TV dysfunction.

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.

Comparing palliation strategies for single-ventricle anatomy with transposed great arteries and systemic outflow obstruction.

Objective: Patients with complex single-ventricle anatomy with transposed great arteries and systemic outflow obstruction (SV-TGA-SOO) undergo varied initial palliation with ultimate goal of Fontan circulation. We examine a longitudinal experience with multiple techniques, including the largest published cohort following palliative arterial switch operation (pASO), to describe outcomes and decision-making factors. Methods: Neonates with SV-TGA-SOO who underwent initial surgical palliation from 1995 to 2022 at a single institution were retrospectively reviewed. Results: In total, 71 neonates with SV-TGA-SOO underwent index surgical palliation at a median age of 7 days (interquartile range, 6-10) by pASO (n = 23), pulmonary artery band (PAB) with or without arch repair (n = 25), or modified Norwood with Damus-Kaye-Stansel aortopulmonary amalgamation (n = 23). Single-ventricle pathology included double-inlet left ventricle (n = 37, 52%), tricuspid atresia (n = 27, 38%), and others (n = 7, 10%). All mortalities (n = 5, 7%) occurred in the first interstage period after PAB (n = 3) and Norwood (n = 2). Subaortic obstruction in the PAB group was addressed by operative resection (n = 10 total, 7 at index operation) and/or delayed aortopulmonary amalgamation (n = 13, 52%). Two patients with pASO (9%) had early postoperative coronary complications, 1 requiring operative revision. Median follow-up for survivors was 10.4 years (interquartile range, 4.5-16.6 years). Comparing patients by their initial palliation type, notable significant differences included size of bulboventricular foramen, weight at initial operation, operation duration, postoperative length of stay, time to second-stage palliation, multiple pulmonary artery reinterventions, and left pulmonary artery interventions. There were no significant differences in overall survival, Fontan completion, reintervention-free survival in the first interstage period, pulmonary artery reintervention-free survival, long-term systemic valve competency, or ventricular dysfunction. Conclusions: Excellent mid- to long-term outcomes are achievable following neonatal palliation for SV-TGA-SOO via pASO, PAB, and modified Norwood, with comparable survival and Fontan completion. Initial palliation strategy should be individualized to optimize anatomy and physiology for successful Fontan by ensuring an unobstructed subaortic pathway and accessible pulmonary arteries. pASO is a reasonable strategy to consider for these heterogeneous lesions.

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.