Christian P. Brizard

Objective: The study objective was to evaluate the outcomes of the extracardiac Fontan operation at a single institution. Methods: We conducted a retrospective study of 398 patients from a single institution who underwent their initial extracardiac Fontan operation between 1997 and 2020. We determined the incidence of and risk factors for failure of the Fontan circulation, which includes death, Fontan takedown, heart transplantation, protein-losing enteropathy, plastic bronchitis, and functional status at the last follow-up. Results: The median follow-up time was 10.3 years (interquartile range, 6.4-14.6). The overall survival was 96% and 86% at 10 and 20 years after extracardiac Fontan operation, respectively. There were 6 early deaths (6/398, 1.5%) and 15 late deaths (15/398, 3.8%). Forty-nine patients (12.5%) developed failure of the Fontan circulation. Freedom from the failure of Fontan circulation was 88% at 10 years and 76% at 20 years. Risk factors for failure of the Fontan circulation were right ventricular dominance (hazard ratio, 4.7; P < .001; 95% CI, 2.1-10.5), aortic atresia (hazard ratio, 5.5; P < .001; 95% CI, 2.3-12.8), and elevated mean pulmonary artery pressure (hazard ratio, 2.3; P = .002; 95% CI, 1.2-6.7). Conclusions: Rates of failure of the Fontan circulation are low after the contemporary extracardiac Fontan operation. Risk factors for failure of the extracardiac Fontan circulation include right ventricular dominance, aortic atresia, and elevated pulmonary artery pressures.

Background: While complete repair of pulmonary atresia with ventricular septal defect and major aortopulmonary collateral arteries (PA/VSD/MAPCAs) is achieved with low surgical mortality, this condition poses ongoing mid- and late-term mortality. The long-term data for repair of pulmonary atresia with ventricular septal defect and major aortopulmonary collateral arteries at a single institution are presented in this paper. Methods: We reviewed the outcomes for 100 patients with PA/VSD/MAPCAs who had surgery between January 1987 and July 2018. Two patients were excluded who had palliative surgery not intended to reach a complete repair. Results: Of the 98 patients who were suitable candidates for complete repair, 13% (13/98) had primary complete repair and 64% (63/98) had staged complete repair. Estimated survival at 1, 5, 10, and 20 years of age was 93%, 87%, 81%, and 76%, respectively. The median age of those patients alive at follow-up was 15 (interquartile range 9-21) years. Freedom from reintervention after staged complete repair was 58% at 5 years after complete repair. Ligating or embolizing MAPCAs was associated with a decreased hazard of death in the staged complete repair group (hazard ratio 0.6, p-value 0.04). Conclusions: A strategy of staged complete repair can be used to achieve complete repair and stable long-term survival for patients with PA/VSD/MAPCAs.

Objective: Patients with Ebstein anomaly (EA) require complex management. A group of experts was commissioned by the American Association for Thoracic Surgery to provide a framework on this topic, focusing on preoperative assessment, indications for intervention, and medical management before and after surgical intervention in older children and adults. This is a companion document to a recently issued document regarding management of EA in neonates and infants. This document addresses children older than 1 year of age and adults. Methods: The EA Writing Group of the Congenital Clinical Practice Standards Committee is a multinational and multidisciplinary group of surgeons and cardiologists with expertise in EA. A citation search in PubMed, Embase, Scopus, and Web of Science was conducted using key words related to EA. The search was restricted to the English language and the year 2000 or later and yielded 455 results. Expert consensus statements with class of recommendation and level of evidence were developed using a modified Delphi method, requiring 80% of members' votes with at least 75% agreement on each statement. Results: In patients with EA, the presence of congestive heart failure, cyanosis, significant left ventricular dysfunction, severe cardiomegaly, and persistent arrhythmia increases the risk for morbidity and mortality. Asymptomatic patients with EA should undergo exercise stress testing with measurement of oxygen consumption to unmask occult exercise intolerance. Cardiac magnetic resonance imaging is recommended for comprehensive imaging of EA for reliable volume assessment of both ventricles, evaluation of tricuspid regurgitation fraction, and right ventricle stroke volume. Surgery is recommended for symptoms that include fatigue, decreased objective exercise tolerance, decreased arterial oxygen saturation (cyanosis), and exertional dyspnea. Surgery can be beneficial in asymptomatic patients with severe tricuspid regurgitation, moderate right ventricular enlargement, and valve anatomy favorable for repair. Bidirectional cavopulmonary shunt is reasonable when there is severe right ventricular dilation, severe right ventricular systolic dysfunction, right atrial pressure: left atrial pressure ratio >1.5, or failure to separate from cardiopulmonary bypass after repair. Concomitant maze procedure at the time of surgery is reasonable when there is paroxysmal or continuous atrial fibrillation. In EA patients with evidence of systolic left ventricular dysfunction, concomitant acquired disease should be excluded, and goal-directed medical therapies is recommended. Conclusions: Management of EA requires a tailored and multidisciplinary approach. The introduction of the cone procedure has revolutionized surgical management, providing excellent outcomes and durability across a wide range of anatomical variations. The timing of intervention, especially in asymptomatic patients, remains a topic of ongoing research and debate. Comprehensive preoperative evaluations, including exercise testing and arrhythmia assessment, are critical for optimizing surgical outcomes. In complex cases, adjunct procedures like bidirectional cavopulmonary shunt or the maze procedure offer additional therapeutic benefits.

Background: Ebstein anomaly is a rare congenital anomaly of the tricuspid valve which presents challenges to cardiac surgeons due to the spectrum of the disease and the technical difficulty of valve repair. The natural history of the anomaly differs between patients presenting in the neonatal period to those presenting in adulthood. Methods: A retrospective review of all patients >15 years of age with Ebstein anomaly, undergoing surgery on the tricuspid valve at 6 centers across Australia and New Zealand was performed. Patients from 1985 to 2019 were included in the study. Results: A total of 125 patients were included in the study, 76 patients (60%) undergoing tricuspid valve repair, of which 23 patients underwent a Cone repair and 49 (40%) had a tricuspid valve replacement. The mean follow-up was 7.9 ± 7.3 years. Postoperatively, early mortality was 3 patients (2%) and 10-year survival was 91.5%. A postoperative pacemaker was required in 24 patients (19%). Reoperation was required in 21 patients (17%). There was no statistically significant difference in survival or reoperation between patients who underwent repair or replacement of the tricuspid valve; however, with a small number of patients in long-term follow-up. Conclusion: Older children and adult patients undergoing surgery for Ebstein anomaly in Australia and New Zealand experience good medium-term postoperative survival. Repair of the valve is achieved in a significant proportion of patients with increasing use and success with the Cone repair technique.

The optimal treatment strategy for symptomatic young infants with tetralogy of Fallot (TOF) is unclear. We sought to compare the outcomes of staged repair (SR) (shunt palliation followed by second-stage complete repair) versus primary repair (PR) at 2 institutions that have exclusively adopted each strategy. We performed propensity score-matched comparison of 143 infants under 4 months of age who underwent shunt palliation at one institution between 1993 and 2021 with 122 infants who underwent PR between 2004 and 2018 at another institution. The primary outcome was mortality. Secondary outcomes were postoperative complications, durations of perioperative support and hospital stays, and reinterventions. Median follow-up was 8.3 years (interquartile range, 8.1-13.4 years). After the initial procedure, hospital mortality (shunt, 2.8% vs PR, 2.5%; P = .86) and 10-year survival (shunt, 95%; 95% confidence interval [CI], 90%-98% vs PR, 90%; 95% CI, 81%-95%; P = .65) were similar. The SR group had a greater risk of early reinterventions but similar rates of late reinterventions. Propensity score matching yielded 57 well-balanced pairs. In the matched cohort, the SR group had similar freedom from reintervention (55%; 95% CI, 39%-68% vs 59%; 95% CI, 43%-71%; P = .85) and greater survival (98%; 95% CI, 88%-99.8% vs 85%; 95% CI, 69%-93%; P = .02) at 10 years, as the result of more noncardiac-related mortalities in the PR group. In symptomatic young infants with TOF operated at 2 institutions with exclusive treatment protocols, the SR strategy was associated with similar cardiac-related mortality and reinterventions as the PR strategy at medium-term follow-up.