OBJECTIVES: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.

Objective:Peritoneal dialysis (PD) commenced early in the postoperative period has the potential to mitigate the postcardiopulmonary bypass inflammatory response. We evaluated the role of early PD on postoperative outcomes after the arterial switch operation (ASO). Methods:Newborns (≤30 days, n = 318) undergoing ASO were classified into those who did (early PD, n = 90) or did not (control, n = 228) receive PD within 6 hours of admission to intensive care unit after surgery. Using observational data and imitating a preplanned clinical trial (target trial framework), we evaluated the role of early PD on postoperative outcomes. Results:Infants in the early PD group had greater serum lactate (median [interquartile range]: 2.6 [2.1, 4.1] vs 2.2 [1.8, 2.9]) and lower central venous saturation (median [interquartile range]: 45.2 [39.3, 51.4] vs 51.3 [42.2, 59.9]) at admission. Early PD was associated with a shorter duration of mechanical ventilation, but this effect was restricted to the subgroup receiving extracorporeal membrane oxygenation (ECMO) in the perioperative period (incidence rate ratio [95% confidence interval]: for early PD/control: 0.28 [0.17-0.47] for those requiring ECMO and 1.14 [0.93-1.39] for those not requiring ECMO, P interaction <.001). Similar results were seen for intensive care unit length of stay. Conclusions:Early PD after ASO was associated with a reduction in duration of mechanical ventilation and intensive care stay for infants who required ECMO in the perioperative period. Future studies of early PD, ideally clinical trials, in high-risk infants (such as those requiring ECMO after cardiac surgery) will be of benefit to either confirm or refute these findings.

Patients with secundum atrial septal defects preferentially undergo device closure;  however, this procedure is not always feasible. Instead, patients can safely undergo surgical closure. At a time when minimally invasive surgery can now be utilized with improved cosmetic results and the same excellent outcomes as a conventional sternotomy for an atrial septal defect closure, we propose the partial lower ministernotomy as the new standard for surgical atrial septal defect closure. We present a surgical case demonstrating this technique.

The patient had rheumatic heart disease, which resulted in severe aortic and mitral valve regurgitation. Repair of both valves was performed at 9 years of age. During surgery, the retracted aortic valve cusps required extension with bovine pericardial patches and suture reduction annuloplasty, and the mitral valve was repaired using a Cosgrove-Edwards (Edwards Lifesciences LLC, Irvine, CA) annuloplasty band. Post-operative echocardiography showed no regurgitation in either valve. The patient is doing well at 2 years of follow-up.

OBJECTIVES:The impact of conotruncal anomalies (CTAs), including tetralogy of Fallot, truncus arteriosus, ventriculo-arterial discordance, double-outlet right ventricle and interrupted aortic arch type B, on long-term outcomes remains poorly described in the Fontan cohort. We sought to review the outcomes of Fontan patients with CTAs in Australia and New Zealand. METHODS:We reviewed the data from 1835 patients who underwent a Fontan operation between 1975 and 2023 from the Australia and New Zealand Fontan Registry. RESULTS:CTAs occurred in 895 patients (49%), including D-TGA (Transposition of Great Arteries) (n = 476), double-outlet right ventricle (n = 360) and L-TGA (n = 170). Patients with CTAs had more heterotaxy syndrome (11.3% vs 6.8%, P < 0.001) and less left-ventricular dominance (53% vs 60%, P = 0.011). Median follow-up was 11.7 years (IQR: 5.3-20.3 years). Overall transplant-free survival and freedom from Fontan failure was 84% (95% confidence interval: 81-87%) and 72% (95% confidence interval: 68-75%) at 20 years, respectively. No difference was demonstrated in survival or freedom from Fontan failure between patients with or without CTAs (P = 0.50 and P = 0.83). Pacemaker implantation was more common in patients with CTAs (11.2% vs 8.3%, P = 0.038). Overall, 45 patients underwent outflow tract reinterventions, including semilunar valves, 'after' Fontan operation. Freedom from these reinterventions was 95% (95% confidence interval: 93-96%) at 30 years and was higher in patients with CTAs (P < 0.001). CONCLUSIONS:Patients with CTAs did not have a demonstrable difference in long-term survival and freedom from Fontan failure to other patients undergoing Fontan operation. Patients with CTAs have higher incidence of outflow tract reinterventions, including semilunar valves, and higher rate of pacemaker implantation.

We present a 12-year-old patient with periosteal osteosarcoma and bilateral pulmonary arterial tumour thrombi. The utility of a three-dimensional model to assess the feasibility of segmental resection is demonstrated.

OBJECTIVES:There is a large and growing population of adult survivors after the arterial switch operation (ASO). We sought to determine late outcomes in adult patients after the ASO. METHODS:Patients who underwent an ASO at a single institution were identified from the hospital database and retrospectively reviewed using hospital records. RESULTS:From 1983 to 2020, 1010 patients with a biventricular circulation underwent an ASO. There were 29 (2.9%, 29/1010) early deaths and 928 local patients with follow-up. There were 128 (14%, 128/928) patients with more than 30 years of follow-up and 47 (5.1%, 47/928) patients with more than 35 years of follow-up. Freedom from any reintervention was 80% (95% confidence interval [CI], 77%-83%), 75% (95% CI, 70%-79%), and 73% (95% CI, 67%-78%) at 20, 30, and 35 years after ASO, respectively. Freedom from reoperation on the neoaortic root or valve was 95% (95% CI, 92%-96%), 89% (95% CI, 85%-92%), and 87% (95% CI, 82%-91%) at 20, 30, and 35 years after ASO, respectively. Risk factors for reoperation on the neoaortic root or valve on multivariate analysis were Taussig-Bing anomaly (P = .009, hazard ratio [HR], 3.2; 95% CI, 1.3-7.7), bicuspid neoaortic valve (P < .001, HR, 6.6; 95% CI, 2.8-15.7), and cardiopulmonary bypass time (P = .001, HR, 1.0; 95% CI, 1.0-1.1). New York Heart Association class at last follow-up was I in 92% (118/128) patients with more than 30 years of follow-up. CONCLUSIONS:One quarter of patients require reoperation at 30 years after the ASO. Neoaortic root or valve surgery, although generally uncommon, is greater in patients with Taussig-Bing anomaly and bicuspid neoaortic valve.

BACKGROUND:The optimal management strategy for symptomatic neonates with tetralogy of Fallot is unclear. We compared the outcomes of staged repair (SR) (shunt palliation followed by complete repair) and primary repair (PR) in two institutions that have each exclusively adopted one of these strategies. METHOD:We retrospectively compared 65 symptomatic neonates who underwent shunt palliation between 1993 and 2021 at the Royal Children's Hospital, Melbourne, Australia with 38 symptomatic neonates who underwent PR between 2005 and 2018 at the Children's National Hospital, Washington, USA. Median follow-up duration was 8.0 (interquartile range 2.9-13.5) years. RESULTS:After the initial procedure, hospital mortality (shunt 4.6% vs PR 7.9%; p=0.50) and 5-year survival (shunt 94%; 95% confidence interval [CI] 84%-98% vs PR 89%; 95% CI 72%-96%; p=0.21) were not significantly different. The SR group had shorter durations of cardiopulmonary bypass and intensive care admission during the neonatal period, but a higher burden of unplanned re-interventions before discharge. Five-year freedom from re-interventions was not significantly different (shunt 63%; 95% CI 49%-74% vs PR 49%; 95% CI 29%-66%; p=0.13). Postoperative morbidity, mortality, and re-interventions were not significantly different among 13 propensity score-matched pairs balanced for operative weight. For neonates weighing <3 kg, PR was associated with significantly more early re-interventions. CONCLUSIONS:In symptomatic neonates with tetralogy of Fallot operated at two institutions with distinct treatment protocols, no statistically significant difference in medium-term survival and re-intervention was observed between the two strategies. SR was associated with lower neonatal morbidity but more unplanned re-interventions before discharge. PR in neonates weighing <3 kg may result in more early re-interventions.

Transcatheter pulmonary valve replacement (TPVR) has been associated with an increased risk of infective endocarditis. However, there are few reports investigating the risk of endocarditis with the specific prostheses used in TPVR-the Melody valve and SAPIEN valve. Rarely, endocarditis of the pulmonary valve may also extend to adjacent tissue, resulting in the need for more complex surgical management. This article reviews current literature on infective endocarditis following TPVR and compares this to rates following surgical pulmonary valve replacement, with a particular focus on the Melody valve, SAPIEN valve, and Contegra conduit. We will also discuss our experiences with pulmonary valve endocarditis and several reported cases of its extension to the aortic valve and paravalvular area.

The patient had a hypoplastic aortic arch with coarctation, a right aberrant subclavian artery, a bicuspid aortic valve, a ventricular septal defect and severe long-segment left ventricular outflow tract obstruction. The patient underwent initial univentricular palliation with subsequent biventricular conversion. Initially the patient had a Norwood operation with a Sano conduit at 23 days of age. The operation was performed on a beating heart with full-body perfusion for optimal preservation of heart cardiac function. At 9 months of age, the patient had a biventricular conversion that required ventricular septal defect enlargement and placement of the valve conduit from the right ventricle to the pulmonary artery (i.e. the Yasui procedure). The patient also had recurrent coarctation and left pulmonary artery stenosis; both were repaired with the heart beating to minimize myocardial ischaemic time.

Maturation of human pluripotent stem (hPS) cell-derived cardiomyocytes is critical for their use as a model system. Here we mimic human heart maturation pathways in the setting of hPS cell-derived cardiac organoids (hCOs). Specifically, transient activation of 5′ AMP-activated protein kinase and estrogen-related receptor enhanced cardiomyocyte maturation, inducing expression of mature sarcomeric and oxidative phosphorylation proteins, and increasing metabolic capacity. hCOs generated using the directed maturation protocol (DM-hCOs) recapitulate cardiac drug responses and, when derived from calsequestrin 2 (CASQ2) and ryanodine receptor 2 (RYR2) mutant hPS cells exhibit a pro-arrhythmia phenotype. These DM-hCOs also comprise multiple cell types, which we characterize and benchmark to the human heart. Modeling of cardiomyopathy caused by a desmoplakin (DSP) mutation resulted in fibrosis and cardiac dysfunction and led to identifying the bromodomain and extra-terminal inhibitor INCB054329 as a drug mitigating the desmoplakin-related functional defect. These findings establish DM-hCOs as a versatile platform for applications in cardiac biology, disease and drug screening. Pocock et al. reveal that transient activation of 5′ AMP-activated protein kinase and estrogen-related receptor drives robust maturation of multicellular human cardiac organoids, enabling modeling of desmoplakin cardiomyopathy dysfunction, which could be rescued using the bromodomain and extra-terminal inhibitor INCB054329.

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.

Isolated right pulmonary artery that comes forms the residual patent ductus arteriosus is rare. Multiple techniques have been described to anastomose the right pulmonary artery to the main pulmonary artery. Herein we detail our technique of reconstruction using pulmonary artery flap and autologous pericardium.