Background Outcomes for repair of Total Anomalous Pulmonary Venous Connection (TAPVC) from individual institutions suggest a significant improvement in mortality over the last several decades. The purpose of this study is to review the outcomes following repair of TAPVC from a large multi-institutional registry. Methods A retrospective review of the multi-institutional database, the Pediatric Cardiac Care Consortium (PCCC) was used to identify patients with the diagnosis of TAPVC who underwent complete correction between the years of 1982 to 2007. Data reviewed included age, decade of primary operation, anatomic type, presentation, and in-hospital mortality. Results Of the 118,084 surgical procedures submitted to the PCCC, 2191 (1.9%) were primary surgical correction of TAPVC. Sixty one percent of the cohort was male with 6.8% reported as premature. Overall in-hospital surgical mortality for simple TAPVC was 13%. Mortality was 20% from 1982 to 1989, 16% from 1990 to 1999, and 8% from 2000 to 2007. Obstruction to the anomalous pulmonary venous connection occurred in 29% with a mortality of 26%. Conclusions Surgical outcomes from repair of congenital cardiac anomalies have significantly improved over the last several decades. Multi-institutional large databases are needed to confirm results published from single institutional experiences. Although improvements in surgical repair of TAPVC have occurred over the last three decades, specific subtypes still experience significant mortality.

Background The long‐term outcomes of patients undergoing interventions for congenital heart disease ( CHD ) remain largely unknown. We linked the Pediatric Cardiac Care Consortium ( PCCC ) with the National Death Index ( NDI ) and the United Network for Organ Sharing Dataset ( UNOS ) registries to study mortality and transplant occurring up to 32 years postintervention. The objective of the current analysis was to determine the sensitivity of this linkage in identifying patients who are known to have died or undergone heart transplant. Methods and Results We used direct identifiers from 59 324 subjects registered in the PCCC between 1982 and 2003 to test for completeness of case ascertainment of subjects with known vital and heart transplant status by linkage with the NDI and UNOS registries. Of the 4612 in‐hospital deaths, 3873 were identified by the NDI as “true” matches for a sensitivity of 84.0% (95% CI, 82.9–85.0). There was no difference in sensitivity across 25 congenital cardiovascular conditions after adjustment for age, sex, race, presence of first name, death year, and residence at death. Of 455 known heart transplants in the PCCC , there were 408 matches in the UNOS registry, for a sensitivity of 89.7% (95% CI , 86.9–92.3). An additional 4851 deaths and 363 transplants that occurred outside the PCCC were identified through 2014. Conclusions The linkage of the PCCC with the NDI and UNOS national registries is feasible with a satisfactory sensitivity. This linkage provides a conservative estimate of the long‐term death and heart transplant events in this cohort.

Objective Surgical coronary revascularisation in children with congenital heart disease (CHD) is a rare event for which limited information is available. In this study, we review the indications and outcomes of surgical coronary revascularisation from the Pediatric Cardiac Care Consortium, a large US-based multicentre registry of interventions for CHD. Methods This is a retrospective cohort study of children (<18 years old) with CHD who underwent surgical coronary revascularisation between 1982 and 2011. Inhospital mortality and graft patency data were obtained from the registry. Long-term transplant-free survival through 2014 was achieved for patients with adequate identifiers via linkage with the US National Death Index and the Organ Procurement and Transplantation Network. Results Coronary revascularisation was accomplished by bypass grafting (n=72, median age 6.8 years, range 3 days–17.4 years) or other operations (n=65, median age 2.6 years, range 5 days–16.7 years) in 137 patients. Most revascularisations were related to the aortic root (61.3%) or coronary anomalies (27.7%), but 10.9% of them were unrelated to either of them. Twenty in-hospital deaths occurred, 70% of them after urgent ‘rescue’ revascularisation in association with another operation. Long-term outcomes were available by external linkage for 54 patients surviving to hospital discharge (median follow-up time 15.0 years, max follow-up 29.8 years) with a 15-year transplant-free survival of 91% (95% CI 83% to 99%). Conclusions Surgical coronary revascularisation can be performed in children with CHD with acceptable immediate and long-term survival. Outcomes are dependent on indication, with the highest mortality in rescue procedures.

Transplant-free survival after TAPVC repair is excellent, with most deaths or transplant events occurring early. Factors associated with the worst long-term outcomes included complex TAPVC, mixed TAPVC, and prolonged postoperative length of stay.

Increasing atrioventricular valve flow reliably induced growth. Valve repair and growth achieved a 2-ventricle repair in all patients.