To ascertain rates of completion of essential cardiac procedures and their overall contribution to longer-term mortality in children with congenital heart disease (CHD). In this cohort study using the United Kingdom National CHD Audit, we described the pathway operations required for treatment for 9 sentinel CHDs-hypoplastic left heart syndrome (HLHS), non-HLHS functionally univentricular heart, ventricular septal defect, tetralogy of Fallot, coarctation, aortic stenosis, atrioventricular septal defect, transposition of the great arteries, and pulmonary atresia-that were undertaken at the population level and report the mortality associated with these pathway operations by age 5 years. Among 28,806 patients, over a median follow-up of 9.8 years (interquartile range, 4.2-15.4 years), 839 (2.9%) had undergone pre-pathway procedures only, 1135 (3.9%) had undergone initial palliation only (inclusive of functionally univentricular and biventricular reparative pathways), 2001 (6.9%) had undergone stage 2 palliation and/or total cavopulmonary connection (TCPC), 2254 (7.8%) had undergone staged biventricular repair after palliation, and 22,572 (78.3%) had undergone primary biventricular repair. Of the 23,239 children with 5 years of follow-up, 1794 (7.7%) had died by age 5 years, consisting of 409 (1.8%) who died following an initial palliative procedure, 43 (0.2%) who died following a stage 2 palliation or TCPC, 379 (1.6%) who died following a biventricular reparative procedure, and 963 (4.1%) who died in other circumstances, such as interstage or following reinterventions. The outcome metrics of surgical pathway completion (biventricular repair or TCPC) and overall mortality at age 5 years can be evaluated using registry data and could contribute to future assessment of overall CHD service quality.

This study sought to investigate the impact of the residual lesion score (RLS) on the long-term outcomes of 5 common congenital heart operations. All infants who underwent definitive operation for complete atrioventricular septal defect, tetralogy of Fallot (TOF), dextro-transposition of the great arteries, single ventricle (Norwood procedure), and coarctation of the aorta with ventricular septal defect between 2000 and 2012 and who survived until discharge were studied. RLS scores (1, no or trivial; 2, minor; 3, major or in-hospital reinterventions or reoperations for such lesions) were assigned on the basis of postrepair clinical and echocardiographic evaluation. The time to events was summarized using the Kaplan-Meier survival method and competing risk models. A total of 1027 patients were included (213 atrioventricular septal defects, 358 TOFs, 308 dextro-transpositions of the great arteries, 127 single ventricles, and 21 coarctations of the aorta with ventricular septal defects), with a median follow-up time of 15 years (interquartile range, 11-18.4 years). Overall, 227 patients (22.1%) had an RLS of 1, 556 patients (54.1%) had an RLS of 2, and 244 patients (23.8%) had an RLS of 3. Freedom from late death or heart transplantation was 93.7% at 15 years. The RLS was not associated with late death or transplantation. Male sex, TOF, and arterial switch operation had the lowest risk for late death or transplantation. Late reinterventions or reoperations were present in 14.5% at 15 years. Younger age, TOF with pulmonary stenosis repair, arterial switch operation, an RLS of 3 (hazard ratio, 2.02; 95% CI, 1.17-3.51; P = .012), intraoperative surgical revision, and in-hospital reintervention or reoperation were associated with late reintervention or reoperation. The RLS does not predict late mortality but predicts late reintervention or reoperation after congenital cardiac disease repair and can be used to target at-risk patients for follow-up.

BackgroundWe sought to determine the management and early outcomes of complete atrioventricular septal defect-tetralogy of Fallot (AVSD-TOF) for a contemporary multicenter cohort.MethodsOf 739 participants in the Congenital Heart Surgeons' Society AVSD cohort (January 2012-May 2021), 40 had AVSD-TOF. We first compared survival differences for patients with AVSD-TOF versus those with isolated AVSD using propensity matching. Secondly, for patients with AVSD-TOF, we compared staged (n = 16) versus primary (n = 24) repair by assessing the following: patient characteristics, progression of atrioventricular valve (AVV) regurgitation, and time-related reoperation and survival.ResultsFive-year survival was similar between matched AVSD-TOF and isolated AVSD groups (80% vs 81%, P = .9). Compared with primary repair patients, staged patients had smaller pulmonary valve annulus Z-score measured at first presentation (-2.2 vs -2.9, P = .006). All staged patients (12 Blalock-Thomas-Taussig shunts, 3 right-ventricular-outflow-tract stents, 1 ductal stent) survived to complete repair. Freedom from AVSD-related reoperation five years post-AVSD-TOF repair was 57% after staged versus 90% after primary repair (P < .05) and left AVV reoperations were the most frequent reintervention. Survival five years after AVSD-TOF repair was 80% (63% after staged vs 90% after primary repair; P = .08).ConclusionsPatients undergoing AVSD-TOF repair have similar survival compared with matched isolated AVSD patients. Although approximately half of AVSD-TOF patients had initial palliation and all survived to complete repair, staged repair patients had lower survival and a higher reintervention rate compared with primary repair patients. The decision to pursue staged versus primary repair for future babies with AVSD-TOF remains challenging and should be chosen based on individual circumstances.

Primary repair in the first six months of life is routine for tetralogy of Fallot, complete atrioventricular septal defect, and ventricular septal defect in high-income countries. The objective of this analysis was to understand the utilization and outcomes of palliative and reparative procedures in high versus middle-income countries. The World Database of Pediatric and Congenital Heart Surgery identified patients who underwent surgery for: tetralogy of Fallot, complete atrioventricular septal defect, and ventricular septal defect. Patients were categorized as undergoing primary repair, repair after prior palliation, or palliation only. Country economic status was categorized as lower middle, upper middle, and high, defined by the World Bank. Multiple logistic regression models were utilized to identify independent predictors of hospital mortality. Economic categories included high (n = 571, 5.3%), upper middle (n = 5,342, 50%), and lower middle (n = 4,793, 49.7%). The proportion of patients and median age with primary repair were: tetralogy of Fallot, 88.6%, 17.7 months; complete atrioventricular septal defect, 83.4%, 7.7 months; and ventricular septal defect, 97.1%, ten months. Age at repair was younger in high income countries (P < .0001). Overall mortality after repair was lowest in high income countries. Risk factors for hospital mortality included prematurity, genetic syndromes, and urgent or emergent operations (all P < .05). Primary repair was selected in >90% of patients, but definitive repair was delayed in lower and upper middle income countries compared with high-income countries. Repair after prior palliation versus primary repair was not a risk factor for hospital mortality. Initial palliation continues to have a small but important role in the management of these three specific congenital heart defects.

It is commonly known that Down syndrome (DS) and congenital cardiovascular abnormalities go hand in hand. Most frequently, complete atrioventricular septal abnormalities have been linked to DS. Along with DS, ventricular septal defect (VSD), atrial septal defect, tetralogy of Fallot, and patent ductus arteriosus have also been reported. We present a case of DS with VSD who underwent VSD correction. Echocardiography prompted the diagnosis, which was then confirmed by surgery. The patient was successfully transferred out of the hospital. After correcting the VSD, the survival and quality of life of the DS patient have improved.