Primary or Delayed Repair for Complete Atrioventricular Septal Defect, Tetralogy of Fallot, and Ventricular Septal Defect: Relationship to Country Economic Status.

OBJECTIVE: 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. METHODS: 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. RESULTS: 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). CONCLUSIONS: 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.

Hospital Mortality and Adverse Events Following Repair of Congenital Heart Defects in Developing Countries.

BACKGROUND: Mortality associated with the correction of congenital heart disease has decreased to approximately 2% in developed countries and major adverse events are uncommon. Outcomes in developing countries are less well defined. The World Database for Pediatric and Congenital Heart Surgery was utilized to compare mortality and adverse events in developed and developing countries. METHODS: A total of 16,040 primary procedures were identified over a two-year period. Centers that submitted procedures were dichotomized to low/middle income (LMI) and high income (HI) by the Gross National Income per capita categorization. Mortality was defined as any death following the primary procedure to discharge or 90 days inpatient. Multiple logistic regression models were utilized to identify independent predictors of mortality. RESULTS: Of the total number of procedures analyzed, 83% (n = 13,294) were from LMI centers. Among all centers, the mean age at operation was 2.2 years, with 36% (n = 5,743) less than six months; 85% (n = 11,307) of procedures were STAT I/II for LMI centers compared with 77% (n = 2127) for HI centers (P < .0001). Overall mortality across the cohort was 2.27%. There was a statistical difference in mortality between HI centers (0.55%) versus LMI centers (2.64%) (P < .0001). After adjustment for other risk factors, the risk of death remained significantly higher in LMI centers (odds ratio: 2.36, 95% confidence interval: 1.707-3.27). CONCLUSION: Although surgical expertise has increased across the globe, there remains a disparity with some outcomes associated with the correction of congenital heart disease between developing and developed countries. Further studies are needed to identify specific opportunities for improvement.

Double aortic arch with atretic proximal left arch.

Aortic arch anomalies are rare and represent about 1% of all congenital cardiac lesions. Double aortic arch with atretic proximal left arch is one of the rare causes of respiratory symptoms in neonates, often missed by preoperative imaging studies. Preoperative identification and differentiating this entity from other arch anomalies is imperative to plan appropriate surgical management.