The purpose of this study is to describe the evolution of pulmonary artery hypertension after repair of complete atrioventricular canal defect and to determine the time to normalisation of pulmonary artery pressure in both patients with and without Trisomy 21.

This is a single centre, retrospective analysis of patients with complete atrioventricular canal defect admitted for surgical repair at the University of Maryland Medical Center from 2005 to 2015. T-test or Mann–Whitney test and Chi-Square or Fisher’s exact tests were used to compare the two groups (patients with Trisomy 21 and those without). Repeated measures of analysis of variance and serial measurement analysis were performed.

Twenty-nine patients meeting the inclusion criteria underwent repair of complete atrioventricular defects during the study period. The right ventricular pressure estimate remained elevated over time and did not show a significant difference between the two groups. Right ventricular to systolic blood pressure ratios for all patients remained > 0.5 over the time periods assessed.

Our study suggests that in patients with complete atrioventricular canal defects, the right ventricular pressure remains elevated and does not normalise on echocardiograms performed up to one year after surgery, suggesting a sustained elevation in pulmonary vascular resistance.

With improvements in technology and surgical technique, paediatric cardiologists are challenging surgeons to repair balanced atrioventricular septal defects in smaller patients. Early repair minimizes aggressive medical therapy to prevent heart failure, maintains growth, and limits exposure to elevated pulmonary pressures. We compare the outcomes of repair among different-sized children.

From December 2002 to July 2005, 92 patients underwent repair of an atrioventricular septal defect with common atrioventricular valvar orifice and balanced ventricles. We reviewed operative and postoperative data. We excluded patients weighing more than 10 kilograms, but included those who underwent concomitant closure of a patent oval foramen or atrial septal defect, or ligation of a patent arterial duct. Those requiring other concomitant procedures were excluded from the analysis.

The median weight at repair was 4.9 kilograms, with a range from 2.93 to 7.9 kilograms, and the median age was 5.1 months, with a range from 0.39 to 9.6 months. Operative data included the time required for cardiopulmonary bypass, aortic cross-clamping, and the overall procedure. These times were not significantly affected by decreasing weight. Postoperative continuous data included duration of ventilation and length of intensive care unit and hospital stay. Stay in intensive care (p = 0.006) and hospital (p = 0.007) both increased significantly with decreasing weight. Postoperative categorical data included presence of residual ventricular septal defects, regurgitation across the left atrioventricular valve, and complications. While there was no difference in residual defects (p = 0.166) or valvar regurgitation (p = 0.729), there was a significantly higher presence of complications with decreasing weight (p = 0.0043). There was no mortality, and no persistent heart block requiring placement of a permanent pacemaker.

Our data shows that, with the exception of a slightly longer and more complicated postoperative course, early surgery for symptomatic patients with atrioventricular septal defects and common atrioventricular valvar orifice can be undertaken safely and effectively in smaller children with excellent outcomes.