Ventricular septal defects – large, surgically closed or small, untreated – have demonstrated lower peak exercise capacity compared with healthy controls. The mechanisms behind these findings are not yet fully understood. Therefore, we evaluated biventricular morphology in adults with a ventricular septal defect using MRI. Adults with either childhood surgically closed or small, untreated ventricular septal defects and healthy controls underwent cine MRI for the evaluation of biventricular volumes and quantitative flow scans for measurement of stroke index. Scans were analysed post hoc in a blinded manner. In total, 20 operated patients (22±2 years) and 20 healthy controls (23±2 years) were included, along with 32 patients with small, unrepaired ventricular septal defects (26±6 years) and 28 controls (27±5 years). Operated patients demonstrated larger right ventricular end-diastolic volume index (103±20 ml/m2) compared with their controls (88±16 ml/m2), p=0.01. Heart rate and right ventricular stroke index did not differ between operated patients and controls. Patients with unrepaired ventricular septal defects revealed larger right ventricular end-diastolic volume index (105±17 ml/m2) compared with their controls (88±13 ml/m2), p<0.01. Furthermore, right ventricular stroke index was higher in unrepaired ventricular septal defects (53±12 ml/minute/m2) compared with controls (46±8 ml/minute/m2), p=0.02, with similar heart rates. Both patient groups’ right ventricles were visually characterised by abundant coarse trabeculation. Positive correlations were demonstrated between right ventricular end-diastolic volume indices and peak exercise capacity in patients. Left ventricle measurements displayed no differences between groups. In conclusion, altered right ventricular morphology was demonstrated in adults 20 years after surgical ventricular septal defect repair and in adults with small, untreated ventricular septal defects.

A male child, with d-transposition of great arteries, a large perimembranous ventricular septal defect, multiple additional ventricular septal defects, small muscle-bound right ventricle, and severe pulmonary stenosis with confluent, moderate-sized branch pulmonary arteries, underwent an emergency right modified Blalock–Taussig shunt on day 15 of life and réparation à l’étageventriculaire procedure with ventricular septal defect closure with takedown of the Blalock–Taussig shunt at 2.5 years of age. On follow-up, he showed a moderate residual upper ventricular septal defect and multiple apical ventricular septal defects, mild mid-right pulmonary artery stenosis, free pulmonary regurgitation, and right ventricular dysfunction. Surgical re-intervention was deemed extremely risky, the upper muscular ventricular septal defect was closed using an 8-mm Amplatzer Muscular Ventricular Septal Defect Occluder Device, and an 18 mm Amplatzer Multi-Fenestrated Septal Occluder – Cribriform was used for the multiple apical muscular ventricular septal defects. After 1 year, his right pulmonary artery stenosis worsened, for which right pulmonary artery angioplasty was carried out using an 8×20 mm cutting balloon followed by a 10×20 mm Tyshak II balloon. This is the only case reported for the paediatric age group using a cribriform septal occluder device for percutaneous closure of multiple apical ventricular septal defects.