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Objectives: Studies evaluating the long-term outcome of adults with ventricular septal defect (VSD) are important to inform patients about prognosis. This study investigated the long-term outcome of patients with perimembranous VSD (pmVSD) followed in the Belgian Registry on Adult Congenital Heart Disease. Methods: All pmVSD patients in the registry were analyzed. Results: Two hundred and sixty-six patients were studied. Fifteen patients had Eisenmenger syndrome. One hundred and seventy-three had isolated pmVSD and 78 had pmVSD with concomitant lesions. Of the patients with isolated pmVSD, 52% were male, median age was 29 years (IQR 24-35 years) and median follow-up duration was 18 years (IQR 10-25 years). Fifty-three (31%) patients underwent VSD closure and 10 (19%) had a residual shunt. Most (93%) patients were in NYHA class I. No patients died. Two (4%) patients developed atrial arrhythmia and 2 (4%) required pacemaker implantation. Seven (14%) developed left ventricular outflow tract obstruction (LVOTO). In the unrepaired pmVSD group, 4 developed endocarditis. In the entire group, moderate or severe aortic regurgitation (AR) occurred in 9 (5%) patients. Conclusions: Long-term survival in patients with isolated pmVSD was not uneventful. Moderate or severe AR might develop and endocarditis occurred in patients without VSD repair. Complications after VSD closure included atrial arrhythmia, pacemaker implantation and LVOTO

Background/objectives Pulmonary arterial hypertension is an important complication in hemodynamically relevant atrial septal defects (ASD) and negatively affects outcome. This retrospective study aimed at (1) estimating the prevalence of pulmonary hypertension (PH) in patients with secundum ASD and (2) identifying predictors of PH development or persistence after ASD closure.Methods Consecutive patients with an isolated secundum ASD from the Belgian Registry on Adult Congenital Heart Disease were studied. Demographic, clinical, echocardiographic and invasive hemodynamic measurements were analyzed. PH was defined upon the echocardiographic PH probability (tricuspid regurgitation velocity ≥ 2.9 m/s).Results PH prevalence in the entire ASD population (295 patients, 68.8% females, mean age 46 ± 21 years) was 15.9% compared to 13.3% in patients after ASD closure. PH after ASD closure was significantly related to mortality (p = 0.001), atrial arrhythmia (p < 0.001) and right heart failure (p = 0.019). Age at repair was the most important predictor for PH (HR 1.11). In the highest tertile of age at repair (> 55 years), PH prevalence was the highest (34%) and mean pulmonary artery pressure (mPAP) at catheterization before was related to PH after closure (HR 1.09). Twenty patients in the PH group had mPAP < 25 mm Hg before closure.Conclusions PH in closed secundum ASD patients is not uncommon. Its prevalence was the highest when the defect was repaired above 55 years of age. Clinical outcome was worse. PH may even develop despite normal mPAP before closure. The present findings raise the question whether the cutoff value for mPAP before closure should be age-adjusted. © 2014 Elsevier Ireland Ltd. All rights reserved.