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Background Discharge from follow-up after closure of isolated congenital shunt lesions in childhood was common practice in the past. The aim of the present study was to recall these patients to evaluate their current status. Methods Patients included in the database of pediatric and congenital heart disease of our tertiary center with repaired secundum atrial septal defect (ASD) or ventricular septal defect (VSD) before the age of 18 years, and discharged from follow-up, were invited for clinical and echocardiographic check-up. Results Forty-six ASD patients (age 30 ± 7 years, 37% male) responded. Median age at ASD repair was 6 (IQR 4–8) years. All but one functioned in NYHA class I. Eight (17%) patients reported palpitations. No patient developed pulmonary hypertension (PH). Right ventricular (RV) dilatation was present in 7 (15%). RV fractional area change (FAC) was < 35% in 7 (15%), TAPSE < 17 mm in 12 (26%). Forty-seven VSD patients (age 34 (IQR 29–40) years, 57% male) participated. Median age at VSD repair was 4 (IQR 1–5) years. Six (13%) patients functioned in NYHA class II. Seventeen (36%) patients reported palpitations. Four (9%) patients presented PH. Left ventricular dilatation was present in 4 (9%), RV dilatation in 6 (13%). RV FAC was < 35% in 7 (15%), TAPSE < 17 mm in 17 (36%). Seven (15%) patients had dilated ascending aorta. Conclusions Patients with closure of isolated secundum-type ASD in childhood do well, but some have persistent RV dilatation and dysfunction. By contrast, more patients after VSD closure were symptomatic and presented with RV dilatation and dysfunction, PH, and a dilated ascending aorta. [ABSTRACT FROM AUTHOR]

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. [ABSTRACT FROM AUTHOR]

Background Pulmonary outflow tract obstruction (POTO) reduces systemic atrioventricular valve (SAVV) regurgitation severity in congenitally corrected transposition of the great arteries (ccTGA). Therefore, pulmonary artery banding is proposed as a palliative intervention. We aimed to investigate the effect of native or surgically induced POTO on event-free survival, defined as the composite of all-cause mortality, heart transplantation, or congestive heart failure (CHF). Methods and results Patients with ccTGA (n = 62; median age 27.5 (IQR 18.4–39.4) years; 39% with POTO) were selected from the Adult Congenital Heart Disease database of a tertiary hospital. At first visit, SAVV regurgitation ≥ 3/4, systemic RV dysfunction ≥ moderate, and CHF were present in 26%, 26%, and 15% of patients, respectively. Over a mean follow-up time of 10.1 ± 6.1 years, all-cause mortality, rate of heart transplantation, and CHF were 18%, 8% and 40%, respectively. SAVV regurgitation (HR: 1.99; 95% CI: 1.01–3.92; P = 0.048) and systemic RV dysfunction severity (HR: 1.89; 95% CI: 1.05–3.37; P = 0.033) were associated with the composite endpoint, independently of age at baseline, POTO, Ebstein-like malformation, and systemic RV dilatation. Patients with POTO had lower risk for developing SAVV regurgitation ≥ 3/4 (HR: 0.18; 95% CI: 0.05–0.58; P = 0.004) and moderate systemic RV dysfunction (HR: 0.34; 95% CI: 0.15–0.78; P = 0.011). When POTO was present, the mean progression-free interval for the composite endpoint increased from 11.2 to 18.1 years ( P = 0.035). Conclusions POTO is associated with an improved event-free survival in adults with ccTGA. [ABSTRACT FROM AUTHOR]