Early pulmonary arterial hypertension immediately after closure of a ventricular or complete atrioventricular septal defect beyond 6 months of age

Background Pulmonary arterial hypertension (PAH) is virtually absent after closure of ventricular septal defect (VSD) in the first six months of life. However the prevalence of PAH in patients, who underwent VSD closure later, is not clear. The aim of this study was to analyse the prevalence of PAH after a successful VSD closure after the age of 6 months and whether there are risk factors for developing PAH. Methods Echocardiographic and right heart catheter data of patients with VSD or complete atrioventricular septal defect, who underwent VSD closure after the age of 6 months in our institution between 01/2005 and 06/2014, were retrospectively analyzed. PAH was defined as mean pulmonary arterial pressure (mPAP) of ≥ 25 mm Hg or tricuspid regurgitation jet velocity of ≥ 3.5 m/s. Results In 228 patients (median age at shunt closure 4.0 years, range 0.5–69) and 174 complete follow-up data (median follow-up 3.7 years, range 0.5–39.4), 9 patients needed pulmonary vasodilator therapy after shunt closure, 4 of them temporarily for up to 79 months. Three patients are still on vasodilator treatment 1, 2.6 and 6 years after surgery, other two were lost to follow-up. Another 6 patients with preoperatively borderline hemodynamics due to elevated mPAP and pulmonary vascular resistance, recovered well without signs of postoperative PAH. Conclusion With the current practice for safe late VSD closure, PAH is very rare at least in the first years of follow-up. In most patients with perioperative PAH, this condition appears to be transient and shows good response on pulmonary vasodilator treatment.