Gabriels Charlien, De Backer Julie, Pasquet Agnes, Paelinck Bernard P, Morissens Marielle, Helsen Frederik, Van De Bruaene Alexander, Budts Werner
University Hospitals, Leuven, Belgium.
Cardiology. 2017;136(3):147-155. doi: 10.1159/000448513. Epub 2016 Sep 21.
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.
All pmVSD patients in the registry were analyzed.
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.
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.
评估成人室间隔缺损（VSD）长期预后的研究对于告知患者预后情况很重要。本研究调查了比利时成人先天性心脏病登记处随访的膜周部室间隔缺损（pmVSD）患者的长期预后。
对登记处所有pmVSD患者进行分析。
共研究了266例患者。15例患有艾森曼格综合征。173例为孤立性pmVSD，78例为合并其他病变的pmVSD。在孤立性pmVSD患者中，52%为男性，中位年龄为29岁（四分位间距24 - 35岁），中位随访时间为18年（四分位间距10 - 25年）。53例（31%）患者接受了VSD封堵术，10例（19%）有残余分流。大多数（93%）患者心功能分级为纽约心脏协会（NYHA）I级。无患者死亡。2例（4%）患者发生房性心律失常，2例（4%）需要植入起搏器。7例（14%）发生左心室流出道梗阻（LVOTO）。在未修复的pmVSD组中，4例发生心内膜炎。在整个研究组中，9例（5%）患者出现中度或重度主动脉瓣反流（AR）。
孤立性pmVSD患者的长期生存并非一帆风顺。未进行VSD修复的患者可能会出现中度或重度AR以及心内膜炎。VSD封堵术后的并发症包括房性心律失常、起搏器植入和LVOTO。
