Long-Term Outcome of Patients with Perimembranous Ventricular Septal Defect

Isolated ventricular septal defect (VSD) is the most common congenital heart defect, apart from bicuspid aortic valve, accounting for 30-40% of all congenital cardiac malformations. Spontaneous closure in the first years of life is common in cases of small defects. VSD is generally diagnosed and, if necessary, treated in childhood. Four subgroups have been defined in the guidelines, according to defect location: perimembranous, muscular, outlet and inlet type. The perimembranous type is the most common in adults (about 80% of all VSDs). The clinical presentation and natural history can vary from small VSD with insignificant left-to-right shunt to VSD with significant left-to-right shunt with left ventricular (LV) volume overload and right ventricular (RV) pressure overload, which, if unrepaired, may cause pulmonary vascular disease and even Eisenmenger syndrome. Patients with a small VSD and insignificant left-to-right shunt or with a repaired VSD usually remain event-free during follow-up. However, several problems may still develop later in life, with the most important being endocarditis, LV dilatation due to volume overload, double-chambered right ventricle, LV outflow tract obstruction (LVOTO), aortic valve regurgitation (AR) and complete heart block (especially in the earlier years of cardiac surgery).

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