Natural history and management of restrictive perimembranous ventricular septal defects in children: insights from a 30-year experience

Abstract Introduction: Perimembranous ventricular septal defects represent the most common form of ventricular septal defects. Although restrictive perimembranous ventricular septal defects (rpVSDs) are generally associated with favourable outcomes, their clinical management remains controversial due to the lack of consensus on closure indications and the variable natural history. This study aims to evaluate the long-term clinical course, treatment needs, and outcomes of children diagnosed with perimembranous ventricular septal defect and restrictive perimembranous ventricular septal defects over a 30-year period. Methods: A retrospective review was conducted on 448 paediatric patients diagnosed with isolated perimembranous ventricular septal defect at a single tertiary centre between 1991 and 2021. Echocardiographic data, clinical presentations, and treatment modalities were assessed. Patients were categorised based on ventricular septal defect size and hemodynamic features, focusing on the spontaneous closure rate, necessity for medicinal treatment, and surgical or interventional procedures. Results: Among the 448 paediatric patients, 20.1% experienced spontaneous closure, while 31.3% required surgical intervention. Medical therapy was administered in 36.6% of the cohort, primarily in symptomatic or hemodynamically significant cases. Among patients with restrictive perimembranous ventricular septal defects, who constituted 82.1% of the cohort, 23.3% experienced spontaneous closure, 22% underwent surgical or transcatheter intervention. Among patients with restrictive perimembranous ventricular septal defects and initial left ventricular end-diastolic diameter z -scores >+2, 91.1% demonstrated regression of left ventricular dilatation without the need for intervention. Conclusion: The majority of perimembranous ventricular septal defects, particularly restrictive types, follow a benign course with favourable remodelling and spontaneous resolution in many cases. Surgical or transcatheter intervention should be reserved for patients with significant hemodynamic compromise or valve involvement. Individualised, risk-based management strategies remain essential to optimise outcomes.