Preliminary study of individualized therapy for ventricular septal defect with severe pulmonary arterial hypertension

BACKGROUND: Pulmonary arterial hypertension (PAH) is a serious complication of congenital heart disease (CHD) with a left-to-right shunt, particularly ventricular septal defect (VSD). In patients presenting with severe PAH, therapeutic decision-making remains challenging because precise and individualized assessment criteria are lacking. In current clinical practice, treatment strategies are often based mainly on isolated hemodynamic parameters obtained by right heart catheterization (RHC), which may not fully reflect disease severity or reversibility. OBJECTIVES: ), pulmonary vascular resistance (PVR), pulmonary-to-systemic blood flow ratio (Qp/Qs), and pulmonary-to-systemic pressure ratio (Pp/Pa). RESULTS: exceeded 93%. The proposed scoring system was associated with observed treatment patterns: patients with total scores ≥ 60 were predominantly managed surgically (with or without preoperative medical therapy), whereas those with scores <40 were mainly treated with medical therapy alone (93.7%). Intermediate score ranges were associated with mixed treatment patterns. CONCLUSIONS: with RHC-derived parameters provides a more comprehensive and individualized assessment of disease severity in VSD-PAH than hemodynamics alone. The proposed multidimensional scoring system may provide a hypothesis-generating framework for cardiovascular specialists.