Atrial Septal Defect Closure With Persistently Elevated Pulmonary Vascular Resistance.

CLINICAL CONDITION: A 32-year-old man presented with a secundum atrial septal defect (ASD) complicated by pulmonary arterial hypertension (PAH) with elevated pulmonary vascular resistance (PVR), creating a major operability dilemma.

KEY QUESTIONS: What is the diagnostic work-up in suspected pulmonary hypertension? Should genetic testing be performed in congenital heart disease-associated PAH? How should ASD patients with elevated PVR be managed? Should closure be pursued despite persistent PVR elevation? What is the surgical strategy in this setting?

OUTCOME: PAH-targeted therapy improved symptoms and reduced PVR, although values remained elevated. After multidisciplinary reassessment, a "treat-to-repair" strategy with fenestrated surgical closure was pursued. Despite transient postoperative right ventricular dysfunction, the patient achieved marked clinical, echocardiographic, biomarker, and hemodynamic recovery.

TAKE-HOME MESSAGES: Operability in ASD-associated pulmonary hypertension requires individualized, multidisciplinary decision-making beyond fixed PVR cutoffs. Fenestrated ASD closure after targeted PAH therapy can balance shunt reduction and right ventricular protection in patients with elevated PVR.