Intravascular ultrasound evaluation of pulmonary hypertension in high-altitude patients with ventricular septal defect: a case report.

Abstract

Background: High-altitude pulmonary hypertension is a rare but serious condition, particularly in individuals with congenital heart disease. Patent ductus arteriosus is a common congenital defect that may progress to irreversible pulmonary hypertension if untreated. However, operability assessment remains challenging in borderline cases. This case highlights the novel application of intravascular ultrasound to directly visualize pulmonary vascular remodeling in a patient with patent ductus arteriosus and high-altitude pulmonary hypertension, offering critical structural insight beyond conventional hemodynamic indices.

Case presentation: A 68-year-old Tibetan woman, a lifelong resident of the Garzê Tibetan Autonomous Prefecture in Sichuan, China (3500 m altitude), presented with a 2-year history of worsening dyspnea, leg swelling, and abdominal distension. Clinical evaluation revealed signs of right heart failure and a systolic heart murmur. Transthoracic echocardiography confirmed a large patent ductus arteriosus with bidirectional shunting and severe pulmonary hypertension. Chest computed tomography and angiography demonstrated dilated pulmonary arteries and pruning of distal branches. Right heart catheterization showed suprasystemic pulmonary artery pressure (130/55 mmHg), elevated pulmonary vascular resistance (15 Wood units), and a pathological pulmonary/systemic vascular resistance ratio of 0.92. Given the borderline operability, intravascular ultrasound was performed via femoral access and revealed severe medial hypertrophy (approximately 70% of wall thickness) and features suggestive of intimal dissection, consistent with irreversible vascular remodeling. The structural abnormalities visualized by intravascular ultrasound confirmed the contraindication to patent ductus arteriosus closure. Medical therapy with macitentan and tadalafil was initiated, alongside recommendations for altitude relocation. Follow-up at 3 months showed symptomatic improvement, although invasive reassessment was not yet performed.

Conclusion: This case demonstrates the first reported use of intravascular ultrasound to assess pulmonary artery structure in a patient with patent ductus arteriosus-associated pulmonary hypertension at extreme altitude. Intravascular ultrasound provided unique real-time insight into vascular remodeling that critically influenced clinical decision-making. It may serve as a valuable adjunct to standard diagnostics in high-risk or borderline operability cases, particularly in settings where histological evaluation is unfeasible. Broader use of intravascular ultrasound in congenital heart disease with pulmonary hypertension may enhance individualized risk assessment and optimize therapeutic strategies.