Ahmed Kheiwa, Vandad Saadat, Joseph Chung, Stephen Nageotte, Anees Razzouk
Loma Linda University Medical Center.
European Heart Journal Case Reports
Eur Heart J Case Rep 2025; 9:
DOI: 10.1093/ehjcr/ytaf527
Abstract
Background: Pulmonary hypertension is often a difficult to diagnose condition, in particular in younger population of patients. Early diagnosis and treatment of this condition is crucial to prevent further morbidity and mortality.
Case summary: A 32-year-old woman with a history of Ebstein anomaly (EA) and secundum atrial septal defect (ASD), who underwent transcatheter ASD closure at age 19, presented with progressive fatigue and exertional dyspnoea. Further evaluation revealed presence of compression of right pulmonary veins (rPVs) by the ASD device resulting in post-capillary pulmonary hypertension (PHTN) and worsening of right ventricular (RV) failure. Following surgical explantation of the ASD device, PHTN resolved with improvement of patient’s functional status.
Discussion: This case depicts a rare haemodynamic entity of worsening PHTN in a 32-year-old patient with EA post-ASD closure. It illustrates a rare complication of compression of rPVs by the ASD device. Therefore, suspicion should be high for evaluating rPVs in EA with worsening PTHN and RV failure post-ASD closure.
Category
Class II. Pulmonary Hypertension Associated with Pulmonary Vein Stenosis
Age Focus: Adult Pulmonary Vascular Disease
Fresh or Filed Publication: Fresh (PHresh). Less than 1-2 years since publication
Article Access
Free PDF File or Full Text Article Available Through PubMed or DOI: Yes