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Secondary pulmonary hypertension in an adult patient with Ebstein anomaly post-atrial septal defect closure; can it be reversible? A case report

Year: 2025

Type: article

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.

Source: European Heart Journal - Case Reports

DOI: 10.1093/ehjcr/ytaf527

Authors: Ahmed Kheiwa, Vandad Saadat, Joseph Chung, Stephen Nageotte, Anees Razzouk

Institutions: Loma Linda University Medical Center, Loma Linda University Health Care

Language: English

Topic: Congenital Heart Disease Studies

Subfield: Epidemiology

Field: Medicine

Domain: Health Sciences

SDG: Good health and well-being

Open Access status: gold

Estimated APC paid: $815