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

TL;DR

A 32-year-old woman with Ebstein anomaly and a history of ASD closure developed reversible pulmonary hypertension due to device-related vein compression.

Contribution

This case highlights a rare complication of ASD device compression on pulmonary veins in Ebstein anomaly patients.

Findings

Pulmonary hypertension resolved after surgical removal of the ASD device.

Compression of right pulmonary veins was identified as the cause of worsening right ventricular failure.

Early suspicion of vein compression is crucial in EA patients with post-ASD PHTN.

Abstract

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.

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.

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.

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