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 history of Ebstein anomaly (EA) and secundum atrial septal defect (ASD), underwent transcatheter ASD closure at age 19, presented with progressive fatigue and exertional dyspnea. 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 hemodynamic entity of worsening PHTN in a 32-year-old patient with Ebstein anomaly 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 Ebstein anomaly with worsening PTHN and RV failure post ASD closure.
Kheiwa et al. (Tue,) studied this question.
Synapse has enriched 5 closely related papers on similar clinical questions. Consider them for comparative context: