Pulmonary Arterial Hypertension With a Secundum Atrial Septal Defect: "Treat-and-Repair" Strategy
Abstract
Background: Pulmonary arterial hypertension (PAH) and congenital heart disease (CHD), particularly in pretricuspid shunts or Eisenmenger syndrome, are associated with significant mortality and limited therapeutic options.
Case summary: A 35-year-old woman presented with hypoxemia and right heart strain at 12 weeks' gestation. Echocardiography and right heart catheterization revealed severe PAH and a large secundum atrial septal defect (ASD). Following pregnancy termination and gradual initiation of triple pulmonary vasodilator therapy, her PAH improved significantly over 8 years. She underwent fenestrated ASD closure with significant improvement in functional status.
Discussion: This case illustrates a "treat-and-repair" strategy in PAH in CHD (PAH-CHD) and highlights how medical therapy can convert a previously inoperable patient into a procedural candidate.
Take-home messages: Combined pulmonary vasodilator therapy can improve PAH and make ASD closure in PAH-CHD feasible. A "treat-and-repair" strategy requires multidisciplinary care.
How strong is the evidence?
Evidence Hub reads this as a case report, done in people. Its evidence level is 5 of 5 (Very low · case / narrative / animal / in vitro / in silico). Case reports, narrative reviews, and animal, cell or computer-model studies give the weakest evidence for human health.
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