JACC. Case reports · 2026
Pulmonary Arterial Hypertension With a Secundum Atrial Septal Defect: "Treat-and-Repair" Strategy.
Клинический случайСтатьяНазвание и аннотация: Перевод в очереди
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Haile Hanna, Malkani Kabir, Satish Mohan, Yuan Su, Kim Luke, Feldman Dmitriy, Bergman Geoffrey, Horn Evelyn, Karas Maria, Singh Harsimran
Аннотация
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.
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.