Hypoplastic right-heart syndrome presenting as multiple miscarriages

Reversible causes of miscarriage are many, but they affect only 1% of women who are trying to conceive. Herein, we describe the case of a 23-year-old woman who presented for evaluation of repeated miscarriages and was found to have hypoxemia and erythrocytosis. Further evaluation revealed hypoplastic right-heart syndrome with an intracardiac shunt. She underwent hybrid repair with pulmonary valve balloon valvuloplasty, followed by surgery to perform atrial septal defect closure and a Glenn anastomosis. The erythrocytosis and hypoxemia resolved, and she was able to conceive and deliver a healthy baby at term 2 years later. This is a unique case of a rare congenital heart defect that went unnoticed until adulthood, when attempts at pregnancy failed because of the associated hypoxemia. Timely and appropriate treatment led to a successful pregnancy after repeated miscarriages. This case exemplifies the need for a comprehensive medical evaluation of every woman with a history of multiple miscarriages to determine whether a reversible cause exists.