Case report
Successful Up-front Combination Therapy in a Patient With Idiopathic Pulmonary Hypertension and Patent Foramen Ovale: An Alternative to Epoprostenol Therapy?

https://doi.org/10.1016/j.healun.2009.03.009Get rights and content

Pulmonary arterial hypertension (PAH) is a life-threatening disease of the pulmonary arterioles, which, in the absence of effective therapy, progresses rapidly to right heart failure and death. Opening of a patent foramen ovale (PFO) is common in patients with severe pulmonary hypertension (PH), resulting in resistive hypoxemia. We report the case of a 40-year-old woman with idiopathic pulmonary hypertension (iPAH) in New York Heart Association (NYHA) class III to IV, who was admitted in the intensive care unit with hemodynamic compromise and severe hypoxemia due to right-to-left shunt throughout a PFO. Combination therapy initially with inhaled iloprost and sildenafil, and then addition of an endothelin A receptor–selective antagonist (sitaxsentan), resulted in impressive improvement in oxygenation with reversal of the right-to-left shunt and marked improvement in functional class of the patient.

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Case Report

A 40-year-old woman was admitted to the hospital with progressive dyspnea and chest pain on exertion during the last 12 months. On admission, she was tachypneic with central cyanosis and, on auscultation of the heart, the second heart sound was increased. Electrocardiogram showed right axis deviation and incomplete right bundle branch block. Chest X-ray indicated cardiomegaly with enlargement of the pulmonary conus and pulmonary arteries. Hematocrit was 53%, erythrocyte count was 6.60 × 106/μl

Discussion

To our knowledge, this is the first report of a patient with critical PH with PFO showing impressive clinical improvement and reversal of a right-to-left shunt with combination therapy as first-line treatment. Our therapeutic approach targeted both the acute vasodilator effects of combined oral sildenafil and inhaled iloprost, together with the long-term anti-remodeling effect of endothelin receptor antagonists.

In patients with advanced PH, and especially those with hemodynamic instability,

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