Case reports
Reversal of pulmonary hypertension and subsequent repair of atrial septal defect after treatment with continuous intravenous epoprostenol

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Abstract

We report the first case in the world literature of a patient with an atrial septal defect, severe pulmonary hypertension, and equalization of pulmonary and systemic pressures, who underwent successful closure of an ASD following prolonged therapy with the intravenous vasodilator epoprostenol. Judicious use of continuous prostacyclin in apparently inoperable patients with congenital heart disease may be associated with significant reversal of pulmonary hypertension, and conversion to an operable state.

Section snippets

Case report

The patient was a 29-year-old Latin American woman who received a diagnosis of primary pulmonary hypertension when referred for consideration of lung transplantation to this center 15 years ago (1989) for symptoms of increasing shortness of breath. A heart murmur present at birth had disappeared shortly thereafter; the patient worked full time and successfully underwent 2 pregnancies and normal vaginal deliveries. Right and left heart catheterizations performed before referral were repeated

Discussion

This patient had an undiagnosed ostium secundum ASD with equalization of pressures, or possibly a small right-to-left shunt from time of earliest presentation. The lack of appropriate diagnosis in spite of several heart catheterizations by several cardiologists reflects the difficulty with diagnosis in the presence of pressure equalization and the superiority of currently available and more sophisticated tools for evaluating patients, such as improvements in transthoracic and transesophageal

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Cited by (56)

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    Theoretically, patients who demonstrate a dramatic response to therapy could be operated on in an attempt to avoid further progression of the disease by abolishing the shunt. While there are case reports of good short-to-mid-term outcomes for this strategy [66–69], the long-term impact of such an approach remains unknown. This uncertainty is reflected in the latest consensus from the 2013 World Congress on Pulmonary Hypertension in Nice, which recommended that even patients with mild elevation of pulmonary vascular resistance (2.3–4.6 Wood units) should be evaluated in tertiary centres, where the pros and cons of a potential defect closure can be properly considered and discussed with the individual patient [70].

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