Case reportsReversal of pulmonary hypertension and subsequent repair of atrial septal defect after treatment with continuous intravenous epoprostenol
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)
Pulmonary arterial hypertension associated with congenital heart disease: Recent advances and future directions
2014, International Journal of CardiologyCitation Excerpt :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].
Hemodynamics of patients developing pulmonary arterial hypertension after shunt closure
2013, International Journal of CardiologyThe role for cardiopulmonary exercise testing in patients with atrial septal defects: A review
2012, International Journal of CardiologyCongenital Heart Disease and Pulmonary Hypertension
2012, Heart Failure ClinicsCitation Excerpt :Intravenous epoprostenol (Flolan) is the most extensively studied medication in patients with PAH-CHD, particularly in patients with Eisenmenger syndrome.134,135 Several small studies have shown that continuous intravenous epoprostenol used in patients with Eisenmenger syndrome significantly improved function class, oxygen saturation, and exercise capacity and decreased PVR.134–137 Treprostinil (Remodulin) is another effective therapy with an acceptable safety profile in patients with PAH.