INTRODUCTION: For patients with a moderate-to-large ventricular septal defect (VSD), surgical repair is recommended in infancy or childhood to prevent pulmonary hypertension (PH) and subsequent right-to-left shunting (Eisenmenger Syndrome, ES). Historically, adults with an unrepaired VSD and ES have a poor prognosis as the operative mortality and risk of post-operative PH prevents surgical correction. We describe an adult with ES who was treated pre-operatively with epoprostenol and ultimately underwent successful surgical repair of his VSD as well as aortic valve replacement (AVR).
CASE PRESENTATION: A 44-year-old man with shortness of breath, hypoxemia, and VSD was referred to our hospital for consideration for heart transplantation. The VSD had been diagnosed during infancy, but had never been surgically corrected. Throughout childhood the patient had been asymptomatic. One year prior to presentation he had been diagnosed with bronchiolitis obliterans-organizing pneumonia (BOOP). He was treated with steroids with improvement in his symptoms, however he continued to require supplemental oxygen. After four months of steroid therapy the patient began to notice worsening dyspnea and hypoxemia in the absence of radiographic changes. At this point he was referred to our institution. At presentation the patient was New York Heart Association (NYHA) functional class 3. An echocardiogram revealed severe mitral regurgitation, severe aortic regurgitation, a dilated left ventricle, a dilated right ventricle with normal systolic function, and a mid-septal muscular 10 mm VSD. Right heart catheterization (RHC) revealed a pulmonary artery pressure (PAP) of 107/36 mm Hg (mean 64) and a wedge pressure of 33 mm Hg. The calculated pulmonary vascular resistance (PVR) was 6.05 U/m2. After inhaled nitric oxide (NO) the PAP decreased to 78/26 mm Hg (mean 43). Examination of oxygen saturations on room air revealed significant hypoxemia as well as right-to-left shunting. In an effort to decrease the PVR and therefore right-to-left shunting, the patient was started on epoprostenol. He noted an almost immediate improvement in symptoms. Repeat RHC one month later revealed a decrease in the PVR to 1.55 U/m2 and reversal of right-to-left shunting. Four months later he underwent cardiac surgery for VSD repair, mitral valve repair and AVR. Post-operatively he temporarily required inhaled NO for elevated PAPs. After the inhaled NO was stopped, sildenafil was started. One month after surgery the patient was NYHA class 1 and no longer required supplemental oxygen.
DISCUSSIONS: In this case we describe the use of epoprostenol in a patient with ES prior to surgical correction of a VSD. The use of prostacyclins in patients with PH associated with congenital heart disease (CHD) has been previously described. To our knowledge, however, only one case report has been published describing the use of prostacyclin in a patient with severe PH prior to surgical repair of CHD. In that report, the patient received epoprostenol for four years prior to the diagnosis of an atrial septal defect (ASD). After repair of the ASD, the patient was weaned off epoprostenol over the next six months. In contrast, our experience suggests a shorter duration of epoprostenol pre-operatively may be sufficient to allow surgical repair of CHD with ES, and post-operatively epoprostenol may be unnecessary.
CONCLUSION: In adult patients with uncorrected CHD and ES, short-term pulmonary vasodilator therapy prior to surgery may allow a subset of patients to undergo surgical repair of CHD who would otherwise not be surgical candidates.
DISCLOSURE: Sarah Schmidt, None.