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A7016 - A High Pressure Situation: Pulmonary Arterial Hypertension and a Large Pericardial Effusion
Author Block: M. Zhang1, M. Petersile1, N. Ruopp2, H. W. Farber3; 1Internal Medicine, Boston Medical Center, Boston, MA, United States, 2Pulmonary/Critical Care, Boston, MA, United States, 3Boston Univ Sch of Med, Boston, MA, United States.
Introduction: The prevalence of pericardial effusion in patients with pulmonary arterial hypertension (PAH) is thought to be 13-29%, with a higher prevalence in patients with PAH secondary to connective tissue disease (CTD), and is associated with a high mortality even in the absence of cardiac tamponade. We present a case of severe PAH, systemic lupus erythematosus (SLE), and a large pericardial effusion that improved with medical management alone.
Case: A 36-year-old female presented with severe dyspnea and fatigue. Chest x-ray was remarkable for an enlarged cardiac silhouette and exam was consistent with right heart failure. A transthoracic echocardiogram (TTE) showed a large circumferential pericardial effusion measuring 2.2cm with a severely dilated right atrium (RA) and right ventricle (RA) without evidence of collapse. Right heart catheterization (RHC) revealed RA mean of 18mmHg, mean pulmonary artery pressure of 57mmHg, normal pulmonary capillary wedge pressure with a cardiac index of 2.2L/min/m2, and pulmonary vascular resistance of 15 Wood units without evidence of tamponade physiology. Subsequent evaluation revealed SLE and, thus, severe CTD-PAH. The patient was initiated on epoprostenol for PAH, as well as hydroxychloroquine and dexamethasone for SLE. Repeat TTE three weeks later showed significant improvement of the pericardial effusion (1.2cm). The patient remained hemodynamically stable throughout her course.
Discussion: Management of large pericardial effusions in the presence of PAH remains controversial. There are no clear guidelines on the management of large pericardial effusions in the absence of cardiac tamponade. Previous 2004 guidelines recommended drainage for pericardial effusions >2cm, even without evidence of tamponade. However, one series of six patients with PAH demonstrated high mortality following drainage of pericardial fluid; in contrast, a study of 14 patients with PAH demonstrated no peri-procedural mortality following pericardiocentesis. It has been hypothesized that the increased intrapericardial pressure conferred by a large effusion protects against RV dilation, and that death following pericardial drainage results from acute RV volume and pressure overload. Furthermore, there is little data guiding management of pericardial effusions in PAH associated with CTD given the possibility of a serositis in these patients. Ultimately, because our patient had no hemodynamic evidence of tamponade physiology, periocardiocentesis was not pursued. With medical management of both PAH and SLE, the pericardial effusion improved substantially. Our case emphasizes the complexity of managing a large pericardial effusion in CTD-PAH and demonstrates a successful outcome with conservative medical management.