INTRODUCTION: Pericardial effusion (PEf) is a feature of pulmonary arterial hypertension (PAH) resulting in a poor prognosis with significant mortality especially when drained. The physiology of Pef formation and its affect in patients with right ventricular (RV) failure is not completely understood. There are no clear strategies for management of PEf secondary to PAH.
CASE PRESENTATION: 46 year old African- American female presents with 1 week history of chest pain and dyspnea. The pain is non-radiating, sharp and intermittent occurring 2-3 times a day and is worse with exertion and improves on leaning forward. Her exercise tolerance is now limited to 15 feet. She has a 10 Ib weight loss, fatigue and decreased appetite with tightening and swelling of arms and legs for the last 2-3 months. She had an asymptomatic PEf diagnosed 2 years ago. She presents with stable vitals, 'fish mouth' appearance of face, diffuse skin tightening, raised jugular venous distension, distant heart sounds and clear lung fields. Elevated ANA with 1: 5120 titer and positive Smith antibodies are found. Chest X-ray and echocardiogram (TTE) are consistent with a large PEf; hyperdynamic left heart and estimated pulmonary artery systolic pressure of 100 mmHg with moderately dilated RV and right atrium (RA) and no tamponade physiology. A pericardial window is performed with 1200 ccs of straw-colored fluid drainage with subsequent PEA arrest and death.
DISCUSSION: Presence of PEf in PAH is an indicator of right ventricular decompensation. Patients with PEf have even higher right atrial pressure (RAP) when compared to those without PEf in PAH. Miller et al hypothesize that elevated RAP contributes to the development of PEf by impeding the myocardial lymphatic drainage or by passive transudative process resulting from increased pressures in RV. Our patient was very symptomatic and there was no tamponade physiology described on the TTE. However, the absence of tamponade physiology with severe PAH is misleading due to the lack of collapse of RA and/or RV secondary to elevated right sided filling pressures. We performed a pericardiocentesis to relieve symptoms but this led to mortality. This is in contrast to the usual treatment for tamponade where drainage of minimal amount of fluid results in rapid clinical improvement. Krikorian and Hancock report 2 deaths among 123 patients undergoing pericardiocentesis. Hemnes et al also report three mortalities in their retrospective case series of six patients where three patients with PAH died after pericardial fluid drainage. It is possible that the presence of PEf in PAH is a sign of end- stage RV failure. Perhaps, the removal of large amounts of pericardial fluid from an over-distended right heart results in loss of RV muscle tone with interventricular septal bowing and decreased left heart filling pressures, causing death. Our case highlights the importance of appropriate initial interventions in patients with PAH and large PEf.
CONCLUSIONS: Awareness of increased mortality associated with drainage of chronic pericardial effusions that are seen in the context of PAH is imperative. Close hemodynamic monitoring may help identify the physiological effects on the RV after drainage of pericardial fluid. Chronic pericardial effusions associated with severe PAH are best managed medically; perhaps with early prostacyclin use. If attempted, pericardiocentesis should be done very slowly with drain in-situ.
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Reference #2 Krikorian JG, Hancock WE. Pericardiocentesis. Am J Med 1978;65 : 8 -814.
Reference #3 A.J. Miller et al. The production of acute pericardial effusion: the effects of varying degrees of interference with venous blood and lymph drainage from the heart muscle in the dog. Am J Cardiol 28 (1971), pp. 463-466.
DISCLOSURE: The following authors have nothing to disclose: Tabassum Nafsi, Suraj Bopanna, Abhishek Vedavalli, Kalil Al-Nassir, Rajive Tandon
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