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A7020 - Significance of Pericardial Effusion in Pulmonary Arterial Hypertension
Author Block: A. Vaughn, Y. Segon, A. Rusk; Medical College of Wisconsin, Milwaukee, WI, United States.
Introduction: Pericardial effusions are a complication of pulmonary arterial hypertension (PAH) associated with increased morbidity and mortality. The pathogenesis and physiology of pericardial effusions in PAH are poorly understood and management is controversial.
Case: A 61-year-old woman presented to the hospital with shortness of breath, bilateral leg swelling, and cough. Her past medical history was significant for non-ischemic cardiomyopathy with baseline ejection fraction 35-40%, severe PAH in the setting of scleroderma, diabetes mellitus type 2, and mycobacterium avium complex pulmonary infection. Medications included sildenafil, macitentan, and trepostinil for PAH, metoprolol, spironolactone, and losartan for heart failure, and rifampin, ethambutol, and azithromycin for pulmonary infection. On arrival, oxygen saturation was 84% on room air and improved to 97% on 4 liters of oxygen. EKG was unchanged from prior, chest x-ray revealed stable cardiomegaly due to known pericardial effusion and unchanged bibasilar fibrosis, elevated B-type natriuretic peptide, normal CBC, and negative troponin. Patient’s hypoxia was determined to be from heart failure exacerbation and intravenous diuretics were initiated. Her course was complicated by periodic hypotension with worsening renal function and she was transferred to the intensive care unit. Dobutamine and norepinephrine were initiated without significant improvement in blood pressure. Echocardiogram showed concern for cardiac tamponade due to moderate pericardial effusion. Pericardiocentesis removed one liter of fluid but she remained hypotensive requiring pressors. Right heart catheterization was consistent with septic shock and she was subsequently found to have an E. coli urinary tract infection with sepsis. Patient improved with antibiotics and was weaned off pressors. She was transferred back to the medicine floor where she continued to improve from infectious and heart failure standpoints.
Discussion: Chronic pericardial effusions are relatively common in patients with PAH and are associated with increased morbidity and mortality. Prevalence of pericardial effusion varies from 15-65% depending on the type and severity of PAH. The pathogenesis of chronic pericardial effusion in this setting is poorly understood and management is controversial. It has been found that pericardial effusions are rarely of hemodynamic significance in patients with PAH, as is highlighted by this case. Tamponade depends on the rate of fluid accumulation within the pericardium, and given the chronic nature of fluid accumulation in PAH, it is less likely to cause tamponade physiology. Drainage of pericardial fluid is associated with poor outcomes in patients with PAH. Alternative measures such as diagnosis and treatment of concomitant infection and vasoactive therapy should be considered.