SESSION TITLE: Pulmonary Vascular Disease Student/Resident Case Report Posters I
SESSION TYPE: Medical Student/Resident Case Report
PRESENTED ON: Tuesday, October 28, 2014 at 01:30 PM - 02:30 PM
INTRODUCTION: The incidence of Pericardial effusion (PCE) in Pulmonary Artery Hypertension (PAH) is 15-65% Compared to PCE from other conditions, presence of PAH significantly alters the echocardiographic and hemodynamic manifestations of cardiac tamponade1,2. Furthermore, drainage of PCE in the presence of severe PAH, is associated with a high mortality due to right ventricular decompensation3.
CASE PRESENTATION: A 33-year-old Caucasian female with Class 1 Idiopathic PAH managed on tadalafil, ambrisentan, treposteionil, digoxin and diuretics presented with progressive dyspnea and orthopnea of two-week duration. Cardiac examination revealed hypotension, tachycardia, pulsus paradoxus, jugular venous distension, muffled heart sounds, loud P2 and holo-systolic murmur at left-lower sternal border. TTE demonstrated a large circumferential PCE with diastolic left atrial (LA) collapse and uncollapsed right atrium (RA). Right Ventricle (RV) was dilated with systolic dysfunction and severe tricuspid regurgitation. RV systolic pressure was 170 mmHg and inferior vena cava was dilated without respiratory variation. Urgent pigtail catheter placement and drainage of 200mL provided hemodynamic improvement. Concerns for RV decompensation precluded rapid drainage, with 2.3L drained over 15 days with gradual LA filling improvement. Serological and microbiological studies were unrevealing and patient was referred for pulmonary rehabilitation, pending heart-lung transplantation.
DISCUSSION: Postulated etiopathogenic mechanisms of PCE in PAH include venous/lymphatic obstruction due to elevated RA/RV pressures with consequent cytokine release1,3. Large PCE present with atypical TTE signs of tamponade due to high RA pressures and can demonstrate isolated LA/LV collapse. Respiratory variation in trans-mitral and trans-tricuspid flows can be unreliable2. Rapid drainage can result in RV ballooning, hemodynamic instability and death1,3,4. Despite high mortality with drainage, isolated reports suggest that gradual drainage may be safer, resulting in hemodynamic improvement.
CONCLUSIONS: This report highlights that gradual drainage of PCE in severe PAH using a pericardial catheter can be hemodynamically well tolerated and should be the preferred method in patients with cardiac tamponade and severe PAH.
Reference #1: 1. Shimony A, Fox BD, Langleben D et al. Incidence and significance of pericardial effusion in patients with pulmonary arterial hypertension. Can J Cardiol. 2013 Jun;29(6):678-82.
Reference #2: 2. Habib G, Torbicki A. The role of echocardiography in the diagnosis and management of patients with pulmonary hypertension. Eur Respir Rev. 2010 Dec;19(118):288-99
Reference #3: 3. Hemnes AR, Gaine SP, Wiener CM. Poor outcomes associated with drainage of pericardial effusions in patients with pulmonary arterial hypertension. South Med J. 2008 May;101(5):490-4. 4. Honeycutt GR, Safdar Z. Pulmonary hypertension complicated by pericardial effusion: a single center experience. Ther Adv Respir Dis. 2013 Jun;7(3):151-9.
DISCLOSURE: The following authors have nothing to disclose: Saraschandra Vallabhajosyula, Swapna Kanuri, Pranathi Sundaragiri, Venkata Alla
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