SWAN-GANZ AND INTRA-PERICARDIAL PRESSURE GUIDED PERICARDIOCENTESIS IN SCLERODERMA-ASSOCIATED PAH

Abstract

TOPIC: Pulmonary Vascular Disease TYPE: Medical Student/Resident Case Reports INTRODUCTION: Large pericardial effusion represents a management dilemma in patients with pulmonary arterial hypertension (PAH). In PAH, the elevated right sided pressures can mask several classic signs of cardiac tamponade. Furthermore, therapeutic drainage can be associated with significant mortality. Here, we present a case of a Swan Ganz and pericardial pressure guided pericardiocentesis in a patient with scleroderma-associated PAH with tamponade physiology. CASE PRESENTATION: The patient is a 57-year-old female with scleroderma-associated PAH (group I). Upon diagnosis, her hemodynamics on RHC were consistent with severe PAH (mRAP 14 mmHg, mPAP 63 mmHg, PAWP 16 mmHg, CO 4.62 L/min, CI 2.49 L/min/m2, and PVR 10.17 WU). She was started on triple combination therapy including intravenous epoprostenol with noted hemodynamic improvement upon repeat RHC (mRAP 12 mmHg, mPAP 34 mmHg, PAWP 14 mmHg, CO 6.14 L/min, CI 3.47 L/min/m2, and PVR 3.25 WU). After maintaining low risk status for 2 years, the patient presented with increased dyspnea on exertion and leg swelling. Cardiac echocardiography showed large pericardial effusion with tamponade physiology. The decision was made for Swan-Ganz and pericardial pressure guided pericardiocentesis via subxiphoid approach. The pericardial pressure was elevated at 20 mmHg with mRAP of 22 mmHg, RVEDP of 22 mmHg, and PAWP of 25 mmHg. Gradual draining of the pericardial fluid (in about 300 cc aliquots) was performed guided by the Swan-Ganz and the pericardial pressure monitoring with serial pressures and cardiac output measurements during the drainage with an eventual total removal of 1100 cc straw-colored fluid. Repeat hemodynamics before removal of the Swan-Ganz and the pericardial drain showed intra-pericardial pressure of 3 mmHg, mRAP 5 mmHg, mPAP 42 mmHg, PAWP 15 mmHg, CO 7.4 L/min, CI 4.3 l/min/m2, and PVR 3.6 WU. DISCUSSION: In PAH patients, the development of pericardial effusion carries poor prognosis. The pericardial effusion can progress into pericardial tamponade that requires drainage. Rapid pericardial drainage in setting of PAH can be associated with hemodynamic collapse and high mortality. It is hypothesized that rapid fluid removal can result in an increase in both right ventricular transmural pressure and venous return in setting of an already compromised right ventricle resulting in further RV dilation and failure with reduction of cardiac output and hemodynamic collapse. During the drainage, we removed small amounts of fluid at a time with repeat pressures and thermodilution cardiac outputs after drainage of each aliquot. CONCLUSIONS: The dual approach of Swan-Ganz and pericardial pressure guided drainage of pericardial effusion can be considered in PAH patients presenting with cardiac tamponade. REFERENCE #1: Ruopp NF, Schoenberg NC, Farber HW. Swan-Ganz and Pericardial Pressure-guided Pericardiocentesis in Pulmonary Arterial Hypertension-associated Cardiac Tamponade. Ann Am Thorac Soc. 2019 Sep;16(9):1189-1191. doi: 10.1513/AnnalsATS.201902-127CC. PMID: 31469306. REFERENCE #2: Sahay S, Tonelli AR. Pericardial effusion in pulmonary arterial hypertension. Pulm Circ. 2013;3(3):467-477. doi:10.1086/674302 DISCLOSURES: Speaker/Speaker's Bureau relationship with United therapeutics Please note: $5001 - $20000 by Karim El-Kersh, source=Web Response, value=Honoraria Removed 04/21/2021 by Karim El-Kersh, source=Web Response Advisory Committee Member relationship with United therapeutics Please note: $1001 - $5000 by Karim El-Kersh, source=Web Response, value=Consulting fee Removed 04/21/2021 by Karim El-Kersh, source=Web Response Advisory Committee Member relationship with Actelion Please note: $5001 - $20000 by Karim El-Kersh, source=Web Response, value=Consulting fee Removed 04/21/2021 by Karim El-Kersh, source=Web Response Advisory Committee Member relationship with United Therapeutics Please note: Current Added 04/23/2021 by Karim El-Kersh, source=Web Response, value=Consulting fee Advisory Committee Member relationship with Actelion Please note: 2019 and 2020 Added 04/23/2021 by Karim El-Kersh, source=Web Response, value=Consulting fee Speaker/Speaker's Bureau relationship with United Therapeutics Please note: Current Added 04/29/2021 by Karim El-Kersh, source=Web Response, value=Honoraria Advisory Committee Member relationship with United Therapeutics Please note: Current Added 04/29/2021 by Karim El-Kersh, source=Web Response, value=Honoraria Advisory Committee Member relationship with Actelion Please note: 2019 and 2020 Added 04/29/2021 by Karim El-Kersh, source=Web Response, value=Honoraria Consultant relationship with Acceleron Pharma Please note: Current Added 04/29/2021 by Karim El-Kersh, source=Web Response, value=Consulting fee No relevant relationships by Matthew Taylor, source=Web Response No relevant relationships by Marianna Weaver, source=Web Response