Pulmonary Vascular Disease: Student/Resident Case Report Poster - Pulmonary Vascular Disease I |

Newly Diagnosed PAH After Orthotopic Liver Transplant FREE TO VIEW

Scott Helgeson, MD; J Saadi Imam, MD; Charles Burger, MD
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Mayo Clinic Florida, Jacksonville, FL

Copyright 2016, American College of Chest Physicians. All Rights Reserved.

Chest. 2016;150(4_S):1208A. doi:10.1016/j.chest.2016.08.1317
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SESSION TITLE: Student/Resident Case Report Poster - Pulmonary Vascular Disease I

SESSION TYPE: Student/Resident Case Report Poster

PRESENTED ON: Tuesday, October 25, 2016 at 01:30 PM - 02:30 PM

INTRODUCTION: Pulmonary arterial hypertension (PAH) is a known complication of liver failure due to portopulmonary hypertension (PoPH). While unusual, PAH has manifested after orthotopic liver transplant (OLT). The current case developed PAH following an OLT that was successfully treated with medications.

CASE PRESENTATION: A 66-year-old woman with history of liver cirrhosis underwent a duct-to-duct biliary anastomosis OLT. Pre-operative autoimmune serology showed an elevated C4 complement of 4.9mg/dL, quantitative C-reactive protein of 46.8mg/dL, and rheumatoid factor of 55IU/mL. An echocardiogram showed an ejection fraction of 72%, normal left and right ventricular (RV) size and wall motion, right atrial pressure (RAP) 5mmHg, right ventricular systolic pressure (RVSP) 23mmHg, and mean pulmonary artery pressure (MPAP) 14mmHg. Saline contrast revealed delayed right-to-left shunt suggestive of intrapulmonary source. Arterial blood gas shunt study revealed calculated shunt fraction of 5%; therefore, mild hepatopulmonary syndrome (HPS) was diagnosed. Pulmonary function testing showed normal spirometry with diffusion capacity of 41% predicted. CT did not show fibrosis, discrete arteriovenous malformations, or chronic thromboembolic disease. She had an uncomplicated OLT, but two months after later she developed severe dyspnea and syncopal episodes. Liver function tests were normal and an echocardiogram revealed severe RV enlargement and hypokinesis. A right heart catherization showed a RAP 3mmHg, MPAP 35mmHg, wedge 4mmHg, transpulmonary gradient 31mmHg, cardiac output 3.1L/min, and a calculated pulmonary vascular resistance (PVR) of 827 dyne*sec cm-5. She declined prostanoid therapy; therefore, she was initiated on sildenafil, ambrisentan, and inhaled treprostinil. The patient’s symptoms dramatically improved and her most recent echocardiogram showed normal RV size and function, RAP 5mmHg, RVSP 18mmHg, and MPAP 15mmHg.

DISCUSSION: Group 1 PAH in association with liver disease and portal hypertension is typically diagnosed prior to OLT. It is unusual to manifest after an uncomplicated OLT with preserved liver function. The patient had mild HPS which may have masked a co-existing component of pulmonary vascular hypertensive vasculopathy. One might speculate that resolution of the pulmonary artery vasodilation with successful OLT resulted in a preponderance of narrowed pulmonary arteries with elevated PVR. Associated PAH in the setting of liver disease has a relatively poor prognosis compared to other subtypes of PAH; therefore, combination PAH treatment was initiated upfront.

CONCLUSIONS: This case reinforces the need for an elevated index of suspicion for the development of PAH after OLT and appreciate the potential for favorable response treatment with PAH therapy.

Reference #1: Koch DG, Caplan M, Reuben A. Pulmonary hypertension after liver transplantation: case presentation and review of the literature. Liver Transpl 2009; 15: 407-12

DISCLOSURE: The following authors have nothing to disclose: Scott Helgeson, J Saadi Imam, Charles Burger

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