CRA49 A MULTIFACETED APPROACH TO DYSPNEA IN CHRONIC LIVER CIRRHOSIS

Authors: Azyan Syamimi khalid1, Yen Shen Wong1, Aisya Natasya Musa1, Fatimah Zaharah Rosli2
1Faculty Of Medicine, Universiti Teknologi Mara (UiTM) Sg Buloh, Selangor, Malaysia
2Pulmonology Department, Sultan Idris Shah Serdang Hospital, Selangor, Malaysia

Introduction 

Hepatopulmonary syndrome (HPS) and portopulmonary hypertension (PoPH) are significant pulmonary vascular complications in advanced liver disease, each with distinct pathophysiology and management1. Here, we present two cases of chronic liver disease patients experiencing unexplained dyspnea despite normal High-resolution computed tomography (HRCT) thorax findings. 

Case report

The first case involved a 75-year-old gentleman with hepatitis C and Child A liver cirrhosis, who presented with progressive exertional dyspnea for three years. HRCT thorax revealed no significant lung pathology, but echocardiogram revealed raised estimated pulmonary arterial systolic pressure, alongside dilated right atrium and right ventricle. Right heart catheterization (RHC) confirmed pre-capillary pulmonary hypertension, with an elevated mean pulmonary artery pressure (mPAP) recorded at 72mmHg. He was managed as liver cirrhosis with portal hypertension and started on Bosentan due to elevated pro-BNP levels.

In the second case, a 41-year-old woman with liver and bone marrow sarcoidosis, confirmed by liver biopsy, presented with worsening dyspnea. Despite being on low-dose prednisolone and azathioprine, full lung function testing showed normal spirometry but reduction in DLCO. CT thorax demonstrated no evidence of lung sarcoidosis and transthoracic echocardiogram findings revealed normal ejection fraction and pulmonary arterial systolic pressure. A targeted echocardiogram with bubble test yielded positive findings, indicating the presence of intrapulmonary shunting. Consequently, she was diagnosed with hepatopulmonary syndrome and considered for liver transplant.

Discussion 

This review offers practical insights into managing PoPH and HPS. Recognizing these differences is essential for tailored patient care and optimal outcomes in chronic liver disease-associated pulmonary complications.

Keywords:

Hepatopulmonary syndrome, portopulmonary hypertension

Raevens S, Geerts A, Van Steenkiste C, Verhelst X, Van Vlierberghe H, Colle I. Hepatopulmonary syndrome and portopulmonary hypertension: recent knowledge in pathogenesis and overview of clinical assessment. Liver International. 2015 Jun;35(6):1646-60.