Pulmonary Manifestations of Systemic Disease: Systemic Disease |

Hypoxaemia in a Portal Vein Thrombosis Patient FREE TO VIEW

Muhammad Ibrahim, MMed; Sazzli Shahlan Kassim, RCP; Soek-Siam Tan, RCP; Ahmad Ismail, CCP
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Respiratory Unit, Universiti Teknologi MARA, Sg Buloh, Malaysia

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

Chest. 2016;149(4_S):A475. doi:10.1016/j.chest.2016.02.494
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SESSION TITLE: Systemic Disease

SESSION TYPE: Case Report Poster

PRESENTED ON: Sunday, April 17, 2016 at 11:45 AM - 12:45 PM

INTRODUCTION: Hepatopulmonary syndrome (HPS) pulmonary complication of liver disease (LD) and/or portal hypertension (PH). HPS is diagnosed based on the presence of LD and/or PH, hypoxia and evidence of intrapulmonary shunting.1,2 Liver transplantation is currently the only effective treatment.1,3

CASE PRESENTATION: A 25-year-old female with liver cirrhosis complicated with portal vein thrombosis and PH (figure 1) presented with breathlessness on exertion over 1 year. On examination, the patient was cyanotic with presence of finger clubbing. She was hypoxic on room air with an SpO2 84%. Respiratory and cardiovascular examinations otherwise were unremarkable. ABG analysis showed type 1 respiratory failure (pO2 7.34 kPa). Erect PA chest x-ray was normal. Due to a history of thrombosis, a CTPA was performed but no abnormalities were found. Subsequent HRCT of the lung was also normal. Contrasted echocardiography was positive with bubbles noted in the left atrium and ventricle after 3 seconds of injection (figure 2). There was however no intracardiac shunt or anomalous pulmonary veins. A cardiac magnetic resonance study did not reveal any structural abnormality. CTA of pulmonary vasculature did not reveal any arterial-venous fistula or malformation. In view of the type 1 respiratory failure and right to left shunt without evidence of intracardiac lesions, a diagnosis of hepatopulmonary syndrome was made. She was started on LTOT and currently is on a waiting list for liver transplant.

DISCUSSION: The hypoxaemia in HPS results from a V/Q mismatch. Pulmonary vascular dilatations due to portal hypertension lead to over perfusion of the alveolar capillary bed with poor oxygenation of red blood cells. This V/Q mismatch is further exacerbated by blunted pulmonary vasoconstriction in poorly oxygenated areas of the lung. The diagnosis of HPS depends on establishing impaired gas exchange due to pulmonary vascular dilatations in a patient with liver disease. The diagnosis is based on arterial blood gases (PaO2 ≤ 80 mmHg or PA-aO2 ≥ 15 mmHg) and a study to detect intrapulmonary shunting (contrasted echocardiography). Patients with oxygenation of less than 60 mmHg will be prioritized for liver transplant.

CONCLUSIONS: HPS remains a fascinating pathophysiological entity of pulmonary complication in liver disease. Prompt recognition of this syndrome and timely referral are important in improving the patients' outcomes.

Reference #1: Grace JA, Angus PW. Hepatopulmonary syndrome: update on recent advances in pathophysiology, investigations, and treatment. Journal of Gastroenterology and Hepatology. 2013;28:213-219.

Reference #2: Khabbaza JE, Krasuski RA, Tonelli AR. Intrapulmonary shunt confirmed by intracardiac echocardiography in the diagnosis of hepatopulmonary syndrome. Hepatology. 2013;58:1514-1515.

Reference #3: Rollan MJ, Munoz AC, Perez T, et al. Value of contrast echocardiography for the diagnosis of hepatopulmonary syndrome. European Journal of Echocardiography. 2007;8:408-410.

DISCLOSURE: The following authors have nothing to disclose: Muhammad Ibrahim, Sazzli Shahlan Kassim, Soek-Siam Tan, Ahmad Ismail

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