INTRODUCTION: Pulmonary syndromes in the setting of hepatic disease with portal hypertension include Portopulmonary hypertension (POPH), Hepatopulmonary syndrome (HPS) and hepatic hydrothorax. POPH is defined as pulmonary arterial hypertension with portal hypertension in the absence of other causes of pulmonary arterial hypertension. HPS is a defect in arterial oxygenation as a result of pulmonary micro vascular dilatation in the setting of liver disease. The coexistence of POPH and HPS is rare but has been reported previously. So far there has been only one case report where HPS and POPH were diagnosed simultaneously
CASE PRESENTATION: A 63-year-old woman came to the emergency department complaining of a one month history of exertional pre-syncope with progressive dyspnea on exertion and leg swelling .She had a past medical history of hepatitis C diagnosed four years prior and could not tolerate pegylated interferon. She was found to be severely hypoxemic with a PaO2 of 53mmHg while breathing room air. Her chest radiograph revealed mild cardiomegaly without any evidence of pulmonary edema. High resolution computed tomography showed a focal area of linear opacities within the right lower lobe in a reticular pattern with honeycombing and associated septal thickening. Laboratory data was indicative of chronic liver disease, including a bilirubin of 2.8, an INR of 1.2, an albumin of 2.8, and a platelet count of 63,000.Abdominal ultrasound showed features suggestive of portal hypertension (ascites, splenomegaly). Transthoracic echocardiogram revealed an elevated right ventricle systolic pressure (49 mmHg) accompanied by a mildly dilated right atrium and right ventricle with normal left ventricular function. POPH was suspected as the etiology of her worsening shortness of breath. Right heart catherization confirmed pulmonary arterial hypertension (PAH). Investigation of other causes of pulmonary hypertension was unrevealing.HPS was suspected as the etiology of her hypoxemia. A contrast echocardiogram was done showing the appearance of micro bubbles in the left ventricle approximately 6 beats after their appearance in the right ventricle supporting the diagnosis of HPS.
DISCUSSION: The pathophysiology of both HPS and POPH is not clearly understood. It is a clinical paradox that both HPS and POPH coexist as the underlying mechanisms of the two diseases states are opposite. Dyspnea on exertion is the most common symptom of POPH. Increased pulmonary artery pressure (PAP) seen on Doppler echocardiography in a patient with portal hypertension is an important clue towards the diagnosis of POPH. The diagnosis of POPH is made by right heart catherization .The presence of hypoxemia in a patient with portal hypertension should raise the suspicion of HPS. Contrast echocardiography is a vital diagnostic tool for HPS. POPH and HPS also differ in terms of their treatment options. Pulmonary vasodilatation is the mainstay of treatment in POPH. The treatment of HPS includes correction of hypoxemia by oxygen and liver transplantation. The treatment strategy in a patient who presents with both HPS and POPH is challenging. Liver transplantation is required for HPS but the presence of POPH carries a poor prognosis before and after liver transplantation.
CONCLUSIONS: There should be a low threshold in getting a transthoracic echocardiogram with a bubble study in liver disease patients with either dyspnea or hypoxemia to investigate the existence of HPS and /or POPH.
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Reference #2 Toya S, Schraufnagel DE. Hepatopulmonary syndrome and pulmonary hypertension in the same patient: A pathophysiological paradox and therapeutic dilemma. Respiratory Medicine Extra 2006; 2:23-26.
Reference #3 Feltracco , Serra E, Brezzi ML, Milevoj M, Furnari M, Barbieri S, Salvaterra F, Ori C. Hemodynamic profile of portopumonary hypertension.Transplant proceedings 2009; 41:1235-1239.
DISCLOSURE: The following authors have nothing to disclose: Faisal Usman, Amita Singh, Ankur Girdhar, Abubakr Bajwa, James Cury
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