Glenda Ernst1, Fernando Grassi1 and Julio F Chertcoff2*
1Pulmonary Lab, British Hospital from Buenos Aires, CABA, Argentina 2Intensive Care Unit, British Hospital from Buenos Aires, CABA, Argentina
Received: 04 January, 2016; Accepted: 27 January, 2016; Published: 29 January, 2016
Julio F. Chertcoff, Intensive Care Unit, British Hospital from Buenos Aires, CABA, Argentina, Perdriel 74, 1° Floor. CP 1280AEB, Tel: (5411) 43096400; Ext 2808; E-mail:
Ernst G, Grassi F, Chertcoff JF (2016) Hepatopulmonary Syndrome and Portopulmonary Hypertension in the Same Patient. J Clin Microbiol Biochem Technol 2(1): 002-005.
© 2016 Ernst G, et al. This is an open-access article distributed under the terms of the Creative Commons Attribution License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original author and source are credited.
Hepatopulmonary syndrome; Portopulmonary hypertension; Liver transplant
HPS: Hepatopulmonary Syndrome; POPH: Portopulmonary Hypertension; RHC: Right Heart Catheterization; PAPm: Pulmonary Arterial Pressure
Hepatopulmonary syndrome (HPS) and portopulmonary hypertension (POPH) are the most common pulmonary vascular complications in patients with liver disease. We present a 71-year old woman with alcoholic cirrhosis and 6 month history of progressive shortness of breath with mild hypoxemia at rest. Results of spirometry was normal and diffusing capacity of the lung for carbon monoxide (DLCO) was severity reduced. Echocardiography with contrast enhancement revealed extra cardiac shunt. Three months after her initial presentation, a new echocardiography was performed with pulmonary systolic pressure increased and in a right heart catheterization (RHC) the mean pulmonary arterial pressure (PAPm) was 50 mmHg, this evidence supported the diagnosis of severe portopulmonary hypertension after HPS.
We consider that patients with HPS can develop POPH during the waiting time for liver transplant. Moderate and severe POPH represents a contraindication for liver transplantation because it could lead to an unacceptable mortality rate. Screening for POPH is relevant in patients while waiting for liver transplant.
Association of chronic liver disease with respiratory symptoms and hypoxia is well recognized, owing to the success of liver transplantation, there has been increasing the importance of recognition of pulmonary vascular complications of hepatic disease states . Hepatopulmonary syndrome (HPS) and portopulmonary hypertension (POPH) are relatively common complications in patients among liver transplantation candidates . HPS and POPH can share similar respiratory symptoms, though the physiology is different and both are considered mutually exclusive.
Pathophysiology of these two entities could be related with an imbalance between vasoconstrictor and vasodilator . Angiogenic factors that escape from hepatic metabolism could play a role in the pulmonary circulation through collateral shunts and the pathogenesis of POPH . HPS has been defined as an oxygenation defect caused by the development of intrapulmonary vascular dilatation in patients with either advanced liver disease and/or portal hypertension . While POPH is characterized by an increase in the pulmonary vascular resistance as a consequence of obstruction to pulmonary arterial blood flow with or without advanced liver disease , the main feature of HPS is vasodilatation.
The role of liver transplantation in reversing these vascular disorders is controversial, although complete resolution of HPS and, less frequently, POPH after liver transplantation has been reported .
A 71 year-old woman with alcoholic cirrhosis presented to the emergency room and referred effort dyspnea for the last six months. She had a past medical history of ex-smoker (10 pack/year), glaucoma and hypothyroidism. She had been abstinent from alcohol for one year and showed no admissions for edematous-ascites syndrome, esophageal varices or spontaneous bacterial peritonitis. Her Model for End-Stage Liver Disease (MELD) score at the time of presentation to our center was 17.
On physical exam, her breath sounds were clear and did not show signs of fluid overload. Cyanosis, clubbing or platypnea were not present, however orthodeoxia was detected: SaO2 of 93% (0.21) in supine position and SaO2 of 89% in upright position. Computer tomography scans showed ground glass areas with and thickening of interlobular septa. Laboratory evaluation showed: hematocrit = 41%, hemoglobin = 14.5 g/dl, platelet count = 83000/mm3, total bilirubin = 2.7 g/dl, alkaline phosphatase =118 U/l, serum albumine = 4.1 g/dl, aspartate aminotransferase = 30UI/l and alanine aminotransferase = 43UI/l. HIV serology was negative and her immunological profile inconsistent with collagen diseases.
Pulmonary function, respiratory gas exchange and ventilation-perfusion relationships were studied. Both forced spirometry results and lung volumes by plethysmography were normal. A severely reduced DLCO (37%) after adequate correction for anaemia was detected (Table 1).
Arterial blood gas (ABG) on room air revealed a pH of 7.43, partial pressure of oxygen (PaO2) was 57.7 mmHg, partial pressure of dioxide (PaCO2) was 30 mmHg, and an alveolar arterial gradient of 43 mmHg. Contrast enhanced transesophageal echocardiography (cTEE) revealed extra cardiac shunt (Figure 1). Shunt calculated was 18%. The RHC showed a mean pulmonary arterial pressure (MPAP) of 28 mmHg and a pulmonary capillary wedge pressure (PCWP) of 11 mmHg.
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