Copyright ©ERS Journals Ltd 2007 Hepatopulmonary syndrome following portopulmonary hypertensionDepts of 1 Sleep Medicine, and 2 Pulmonary, Allergy, and Critical Care Medicine, Cleveland Clinic, Cleveland, OH, USA. CORRESPONDENCE: O. C. Ioachimescu, Cleveland Clinic, Dept of Sleep Medicine, 9500 Euclid Ave FA20, Cleveland, OH 44195, USA. Fax: 1 2166360090. E-mail: oioac{at}yahoo.com Keywords: Cardiopulmonary pathophysiology, cardiorespiratory, hepatopulmonary problems, hepatopulmonary syndrome, pulmonary circulation physiopathology, pulmonary hypertension
Received: October 28, 2006
Portopulmonary hypertension (PPHTN) and hepatopulmonary syndrome (HPS) are distinct clinical entities that may accompany liver disease.
While PPHTN and HPS have been infrequently described as occurring in the same patient, to the present authors knowledge, the order of occurrence has always been the initial onset of HPS, with pulmonary hypertension developing either concurrently or subsequently. In some instances, liver transplantation has been undertaken for HPS, followed by resolution of the HPS and subsequent development of pulmonary hypertension.
The current case study presents a patient with hepatitis C-related cirrhosis in whom PPTHN developed initially, followed 2 yrs later by the development of the HPS.
The current authors speculate that progressive imbalance in favour of endogenous vasodilators over vasoconstrictive factors led to normalisation of the pulmonary artery pressures.
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