PT  - JOURNAL ARTICLE
AU  - O. C. Ioachimescu
AU  - A. C. Mehta
AU  - J. K. Stoller
TI  - Hepatopulmonary syndrome following portopulmonary hypertension
AID  - 10.1183/09031936.00140306
DP  - 2007 Jun 01
TA  - European Respiratory Journal
PG  - 1277--1280
VI  - 29
IP  - 6
4099  - http://erj.ersjournals.com/content/29/6/1277.short
4100  - http://erj.ersjournals.com/content/29/6/1277.full
SO  - Eur Respir J2007 Jun 01; 29
AB  - 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.