RELATIONSHIP BETWEEN PULMONARY AND PORTAL -HYPERTENSION ILLUSTRATED BY 2 CASES WITH DIFFERENT TYPES OF PRESINUSOIDAL PORTAL-HYPERTENSION

Since its first description in 1951 by Mantz and Craig pulmonary hyper tension in combination with portal hypertension has been observed more and more frequently. In a recent prospective study Hadengue et al. re ported an incidence of 2%. Thus this simultaneons occurrence can no lo nger be considered to be coincidental. The etiology remains still uncl ear. It is most probable that the development is due to vasoactive sub stances which bypass the liver or which are produced in the lung itsel f, and which, due to a long-term vasoconstriction, causes irreparable damage to the arterioles and arteries in the lung. Such pulmonary hype rtension can develop in the presence of a pre- as well as an intrahepa tic block, even when the portal hypertension is partially or completel y alleviated by a portosystemic anastomosis. This last circumstance ca n be illustrated by two cases which were observed by our group. Case A is of particular interest because it is the first documentation of a case of an intrahepatic black due to a (so-called) macronodular transf ormation of the liver in the absence of portal thrombosis (a socalled NRH: nodular regenerative hyperplasia) in combination with pulmonary h ypertension. This type of non-cirrhotic portal hypertension can be ass ociated with micronodular transformation of the liver as well. of the liver as well. Posthepatic blocks or the so-called BUDD-CHIARI Syndrom e type appear to carry no risk of development of pulmonary hypertenson . It remains unclear which particular etiologies increase susceptibili ty to later development of pulmonary hypertension.