Reduction of intrapulmonary shunt and resolution of digital clubbing associated with primary biliary cirrhosis after liver transplantation

✍ Scribed by James K. Stoller; Douglas Moodie; William A. Schiavone; David Vogt; Thomas Broughan; Eugene Winkelman; Patrice K. Rehm; William D. Carey

• Publisher: John Wiley and Sons
• Year: 1990
• Tongue: English
• Weight: 596 KB
• Volume: 11
• Category: Article
• ISSN: 0270-9139
• DOI: 10.1002/hep.1840110111

✦ Synopsis

This report describes a patient with marked hypoxemia caused by intrapulmonary shunt associated with primary biliary cirrhosis. Liver transplantation resulted in resolution of d i g i a clubbing and reduction of intrapulmonary shunt as demonstrated by normalization of room air arterial blood gases, reduction in shunt fraction and normalization of the indocyanin-enhanced echocardiogram and perfusion lung scan. This patient's course challenges the conventional notion that intrapulmonary shunting associated with chronic liver disease does not reverse after liver transplantation. (HEPATOLOGY 1-1 1:51-58.)

Mechanisms of the hypoxemia that may accompany chronic liver disease include ventilation-perfusion mismatching (e.g., caused by atelectasis and/ or pleural effusions) and the development of intrapulmonary arteriovenous channels (1)(2)(3)(4)(5)(6)(7)(8)(9)(10)(11). Concomitant pulmonaryrelated features of chronic liver disease include digital clubbing (4, 7, 10, 11) and a diffusion impairment (1, 2, ll), the latter ascribed to interstitial scarring or to the increased distance for oxygen transit within dilated pulmonary capillaries, a phenomenon named "diffusion-perfusion" impairment (1, 2, 11, 12). Although the pathogenesis of these pulmonary changes remains obscure, limited transplantation experience with hypoxemic cirrhotic patients (13) suggests that, once established, arteriovenous channels are irreversible (1, 14). Furthermore, because postoperative changes may worsen hypoxemia after upper abdominal surgery (151, such shunting is commonly deemed a contraindication to liver transplantation when the accompanying hypoxemia is severe (1,16, 17).

To challenge this notion, the current report presents a patient with PBC in whom clubbing and severe hy-