Improvement of hepatopulmonary syndrome after transjugular intrahepatic portasystemic shunting: Case report and review of literature

Abstract

The hepatopulmonary syndrome has been described in as many as 5-29% of patients with liver disease. Patients with this syndrome may suffer from chronic hypoxemia, and mortality rates of liver patients with this syndrome are as high as 41%. Early diagnosis of such patients is essential. Currently, liver transplantation is the only effective therapy for such patients, and reversal of this syndrome is seen in up to 80% of patients post-transplant. Transjugular intrahepatic portasystemic shunting (TIPS) as a therapeutic maneuver for this syndrome has been described in five patients to date with mixed results. Reduction in portal hypertension with consequent redistribution of blood flow and altered synthesis of vasodilatory chemicals have been postulated to help resolve this disease. In this report, we describe an 11-yr-old female with biliary atresia and hepatopulmonary syndrome. Her disease was complicated with recurrent variceal bleeding. TIPS achieved a therapeutic response of both her bleeding and respiratory complications.

The hepatopulmonary syndrome

“…These findings suggest that improved oxygenation may not have been caused by reversal of intrapulmonary vasodilatation. In the case reports of an 11-year-old girl with biliary atresia and a 46-year-old woman with presumed alcohol related cirrhosis, oxygenation and intrapulmonary shunting were improved and sustained over 8 months and 3 years, respectively, following TIPS placement [78,79]. There is a seventh report of failure of TIPS to improve oxygenation in one patient and identification of two patients where HPS developed in the setting of a functional TIPS [80].…”

The role of transjugular intrahepatic portosystemic shunt in the management of portal hypertension

“…114 Six patients have been reported who had hepatopulmonary syndrome and received a TIPS; 5 of the 6 showed improvement in oxygenation, and some but not all showed a decrease in intrapulmonary shunts. 115 The mechanism through which TIPS may improve intrapulmonary shunting in patients with portal hypertension is unclear. …”

Dyspnoea, Cyanosis and Digital Clubbing in a 28-Year-Old Patient as a Result of Hepatopulmonary Syndrome

“…The results obtained are contradictory, as of today there are no clear-cut criteria that would allow to isolate a group of patients with HPS obtaining measurable benefits by the application of the vascular anastomosis. [14][15][16] As a palliative procedure, embolisaton of thickened vessels or local resections of the pulmonary tissue that contains them is adopted [7]. In connection with the values obtained in gasometry in the patient described herein, the patient was directly qualified to lung transplantation as the target treatment.…”