Triad of HPS

Q: Describe the triad of Hepatopulmonary syndrome (HPS)?

Answer: HPS is a triad created by the interlink pathophysiology of abnormal arterial oxygenation caused by intrapulmonary vascular dilatations (IPVD) in the setting of liver disease portal hypertension, or congenital portosystemic shunts.

Unfortunately, the only curative treatment is liver transplant. And while waiting for transplant, support with oxygen to keep saturation > 88%. There is a rare form of HPS called granulomatous hepatitis, which is responsive to steroids and can be cured.

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References:

1. Rodríguez-Roisin R, Krowka MJ. Hepatopulmonary syndrome--a liver-induced lung vascular disorder. N Engl J Med 2008; 358:2378.

2. Krowka MJ, Fallon MB, Kawut SM, et al. International Liver Transplant Society Practice Guidelines: Diagnosis and Management of Hepatopulmonary Syndrome and Portopulmonary Hypertension. Transplantation 2016; 100:1440.

3. Tzovaras N, Stefos A, Georgiadou SP, et al. Reversion of severe hepatopulmonary syndrome in a non cirrhotic patient after corticosteroid treatment for granulomatous hepatitis: a case report and review of the literature. World J Gastroenterol 2006; 12:336.