Hepatopulmonary syndrome and portopulmonary hypertension coexisting in a case of wilson's disease cirrhosis: “Double trouble”

Abstract

Wilson's disease is one of the infrequent causes of hepatic failure with a wide clinical spectrum. Hepatic and neuropsychiatric manifestations are the most common, whereas atypical presentations were also reported as the complications of hepatic failure such as hepatopulmonary syndrome (HPS) and portopulmonary hypertension (POPH). These are considered pulmonary complications of hepatic disease with underlying complex pathology and various vasoactive substances such as endothelin 1 and nitric oxide on the pulmonary blood vessels. HPS usually presents with breathlessness and central cyanosis. The triad of HPS comprises dilatation of pulmonary vasculature, intrapulmonary shunting, and hypoxia, whereas POPH causes vasoconstriction and vascular remodeling leading to increased resistance in pulmonary blood vessels and subsequent right ventricular dysfunction. In this case report, we highlight two antagonistic pathological processes coexisting in the same patient.

Keywords

• endothelin
• hepatic
• hypertension
• hypoxia
• neuropsychiatric