Portopulmonary hypertension (PoPH) is a form of pulmonary arterial hypertension (PAH). PoPH occurs in approximately 15% of patients with PAH, and is reportedly found in 2-6% of patients with portal hypertension and 1-2% of patients with liver cirrhosis according to studies from Europe and America. However, the real-world data on PoPH in Japan are largely unknown, with many questions on the condition’s etiology and prevalence.

Led by doctorate student Shun-ichi Wakabayashi of Shinshu University, the goal of this investigation is to clarify the actual state of PoPH among patients with chronic liver disease by screening all such patients treated at Shinshu University Hospital.

Although there is considerable uncertainty on the impact of PoPH, it is known that the prognosis of liver disease becomes poor when PoPH is also present. As there are no symptoms specific to PoPH and patients with chronic liver disease may exhibit symptoms similar to PoPH with disease progression, a brief but reliable questionnaire is needed to identify accompanying PoPH.

The corresponding author of the study, associate professor Satoru Joshita, states, “In this single-center study, we will clarify the frequency, etiology, and pathophysiology of PoPH by screening all patients with chronic liver disease for PoPH at our institution. We seek to clarify the risk factors for PoPH onset to ultimately improve the prognosis of PoPH patients by early diagnosis and timely treatment.”

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