Porto-Pulmonary Hypertension (POPH) in a Female with Non-Cirrhotic Portal Hypertension - Early Suspicion and Early Diagnosis is the Key

Liver disease and portal venous disease can present with a number of pulmonary complications, such as hepatic hydrothorax, hepatopulmonary syndrome and Portopulmonary hypertension (POPH). Porto-pulmonary hypertension is a rare but serious condition. Increased vascular pressure occurs due to progressive vasoconstriction, causing respiratory discomfort to the patient and ultimately progressing to right heart failure. Porto-pulmonary hypertension falls into World Health Organization (WHO) group 1 of pulmonary hypertension, indicating that the mechanism involved in the development of the disease is pulmonary vascular remodelling. It is the rarest of all pulmonary complications of liver disease, but perhaps the most destructive, as it is impossible to reverse the changes at the vascular level. Early diagnosis is essential to decrease mortality, and investigations, such as echocardiography and right heart catheterization are available. As POPH is uncommon, not much is known about its treatment. The best therapy is proper and timely management of portal hypertension, to prevent its occurrence altogether. We present a case of a 36-year-old female, who presented with features typical of portal hypertension, but on further examination, patient was found to have pulmonary hypertension as well. Imaging findings suggested non cirrhotic portal fibrosis to have caused portal hypertension, and eventually pulmonary hypertension.