pulmonary disorders
Portopulmonary Hypertension
Introduction
Portopulmonary hypertension is pulmonary arterial hypertension associated with portal hypertension without other secondary causes. Pulmonary Hypertension occurs in patients with various conditions that involve Abnormalities in bone morphogenetic protein 9 (BMP9) signaling are linked to the development of pulmonary hypertension. BMP9 and BMP10 are produced in the liver and are ligands for the BMP2 receptor. Patients with portopulmonary hypertension have been noted to have a significantly reduced BMP9 level when compared to control patients with advanced liver disease. There was no difference in BMP10 levels when comparing patients with portopulmonary hypertension to patients with advanced liver disease without portopulmonary hypertension ( General references ). Presenting symptoms are dyspnea and fatigue. Chest pain and hemoptysis can also occur. Patients have physical findings and ECG abnormalities consistent with pulmonary hypertension and may develop evidence of cor pulmonale (elevated jugular venous pulse, edema). Tricuspid regurgitation is common. The diagnosis is suspected based on Echocardiography findings and confirmed by Treatment of portopulmonary hypertension is the same as that of Treatment except that hepatotoxic drugs and anticoagulants should be avoided. Beta-blockers, frequently used in portal hypertension, should also be avoided in portopulmonary hypertension due to hemodynamic instability ( 