Liver and lung: How to manage portopulmonary hypertension

Initially, most patients who have portopulmonary hypertension (portoPH) are asymptomatic or have subtle symptoms, and the disorder may be recognized only after the patient is anesthetized for orthotopic liver transplantation (OLT). The initial appearance of symptoms usually is associated with right ventricular dysfunction or low cardiac output. Dyspnea on exertion, peripheral edema, chest pain, and syncope are most common, but fatigue may occur also. Usually, a high degree of clinical suspicion, screening by Doppler echocardiography, and confirmation by right heart catheterization are needed to make the diagnosis. Various vasodilators, such as nitric oxide and epoprostenol, may be given. PortoPH patients with mean pulmonary artery pressures of 35 to 50 mm Hg and pulmonary vascular resistance of less than 250 dynes/s/cm⁵ may be good candidates for OLT.