Pulmonary hypertension Pulmonary Hypertension Pulmonary hypertension is increased pressure in the pulmonary circulation. It has many secondary causes; some cases are idiopathic. In pulmonary hypertension, pulmonary vessels may become constricted... read more occurs in patients with various conditions that involve portal hypertension Portal Hypertension Portal hypertension is elevated pressure in the portal vein. It is caused most often by cirrhosis (in North America), schistosomiasis (in endemic areas), or hepatic vascular abnormalities. Consequences... read more with or without cirrhosis Cirrhosis Cirrhosis is a late stage of hepatic fibrosis that has resulted in widespread distortion of normal hepatic architecture. Cirrhosis is characterized by regenerative nodules surrounded by dense... read more . Portopulmonary hypertension occurs less commonly than hepatopulmonary syndrome Hepatopulmonary Syndrome Hepatopulmonary syndrome is hypoxemia caused by pulmonary microvascular vasodilation in patients with portal hypertension; dyspnea and hypoxemia are worse when the patient is upright. Hepatopulmonary... read more in patients with chronic liver disease (3.5 vs 12%).
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 (1 General references Portopulmonary hypertension is pulmonary arterial hypertension associated with portal hypertension without other secondary causes. Pulmonary hypertension occurs in patients with various conditions... read more ).
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 Echocardiography This photo shows a patient having echocardiography. This image shows all 4 cardiac chambers and the tricupsid and mitral valves. Echocardiography uses ultrasound waves to produce an image of... read more findings and confirmed by right heart catheterization Right heart catheterization Cardiac catheterization is the passage of a catheter through peripheral arteries or veins into cardiac chambers, the pulmonary artery, and coronary arteries and veins. Cardiac catheterization... read more .
Treatment of portopulmonary hypertension is the same as that of pulmonary arterial hypertension Treatment Pulmonary hypertension is increased pressure in the pulmonary circulation. It has many secondary causes; some cases are idiopathic. In pulmonary hypertension, pulmonary vessels may become constricted... read more 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 (2 General references Portopulmonary hypertension is pulmonary arterial hypertension associated with portal hypertension without other secondary causes. Pulmonary hypertension occurs in patients with various conditions... read more ). Some patients benefit from vasodilator therapy. The underlying liver disease is a major determinant of outcome. Portopulmonary hypertension is a relative contraindication to liver transplantation because of increased morbidity and mortality from the procedure. However, in some patients who receive a transplant, particularly those with mild pulmonary hypertension, pulmonary hypertension regresses. Some centers consider transplantation in patients who have mean pulmonary arterial pressures < 35 mm Hg after a trial of vasodilator therapy.
1. Rochon ER, Krowka MJ, Bartolome S, et al: BMP 9/10 in pulmonary vascular complications of liver disease. Am J Respir Crit Care Med 201 (11):1575–1578, 2020. doi: 10.1164/rccm.201912-2514LE
2.Galiè N, Humbert M, Vachiery JL, et al: 2015 ESC/ERS Guidelines for the diagnosis and treatment of pulmonary hypertension: The Joint Task Force for the Diagnosis and Treatment of Pulmonary Hypertension of the European Society of Cardiology (ESC) and the European Respiratory Society (ERS): Endorsed by: Association for European Paediatric and Congenital Cardiology (AEPC), International Society for Heart and Lung Transplantation (ISHLT). Eur Heart J 37(1): 67-119, 2016. doi: 10.1093/eurheartj/ehv317