Alerts and Notices
SynopsisPortopulmonary hypertension (PPHTN) is defined as pulmonary arterial hypertension (PAH) with concomitant portal hypertension in the absence of other common causes of PAH, such as connective tissue disease or congenital heart disease. This is usually seen in patients with chronic liver disease, although it can develop in patients with non-cirrhotic portal hypertension. The pathogenesis of PPHTN involves endothelial and smooth muscle proliferation. The cause of this proliferation is unknown, but one theory is that a hormonal agent that is typically cleared by the liver enters the pulmonary blood stream via collaterals. Other theories include genetic predisposition, thromboembolic disease from portosystemic shunts, and increased blood flow through pulmonary system in patients with liver disease. PPHTN affects approximately 1%-2% of patients with portal hypertension or cirrhosis and 5%-10% of patients who are referred for liver transplantation.
Clinical presentation of PPHTN includes manifestations of portal hypertension (ascites, lower extremity edema, encephalopathy, varices, and splenomegaly) along with symptoms of PAH (dyspnea on exertion, orthopnea, fatigue, chest pain, and syncope).
I27.29 – Other secondary pulmonary hypertension
445237003 – Portopulmonary Hypertension
Differential Diagnosis & Pitfalls
- Pulmonary artery hypertension unrelated to liver disease
- Hepatopulmonary syndrome
- Interstitial lung disease
- Obstructive sleep apnea
- Chronic obstructive pulmonary disease (COPD)
- Drug toxicity (chemotherapeutics such as cyclophosphamide and bleomycin; amiodarone, fenfluramine / phentermine, aminorex, toxic rapeseed oil, cocaine, amphetamines)
- Radiation-induced lung disease
- Mitral stenosis
- Alpha-1 antitrypsin deficiency
- Congestive heart failure