Portopulmonary hypertension
Portopulmonary hypertension is pulmonary arterial hypertension arising in a patient with portal hypertension, confirmed by right heart catheterization showing an elevated mean pulmonary artery pressure and pulmonary vascular resistance with a normal or low wedge pressure. It carries a high mortality rate, and severity by mean pulmonary artery pressure and right ventricular function determines candidacy for liver transplantation, which is not curative on its own.
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What this article covers
- Overview
Definition: portopulmonary hypertension (POPH) is pulmonary arterial hypertension (Group 1 PAH) developing in a patient with portal hypertension, from cirrhosis or another cause, without another explanation for the pulmonary vasculopathy.
- Epidemiology
Less common than hepatopulmonary syndrome: POPH occurs in fewer patients with cirrhosis and portal hypertension than hepatopulmonary syndrome does, but carries a higher mortality rate when unrecognized.
- Risk factors
Portal hypertension of any cause: the obligatory substrate; POPH does not occur without it.
- Pathophysiology
Portal hypertension is the initiating condition: elevated portal pressure and portosystemic shunting expose the pulmonary circulation to vasoactive and inflammatory mediators normally cleared by the liver.
- Clinical features
Dyspnea on exertion: the presenting and most common symptom, and the finding that should prompt suspicion of POPH in any patient with known cirrhosis and portal hypertension.
- Differential diagnosis
Hepatopulmonary syndrome: dyspnea and hypoxemia from intrapulmonary vascular dilation and right-to-left shunting, with orthodeoxia and platypnea; confirmed by intrapulmonary shunting on agitated…
- Diagnosis
Confirm with right heart catheterization showing precapillary pulmonary hypertension in a patient with portal hypertension: a mean pulmonary artery pressure of 25 mm Hg or higher at rest (some…
- Management
Refer to a combined pulmonary hypertension and hepatology/transplant program as soon as right heart catheterization confirms the diagnosis, because drug selection, titration, and the decision to…
- Complications
Right ventricular failure: the dominant cause of morbidity and death, from sustained pressure overload of a ventricle not built for systemic-level afterload.
- Prognosis
Untreated or unrecognized POPH carries a high mortality rate, the central reason for systematic screening in patients with cirrhosis and portal hypertension who report exertional dyspnea.
- Prevention
Screen every patient with cirrhosis and portal hypertension who reports new or progressive exertional dyspnea with transthoracic echocardiography, rather than attributing it to deconditioning or the liver disease itself.
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