- Pulmonary HTN probably due to impaired hepatic metabolism of an unknown vasoconstrictor substance (supported by observed plexogenic pulmonary arteriopathy in cases of congenital protal vein agenesis)/ possible role of microemboli from portal circulation
- Echo: pulmonary HTN may be severe in these cases (at levels similar to PPH/ CTEPH)
- Pulmonary Hypertension (see Pulmonary Hypertension, [[Pulmonary Hypertension]])
- Pulmonary vasodilator trial: NO or Prostacyclin with Swan in place
- If responsive, may consider Calcium Channel Blockers
- Liver transplant: anecdotal evidence that this may resolve pulmonary HTN
The development of PAH in association with elevated pressure in the portal circulation is known as portopulmonary hypertension (POPH) (39,40).
Portal hypertension, rather than the presence of underlying liver disease, is the main determining risk factor for the development of POPH. Prospective hemodynamic studies have shown that 2% to 6% of patients with portal hypertension have PH (41,42).
Right heart catheterization is absolutely mandatory for the definitive diagnosis of POPH because several factors may increase pulmonary arterial pressure (PAP) in the setting of ad- vanced liver disease (e.g., high flow associated with the hyperdynamic circulatory state and increased pulmonary capillary wedge pressure owing to fluid overload and/or diastolic dysfunction).
Pulmonary vascular resistance (PVR) is usually normal in these cases.
Pathologic changes in the small arteries appear identical to those seen in IPAH. A recent multicenter case-control study identified 2 risk fac- tors for the development of POPH: female sex and auto- immune hepatitis (43).
Interestingly, hepatitis C infection was associated with a decreased risk. A recent, large cohort study of POPH showed that long-term prognosis was related to the presence and severity of cirrhosis and to cardiac function (44).
- 39 Pulmonary vascular disorders in portal hypertension. Eur Respir J 1998;11:1153– 66.
- 40 On behalf of the ERS Task Force Pulmonary-Hepatic Vascular Disorders Scien-tific Committee. Pulmonary-hepatic vascular disorders. Eur Respir J 2004;24:861– 80.
- 41 Pulmonary hypertension complicating portal hypertension: prevalence and rela- tion to splanchnic hemodynamics. Gastroenterology 1991;100: 520–8.
- 42 Portopulmonary hypertension: results from a 10-year screening algo- rithm. Hepatology 2006;44:1502–10.
- 43 Clinical risk factors for portopulmonary hypertension. Hepatology 2008;48:196 –203.
- 44 Portopulmonary hypertension: survival and prognostic factors. Am J Respir Crit Care Med 2008; 178:637– 43