Published online Feb 14, 2016. doi: 10.3748/wjg.v22.i6.2024
Peer-review started: July 3, 2015
First decision: September 29, 2015
Revised: October 21, 2015
Accepted: December 19, 2015
Article in press: December 19, 2015
Published online: February 14, 2016
Processing time: 208 Days and 11.2 Hours
Pulmonary vascular disorders including portopulmonary hypertension (PoPHT) are among the common complications of liver disease and are prognostically significant. Survival is very low without medical treatment and liver transplantation. With advances in medical therapy for elevated pulmonary artery pressure (PAP) and liver transplant surgery, survival of patients with PoPHT and advanced liver disease is significantly improved. Because of the prognostic significance of PoPHT and the limited donor pool, a comprehensive preoperative cardio-pulmonary assessment is of great importance in cirrhotic patients prior to transplant surgery. Therefore, a detailed transthoracic Doppler echocardiographic examination must be an essential component of this evaluation. Patients with mild PoPHT can safely undergo liver transplant surgery. In cases of moderate to severe PoPHT, right heart catheterization (RHC) should be performed. In patients with moderate to severe PoPHT on RHC (mean PAP 35-45 mmHg), vasodilator therapy should be attempted. Liver transplantation should be encouraged in cases that demonstrate a positive response. Bridging therapy with specific pulmonary arterial hypertension treatment agents should be considered until the transplant surgery and should be continued during the peri- and post-operative periods as needed.
Core tip: Portopulmonary hypertension (PoPHT) is one of the most common findings on preoperative assessment of cirrhotic patients prior to liver transplant surgery. Since it has prognostic significance, diagnosis of PoPHT by Doppler echocardiography and further characterization by right heart catheterization is critical in classifying these patients. Therapy with pulmonary arterial hypertension (PAH)-specific agents should be started when PoPHT is moderate to severe. Patients with a positive response should be encouraged to undergo liver transplant surgery. Bridging therapy with these agents should be considered until the time of transplant surgery and continued during the peri and postoperative periods as needed.