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IPVDs decrease survival in portopulmonary hypertension

Intrapulmonary vascular dilatations occurred more commonly in patients with portopulmonary hypertension and were associated with decreased overall survival, according to study data published in Liver Transplantation.

Michael J. Krowka, MD, of the division of pulmonary and critical care medicine, Mayo Clinic, and colleagues conducted a retrospective chart review of 80 patients listed for liver transplant diagnosed with portopulmonary hypertension (POPH) at the Mayo Clinic between January 2002 and June 2014, and sought to determine the prevalence of intrapulmonary vascular dilatations (IPVD), evidence of contrast-enhanced transthoracic echocardiography (cTTE), pulmonary hemodynamics, oxygenation and survival.

Michael J. Krowka

“To date, there has been no systematic cTTE study in POPH to evaluate for the coexistence of IPVD findings typical for [hepatopulmonary syndrome],” the researchers wrote.

Of all the patients, 42% underwent cTTE upon POPH diagnosis (n = 34), and were used as the primary study cohort in the final analysis.

IPVDs were found in 59% of the patients via positive cTTE (n = 20), with four of these patients also showing evidence of right-to-left atrial shunt. Of the remaining patients without IPVD (n = 14), four also had right-to-left atrial shunt, one had bidirectional atrial shunt and nine had a negative cTTE with no shunting.

The number of surviving patients with IPVD decreased at 1-year, 3 and 5 years from baseline (71% vs. 18% vs. 9%; log-rank, P = .003). Over the course of follow-up, 17 of the 34 patients died, with a majority being patients in the IPVD group (n = 13). Of the patients who died, five were related to progression or complications of pulmonary hypertension and three to progression or complications of liver disease.

There were no significant differences in baseline patient characteristics, PaO₂, median pulmonary artery pressure and median right ventricular systolic pressure (RVSP) and right ventricle (RV) proportions between the patients who had IPVD and those who did not.

“The combination of ‘positive’ contrast echo and well-documented portopulmonary hypertension was unexpected, not uncommon and may have clinical importance and deserves prospective analysis by others too,” Krowka told Healio.com/Hepatology.

The researchers concluded: “The presence of IPVD seems to confer a survival disadvantage in patients with POPH. These findings suggest the possibility of a pathophysiological overlap between POPH and [hepatopulmonary syndrome] in patients with [end-stage liver disease].” – by Melinda Stevens

Disclosure: The researchers report no relevant financial disclosures.