Chest
Original ResearchPulmonary Vascular DiseaseSafety and Efficacy of Ambrisentan for the Treatment of Portopulmonary Hypertension
Section snippets
Materials and Methods
The Mayo Clinic institutional review board approved the Pulmonary Vascular Complications of Liver Disease (PVCLD) data collection. We prospectively identified and followed consecutive adult (≥ 18 years old) patients with POPH evaluated at the Mayo Clinic from January 2007 to December 2009 who were treated with ambrisentan as monotherapy (group 1, World Health Organization [WHO] functional class 2 or 3). Three patients refused the suggested IV prostacyclin therapy; no patient seen during this
Results
During the observation period, we identified 13 patients with POPH who were started on ambrisentan as monotherapy. Baseline clinical characteristics of the cohort are described in Table 1. All patients were considered to have at least moderate POPH, with an mPAP > 35 mm Hg. The patients were followed for a median of 613 days (IQR, 385-1,011). One patient stopped the medication after 2 weeks of therapy because of bilateral periorbital bleeding, peripheral edema, and a weight gain of 8 pounds,
Discussion
This single-institution observational study demonstrates that monotherapy with ambrisentan is effective and safe for the treatment of patients with POPH, as evidenced by significant improvements in hemodynamic measurements (mPAP, PVR, and cardiac output), biomarkers (BNP), and symptoms (WHO functional class) with no deterioration in systemic BP, liver function test results, or renal function. Ambrisentan was well tolerated, with only one patient stopping the medication because of side effects
Acknowledgments
Author contributions: All authors have directly contributed to the content of this manuscript and reviewed the final version.
Dr Cartin-Ceba: wrote the manuscript.
Dr Swanson: helped draft all portions of the manuscript.
Dr Iyer: helped draft all portions of the manuscript.
Dr Wiesner: helped draft all portions of the manuscript.
Dr Krowka: designed the study and reviewed and revised the final manuscript.
Financial/nonfinancial disclosures: The authors have reported to CHEST that no potential
References (22)
- et al.
Updated clinical classification of pulmonary hypertension
J Am Coll Cardiol
(2009) - et al.
Pulmonary hemodynamics and perioperative cardiopulmonary-related mortality in patients with portopulmonary hypertension undergoing liver transplantation
Liver Transpl
(2000) - et al.
Portopulmonary hypertension: a tale of two circulations
Chest
(2003) - et al.
Accuracy of Doppler echocardiography in the assessment of pulmonary hypertension in liver transplant candidates
Liver Transpl
(2000) - et al.
Survival in portopulmonary hypertension: Mayo Clinic experience categorized by treatment subgroups
Am J Transplant
(2008) - et al.
Endothelin-receptor antagonist treatment of portopulmonary hypertension
Clin Gastroenterol Hepatol
(2004) - et al.
Use of a mixed endothelin receptor antagonist in portopulmonary hypertension: a safe and effective therapy?
Gastroenterology
(2005) - et al.
Improvement in pulmonary hemodynamics during intravenous epoprostenol (prostacyclin): a study of 15 patients with moderate to severe portopulmonary hypertension
Hepatology
(1999) - et al.
Portopulmonary hypertension: survival and prognostic factors
Am J Respir Crit Care Med
(2008) - et al.
Portopulmonary hypertension in decompensated cirrhosis with refractory ascites
Gut
(2003)
Hepatopulmonary syndrome and portopulmonary hypertension: a report of the multicenter liver transplant database
Liver Transpl
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