Long‐term outcome in liver transplantation candidates with portopulmonary hypertension. Issue 5 (14th March 2017)

• Record Type: Journal Article
• Authors: Savale, Laurent; Sattler, Caroline; Coilly, Audrey; Conti, Filoména; Renard, Sébastien; Francoz, Claire; Bouvaist, Hélène; Feray, Cyrille; Borentain, Patrick; Jaïs, Xavier; Montani, David; Parent, Florence; O'Connell, Caroline; Hervé, Philippe; Humbert, Marc; Simonneau, Gérald; Samuel, Didier; Calmus, Yvon; Duvoux, Christophe; Durand, François; Duclos‐Vallée, Jean Charles; Sitbon, Olivier
• Is Part Of: Hepatology. Volume 65:Issue 5(2017)
• Page Start: 1683
• Page End: 1692
• Publication Date: 2017-03-14
• Subjects: Heart -- Diseases -- Nursing -- Periodicals; Lungs -- Diseases -- Nursing -- Periodicals; Intensive care nursing -- Periodicals; Foie -- Maladies -- Périodiques; 616.362
• DOI: 10.1002/hep.28990
• Languages: English
• ISSNs: 0270-9139

Abstract

Abstract : Portopulmonary hypertension (PoPH) is diagnosed in 2‐6% of liver transplantation (LT) candidates. We studied outcomes of candidates for LT suffering from PoPH. Data were collected retrospectively from a prospective registry. Pulmonary hemodynamic variables were collected at the time of PoPH diagnosis, at last evaluation before LT, and within 6 months and beyond 6 months after LT. Forty‐nine patients (35 males, 48 ± 8 years) were analyzed (median Model for End‐Stage Liver Disease score 20). At baseline, mean pulmonary artery pressure (mPAP) was 44 ± 10 mm Hg (range 26‐73 mm Hg), cardiac index was 3.5 ± 0.9 L/min/m 2, and pulmonary vascular resistance was 5.6 ± 2.8 Wood units. Hemodynamic reassessment performed in 35 patients who were treated with pulmonary arterial hypertension–targeted therapies before LT resulted in significant decreases in both mPAP (36 ± 7 versus 47 ± 10 mm Hg, P < 0.0001) and pulmonary vascular resistance (3.0 ± 1.4 versus 6.1 ± 3.1 Wood units, P < 0.0001). Fourteen patients (29%) died without having had access to LT. Thirty‐five patients underwent LT and were followed up for a median of 38 months. Eight patients (23%) died after LT including 5 due to PoPH (after 1 day to 6 months). Among survivors (n = 27), all patients treated with intravenous epoprostenol were weaned off post‐LT, and endothelin receptor antagonist or phosphodiesterase type 5 inhibitors were continued in 15/27 patients (55%). At last evaluation, 20/27 patients (74%) had mPAP <35 mm Hg and 8 of them (30%) had mPAP <25 mm Hg. Overall survival estimates after LT were 80%, 77%, and 77% at 6 months, 1 year, and 3 years, respectively. Conclusion : Stabilization or reversibility of PoPH seems to be an attainable goal using the combination of pulmonary arterial hypertension–targeted therapies and LT in patients who are transplantation candidates. (Hepatology 2017;65:1683‐1692).