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Long‐term outcome in liver transplantation candidates with portopulmonary hypertension
Author(s) -
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,
DuclosVallée Jean Charles,
Sitbon Olivier
Publication year - 2017
Publication title -
hepatology
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 5.488
H-Index - 361
eISSN - 1527-3350
pISSN - 0270-9139
DOI - 10.1002/hep.28990
Subject(s) - portopulmonary hypertension , medicine , liver transplantation , pulmonary hypertension , vascular resistance , pulmonary artery , cardiac index , cardiology , portal hypertension , hemodynamics , liver disease , transplantation , surgery , cardiac output , cirrhosis
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. (H epatology 2017;65:1683‐1692).