Long-term outcomes of pulmonary arterial hypertension under specific drug therapy in Eisenmenger syndrome

JOURNAL OF HEART AND LUNG TRANSPLANTATION

卷:36

Article

Hascoet, Sebastien¹  Baruteau, Alban-Elouen¹  Humbert, Marc^2,3,4  Simonneau, Gerald^2,3,4  Jais, Xavier^2,3,4  Petit, Jerome¹  Laux, Daniela¹  Sitbon, Olivier^2,3,4  Lambert, Virginie^1,4  Capderou, Andre^3,4,5 

[1] Hop Marie Lannelongue, Pole Cardiopathies Congenitales Enfant & Adulte, Ctr Reference Malformat Cardiaques Congenitales C, Le Plessis Robinson, France

[2] Hop Bicetre, Serv Pneumol, Ctr Reference Hypertens Pulm Severe, DHU Thorax Innovat, Le Kremlin Bicetre, France

[3] Univ Paris Saclay, Univ Paris Sud, Fac Med Paris Sud, Le Kremlin Bicetre, France

[4] Univ Paris Saclay, Univ Paris Sud, Inserm UMR S 999, Hop Marie Lannelongue, Le Kremlin Bicetre, France

[5] Hop Marie Lannelongue, Physiol Lab, Le Plessis Robinson, France

关键词: Eisenmenger syndrome;    catheterization;    drug therapy;    outcome;    pulmonary arterial hypertension;    congenital heart diseases;   

DOI  :  10.1016/j.healun.2016.10.006

来源: Elsevier

【 摘 要 】

BACKGROUND: The long-term effectiveness of pulmonary arterial hypertension specific drug therapy (PAH-SDT) in Eisenmenger syndrome is controversial. We investigated short-term and long-term hemodynamic changes under PAH-SDT and their associations with outcomes in a bicentric cohort. METHODS: Over 20 years, we included 69 patients with congenital heart disease, an indexed pulmonary vascular resistance (PVRi) > 8 WU . m(2), and 292 standardized catheterizations at baseline and after PAH-SDT initiation or intensification. Oxygen consumption was measured and the Fick principle applied to calculate indexed pulmonary output (Qpi) and PVRi. RESULTS: After PAH-SDT initiation or intensification, median (interquartile range) PVRi decrease was 5.1 WU . m(2) (-1.4, 12.6) (p < 0.0001). Median Qpi and 6-minute walk test increases were +0.4 liter/min/m(2) (0.0, +0.9) (p < 0.0001) and +49 m (+15, +93) (p = 0.0003), respectively. Hemodynamic response combining increased Qpi with decreases in transpulmonary gradient and PVRi occurred in 68.0% of patients. After a median of 4.9 years, PVRi and Qpi changes were no longer significant. Over a median of 7.2 years, 23 (33.3%) patients met a composite criterion (death, n = 8; heart-lung transplantation or listing for transplantation, n = 15). The 15-year cumulative event rate was 49.2%. By multivariate analysis, independent predictors of events were superior vena cava oxygen saturation and hemodynamic response (p = 0.048 and p < 0.0001). CONCLUSIONS: In Eisenmenger syndrome, PAH-SDT induces early hemodynamic improvements, which decline over time. Hemodynamic changes under PAH-SDT vary across patients. Hemodynamic parameters at baseline and under PAH-SDT are associated with events. PAH-SDT may need to be individualized based on hemodynamic changes. (C) 2017 International Society for Heart and Lung Transplantation. All rights reserved.

【 授权许可】

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