Ethnicity in Pulmonary Arterial Hypertension: Possibilities for Novel Phenotypes in the Age of Personalized Medicine

Chest: The Journal of Circulation, Respiration and Related Systems

Sandeep Sahay^2¹  Sarah K. Medrek^1² 

[1]Department of Medicine, Weill Cornell Medical College and Institute of Academic Medicine, Houston Methodist Hospital, Houston, TX^2

[2]Division of Pulmonary, Critical Care, and Sleep Medicine, Baylor College of Medicine, Houston, TX^1

关键词: ethnicity;    phenotypes in pulmonary arterial hypertension;    pulmonary arterial hypertension;    race;    CTD-PAH;    connective tissue disease-associated pulmonary arterial hypertension;    ERA;    endothelin-receptor antagonist;    HPAH;    hereditary pulmonary arterial hypertension;    IPAH;    idiopathic pulmonary arterial hypertension;    LV;    left ventricular;    MESA;    Multi-Ethnic Study of Atherosclerosis;    NIH;    National Institutes of Health;    PAH;    pulmonary arterial hypertension;    PH;    pulmonary hypertension;    PoPH;    portopulmonary hypertension;    REVEAL;    Registry to Evaluate Early and Long-Term PAH Disease Management;    RV;    right ventricular;    RVEF;    right ventricular ejection fraction;    Scl-APAH;    scleroderma-associated pulmonary arterial hypertension;    SLE;    systemic lupus erythematosus;   

DOI  :  10.1016/j.chest.2017.08.1159

学科分类：呼吸医学

来源: American College of Chest Physicians

【 摘 要 】

In the past decade and a half, the introduction of new therapeutic agents has revolutionized the management of pulmonary arterial hypertension (PAH). These new treatment options have improved the quality of life and survival in PAH. With an armamentarium of options available, the identification of unique phenotypes can help practitioners choose tailored treatment regimens. Experts in other cardiovascular diseases, such as congestive heart failure and hypertension, have recommended race-specific treatments in their fields based on data highlighting variations in response to therapies. With this perspective, we review evidence supporting the hypothesis that ethnicity or race plays an important role in the management of PAH. Preliminary research suggests that races/ethnicities have differences in the presentation and outcome of PAH and could respond to PAH-specific medications with varying efficacy. Genetic, physiological, and anatomic differences exist between races, particularly regarding the structure and function of the right ventricle. Unfortunately, clinical trials have not adequately included minorities, and registry data often omit inclusion of this demographic information. Further studies are needed to characterize the role that ethnicity plays in the prevalence, presentation, outcomes, and optimal treatment of PAH.

【 授权许可】

CC BY