TY - JOUR
T1 - An official American Thoracic Society statement
T2 - Pulmonary hypertension phenotypes
AU - Dweik, Raed A.
AU - Rounds, Sharon
AU - Erzurum, Serpil C.
AU - Archer, Stephen
AU - Fagan, Karen
AU - Hassoun, Paul M.
AU - Hill, Nicholas S.
AU - Humbert, Marc
AU - Kawut, Steven M.
AU - Krowka, Michael
AU - Michelakis, Evangelos
AU - Morrell, Nicholas W.
AU - Stenmark, Kurt
AU - Tuder, Rubin M.
AU - Newman, John
AU - Bloch, Kenneth
AU - Gaston, Benjamin
PY - 2014/2/1
Y1 - 2014/2/1
N2 - Background: Current classification of pulmonary hypertension (PH) is based on a relatively simple combination of patient characteristics and hemodynamics. This limits customization of treatment, and lacks the clarity of a more granular identification based on individual patient phenotypes. Rapid advances in mechanistic understanding of the disease, improved imaging methods, and innovative biomarkers now provide an opportunity to define PH phenotypes on the basis of biomarkers, advancedimaging,andpathobiology. Thisdocumentorganizesour current understanding of PH phenotypes and identifies gaps in our knowledge. Methods: A multidisciplinary committee with expertise in clinical care (pulmonary, cardiology, pediatrics, and pathology), clinical research, and/or basic science in the areas of PH identified important questions and reviewed and synthesized the literature. Results: This document describes selected PH phenotypes and serves as an initial platform to define additional relevant phenotypes as new knowledge is generated. The biggest gaps in our knowledge stem from the fact that our present understanding of PH phenotypes has not come from any particularly organized effort to identify such phenotypes, but rather from reinterpreting studies and reports that were designed and performed for other purposes. Conclusions: Accurate phenotyping of PH can be used in research studies to increase the homogeneity of study cohorts. Once the ability of the phenotypes to predict outcomes has been validated, phenotyping may also be useful for determining prognosis and guiding treatment. This important next step in PH patient care can optimally be addressed through a consortium of study sites with well-defined goals, tasks, and structure. Planning and support for this could include the National Institutes of Health and the U.S. Food and Drug Administration, with industry and foundation partnerships.
AB - Background: Current classification of pulmonary hypertension (PH) is based on a relatively simple combination of patient characteristics and hemodynamics. This limits customization of treatment, and lacks the clarity of a more granular identification based on individual patient phenotypes. Rapid advances in mechanistic understanding of the disease, improved imaging methods, and innovative biomarkers now provide an opportunity to define PH phenotypes on the basis of biomarkers, advancedimaging,andpathobiology. Thisdocumentorganizesour current understanding of PH phenotypes and identifies gaps in our knowledge. Methods: A multidisciplinary committee with expertise in clinical care (pulmonary, cardiology, pediatrics, and pathology), clinical research, and/or basic science in the areas of PH identified important questions and reviewed and synthesized the literature. Results: This document describes selected PH phenotypes and serves as an initial platform to define additional relevant phenotypes as new knowledge is generated. The biggest gaps in our knowledge stem from the fact that our present understanding of PH phenotypes has not come from any particularly organized effort to identify such phenotypes, but rather from reinterpreting studies and reports that were designed and performed for other purposes. Conclusions: Accurate phenotyping of PH can be used in research studies to increase the homogeneity of study cohorts. Once the ability of the phenotypes to predict outcomes has been validated, phenotyping may also be useful for determining prognosis and guiding treatment. This important next step in PH patient care can optimally be addressed through a consortium of study sites with well-defined goals, tasks, and structure. Planning and support for this could include the National Institutes of Health and the U.S. Food and Drug Administration, with industry and foundation partnerships.
KW - Biomarkers
KW - Consortium
KW - Metabolism
KW - Pathobiology
KW - Pulmonary circulation
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U2 - 10.1164/rccm.201311-1954ST
DO - 10.1164/rccm.201311-1954ST
M3 - Article
C2 - 24484330
AN - SCOPUS:84893573008
SN - 1073-449X
VL - 189
SP - 345
EP - 355
JO - American journal of respiratory and critical care medicine
JF - American journal of respiratory and critical care medicine
IS - 3
ER -