An official American Thoracic Society Statement: pulmonary hypertension phenotypes.
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, advanced imaging, and pathobiology. This document organizes our 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.
Raed A
RA
Dweik
Sharon
S
Rounds
Serpil C
SC
Erzurum
Stephen
S
Archer
Karen
K
Fagan
Paul M
PM
Hassoun
Nicholas S
NS
Hill
Marc
M
Humbert
Steven M
SM
Kawut
Michael
M
Krowka
Evangelos
E
Michelakis
Nicholas W
NW
Morrell
Kurt
K
Stenmark
Rubin M
RM
Tuder
John
J
Newman
D000367:Age Factors; D015415:Biomarkers / Q000378:metabolism; D003240:Connective Tissue Diseases / Q000150:complications; D003952:Diagnostic Imaging; D015658:HIV Infections / Q000150:complications; D006801:Humans; D006975:Hypertension, Portal / Q000150:complications; D006976:Hypertension, Pulmonary / Q000175:diagnosis* / Q000209:etiology / Q000378:metabolism; D017380:Hypertrophy, Right Ventricular / Q000175:diagnosis / Q000209:etiology; D008487:Medical History Taking; D024821:Metabolic Syndrome / Q000150:complications; D010641:Phenotype; D010808:Physical Examination; D012720:Severity of Illness Index
D015415:Biomarkers