000			05802nam a22007577a 4500
008			220222s20222022 xxu||||| |||| 00| 0 eng d
024			_a10.1001/jama.2021.24674 [doi]
024			_a2788528 [pii]
024			_aPMC8808323 [pmc]
040			_aOvid MEDLINE(R)
099			_a35103767
245			_aPrevalence and Cumulative Risk of Familial Idiopathic Dilated Cardiomyopathy.
251			_aJAMA. 327(5):454-463, 2022 02 01.
252			_aJAMA. 327(5):454-463, 2022 02 01.
252			_zJAMA. 327(5):454-463, 2022 02 01.
253			_aJAMA
260			_c2022
260			_fFY2022
260			_p2022 02 01
265			_sppublish
266			_d2022-02-22
268			_aJAMA. 327(5):454-463, 2022 02 01.
269			_fFY2022
501			_aAvailable online from MWHC library: 1998 - present, Available in print through MWHC library: 1999 - present
520			_aConclusions and Relevance: In a US cross-sectional study, there was substantial estimated prevalence of familial DCM among probands and modeled cumulative risk of DCM among their first-degree relatives.
520			_aDesign, Setting, and Participants: A family-based, cross-sectional study conducted by a multisite consortium of 25 US heart failure programs. Participants included patients with DCM (probands), defined as left ventricular systolic dysfunction and left ventricular enlargement after excluding usual clinical causes, and their first-degree relatives. Enrollment commenced June 7, 2016; proband and family member enrollment concluded March 15, 2020, and April 1, 2021, respectively.
520			_aExposures: The presence of DCM in a proband.
520			_aImportance: Idiopathic dilated cardiomyopathy (DCM) aggregates in families, and early detection in at-risk family members can provide opportunity to initiate treatment prior to late-phase disease. Most studies have included only White patients, yet Black patients with DCM have higher risk of heart failure-related hospitalization and death.
520			_aMain Outcomes and Measures: Familial DCM defined by DCM in at least 1 first-degree relative; expanded familial DCM defined by the presence of DCM or either left ventricular enlargement or left ventricular systolic dysfunction without known cause in at least 1 first-degree relative.
520			_aObjective: To estimate the prevalence of familial DCM among DCM probands and the age-specific cumulative risk of DCM in first-degree relatives across race and ethnicity groups.
520			_aResults: The study enrolled 1220 probands (median age, 52.8 years [IQR, 42.4-61.8]; 43.8% female; 43.1% Black and 8.3% Hispanic) and screened 1693 first-degree relatives for DCM. A median of 28% (IQR, 0%-60%) of living first-degree relatives were screened per family. The crude prevalence of familial DCM among probands was 11.6% overall. The model-based estimate of the prevalence of familial DCM among probands at a typical US advanced heart failure program if all living first-degree relatives were screened was 29.7% (95% CI, 23.5% to 36.0%) overall. The estimated prevalence of familial DCM was higher in Black probands than in White probands (difference, 11.3% [95% CI, 1.9% to 20.8%]) but did not differ significantly between Hispanic probands and non-Hispanic probands (difference, -1.4% [95% CI, -15.9% to 13.1%]). The estimated prevalence of expanded familial DCM was 56.9% (95% CI, 50.8% to 63.0%) overall. Based on age-specific disease status at enrollment, estimated cumulative risks in first-degree relatives at a typical US advanced heart failure program reached 19% (95% CI, 13% to 24%) by age 80 years for DCM and 33% (95% CI, 27% to 40%) for expanded DCM inclusive of partial phenotypes. The DCM hazard was higher in first-degree relatives of non-Hispanic Black probands than non-Hispanic White probands (hazard ratio, 1.89 [95% CI, 1.26 to 2.83]).
520			_aTrial Registration: ClinicalTrials.gov Identifier: NCT03037632.
546			_aEnglish
650			_a*Cardiomyopathy, Dilated/ep [Epidemiology]
650			_a*Family Health/sn [Statistics & Numerical Data]
650			_aAdult
650			_aAge Factors
650			_aCardiomyopathy, Dilated/di [Diagnosis]
650			_aCardiomyopathy, Dilated/eh [Ethnology]
650			_aConfidence Intervals
650			_aCross-Sectional Studies
650			_aEarly Diagnosis
650			_aFamily Health/eh [Ethnology]
650			_aFemale
650			_aHumans
650			_aHypertrophy, Left Ventricular/di [Diagnosis]
650			_aHypertrophy, Left Ventricular/eh [Ethnology]
650			_aHypertrophy, Left Ventricular/ep [Epidemiology]
650			_aMale
650			_aMiddle Aged
650			_aPrevalence
650			_aRisk
650			_aUnited States/ep [Epidemiology]
650			_aVentricular Dysfunction, Left/di [Diagnosis]
650			_aVentricular Dysfunction, Left/eh [Ethnology]
650			_aVentricular Dysfunction, Left/ep [Epidemiology]
651			_aMedStar Heart & Vascular Institute
657			_aJournal Article
657			_aMulticenter Study
657			_aResearch Support, N.I.H., Extramural
700			_aHofmeyer, Mark
700			_aNOT FOUND: DCM Precision Medicine Study of the DCM Consortium
790			_aAaronson KD, Burke W, DCM Precision Medicine Study of the DCM Consortium, Ewald GA, Fishbein DP, Garg S, Gottlieb SS, Haas GJ, Hershberger RE, Hofmeyer M, Huggins GS, Jimenez J, Jordan E, Judge DP, Katz S, Kinnamon DD, Kransdorf E, Lowes B, Mead JO, Moore CK, Morris AA, Ni H, Owens A, Pamboukian SV, Pan S, Shah P, Smart F, Stoller D, Sweitzer NK, Tang WHW, Trachtenberg BH, Wang J, Wheeler MT, Wilcox JE
856			_uhttps://dx.doi.org/10.1001/jama.2021.24674 _zhttps://dx.doi.org/10.1001/jama.2021.24674
942			_cART _dArticle
999			_c804 _d804