PMID- 31799990
OWN - NLM
STAT- MEDLINE
DCOM- 20210111
LR  - 20221207
IS  - 2380-6591 (Electronic)
IS  - 2380-6583 (Print)
VI  - 5
IP  - 1
DP  - 2020 Jan 1
TI  - Association of Race With Disease Expression and Clinical Outcomes Among Patients 
      With Hypertrophic Cardiomyopathy.
PG  - 83-91
LID - 10.1001/jamacardio.2019.4638 [doi]
AB  - IMPORTANCE: Racial differences are recognized in multiple cardiovascular 
      parameters, including left ventricular hypertrophy and heart failure, which are 2 
      major manifestations of hypertrophic cardiomyopathy. The association of race with 
      disease expression and outcomes among patients with hypertrophic cardiomyopathy 
      is not well characterized. OBJECTIVE: To assess the association between race, 
      disease expression, care provision, and clinical outcomes among patients with 
      hypertrophic cardiomyopathy. DESIGN, SETTING, AND PARTICIPANTS: This 
      retrospective cohort study included data on black and white patients with 
      hypertrophic cardiomyopathy from the US-based sites of the Sarcomeric Human 
      Cardiomyopathy Registry from 1989 through 2018. EXPOSURES: Self-identified race. 
      MAIN OUTCOMES AND MEASURES: Baseline characteristics; genetic architecture; 
      adverse outcomes, including cardiac arrest, cardiac transplantation or left 
      ventricular assist device implantation, implantable cardioverter-defibrillator 
      therapy, all-cause mortality, atrial fibrillation, stroke, and New York Heart 
      Association (NYHA) functional class III or IV heart failure; and septal reduction 
      therapies. The overall composite outcome consists of the first occurrence of any 
      component of the ventricular arrhythmic composite end point, cardiac 
      transplantation, left ventricular assist device implantation, NYHA class III or 
      IV heart failure, atrial fibrillation, stroke, or all-cause mortality. RESULTS: 
      Of 2467 patients with hypertrophic cardiomyopathy at the time of analysis, 205 
      (8.3%) were black (130 male [63.4%]; mean [SD] age, 40.0 [18.6] years) and 2262 
      (91.7%) were white (1351 male [59.7%]; mean [SD] age, 45.5 [20.5] years). 
      Compared with white patients, black patients were younger at the time of 
      diagnosis (mean [SD], 36.5 [18.2] vs 41.9 [20.2] years; P < .001), had higher 
      prevalence of NYHA class III or IV heart failure at presentation (36 of 205 
      [22.6%] vs 174 of 2262 [15.8%]; P = .001), had lower rates of genetic testing 
      (111 [54.1%] vs 1404 [62.1%]; P = .03), and were less likely to have sarcomeric 
      mutations identified by genetic testing (29 [26.1%] vs 569 [40.5%]; P = .006). 
      Implantation of implantable cardioverter-defibrillators did not vary by race; 
      however, invasive septal reduction was less common among black patients (30 
      [14.6%] vs 521 [23.0%]; P = .007). Black patients had less incident atrial 
      fibrillation (35 [17.1%] vs 608 [26.9%]; P < .001). Black race was associated 
      with increased development of NYHA class III or IV heart failure (hazard ratio, 
      1.45; 95% CI, 1.08-1.94) which persisted on multivariable Cox proportional 
      hazards regression (hazard ratio, 1.97; 95% CI, 1.34-2.88). There were no 
      differences in the associations of race with stroke, ventricular arrhythmias, 
      all-cause mortality, or the overall composite outcome. CONCLUSIONS AND RELEVANCE: 
      The findings suggest that black patients with hypertrophic cardiomyopathy are 
      diagnosed at a younger age, are less likely to carry a sarcomere mutation, have a 
      higher burden of functionally limited heart failure, and experience inequities in 
      care with lower use of invasive septal reduction therapy and genetic testing 
      compared with white patients. Further study is needed to assess whether higher 
      rates of heart failure may be associated with underlying ancestry-based disease 
      pathways, clinical management, or structural inequities.
FAU - Eberly, Lauren A
AU  - Eberly LA
AD  - Division of Cardiovascular Medicine, Brigham and Women's Hospital, Boston, 
      Massachusetts.
FAU - Day, Sharlene M
AU  - Day SM
AD  - Department of Internal Medicine, University of Michigan, Ann Arbor.
FAU - Ashley, Euan A
AU  - Ashley EA
AD  - Stanford Center for Inherited Heart Disease, Palo Alto, California.
FAU - Jacoby, Daniel L
AU  - Jacoby DL
AD  - Section of Cardiovascular Medicine, Yale University, New Haven, Connecticut.
FAU - Jefferies, John Lynn
AU  - Jefferies JL
AD  - Heart Institute and the Division of Human Genetics, Cincinnati Children's 
      Hospital Medical Center, Cincinnati, Ohio.
FAU - Colan, Steven D
AU  - Colan SD
AD  - Department of Cardiology, Boston Children's Hospital, Boston, Massachusetts.
FAU - Rossano, Joseph W
AU  - Rossano JW
AD  - Division of Cardiology, Children's Hospital of Philadelphia, Philadelphia, 
      Pennsylvania.
FAU - Semsarian, Christopher
AU  - Semsarian C
AD  - Agnes Ginges Centre for Molecular Cardiology, Centenary Institute and The 
      University of Sydney, Sydney, New South Wales, Australia.
FAU - Pereira, Alexandre C
AU  - Pereira AC
AD  - Heart Institute (Instituto do Coracao da Universidade de Sao Paulo), University 
      of Sao Paulo Medical School, Sao Paulo, Brazil.
FAU - Olivotto, Iacopo
AU  - Olivotto I
AD  - Cardiomyopathy Unit and Genetic Unit, Careggi University Hospital, Florence, 
      Italy.
FAU - Ingles, Jodie
AU  - Ingles J
AD  - Agnes Ginges Centre for Molecular Cardiology, Centenary Institute and The 
      University of Sydney, Sydney, New South Wales, Australia.
FAU - Seidman, Christine E
AU  - Seidman CE
AD  - Division of Cardiovascular Medicine, Brigham and Women's Hospital, Boston, 
      Massachusetts.
FAU - Channaoui, Nadine
AU  - Channaoui N
AD  - Division of Cardiovascular Medicine, Brigham and Women's Hospital, Boston, 
      Massachusetts.
FAU - Cirino, Allison L
AU  - Cirino AL
AD  - Division of Cardiovascular Medicine, Brigham and Women's Hospital, Boston, 
      Massachusetts.
FAU - Han, Larry
AU  - Han L
AD  - Division of Cardiovascular Medicine, Brigham and Women's Hospital, Boston, 
      Massachusetts.
AD  - Department of Biostatistics, Harvard T. H. Chan School of Public Health, Boston, 
      Massachusetts.
FAU - Ho, Carolyn Y
AU  - Ho CY
AD  - Division of Cardiovascular Medicine, Brigham and Women's Hospital, Boston, 
      Massachusetts.
FAU - Lakdawala, Neal K
AU  - Lakdawala NK
AD  - Division of Cardiovascular Medicine, Brigham and Women's Hospital, Boston, 
      Massachusetts.
LA  - eng
GR  - T32 LM012411/LM/NLM NIH HHS/United States
GR  - UL1 TR001863/TR/NCATS NIH HHS/United States
GR  - HHMI/Howard Hughes Medical Institute/United States
PT  - Journal Article
PT  - Research Support, N.I.H., Extramural
PT  - Research Support, Non-U.S. Gov't
PL  - United States
TA  - JAMA Cardiol
JT  - JAMA cardiology
JID - 101676033
SB  - IM
MH  - Adult
MH  - *Black or African American
MH  - Aged
MH  - Atrial Fibrillation/epidemiology/ethnology
MH  - Cardiomyopathy, Hypertrophic/*ethnology/physiopathology/therapy
MH  - Cohort Studies
MH  - Death, Sudden, Cardiac/epidemiology/prevention & control
MH  - Defibrillators, Implantable/statistics & numerical data
MH  - Female
MH  - Genetic Testing/*statistics & numerical data
MH  - Health Services Accessibility
MH  - Healthcare Disparities/*ethnology
MH  - Heart Failure/epidemiology/ethnology
MH  - Heart Septum/*surgery
MH  - Heart Transplantation
MH  - Heart-Assist Devices/statistics & numerical data
MH  - Humans
MH  - Male
MH  - Middle Aged
MH  - Mortality/*ethnology
MH  - Phenotype
MH  - Quality of Health Care
MH  - Stroke/epidemiology/ethnology
MH  - United States/epidemiology
MH  - *White People
PMC - PMC6902181
COIS- Conflict of Interest Disclosures: Dr Day reported receiving grants from MyoKardia 
      during the conduct of the study. Dr Ashley reported receiving personal fees from 
      Apple, DeepCell, Inc, and Personalis, Inc during the conduct of the study. Dr 
      Jacoby reported receiving grants and personal fees from MyoKardia during the 
      conduct of the study and receiving personal fees from Alnylam and Abbott outside 
      the submitted work. Dr Rossano reported receiving personal fees from Novartis, 
      Amgen, CSL Behring, and Bayer outside the submitted work. Dr Olivotto reported 
      receiving grants from MyoKardia during the conduct of the study; receiving grants 
      from the Menarini Group and Bayer; and receiving grants and personal fees from 
      Sanofi Genzyme and Shire (now Takeda Pharmaceutical Company Ltd) outside the 
      submitted work. Dr Seidman reported receiving grants from Howard Hughes Medical 
      Institute during the conduct of the study. Dr Ho reported receiving grants and 
      personal consulting fees from MyoKardia during the conduct of the study. Dr 
      Lakdawala reported receiving personal fees from MyoKardia outside the submitted 
      work. No other disclosures were reported.
EDAT- 2019/12/05 06:00
MHDA- 2021/01/12 06:00
PMCR- 2020/12/04
CRDT- 2019/12/05 06:00
PHST- 2019/12/05 06:00 [pubmed]
PHST- 2021/01/12 06:00 [medline]
PHST- 2019/12/05 06:00 [entrez]
PHST- 2020/12/04 00:00 [pmc-release]
AID - 2755970 [pii]
AID - hoi190081 [pii]
AID - 10.1001/jamacardio.2019.4638 [doi]
PST - ppublish
SO  - JAMA Cardiol. 2020 Jan 1;5(1):83-91. doi: 10.1001/jamacardio.2019.4638.