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Cited by 328

The DOI matches its Crossref record; 35 of the 35 references it cites were found in a registry or an index.

Distinct Subgroups in Hypertrophic Cardiomyopathy in the NHLBI HCM Registry

Open access
Neubauer, Stefan, Kolm, Paul, Ho, Carolyn Y., Kwong, Raymond Y., Desai, Milind Y., Dolman, Sarahfaye F., Appelbaum, Evan, Desvigne-Nickens, Patrice, DiMarco, John P., Friedrich, Matthias G., Geller, Nancy, Harper, Andrew R., Jarolim, Petr, Jerosch-Herold, Michael, Kim, Dong-Yun, Maron, Martin S., Schulz-Menger, Jeanette, Piechnik, Stefan K., Thomson, Kate, Zhang, Cheng, Watkins, Hugh, Weintraub, William S., Kramer, Christopher M., Mahmod, Masliza, Jacoby, Daniel, White, James, Chiribiri, Amedeo, Helms, Adam, Choudhury, Lubna, Michels, Michelle, Bradlow, William, Salerno, Michael, Heitner, Stephen, Prasad, Sanjay, Mohiddin, Saidi, Swoboda, Peter, Mahrholdt, Heiko, Bucciarelli-Ducci, Chiara, Weinsaft, Jonathan, Kim, Han, McCann, Gerry, van Rossum, Albert, Williamson, Eric, Flett, Andrew, Dawson, Dana, Mongeon, F. Pierre, Olivotto, Iacopo, Crean, Andrew, Owens, Anjali, Anderson, Lisa, Biagini, Elena, Newby, David, Berry, Colin, Kim, Bette, Larose, Eric, Abraham, Theodore, Sherrid, Mark, Nagueh, Sherif, Rimoldi, Ornella, Elstein, Eleanor, Autore, Camillo·2019·Journal of the American College of Cardiology
DOI 10.1016/j.jacc.2019.08.1057
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Abstract

Background: The Hypertrophic Cardiomyopathy Registry (HCMR) is a NHLBI-funded, prospective registry of 2755 patients with HCM recruited from 44 sites in 6 countries. Objectives: To improve risk prediction in HCM by incorporating cardiovascular magnetic resonance (CMR), genetic, and biomarker data. Methods: Demographic and echocardiographic data were collected. Patients underwent CMR including cine imaging, late gadolinium enhancement imaging (LGE, replacement fibrosis) and T1 mapping for measurement of extracellular volume (ECV) as a measure of interstitial fibrosis. Blood was drawn for biomarker (N-terminal pro brain natriuretic peptide [NTproBNP] and high sensitivity troponin T [cTnT]) and genetic analysis. Results: 2,755 patients were studied. Mean age was 49±11 years, 71% were male, and 17% non-white. Mean ESC risk score was 2.48±0.56. Eighteen % had a resting left ventricular outflow tract (LVOT) gradient ≥30mmHg. Thirty six percent had a sarcomere mutation identified and 50 percent had any LGE. Sarcomere mutation (+) patients were more likely to have reverse septal curvature morphology, LGE, and no significant resting LVOT obstruction. Those that were sarcomere mutation (−), had isolated basal septal hypertrophy, less LGE, and more LVOT obstruction. Interstitial fibrosis was present in segments both with and without LGE. Serum NTproBNP and cTnT levels correlated with increasing LGE and ECV in a graded fashion. Conclusions: The HCMR population has characteristics of low risk HCM. 93% had no or only mild functional limitation. Baseline data separated patients broadly into 2 categories. One group was sarcomere mutation (+), more likely had reverse septal curvature morphology, more fibrosis, but less resting obstruction whereas the other was sarcomere mutation (−), more likely had isolated basal septal hypertrophy with obstruction but less fibrosis. Further follow-up will allow better understanding of these subgroups and development of an improved risk prediction model incorporating all these markers.

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Metadata

Authors
Neubauer, S., Kolm, P., Ho, C. Y., Kwong, R. Y., Desai, M. Y., Dolman, S. F., Appelbaum, E., Desvigne-Nickens, P., DiMarco, J. P., Friedrich, M. G., Geller, N., Harper, A. R., Jarolim, P., Jerosch-Herold, M., Kim, D.-Y., Maron, M. S., Schulz-Menger, J., Piechnik, S. K., Thomson, K., Zhang, C., Watkins, H., Weintraub, W. S., Kramer, C. M., Mahmod, M., Jacoby, D., White, J., Chiribiri, A., Helms, A., Choudhury, L., Michels, M., Bradlow, W., Salerno, M., Heitner, S., Prasad, S., Mohiddin, S., Swoboda, P., Mahrholdt, H., Bucciarelli-Ducci, C., Weinsaft, J., Kim, H., McCann, G., van Rossum, A., Williamson, E., Flett, A., Dawson, D., Mongeon, F. P., Olivotto, I., Crean, A., Owens, A., Anderson, L., Biagini, E., Newby, D., Berry, C., Kim, B., Larose, E., Abraham, T., Sherrid, M., Nagueh, S., Rimoldi, O., Elstein, E., & Autore, C.
Venue
Journal of the American College of Cardiology
Publisher
Elsevier BV
Published
2019-11
Volume
74
Issue
19
Pages
2333-2345
ISSN
0735-1097 (ISSN-L)
DOI
10.1016/j.jacc.2019.08.1057
OpenAlex
W2983101720
Cited by
328
References
35
FWCI
16.79
Type
journal article
Language
en
Open access
bronze

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Neubauer, S., Kolm, P., Ho, C. Y., Kwong, R. Y., Desai, M. Y., Dolman, S. F., Appelbaum, E., Desvigne-Nickens, P., DiMarco, J. P., Friedrich, M. G., Geller, N., Harper, A. R., Jarolim, P., Jerosch-Herold, M., Kim, D.-Y., Maron, M. S., Schulz-Menger, J., Piechnik, S. K., Thomson, K., . . . Autore, C. (2019). Distinct Subgroups in Hypertrophic Cardiomyopathy in the NHLBI HCM Registry. Journal of the American College of Cardiology. https://doi.org/10.1016/j.jacc.2019.08.1057

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Contains information from the Semantic Scholar Academic Graph Datasets, made available under the ODC Attribution License (ODC-BY 1.0).

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