The DOI matches its Crossref record; 31 of the 31 references it cites were found in a registry or an index.
Prevalence, Clinical Significance, and Natural History of Left Ventricular Apical Aneurysms in Hypertrophic Cardiomyopathy
Open accessAbstract
Background— Hypertrophic cardiomyopathy (HCM) is the most common genetic heart disease characterized by a diverse clinical and phenotypic spectrum. This study reports the prevalence, morphology, clinical course, and management of an underrecognized subgroup of HCM patients with left ventricular apical aneurysms. Methods and Results— Of 1299 HCM patients, 28 (2%) were identified with left ventricular apical aneurysms, including a pair of identical twins. Aneurysms were recognized at a wide age range (26 to 83 years), including 12 patients (43%) who were ≤50 years of age. Apical aneurysms varied considerably in size (maximum dimension, 10 to 66 mm), were dyskinetic/akinetic with thin rims, and were associated with transmural (and often more extensive) myocardial scarring identified by late gadolinium enhancement cardiovascular magnetic resonance. Apical aneurysms were recognized by echocardiography in only 16 of 28 patients (57%) but by cardiovascular magnetic resonance in the 12 patients undetected by echocardiography. Left ventricular chamber morphology varied; however, 19 patients (68%) showed an “hourglass” contour, with midventricular hypertrophy producing muscular narrowing and intracavitary gradients in 9 patients (74±42 mm Hg). Sarcomeric protein missense mutations known to cause other phenotypic expressions of HCM were present in 3 patients. Over 4.1±3.7 years of follow-up, 12 patients (43%) with left ventricular apical aneurysms experienced adverse disease complications (event rate, 10.5%/y), including sudden death, appropriate implantable cardioverter-defibrillator discharges, nonfatal thromboembolic stroke, and progressive heart failure and death. Conclusions— Patients with left ventricular apical aneurysms represent an underappreciated subset in the heterogeneous HCM disease spectrum with important clinical implications, often requiring a high index of suspicion and cardiovascular magnetic resonance for identification. Apical aneurysms in HCM are associated with substantial cardiovascular morbidity and mortality and raise novel treatment considerations.
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Cites
Hypertrophic Cardiomyopathy: A Systematic Review
Maron, B. J. (2002) · JAMA · doi:10.1001/jama.287.10.1308 · Cited by 2,224
Self-citationMaron, B. J., McKenna, W. J., Danielson, G. K., Kappenberger, L. J., Kuhn, H. J., Seidman, C. E., Shah, P. M., Spencer, W. H., Spirito, P., Ten Cate, F. J., et al. (2003) · Journal of the American College of Cardiology · doi:10.1016/s0735-1097(03)00941-0 · Cited by 1,578
Self-citationRichard, P., Charron, P., Carrier, L., Ledeuil, C., Cheav, T., Pichereau, C., Benaiche, A., Isnard, R., Dubourg, O., Burban, M., et al. (2003) · Circulation · doi:10.1161/01.cir.0000066323.15244.54 · Cited by 1,279
Maron, M. S., Olivotto, I., Betocchi, S., Casey, S. A., Lesser, J. R., Losi, M. A., Cecchi, F., & Maron, B. J. (2003) · New England Journal of Medicine · doi:10.1056/nejmoa021332 · Cited by 1,259
Self-citationMagnitude of Left Ventricular Hypertrophy and Risk of Sudden Death in Hypertrophic Cardiomyopathy
Spirito, P., Bellone, P., Harris, K. M., Bernabò, P., Bruzzi, P., & Maron, B. J. (2000) · New England Journal of Medicine · doi:10.1056/nejm200006153422403 · Cited by 1,143
Self-citationThe Genetic Basis for Cardiomyopathy
Seidman, J. G., & Seidman, C. (2001) · Cell · doi:10.1016/s0092-8674(01)00242-2 · Cited by 1,046
The Management of Hypertrophic Cardiomyopathy
Spirito, P., Seidman, C. E., McKenna, W. J., & Maron, B. J. (1997) · New England Journal of Medicine · doi:10.1056/nejm199703133361107 · Cited by 1,007
Self-citationSudden death in hypertrophic cardiomyopathy: identification of high risk patients
Elliott, P. M., Poloniecki, J., Dickie, S., Sharma, S., Monserrat, L., Varnava, A., Mahon, N. G., & McKenna, W. J. (2000) · Journal of the American College of Cardiology · doi:10.1016/s0735-1097(00)01003-2 · Cited by 978
Maron, B. J., Spirito, P., Shen, W.-K., Haas, T. S., Formisano, F., Link, M. S., Epstein, A. E., Almquist, A. K., Daubert, J. P., Lawrenz, T., et al. (2007) · JAMA · doi:10.1001/jama.298.4.405 · Cited by 804
Self-citationHypertrophic Cardiomyopathy: Clinical Spectrum and Treatment
Wigle, E. D., Rakowski, H., Kimball, B. P., & Williams, W. G. (1995) · Circulation · doi:10.1161/01.cir.92.7.1680 · Cited by 778
Cited by
2014 ESC Guidelines on diagnosis and management of hypertrophic cardiomyopathy
Authors/Task Force members, Elliott, P. M., Anastasakis, A., Borger, M. A., Borggrefe, M., Cecchi, F., Charron, P., Hagege, A. A., Lafont, A., Limongelli, G., et al. (2014) · European Heart Journal · doi:10.1093/eurheartj/ehu284 · Cited by 4,332
2023 ESC guidelines for the management of cardiomyopathies
Arbelo, E., Protonotarios, A., Gimeno, J. R., Arbustini, E., Barriales-Villa, R., Basso, C., Bezzina, C. R., Biagini, E., Blom, N. A., de Boer, R. A., et al. (2023) · European Heart Journal · doi:10.1093/eurheartj/ehad194 · Cited by 2,844
Gersh, B. J., Maron, B. J., Bonow, R. O., Dearani, J. A., Fifer, M. A., Link, M. S., Naidu, S. S., Nishimura, R. A., Ommen, S. R., Rakowski, H., et al. (2011) · Circulation · doi:10.1161/cir.0b013e318223e2bd · Cited by 2,104
Self-citation2011 ACCF/AHA Guideline for the Diagnosis and Treatment of Hypertrophic Cardiomyopathy
Gersh, B. J., Maron, B. J., Bonow, R. O., Dearani, J. A., Fifer, M. A., Link, M. S., Naidu, S. S., Nishimura, R. A., Ommen, S. R., Rakowski, H., et al. (2011) · Journal of the American College of Cardiology · doi:10.1016/j.jacc.2011.06.011 · Cited by 1,276
Self-citationMaron, B. J., & Maron, M. S. (2013) · The Lancet · doi:10.1016/s0140-6736(12)60397-3 · Cited by 1,231
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Metadata
- Authors
- Maron, M. S., Finley, J. J., Bos, J. M., Hauser, T. H., Manning, W. J., Haas, T. S., Lesser, J. R., Udelson, J. E., Ackerman, M. J., & Maron, B. J.
- Venue
- Circulation
- Published
- 2008-10-07
- Volume
- 118
- Issue
- 15
- Pages
- 1541-1549
- ISSN
- 0009-7322 (ISSN-L)
- 1524-4539
- OpenAlex
- W2049057276
- Cited by
- 567
- References
- 31
- FWCI
- 16.72
- Type
- journal article
- Language
- en
- Open access
- bronze
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- Maron, M. S., Finley, J. J., Bos, J. M., Hauser, T. H., Manning, W. J., Haas, T. S., Lesser, J. R., Udelson, J. E., Ackerman, M. J., & Maron, B. J. (2008). Prevalence, Clinical Significance, and Natural History of Left Ventricular Apical Aneurysms in Hypertrophic Cardiomyopathy. Circulation. https://doi.org/10.1161/circulationaha.108.781401
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