Dilated-Hypokinetic Evolution of Hypertrophic Cardiomyopathy: Prevalence, Incidence, Risk Factors, and Prognostic Implications in Pediatric and Adult Patients

作者: Elena Biagini , Fabio Coccolo , Marinella Ferlito , Enrica Perugini , Guido Rocchi

DOI: 10.1016/J.JACC.2005.04.062

关键词: Internal medicineRisk factorIncidence (epidemiology)Hypertrophic cardiomyopathySeptal myectomyFamily historyCardiomyopathyProspective cohort studyPediatricsCardiologyMedicineSudden death

摘要: Objectives This study sought to investigate the incidence, risk factors, and prognosis of dilated-hypokinetic evolution in a large cohort patients with hypertrophic cardiomyopathy (HCM) followed up at cardiology center serving both pediatric adult population. Background The available data on this HCM mainly regards prevalence (rather than incidence) adults, very little being known about Methods A total 222 consecutive (65% men, 19% ≤18 years old) were prospectively evaluated for mean follow-up 11 ± 9 years. Results diagnosis was made 12 first evaluation (11 without previous septal myectomy surgery; prevalence, 4.9%). Twelve 210 classic underwent (incidence, 5.3/1,000 patient-years). Patients prevalent/incident younger (32 14 vs. 41 21 years, p = 0.04) more often had family history (61% 26%, 0.002) or sudden death (43% 19%, 0.01) respect who maintained HCM. Moreover, they showed greater interventricular septum (23 3 mm 19 6 mm, 0.004) posterior wall (15 13 4 0.006) thickness. Cardiovascular death-free survival lower among (p Conclusions Dilated-hypokinetic is rare but not exceptional Young age diagnosis, HCM, thickness are incremental factors which carries an ominous prognosis.

参考文章(31)
Barry J. Maron, Robert O. Bonow, Richard O. Cannon, Martin B. Leon, Stephen E. Epstein, Hypertrophic cardiomyopathy. Interrelations of clinical manifestations, pathophysiology, and therapy (1). The New England Journal of Medicine. ,vol. 316, pp. 780- 789 ,(1987) , 10.1056/NEJM198703263161305
Yoshinori L. Doi, Hiroaki Kitaoka, Nobuhiko Hitomi, Manatsu Satoh, Akinori Kimura, Clinical Expression in Patients With Hypertrophic Cardiomyopathy Caused by Cardiac Myosin-Binding Protein C Gene Mutation Circulation. ,vol. 100, pp. 446- 449 ,(1999) , 10.1161/CIRC.100.4.446/-C
David R. Cox, Regression Models and Life-Tables Springer Series in Statistics. ,vol. 34, pp. 527- 541 ,(1992) , 10.1007/978-1-4612-4380-9_37
F.J. ten Cate, J. Roelandt, Progression to left ventricular dilatation in patients with hypertrophic obstructive cardiomyopathy American Heart Journal. ,vol. 97, pp. 762- 765 ,(1979) , 10.1016/0002-8703(79)90012-7
Hiromasa Kokado, Masami Shimizu, Hiroyuki Yoshio, Hidekazu Ino, Kazuyasu Okeie, Yorito Emoto, Toru Matsuyama, Masato Yamaguchi, Toshihiko Yasuda, Noboru Fujino, Hideki Ito, Hiroshi Mabuchi, Clinical Features of Hypertrophic Cardiomyopathy Caused by a Lys183 Deletion Mutation in the Cardiac Troponin I Gene Circulation. ,vol. 102, pp. 663- 669 ,(2000) , 10.1161/01.CIR.102.6.663
Paolo Spirito, Barry J. Maron, Robert O. Bonow, Stephen E. Epstein, Severe functional limitation in patients with hypertrophic cardiomyopathy and only mild localized left ventricular hypertrophy Journal of the American College of Cardiology. ,vol. 8, pp. 537- 544 ,(1986) , 10.1016/S0735-1097(86)80180-2
Jamshid Shirani, Barry J. Maron, Richard O. Cannon, Sheyda Shahin, William C. Roberts, Clinicopathologic features of hypertrophic cardiomyopathy managed by cardiac transplantation The American Journal of Cardiology. ,vol. 72, pp. 434- 440 ,(1993) , 10.1016/0002-9149(93)91136-6
Sayid Fighali, Zvonimir Krajcer, Sidney Edelman, Robert D. Leachman, Progression of hypertrophic cardiomyopathy into a hypokinetic left ventricle: Higher incidence in patients with midventricular obstruction Journal of the American College of Cardiology. ,vol. 9, pp. 288- 294 ,(1987) , 10.1016/S0735-1097(87)80377-7