Hypertrophic Cardiomyopathy Morphology and Significance of the Left Ventricular Collagen Network in Young Patients With Hypertrophic Cardiomyopathy and Sudden Cardiac Death

METHODS We analyzed the amount, distribution and structure of the cardiac collagen network in transmural sections of the ventricular septum (thickness 17 to 40 mm, mean 25 mm) in 16 previously asymptomatic children and young adults with HCM (11 to 31 years of age, mean 20 years) who died suddenly. The morphologic appearance and volume fractions of interstitial (matrix) and perivascular (adventitial) collagen were analyzed with polarization microscopy and computerized videodensitometry in picrosirius red-stained sections. Findings were compared with 16 structurally normal hearts, 5 with systemic hypertension and 6 infants who died of HCM.

[1]  B. Maron,et al.  Implications of left ventricular remodeling in hypertrophic cardiomyopathy. , 1998, The American journal of cardiology.

[2]  D. Kass,et al.  Marked discordance between dynamic and passive diastolic pressure-volume relations in idiopathic hypertrophic cardiomyopathy. , 1996, Circulation.

[3]  B. Maron,et al.  Phenotypic spectrum and patterns of left ventricular hypertrophy in hypertrophic cardiomyopathy: morphologic observations and significance as assessed by two-dimensional echocardiography in 600 patients. , 1995, Journal of the American College of Cardiology.

[4]  A. Marian,et al.  Recent advances in the molecular genetics of hypertrophic cardiomyopathy. , 1995, Circulation.

[5]  M. Komajda,et al.  Molecular basis of familial cardiomyopathies. , 1995, Circulation.

[6]  M. Burch,et al.  Hypertrophic cardiomyopathy. , 1994, Archives of disease in childhood.

[7]  R. Kloner,et al.  Quantitative assessment of myocardial collagen with picrosirius red staining and circularly polarized light , 1994, Basic Research in Cardiology.

[8]  W. Mckenna,et al.  Management of hypertrophic cardiomyopathy. , 1993 .

[9]  W. Williams,et al.  Transesophageal Doppler echocardiography in obstructive hypertrophic cardiomyopathy: clarification of pathophysiology and importance in intraoperative decision making. , 1992, Journal of the American College of Cardiology.

[10]  W. Roberts,et al.  Diversity of Structural Mitral Valve Alterations in Hypertrophic Cardiomyopathy , 1992, Circulation.

[11]  W. Roberts,et al.  Anomalous Insertion of Papillary Muscle Directly Into Anterior Mitral Leaflet in Hypertrophic Cardiomyopathy: Significance in Producing Left Ventricular Outflow Obstruction , 1991, Circulation.

[12]  K. Weber,et al.  Pathological Hypertrophy and Cardiac Interstitium: Fibrosis and Renin‐Angiotensin‐Aldosterone System , 1991, Circulation.

[13]  S. Factor,et al.  Pathologic fibrosis and matrix connective tissue in the subaortic myocardium of patients with hypertrophic cardiomyopathy. , 1991, Journal of the American College of Cardiology.

[14]  Y. Koga,et al.  Decreased coronary vasodilatory capacity in hypertrophic cardiomyopathy determined by split-dose thallium-dipyridamole myocardial scintigraphy. , 1990, The American journal of cardiology.

[15]  B. Maron,et al.  Relation between extent of left ventricular hypertrophy and diastolic filling abnormalities in hypertrophic cardiomyopathy. , 1990, Journal of the American College of Cardiology.

[16]  K. Weber,et al.  Cardiac interstitium in health and disease: the fibrillar collagen network. , 1989, Journal of the American College of Cardiology.

[17]  M. Shimizu,et al.  Quantitation of myocardial fibrosis and its relation to function in essential hypertension and hypertrophic cardiomyopathy , 1988, Clinical cardiology.

[18]  R. Bonow,et al.  Noninvasive assessment of left ventricular diastolic function by pulsed Doppler echocardiography in patients with hypertrophic cardiomyopathy. , 1987, Journal of the American College of Cardiology.

[19]  S. Seifter,et al.  Morphology, composition, and function of struts between cardiac myocytes of rat and hamster , 1987, Cell and Tissue Research.

[20]  An Integrated Mechanism for Systolic Anterior Motion of the Mitral Valve in Hypertrophic Cardiomyopathy Based on Echocardiographic Observations , 1987 .

[21]  M. Matsuda,et al.  Pathogenetic role of myocardial fiber disarray in the progression of cardiac fibrosis in normal hearts, hypertensive hearts and hearts with hypertrophic cardiomyopathy. , 1987, Japanese circulation journal.

[22]  W. Roberts,et al.  Regional myocyte hypertrophy and increased interstitial myocardial fibrosis in hypertrophic cardiomyopathy. , 1987, The American journal of cardiology.

[23]  B. Maron,et al.  Development and progression of left ventricular hypertrophy in children with hypertrophic cardiomyopathy. , 1986, The New England journal of medicine.

[24]  W. Roberts,et al.  Intramural ("small vessel") coronary artery disease in hypertrophic cardiomyopathy. , 1986, Journal of the American College of Cardiology.

[25]  C Kawai,et al.  Quantitative analysis of myocardial fibrosis in normals, hypertensive hearts, and hypertrophic cardiomyopathy. , 1986, British heart journal.

[26]  W. Williams,et al.  Hypertrophic cardiomyopathy. The importance of the site and the extent of hypertrophy. A review. , 1985, Progress in cardiovascular diseases.

[27]  R. Bonow,et al.  Myocardial ischemia in patients with hypertrophic cardiomyopathy: contribution of inadequate vasodilator reserve and elevated left ventricular filling pressures. , 1985, Circulation.

[28]  S. M. Collins,et al.  Computer-assisted quantitation of myocardial fibrosis in histologic sections. , 1984, Archives of pathology & laboratory medicine.

[29]  S. Factor,et al.  Skeletal framework of mammalian heart muscle. Arrangement of inter- and pericellular connective tissue structures. , 1983, Laboratory investigation; a journal of technical methods and pathology.

[30]  Y. Hamashima,et al.  Number and size of myocytes and amount of interstitial space in the ventricular septum and in the left ventricular free wall in hypertrophic cardiomyopathy. , 1983, The American journal of cardiology.

[31]  A. Fishman,et al.  Muscle fiber orientation and connective tissue content in the hypertrophied human heart. , 1982, Laboratory investigation; a journal of technical methods and pathology.

[32]  M. Cheitlin,et al.  The Distribution of Fibrosis in the Left Ventricle in Congenital Aortic Stenosis and Coarctation of the Aorta , 1980, Circulation.

[33]  W. Roberts,et al.  Hypertrophic cardiomyopathy and transmural myocardial infarction without significant atherosclerosis of the extramural coronary arteries. , 1979, The American journal of cardiology.

[34]  W. Roberts,et al.  Quantitative Analysis of Cardiac Muscle Cell Disorganization in the Ventricular Septum of Patients with Hypertrophic Cardiomyopathy , 1979, Circulation.

[35]  D TEARE,et al.  ASYMMETRICAL HYPERTROPHY OF THE HEART IN YOUNG ADULTS , 1958, British heart journal.

[36]  J. Towbin,et al.  Impact of Laboratory Molecular Diagnosis on Contemporary Diagnostic Criteria for Genetically Transmitted Cardiovascular Diseases : Hypertrophic Cardiomyopathy , Long-QT Syndrome , and Marfan Syndrome , 1998 .