Development and progression of left ventricular hypertrophy in children with hypertrophic cardiomyopathy. 1986

B J Maron, and P Spirito, and Y Wesley, and J Arce

Whether the magnitude and distribution of left ventricular hypertrophy in patients with hypertrophic cardiomyopathy are established at birth, or whether they evolve during the first years of life, is unknown. Accordingly, we conducted serial echocardiographic studies in 39 children with a family history or morphologic evidence of hypertrophic cardiomyopathy. The patients were initially evaluated at 4 to 15 years of age (mean, 11) and most recently at 9 to 20 years (mean, 16). During a follow-up period of 2.5 to 6.8 years (mean, 4), the magnitude and extent of preexisting left ventricular hypertrophy markedly increased in 17 patients and the morphologic appearance of the heart evolved from normal to hypertrophic in 5 others. In these 22 patients the left ventricular wall thickness increased strikingly (by 6 to 23 mm, a change of 101 +/- 62 percent); these increases significantly exceeded those expected as a consequence of normal growth (13 +/- 10 percent; P less than 0.001) and were not associated with symptomatic deterioration or related to subaortic obstruction. We conclude that left ventricular hypertrophy may develop or progress spontaneously in patients with hypertrophic cardiomyopathy during childhood, when body growth is considerable. Since echocardiograms may be normal during childhood--before the morphologic features of hypertrophic cardiomyopathy develop--a single echocardiographic examination of young relatives of patients with hypertrophic cardiomyopathy may not exclude this disease.

UI MeSH Term Description Entries
D008297 Male Males
D002312 Cardiomyopathy, Hypertrophic A form of CARDIAC MUSCLE disease, characterized by left and/or right ventricular hypertrophy (HYPERTROPHY, LEFT VENTRICULAR; HYPERTROPHY, RIGHT VENTRICULAR), frequent asymmetrical involvement of the HEART SEPTUM, and normal or reduced left ventricular volume. Risk factors include HYPERTENSION; AORTIC STENOSIS; and gene MUTATION; (FAMILIAL HYPERTROPHIC CARDIOMYOPATHY). Cardiomyopathy, Hypertrophic Obstructive,Cardiomyopathies, Hypertrophic,Cardiomyopathies, Hypertrophic Obstructive,Hypertrophic Cardiomyopathies,Hypertrophic Cardiomyopathy,Hypertrophic Obstructive Cardiomyopathies,Hypertrophic Obstructive Cardiomyopathy,Obstructive Cardiomyopathies, Hypertrophic,Obstructive Cardiomyopathy, Hypertrophic
D002648 Child A person 6 to 12 years of age. An individual 2 to 5 years old is CHILD, PRESCHOOL. Children
D002675 Child, Preschool A child between the ages of 2 and 5. Children, Preschool,Preschool Child,Preschool Children
D004452 Echocardiography Ultrasonic recording of the size, motion, and composition of the heart and surrounding tissues. The standard approach is transthoracic. Echocardiography, Contrast,Echocardiography, Cross-Sectional,Echocardiography, M-Mode,Echocardiography, Transthoracic,Echocardiography, Two-Dimensional,Transthoracic Echocardiography,2-D Echocardiography,2D Echocardiography,Contrast Echocardiography,Cross-Sectional Echocardiography,Echocardiography, 2-D,Echocardiography, 2D,M-Mode Echocardiography,Two-Dimensional Echocardiography,2 D Echocardiography,Cross Sectional Echocardiography,Echocardiography, 2 D,Echocardiography, Cross Sectional,Echocardiography, M Mode,Echocardiography, Two Dimensional,M Mode Echocardiography,Two Dimensional Echocardiography
D005260 Female Females
D005500 Follow-Up Studies Studies in which individuals or populations are followed to assess the outcome of exposures, procedures, or effects of a characteristic, e.g., occurrence of disease. Followup Studies,Follow Up Studies,Follow-Up Study,Followup Study,Studies, Follow-Up,Studies, Followup,Study, Follow-Up,Study, Followup
D006128 Growth Gradual increase in the number, the size, and the complexity of cells of an individual. Growth generally results in increase in ORGAN WEIGHT; BODY WEIGHT; and BODY HEIGHT.
D006332 Cardiomegaly Enlargement of the HEART, usually indicated by a cardiothoracic ratio above 0.50. Heart enlargement may involve the right, the left, or both HEART VENTRICLES or HEART ATRIA. Cardiomegaly is a nonspecific symptom seen in patients with chronic systolic heart failure (HEART FAILURE) or several forms of CARDIOMYOPATHIES. Cardiac Hypertrophy,Enlarged Heart,Heart Hypertrophy,Heart Enlargement,Cardiac Hypertrophies,Enlargement, Heart,Heart Hypertrophies,Heart, Enlarged,Hypertrophies, Cardiac,Hypertrophies, Heart,Hypertrophy, Cardiac,Hypertrophy, Heart
D006801 Humans Members of the species Homo sapiens. Homo sapiens,Man (Taxonomy),Human,Man, Modern,Modern Man

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