Restrictive cardiomyopathies in childhood: Etiologies and natural history

Susan W. Denfield, Geoffrey Rosenthal, Robert J. Gajarski, J. Timothy Bricker, Kenneth O. Schowengerdt, Julia K. Price, Jeffrey Towbin

Research output: Contribution to journalArticle

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Abstract

Restrictive cardiomyopathy is rare in childhood and little is known about the causes and outcome. This lack of information results in extrapolation of adult data to the care and management of children, who might require different treatment from that of adults. This study was undertaken retrospectively to evaluate the causes and natural history of restrictive cardiomyopathy in childhood. Twelve cases of restrictive cardiomyopathy were identified by database review of patient records from 1967 to 1994. The cases were selected on the basis of echocardiographic and cardiac catheterization criteria. Charts were reviewed for the following variables: age, sex, cause, right- and left-sided hemodynamics, pulmonary vascular resistance index, shortening fraction, therapy, and outcome. There were 6 males and 6 females with a mean age of 4.6 years at presentation (median, 3.4 yr; range. 0.9 to 12.3 yr). Etiologies included hypertrophic cardiomyopathy in 3 patients, cardiac hypertrophy with restrictive physiology in 3, idiopathic in 2, familial in 2 (twins), 'chronic eosinophilia' in 1, and 'post inflammatory' with no definitive causes in 1. At presentation the mean shortening fraction was 33% ± 2% (mean ± SEM), average right ventricular pressures were 44/13 ± 3/1, average left ventricular pressures were 88/25 ± 4/3, and the mean pulmonary vascular resistance index was 3.4 ± 1.3 U · m2 (n=9), but increased to 9.9 ± 3.1 U · m2 (n=5, p=0.04) by 1 to 4 years after diagnosis. Four of the 12 patients had embolic events (1, recurrent pulmonary emboli; 1, saddle femoral embolus; 2, cerebrovascular accidents) and 9 of 12 died within 6.3 years despite medical therapies, which included diuretics, verapamil, propranolol, digoxin, and captopril. In conclusion, restrictive cardiomyopathy in childhood is commonly idiopathic or associated with cardiac hypertrophy, and the prognosis is poor. Embolic events occurred in 33% of our patients, and 9 of 12 patients died within 6.3 years. Within 1 to 4 years of diagnosis, patients may develop a markedly elevated pulmonary vascular resistance index; therefore, transplantation should be considered early.

Original languageEnglish (US)
Pages (from-to)38-44
Number of pages7
JournalTexas Heart Institute Journal
Volume24
Issue number1
StatePublished - Apr 1 1997
Externally publishedYes

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Restrictive Cardiomyopathy
Natural History
Vascular Resistance
Cardiomegaly
Ventricular Pressure
Embolism
Hypertrophic Cardiomyopathy
Digoxin
Captopril
Eosinophilia
Cardiac Catheterization
Child Care
Verapamil
Thigh
Diuretics
Propranolol
Therapeutics
Transplantation
Hemodynamics
Stroke

All Science Journal Classification (ASJC) codes

  • Cardiology and Cardiovascular Medicine

Cite this

Denfield, S. W., Rosenthal, G., Gajarski, R. J., Bricker, J. T., Schowengerdt, K. O., Price, J. K., & Towbin, J. (1997). Restrictive cardiomyopathies in childhood: Etiologies and natural history. Texas Heart Institute Journal, 24(1), 38-44.

Restrictive cardiomyopathies in childhood : Etiologies and natural history. / Denfield, Susan W.; Rosenthal, Geoffrey; Gajarski, Robert J.; Bricker, J. Timothy; Schowengerdt, Kenneth O.; Price, Julia K.; Towbin, Jeffrey.

In: Texas Heart Institute Journal, Vol. 24, No. 1, 01.04.1997, p. 38-44.

Research output: Contribution to journalArticle

Denfield, SW, Rosenthal, G, Gajarski, RJ, Bricker, JT, Schowengerdt, KO, Price, JK & Towbin, J 1997, 'Restrictive cardiomyopathies in childhood: Etiologies and natural history', Texas Heart Institute Journal, vol. 24, no. 1, pp. 38-44.
Denfield SW, Rosenthal G, Gajarski RJ, Bricker JT, Schowengerdt KO, Price JK et al. Restrictive cardiomyopathies in childhood: Etiologies and natural history. Texas Heart Institute Journal. 1997 Apr 1;24(1):38-44.
Denfield, Susan W. ; Rosenthal, Geoffrey ; Gajarski, Robert J. ; Bricker, J. Timothy ; Schowengerdt, Kenneth O. ; Price, Julia K. ; Towbin, Jeffrey. / Restrictive cardiomyopathies in childhood : Etiologies and natural history. In: Texas Heart Institute Journal. 1997 ; Vol. 24, No. 1. pp. 38-44.
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