Outcome of idiopathic restrictive cardiomyopathy in children
Section snippets
Patient population
All children with IRC referred to Columbia-Presbyterian Medical Center from February 1984 to December 1995 and to Royal Children’s Hospital from January 1977 to December 1995 were studied retrospectively with follow-up until January 2000. Most patients seen at Columbia-Presbyterian Medical Center were referred for cardiac transplant evaluation. All patients fulfilled the following diagnostic criteria for IRC: (1) severe diastolic dysfunction as evidenced by elevated left, right, or
Patient profile at diagnosis
Table 1lists the clinical profiles of 18 children who fulfilled the criteria for IRC during the study period. The diagnosis was made either by a local cardiologist or by a cardiologist at 1 of the study institutions. The mean age at diagnosis was 4.5 ± 4.0 years (range 0.1 to 16). At the time of diagnosis, all patients had normal left ventricular systolic function by echocardiography or angiography. The mean length of follow-up from diagnosis was 72 months (range 1 to 223).
Four patients were
Discussion
The presentation of children with IRC may be subtle, due to the predominance of diastolic dysfunction in these patients. In this series, 78% of children with IRC presented with only mild cardiac compromise. This is similar to series reported by Denfield et al6and Gewillig et al8; however, other series have reported 75% to 100% of children presenting with significant low CO.3, 4 Failure to thrive can be an early sign of cardiac compromise; this occured before clinical deterioration in 33% of
References (17)
Clinical profile and outcome of restrictive cardiomyopathy in children
Am Heart J
(1992)- et al.
Idiopathic restrictive cardiomyopathy in childhooddiagnostic features and clinical course
Mayo Clin Proc
(1995) - et al.
Clinical spectrum of restrictive cardiomyopathy in children
J Heart Lung Transplant
(2001) - et al.
Spectrum of restrictive cardiomyopathyreport of the national survey in Japan
Am Heart J
(1990) - et al.
Idiopathic restrictive cardiomyopathy in the youngreport of two cases
Int J Cardiol
(1990) - et al.
Constrictive pericarditis versus restrictive cardiomyopathyA reappraisal and update of diagnostic criteria
Am Heart J
(1991) - et al.
Comparison of new Doppler echocardiographic methods to differentiate constrictive pericardial heart disease and restrictive cardiomyopathy
Am J Cardiol
(2001) - et al.
Pulmonary vascular resistance and reactivity in children with end-stage cardiomyopathy
J Heart Lung Transplant
(2000)
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