IDIOPATHIC RESTRICTIVE CARDIOMYOPATHY IN CHILDHOOD - DIAGNOSTIC FEATURES AND CLINICAL COURSE

Objective: To describe the clinical course and outcome of children wit h idiopathic restrictive cardiomyopathy (IRCM) and to present the Dopp ler echocardiographic features of this disease in childhood. Design: W e reviewed the Mayo Clinic patient database for the period from 1975 t o 1993 to identify children who underwent assessment for IRCM. Materia l and Methods: Clinical records and diagnostic studies, including two- dimensional (2-D), M-mode, and Doppler echocardiograms, were reviewed for each patient. Characteristics were analyzed statistically to deter mine potential predictors of outcome. Results: Eight children (five gi rls and three boys) were diagnosed with IRCM between 1975 and 1993 at our institution. The median age at diagnosis was 11 years, and the med ian duration of follow-up was 11.5 years. Of the eight patients, five died (the median time from initial examination to death was 1 year). A ll five of these patients had clinical and radiographic evidence of pu lmonary venous congestion. In all patients, 2-D and M-mode echocardiog raphy revealed atrial enlargement without ventricular dilatation or hy pertrophy. The four patients who underwent detailed diastolic Doppler assessment had findings consistent with restrictive filling and increa sed left ventricular end-diastolic pressure: (1) short mitral decelera tion time, (2) increased pulmonary vein atrial reversal velocity and d uration, and (3) pulmonary vein atrial reversal duration greater than mitral A-wave duration. Conclusion: The prognosis for children with IR CM is poor. In this small group of patients, absence of pulmonary veno us congestion most consistently predicted extended survival. A combine d 2-D and Doppler echocardiographic examination provides a reliable no ninvasive means of assessing the physiologic and morphologic features of IRCM in children.