Outcome of idiopathic restrictive cardiomyopathy in children

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Abstract

Eighteen children with idiopathic restrictive cardiomyopathy (IRC) were studied in an attempt to identify potential predictors of poor outcome. Four patients presented with low cardiac output (CO) syndrome. Fourteen remaining patients were minimally symptomatic at presentation but developed a low CO syndrome at a mean of 2.8 ± 2.3 years after diagnosis. At the time of development of low CO in the 18 patients, mean left ventricular end-diastolic pressure was 27 mm Hg, right ventricular end-diastolic pressure was 18 mm Hg, cardiac index was 2.5 L/min/m2, and pulmonary vascular resistance index (PVRI) was 8.8 U-m2. Eleven of the 18 patients underwent cardiac transplantation. One died perioperatively from donor right-sided cardiac failure and 10 survived. Six were not transplanted and died, including 3 in whom transplantation was precluded secondary to extremely elevated PVRI. One patient is alive with right-sided cardiac failure. Ten of our 18 patients had pulmonary hypertension (PVRI >6 U-m2) at the time of referral for cardiac transplant and/or development of low CO syndrome. In comparison, children with dilated cardiomyopathy who were referred for heart transplant during the same time period had a PVRI that was significantly lower (5.2 U-m2). Elevated PVRI was associated with death (p <0.01) and 40% of our children with pulmonary hypertension were precluded from receiving an orthotopic heart transplant because their pulmonary hypertension was so severe. No risk factors for the development of pulmonary hypertension were identified; therefore, all children with IRC should undergo serial monitoring of their PVRI, and any increase should prompt a transplant evaluation.

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

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