[Clinical aspects of dilated cardiomyopathy in pediatric age]. / Aspetti clinici della cardiomiopatia dilatativa in età pediatrica.

ABSTRACT

Natural history and prognostic factors of dilated cardiomyopathy in pediatric age are not well identified so far. The course of the disease, often, presents "odd" characteristics some of the patients show clinical improvement, someone remains unchanged and some of them have a negative evolution in a very short time. It is necessary to identify the group of patients having a poor outcome in order to define prognostic factors of impending death so that a cardiac transplant could be offered to them as a reasonable therapeutic choice. Twenty-two children, 9 males and 13 females (median age 5 +/- 5 years) with dilated cardiomyopathy were studied. Their natural history was followed for a period of 40 +/- 30 months. Clinical data, laboratory studies (electrocardiogram, Holter monitoring, echocardiogram M-mode, 2-D, Doppler and chest X-ray) were taken into consideration. At the end of follow-up period 7 patients showed improvement in cardiac status (31.8%), 7 remained unchanged (31.8%) and 8 (36%) got worse; 9 died. The survival curve showed a rate of 72% at 12 months and of 59.3% at 60 months. Of 9 died patients, 2 died suddenly and seven for congestive heart failure. Clinical findings and laboratory investigations of 13 survived patients (Group I) and 9 died patients (Group II) were compared to establish the risk factors of poor outcome. The fractional shortening is the only significant factor of poor outcome (p less than 0.025). Other negative factors were identified more in Group II than in Group I.(ABSTRACT TRUNCATED AT 250 WORDS)