We describe the clinical features of 2 female pediatric patients with idiopathic restrictive cardiomyopathy (IRCM), an uncommon disease in childhood. Patient I was diagnosed early because the symptoms of pulmonary venous congestion manifested at 14 months of age, and had a short-term unfavorable outcome. In contrast, patient 2, who suffered from skeletal myopathy, was diagnosed as having IRCM at 8 years of age; despite a severe cardiac involvement documented by ECG and echocardiogram, this patient has no cardiac symptoms at the age of 16. This study confirms that IRCM has a malignant outcome in children whenever the clinical manifestations of pulmonary congestion occur early, whereas absence of symptoms is associated with a better survival.
|Number of pages||4|
|Journal||Italian Journal of Pediatrics|
|Publication status||Published - Oct 2002|
- Congestive heart failure
ASJC Scopus subject areas
- Pediatrics, Perinatology, and Child Health