Noncompacted cardiomyopathy in infants and children. Clinical findings and diagnostic techniques

Abstract

Noncompaction of the ventricular myocardium (NcCM) represents an arrest in the normal process of myocardial compaction, and has only recently been recognized as a distinct form of cardiomyopathy. It was previously termed as "spongy myocardium" and it is an extremely rare form of CM especially in infants and children. The last 20 years 23 cases aged 3 days-17 years mean: 60,3 months were diagnosed with NcCM (previously "spongy CM") based on echo-morphological criteria in our Institute. Our purpose was to assess the diagnostic value of Echo/ and MRI using conventional Echo and TDI parameters. 0.5-17 years, mean 6.3 years. We introduced in our country the MRI, also in the pediatric population with NcCM. Echo/MRI diagnosis was in good agreement in the diagnosis of pediatric NcCM. TEI index correlated well with MRI EF (r: 0.96, p<0.01). The prognosis of NcCM in infants was very poor, with a 43% half year mortality, with one successful heart transplantation. All children were stable hemodynamic condition for a longer period. We think our result will contribute to the early diagnosis, adequate treatment of NcCM with improvement of the prognosis, and to the better knowledge of prevalence and family screening of this very severe disease.