title: Left ventricular non-compaction cardiomyopathy in children: Is segmental fibrosis the cause of tissue Doppler alterations and of EF reduction?
abstract: Noncompaction of the ventricular myocardium (LVNC) is a rare congenital cardiomyopathy resulting from an arrest in normal endomyocardial embryogenesis. In 2002 Jenni et al. [Jenni R, Wyss CA, Oechslin EN, Kaufmann PA. Isolated ventricular noncompaction is associated with coronary microcirculatory dysfunction. J Am Coll Cardiol 2002; 39:450-454.] reported a microvascular dysfunction in 12 patients affected by non compaction: areas of restricted myocardial perfusion have been documented by scintigraphy, suggesting a reduction of Coronary flow reserve. McMahon et al reported in a recent article a reduction of TD velocities in children with noncompaction of the left ventricle, compared with normal controls. The authors concluded their work saying that the reduction of lateral mitral Ea velocity helps to predict children with LVNC who are at risk of adverse clinical outcomes including death and need for cardiac transplantation. In a precedent report our group reported a strong correlation between pathological tissue Doppler and reduction of ejection fraction. Recently we scanned with a Signa HD 1.5 T (GE, Milwaukee, USA) 8 patients affected by non compaction. Transmural Gd-enhancement was detected in 5/8 patients (62%). In all patients with late enhancement a reduction of EF has demonstrated. In our opinion the late enhancement can depend on a CFR, and is the determinant of the tissue Doppler alterations. So the TD alteration is associated with EF, and is an indirect index of poor clinical outcome, like EF.
authors: Giovanni
authors: G
authors: Fazio
authors: Giuseppina
authors: G
authors: Novo
authors: Calogero
authors: C
authors: Casalicchio
authors: Gabriele
authors: G
authors: Di Gesaro
authors: Loredana
authors: L
authors: Sutera
authors: Emanuele
authors: E
authors: Grassedonio
authors: Eliana
authors: E
authors: Cracolici
authors: Valentina
authors: V
authors: Pitruzzella
authors: Egle
authors: E
authors: Incalcaterra
authors: Salvatore
authors: S
authors: Pipitone
authors: Massimo
authors: M
authors: Midiri
authors: Salvatore
authors: S
authors: Novo
mesh_terms: D009202:Cardiomyopathies / Q000151:congenital / Q000000981:diagnostic imaging* / Q000473:pathology / Q000503:physiopathology*; D015150:Echocardiography, Doppler; D005355:Fibrosis; D006352:Heart Ventricles / Q000473:pathology* / Q000503:physiopathology*; D006801:Humans; D013318:Stroke Volume