title: Different types of cardiomyopathy associated with isolated ventricular noncompaction.
abstract: Although mainly described in the context of dilated and hypokinetic left ventricles, it is unclear whether isolated ventricular noncompaction (IVNC) is a distinct cardiomyopathy, a subtype of dilated cardiomyopathy, or a morphogenetic disorder. To investigate the spectrum of cardiomyopathies associated with IVNC, children and adults with stringent echocardiographic diagnoses of IVNC were reviewed. Seventy-three patients (12 children aged <15 years) seen since 1994 satisfied stringent echocardiographic criteria for IVNC. Sixty-five patients (89%; 11 children) had dilated cardiomyopathy, 2 adults had clear-cut hypertrophic cardiomyopathy, 1 adult had restrictive cardiomyopathy (to the investigators' knowledge, the first reported example of this particular association), and 5 patients (1 child) had normal left ventricular morphology and function. In conclusion, knowledge that IVNC can co-exist with restrictive and hypertrophic cardiomyopathy (in addition to the dilated form) supports the concept that IVNC is a morphologic trait rather than a distinct cardiomyopathy. This knowledge should be taken into account during echocardiographic examination and encourage the use of contrast echocardiography (and magnetic resonance) and could also orient molecular biology studies.
authors.affiliation: Institute of Cardiology, University of Bologna and S. Orsola-Malpighi Hospital, Bologna, Italy.
authors.forename: Elena
authors.initials: E
authors.lastname: Biagini
authors.forename: Luca
authors.initials: L
authors.lastname: Ragni
authors.forename: Marinella
authors.initials: M
authors.lastname: Ferlito
authors.forename: Ferdinando
authors.initials: F
authors.lastname: Pasquale
authors.forename: Carla
authors.initials: C
authors.lastname: Lofiego
authors.forename: Ornella
authors.initials: O
authors.lastname: Leone
authors.forename: Guido
authors.initials: G
authors.lastname: Rocchi
authors.forename: Enrica
authors.initials: E
authors.lastname: Perugini
authors.forename: Silvia
authors.initials: S
authors.lastname: Zagnoni
authors.forename: Angelo
authors.initials: A
authors.lastname: Branzi
authors.forename: Fernando M
authors.initials: FM
authors.lastname: Picchio
authors.forename: Claudio
authors.initials: C
authors.lastname: Rapezzi
mesh_terms: D000293:Adolescent; D000328:Adult; D000368:Aged; D009202:Cardiomyopathies / Q000145:classification / Q000000981:diagnostic imaging* / Q000473:pathology; D002311:Cardiomyopathy, Dilated / Q000000981:diagnostic imaging / Q000473:pathology; D002313:Cardiomyopathy, Restrictive / Q000000981:diagnostic imaging / Q000473:pathology; D002648:Child; D002675:Child, Preschool; D004452:Echocardiography; D006352:Heart Ventricles / Q000000981:diagnostic imaging* / Q000473:pathology; D006801:Humans; D007223:Infant; D007231:Infant, Newborn; D008875:Middle Aged; D009206:Myocardium / Q000473:pathology