title: Isolated ventricular noncompaction: a case report.
abstract: Isolated ventricular noncompaction is an extremely rare cardiomyopathy, not fully clarified.It is characterized by persistent embryonic myocardium morphology without associated cardiac abnormalities.Since first description in 1984, few clinical studies were done. Data in the literature are lacking and most reports consist on a few case studies.Doppler ecocardiogram is considered the reference method for diagnosis.Diagnosis remains difficult since there are similarities with other cardiac defects, clinical manifestations are non-specific and echocardiographic criteria are not universally accepted.As a consequence diagnosis may be easily missed.Moreover, clinical and echocardiographic features were just recently clarified.Treatment is directed towards important clinical manifestations (heart failure, arrhythmias and embolic events).We present a clinical case of severe cardio-respiratory failure in previously healthy and asymptomatic young male, which was the initial presentation of an isolated ventricular noncompaction.A brief review of available literature is done concerning to this case study.
authors.affiliation: Pulmonology Department, Hospital S João, Alameda Professor Hernâni Monteiro 4300-319, Porto, Portugal.
authors.forename: João
authors.initials: J
authors.lastname: Bento
authors.forename: Filipe
authors.initials: F
authors.lastname: Monteiro
authors.forename: Luis
authors.initials: L
authors.lastname: Sargento
authors.forename: Joaquin
authors.initials: J
authors.lastname: Vizcaino
authors.forename: Jorge
authors.initials: J
authors.lastname: Monteiro
authors.forename: Pilar
authors.initials: P
authors.lastname: Azevedo
authors.forename: Gabriela
authors.initials: G
authors.lastname: Brum