title: Long-term survival of a patient with isolated noncompaction of the ventricular myocardium.
abstract: Isolated noncompaction of the myocardium (INCM) is a rare, congenital, unclassified cardiomyopathy. It is caused by a disorder in endomyocardial morphogenesis in the absence of other structural disease. INCM is characterized by numerous prominent trabeculations and deep intertrabecular recesses in the myocardium. Frequently, INCM is associated with an increased incidence of heart failure, arrhythmias, and cardioembolic events with high morbidity and mortality. We describe a 28-year-old woman experiencing symptoms of heart failure since she was 4 years old. She had been intensively investigated and misdiagnosed as having dilated, restrictive, and apical hypertrophic cardiomyopathies. Cardiac magnetic resonance and echocardiography recently revealed the actual diagnosis of INCM. The patient is alive and well, taking vasodilators and warfarin. Herein, we describe the long-term follow-up of this patient and demonstrate that some patients have a favorable prognosis. In addition, the improvement in noninvasive cardiac imaging has revealed a higher prevalence of INCM, previously undetected.
authors.affiliation: Cardiomyopathy Unit, University of São Paulo Medical School, São Paulo, Brazil. verasalemi@uol.com.br
authors.forename: Vera M C
authors.initials: VM
authors.lastname: Salemi
authors.forename: Carlos E
authors.initials: CE
authors.lastname: Rochitte
authors.forename: Pedro
authors.initials: P
authors.lastname: Lemos
authors.forename: Luiz A
authors.initials: LA
authors.lastname: Benvenuti
authors.forename: Cristiane G
authors.initials: CG
authors.lastname: Pita
authors.forename: Charles
authors.initials: C
authors.lastname: Mady
mesh_terms: D000925:Anticoagulants / Q000627:therapeutic use; D009202:Cardiomyopathies / Q000151:congenital* / Q000000981:diagnostic imaging* / Q000188:drug therapy; D002675:Child, Preschool; D003937:Diagnosis, Differential; D005260:Female; D006801:Humans; D008137:Longitudinal Studies; D016019:Survival Analysis; D013997:Time Factors; D014463:Ultrasonography; D014665:Vasodilator Agents / Q000627:therapeutic use; D018487:Ventricular Dysfunction, Left / Q000151:congenital* / Q000000981:diagnostic imaging* / Q000188:drug therapy; D014859:Warfarin / Q000627:therapeutic use
chemical_list: D000925:Anticoagulants; D014665:Vasodilator Agents; D014859:Warfarin