Isolated Noncompaction of the Myocardium in Adults

To describe the entity of isolated ventricular noncompaction (IVNC) and present a series of cases of this rare disorder in an adult population. We review a 10-year experience with the diagnosis of IVNC and discuss the clinical, echocardiographic, and pathologic features of this condition. Echocardio...

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Bibliographic Details
Published in:Mayo Clinic proceedings 1997-01, Vol.72 (1), p.26-31
Main Authors: Ritter, Manfred, Oechslin, Erwin, Sütsch, Gabor, Attenhofer, Christine, Schneider, Jakob, Jenni, Rolf
Format: Article
Language:English
Subjects:
Adult
Aged
Biological and medical sciences
Cardiology. Vascular system
Congenital heart diseases. Malformations of the aorta, pulmonary vessels and vena cava
Diagnosis, Differential
Echocardiography
Female
Heart
Heart Defects, Congenital - complications
Heart Defects, Congenital - diagnosis
Heart Defects, Congenital - diagnostic imaging
Heart Defects, Congenital - pathology
Heart Failure - etiology
Heart Ventricles - abnormalities
Heart Ventricles - diagnostic imaging
Humans
Male
Medical sciences
Middle Aged
Retrospective Studies
Tachycardia, Ventricular - etiology
Thromboembolism - etiology
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Summary:To describe the entity of isolated ventricular noncompaction (IVNC) and present a series of cases of this rare disorder in an adult population. We review a 10-year experience with the diagnosis of IVNC and discuss the clinical, echocardiographic, and pathologic features of this condition. Echocardiographic diagnostic criteria included the absence of coexisting cardiac abnormalities, the presence of prominent and excessive trabeculations of one or more ventricular wall segments, and intertrabecular spaces perfused from the ventricular cavity. Pathologic examination focused on regions with exaggerated trabeculations and deep intertrabecular spaces. IVNC is an unexplained arrest of myo-cardial morphogenesis previously encountered mainly in pediatrie patients. Among 37,555 transthoracic echocardiographic studies performed at our hospital between January 1984 and October 1993, 17 cases of IVNC were identified in adult subjects (14 men and 3 women, 18 to 71 years of age). The mean time from onset of symptoms to correct diagnosis was 3.5 ± 5.7 years, and the mean duration of follow-up was 30 ± 28 months. Common clinical symptoms were heart failure, ventricular arrhythmias, and a history of embolie events. Two-dimensional echocardiog-raphy revealed 10 patients with left ventricular and 7 (41%) with biventricular IVNC. During a 6-year follow-up period, eight patients died and two underwent heart transplantation. Although the diagnosis of IVNC in an adult population is often delayed because of similarities with more frequently diagnosed conditions, two-dimensional echocardiography will facilitate the diagnosis of IVNC in this subset of patients. Because of the high incidence of heart failure, ventricular arrhythmias, and embolization in adults with IVNC, early diagnosis is important.
ISSN:0025-6196
1942-5546
DOI:10.4065/72.1.26