Non-compacted Cardiomyopathy: Clinical-Echocardiographic Study

Nilda Espinola-Zavaleta; M. Elena Soto; Luis Muñóz Castellanos; Silvio Játiva-Chávez; Candace Keirns

Disclosures

Cardiovasc Ultrasound. 2006;4(1) 

In This Article

Abstract and Background

Abstract

The aim of the present study was to describe the clinical and echocardiographic findings of ventricular noncompaction in adult patients. Fifty-three patients underwent complete clinical history, electrocardiogram, Holter and transthoracic echocardiogram. Forty patients (75%) were in class I/II of the New York Heart Association, and 13 (25%) in class III/IV. Ventricular and supraventricular escape beats were found in 40% and 26.4%, respectively. Holter showed premature ventricular contractions in 32% and sustained ventricular tachycardia in 7.5%. Ventricular noncompaction was an isolated finding in 74% of cases and was associated with other congenital heart disease in 26%. Noncompacted ventricular myocardium involved only left ventricle in 62% of the patients and both ventricles in 38%. The mean ratio of noncompacted to compacted myocardial layers at the site of maximal wall thickness was 3.4 ± 0.87 mm (range 2.2-7.5). The presence of ventricular noncompaction in more than three segments was associated with a functional class greater than II and ventricular arrhythmia with demonstrable statistical significance by X 2(p < 0.003).
Conclusion: a) Noncompacted cardiomyopathy is a congenital pathological entity that can occur in isolated form or associated with other heart disease and often involves both ventricles. b) A ratio of noncompacted to compacted myocardium greater than 3 and involvement of three or more segments are indicators of poor prognosis. c) Since the clinical manifestations are not sufficient to establish diagnosis, echocardiography is the diagnostic tool that makes it possible to document ventricular noncompaction and establish prognostic factors.

Background

Noncompaction of ventricular myocardium is recently included in the 2006 classsification of cardiomyopathies as a Genetic Cardiomyopathy.[1,2] Ventricular noncompaction occurs because of a disorder of endomyocardial morphogenesis that results in a failure of trabecular compaction of the developing myocardium.[3,4,5] Ventricular noncompaction is often associated with other congenital cardiac malformations.[6,7,8] In adult patients one or more segments of the left ventricle, and sometimes both ventricles, are characterized by numerous sinusoids or trabeculae that are excessive in number and abnormal in prominence and by deep intratrabecular recesses covered by endothelium that exhibits continuity with ventricular endocardium. Two-dimensional echocardiography provides definition of typical anatomic features. Jenni et al.[9] established four morphologic criteria for echocardiographic diagnosis that allow accurate differentiation from other forms of left ventricular hypertrophy. On the basis of echocardiographic studies, the prevalence of ventricular noncompaction has been estimated at 0.05% in the general population.[3,10]

The aim of the present study was to describe the clinical and echocardiographic data of isolated and non-isolated ventricular noncompaction in adult patients from the Outpatient Clinic of the Instituto Nacional de Cardiología "Ignacio Chávez".

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