Miocardiopatía no compactada: una serie de 15 casos / Non compaction cardiomyopathy: a series of 15 cases
Rev. méd. Chile
;
139(7): 864-871, jul. 2011. ilus, tab
Article
in Spanish
| LILACS
| ID: lil-603138
ABSTRACT
Background:
Non compaction cardiomyopathy is a rare disorder caused by the arrest of myocardial compaction during embryogenesis, leading to a non compacted endocardial layer with marked hypertrabeculation and deep recesses.Aim:
To report the clinical and echocardiographic characteristics of a series of 15 adult patients with non-compaction cardiomyopathy. Patients andMethods:
We included a total of 15 patients aged 52 ± 17 years (40 percent males) diagnosed at our echocardiography laboratory between January 2001 and July 2010.Results:
Theform of presentation was heart failure in 53 percent of subjects, syncope in 20 percento, ventricular arrhythmias in 13 percento and stroke in 7 percent>. Left ventricular end-diastolic diameter was 66 ±11 mm and estimated ejection fraction was 27 ± 10 percent>. Apical and/or mid-ventricular segments of the left ventricle were involved in all the cases. Pulmonary hypertension was present in 40 percento. The average follow-up was 19 months and no patient died during this period. Sixty seven percent ofthe patients had manifestations of heart failure, 27 percento presented sustained ventricular arrhythmias and 20 percent> had atrial fibrillation orflutter, whereas 13 percento had cerebral embolic events. An automated internal cardioverter defibrillator was implanted in 47 percento of patients.Conclusions:
Non-compaction cardiomyopathy is associated with high cardiovascular morbidity. The diagnosis is made in advanced stages of the disease, with significant dilation and ventricular dysfunction.
Full text:
Available
Index:
LILACS (Americas)
Main subject:
Rare Diseases
/
Isolated Noncompaction of the Ventricular Myocardium
Type of study:
Observational study
/
Prognostic study
/
Risk factors
Limits:
Adolescent
/
Adult
/
Aged
/
Female
/
Humans
/
Male
Language:
Spanish
Journal:
Rev. méd. Chile
Journal subject:
Medicine
Year:
2011
Type:
Article
Affiliation country:
Chile
Institution/Affiliation country:
Pontificia Universidad Católica de Chile/CL
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