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 and Methods: 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.
Subject(s)
Adolescent , Adult , Aged , Female , Humans , Male , Middle Aged , Young Adult , Isolated Noncompaction of the Ventricular Myocardium/diagnosis , Rare Diseases/diagnosis , Echocardiography , Follow-Up Studies , Isolated Noncompaction of the Ventricular Myocardium/complications , Magnetic Resonance Imaging , Retrospective StudiesABSTRACT
There is an increased incidence of celiac disease in patients with idiopathic dilated cardiomyopathy. We report a 4 7 year-old female presenting with heart failure secondary to dilated cardiomyopathy of unknown etiology. During the five months following the first hospitalization the patient had multiple hospital admissions due to decompensate heart failure. Due to a history of intermittent diarrhea and weight loss, a celiac disease was suspected. Antiendomysial antibodies were positive and there was a villous atrophy in duodenal mucosa. A gluten free diet was started with a concomitant recovery of her functional capacity. After one month of gluten free diet a new echocardiogram showed a normal left ventricle and systolic function.