Noncompaction cardiomyopathy
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Kalayci1 · Y. Guler1 · C.Y. Karabay1 · A. Guler2 · S.M. Aung3 · C. Kirma1 1 Cardiology Clinic, Koşuyolu Heart & Research Hospital, Istanbul 2 Cardiology Clinic, Kafkas University Faculty of Medicine, Kars 3 Cardiology Clinic, Sema Hospital, Istanbul
Noncompaction cardiomyopathy Is it more than noncompaction?
Additional material online This article includes a supplementary video. This supplemental material is available at dx.doi.org/10.1007/s00059-012-3667-0
Case presentation A 77-year-old man who had a history of dyslipidemia and smoking presented with effort dyspnea, palpitation and chest pain. Physical examination did not reveal any characteristic finding, and atrial fibrillation with rapid ventricular response was seen on his ECG. Echocardiography performed after medical stabilization showed that both ventricles were hyper-trabeculated (noncompacted to compacted ratio was 4.7 in the apical wall) and was consistent with noncompaction cardiomyopathy (NC). Dilatation of the left ventricle (end-diastolic volume 168 ml) and a restrictive diastolic filling pattern (mitral E/E’ ratio was 18) were detected. The left ventricular ejection fraction was 37% assessed by the biplane Simpson method (. Fig. 1a, b, c and supplementary Video). There was no valvular pathology of note. The myocardial perfusion scintigraphy revealed ischemia of the apex and coronary angiography was done. There was no stenosis but multiple coronary–cameral fistulae were found at the distal left anterior descending artery (. Fig. 1d). Cardiovascular disease was not found in the relatives (a brother and two sons) of patient.
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The patient was followed medically with carvedilol 12.5 mg/day, spirinolactone 25 mg/day and warfarin therapy.
Discussion NC, a rare congenital cardiomyopathy, is characterized by increased trabeculation in one or more segments of the ventricle, which in turn is caused by the cessation of compaction during intrauterine development. It was first defined in 1981 and its prevalence was reported to be 0.05% in the general population. Since then a growing number of NC cases have been reported, so the true incidence of NC may have been previously underestimated [1, 2]. NC is a genetic disorder and can be found alone or together with other congenital heart diseases [3, 4, 5, 6, 7, 8, 9, 10, 11, 12]. It is rarely reported together with coronary artery anomalies such as coronary–cameral fistulae [13, 14, 15, 16, 17]. Malignant ventricular arrhythmias, bradyarrhythmias and systemic embolism have also been documented in the literature [18, 19, 20, 21, 22, 23]. Our case presented with signs of heart failure and atrial fibrillation. The symptoms of heart failure are assumed to be the composite effect of NC, coronary–cameral fistulae and tachyarrhythmia. The human heart, in its different stages of development, shows resemblance to different classes of vertebrates. It resembles a fish heart, possessing only two chambers in a series in its early embryon-
ic stage. Later, as in reptiles, the atrium is divided into two and
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