Ultrasound diagnostics of left ventricular noncompaction

The clinical cases of rare, genetically heterogeneous cardiomyopathy are described - the left ventricular noncompaction (LVNC) cardiomyopathy for four children (three boys and one girl) in age from two months to 6 years. Description of echocardiography signs is presented in accordance with modern diagnostic criteria. Noncompaction of the left ventricle is recognized in three patients, of the left and right ventricle - in one. In three patients out of four, LVNC was a separate disease, and yet in one it was associated with neurofibromatosis type 1, in combination with dilatation of coronary arteries and pericardial effusion. Three of four patients were admitted with congestive heart failure (CHF) of New York Heart Association (NYHA) functional class 3-4 (FC), and one - with CHF 2 FC. The echocardiograms are brought with description of characteristic features of LVNC (hypertrabecularity, spongy myocardium on the apex of the left ventricle, typical correlation of incompact and compact layer from 2.0 to 2.6). All four patients received treatment for CHF (diuretics, beta-blockers, captopril, in one case - digoxin); two out of four got antithrombotic therapy (aspirin, warfarin). Results of follow-up observation of the patients are described with the estimation of echocardiography signs, including dilatation of coronary arteries. Left ventricular ejection fraction - both by Simpson and Teicholz - increased in two patients, with therapy of chronic heart failure at a follow-up visits over > 5 years simultaneously with the decline of severity of CHF. Another two patients have been examined from 2 months to 5 years and have had CHF 3 FC; their left ventricular ejection fraction increased too, but cardiomegaly and right ventricular heart failure preserved.

noncompaction of the left ventricular myocardium, ultrasound diagnosis, echocardiography, cardiomyopathy, children, noncompaction of the left ventricular myocardium, ultrasound diagnosis, echocardiography, cardiomyopathy, children

1. Агранович Н.В., Бабашева Г.Г., Агранович И.С., Крон Е.Ю., Игнатенко И.В., Юндина Е.Е. Эхокардиография в диагностике некомпактного миокарда: научный обзор // Современные проблемы науки и образования. - 2015. - № 5. - С. 69

2. Asfalou I., Boulaamayl S., Mouine M.R.N., Sabry M., Kheyi J., Doghmi N., Benyassa A. (2017). Left ventricular noncompaction - A rare form of cardiomyopathy: Revelation modes and predictors of mortality in adults through 23 cases. J Saudi Heart Association, 29, 102-109.

3. Barry M., Towbin J., Thiene G., Antzelevitch C., Corrado D., Moss A.J., Seidman C.E., Young J.B. (2006). Contemporary definitions and classifications of the cardiomyopathies. J Am Heart Assoc, 113, 1807.

4. Chin T.K., Perloff J.K., Williams R.G. (1990). Isolated noncompaction of left ventricular myocardium. A study of eight cases. Circulation, 82, 507.

5. Dallaire F., Dandah N. (2011). New equations and a critical appraisal of coronary artery Z scores in healthy children. J Am Soc Echocardiogr, 24, 60-74.

6. Engberding R., Bender F. (1984). Identification of a rare congenital anomaly of the myocardium by two-dimensional echocardiography: Persistence of isolated myocardial sinusoids. Am J Cardiol, 6, 53.

7. Espinola-Zavaleta N., Soto E., Castellanos L.M., Játiva-Chávez S, Keirns C. (2006). Non-compacted cardiomyopathy: clinical echographic study. Cardiovasc Ultrasound, 4, 35.

8. Haland T., Saberniak J., Leren I.S., Edvardsen T., HaugaaHaland K.H. (2017). Echocardiographic comparison between left ventricular non-compaction and hypertrophic cardiomyopathy. Int J Cardiol, 228, 900-905.

9. Jenni R., Oechslin E., Schneider J., Jost C., Kaufmann P. (2001). Echocardiographic and pathoanatomical characteristics of isolated left ventricular non-compaction: a step towards classification as a distinct cardiomyopathy. Heart, 86, 666-671.

10. Kalavakunta J., Tokala H., Gosavi A., Gupta V. (2010). Left ventricular noncompaction and myocardial fibrosis: a case report. Int Arch Med, 3, 20.

11. Kampmann C. Wiethoff C., Wenzel A., Stolz G., Betancor M., Wippermann C.F., Huth C.R., Habermehl P., Knuf M., Emschermann T., Stopfkuchen H. (2000). Normal values of M mode echocardiographic measurements of more than 2000 healthy infants and children in central Europe. Heart, 83, 72.

12. Lang R.M., Bierig M., Devereux R.B., Flachskampf F.A., Foster E., Pellikka P.A., Picard M.H., Roman M.J., Seward J., Shanewise J., Solomon S., Spencer K.T., Sutton M.S., Stewart W. (2012). Рекомендации по количественной оценке структуры и функции камер сердца // Российский кардиологический журнал. - № 3. - С. 95.

13. Nugent A.W., Daubeney P.F., Chondros P.W. (2003). The epidemiology of childhood cardiomyopathy. New Engl J Med, 348, 1639-1646.

14. Oechslin E., Jenni R. (2005). Non-compaction of the left ventricular myocardium - from clinical observation to the discovery of a new disease. Cardiology, 1, 23-24.

15. Pignatelli R.H., McMahon C.J., Dreyer W.J. (2008). Clinical characterization of left ventricular noncompaction in children: a relatively common form of cardiomyopathy. Circulation, 108, 2672.

16. Stollberger C., Finsterer J., Blazek G.C. (2002). Left ventricular hypertrabeculation/noncompaction and association with additional cardiac abnormalities and muscular disorder. Am J Cardiol, 90, 899-902.

17. Weiford B.C., Subbarao V.D., Mulhern K.M. (2004). Noncompaction of the ventricular myocardium. Circulation, 109, 24