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The variable clinical presentation of, and outcome for, noncompaction of the ventricular myocardium in infants and children, an under-diagnosed cardiomyopathy

Published online by Cambridge University Press:  21 January 2005

Sulafa K. M. Ali  and
Michael J. Godman
Sulafa K. M. Ali
Affiliation:
King Abdulaziz Cardiac Centre, King Fahad National Guard Hospital, Department of Cardiac Sciences, Division of Paediatric Cardiology, Riyadh, Kingdom of Saudi Arabia
Michael J. Godman
Affiliation:
King Abdulaziz Cardiac Centre, King Fahad National Guard Hospital, Department of Cardiac Sciences, Division of Paediatric Cardiology, Riyadh, Kingdom of Saudi Arabia
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Abstract

Noncompaction of the ventricular myocardium is increasingly recognized as an important cause of cardiomyopathy. Its echocardiographic definition, however, is not yet clearly refined, and differentiation from other conditions with hypertrabeculation can be difficult. We report a prospective short-term follow-up of 15 children with noncompaction, excluding those with associated complex congenital cardiac disease.

The clinical presentation and outcome were variable, with 2 patients being asymptomatic. For 5 patients, presentation was with cardiac failure due to depressed myocardial function. The function deteriorated in two, remained the same in two, and improved in the other patient. Cardiac failure due to mitral regurgitation was the mode of presentation in 2 patients with preserved myocardial function, one of whom needed replacement of the mitral valve. In 6 patients (40%), symptoms of cardiac failure were due to noncomplex congenital cardiac disease. All of them had ventricular septal defects. In addition, two had cleft mitral valves, and one had a large persistently patent arterial duct. The diagnosis of noncompaction was initially missed on more than one echocardiographic study in one-third of our patients. We conclude that noncompaction is under-diagnosed, and is not as rare as is thought. In children, it is often associated with other cardiac lesions that can cause cardiac failure in the presence of preserved myocardial function.

Keywords

Ventricular masscongenital heart diseaseechocardiography
Type
Original Article
Information
Cardiology in the Young , Volume 14 , Issue 4 , August 2004 , pp. 409 - 416
Copyright
© 2004 Cambridge University Press

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References

Tong KL, Ding ZP. Isolated non-compaction of ventricular myocardium: a report of three cases. Ann Acad Med (Singapore) 2001; 30: 539–541.Google Scholar
Grillo R, Pipitone S, Mongiovi M, Cipolla T, Giudice G, Gagliano S, Sperandeo V. Isolated non-compaction of left ventricle in childhood: clinical experience with 5 cases. Ital Heart J 2002; 3 (Suppl 8): 858–863.Google Scholar
Ozkutlu S, Ayabakan C, Celiker A, Elshershari H. Noncompaction of ventricular myocardium: a study of twelve patients. J Am Soc Echocardiogr 2002; 15: 1523–1528.Google Scholar
Chin TK, Perloff JK, Williams RG, Jue K, Mohrmann R. Isolated noncompaction of left ventricular myocardium, a study of eight cases. Circulation 1990; 82: 507–513.Google Scholar
Ichida F, Hamamichi Y, Miyawaki T, Ono Y, Kamiya T, Akagi T, et al. Clinical features of isolated noncompaction of the ventricular myocardium: long-term clinical course, hemodynamic properties, and genetic background. J Am Coll Cardiol 1999; 34: 233–240.Google Scholar
Jenni R, Oechslin E, Schneider J, Jost CA, Kaufmann PA. Echocardiographic and pathoanatomical characteristics of patients with isolated left ventricular non-compaction: a step towards classification as a distinct cardiomyopathy. Heart 2001; 86: 666–667.Google Scholar
Oechslin EN, Attenhofer Jost CH, Rojas JR, Kaufmann PA, Jenni R. Long-term follow up of 34 adults with isolated left ventricular noncompaction: a distinct cardiomyopathy with poor prognosis. J Am Coll Cardiol 2000; 36: 493–500.Google Scholar
Stollberger C, Finsterer J. Left ventricular hypertrabeculation/noncompaction. J American Soc Echo 2004; 17 (1): 91–100.Google Scholar
Anderson RH. Regarding isolated ventricular noncompaction. Heart 2001; (electronic pages).
Pignatelli RH, McMahon CJ, Dreyer WJ, Denfield SW, Price J, Belmont JW, et al. Clinical characterization of left ventricular noncompaction in children: a relatively common form of cardiomyopathy. Circulation 2003; 108: 2672–2678.Google Scholar
Ben-Shachar G, Arcilla RA, Lucas RV, Manasek FJ. Ventricular trabeculations in the chick embryo heart and their contribution to ventricular and muscular septal development. Circ Res 1985; 57: 759–766.Google Scholar
Lamers WH, Wessels A, Verbeek FJ, Moorman AF, Viragh S, Wenink AC, et al. New findings concerning ventricular septation in the human heart: implications for maldevelopment. Circulation 1992; 86: 1194–1205.Google Scholar
Lee Y, Song AJ, Baker R, Micales B, Conway SJ, Lyons GE. Jumonji, a nuclear protein that is necessary for normal heart development. Circulation Research 2000; 86: 932–938.Google Scholar
Soler R, Rodriguez E, Monserrat L, Alvarez N. MRI of subendocardial perfusion deficits in isolated left ventricular noncompaction. J Comput Assist Tomogr 2002; 26: 373–375.Google Scholar
Junga G, Kneifel S, Von Smekal A, Steinert H, Bauersfeld U. Myocardial ischaemia in children with isolated ventricular non-compaction. Eur Heart J 1999; 20: 910–912.Google Scholar
Bleyl SB, Mumford BR, Brown-Harrison MC, Pagotto LT, Carey JC, et al. Xq28 – linked noncompaction of the left ventricular myocardium: prenatal diagnosis and pathological analysis of affected individuals. Am J Med Genet 1997; 72: 257–265.Google Scholar
Nugent AW, Daubeney PE, Chondros P, Carlin JB, Cheung M, Wilkinson LC, et al. The epidemiology of childhood cardiomyopathy in Australia. N Engl J Med 2003; 348: 1639–1646.Google Scholar
Hughes ML, Willkinson JL, Weintraub RG. The spectrum of left ventricular noncompaction in children with congenital heart disease. J Am Coll Cardiol 2003; 41 (Suppl B): 485.Google Scholar