Noncompaction of Ventricular Myocardium in a Patient with Congenitally Corrected Transposition of the Great Arteries Treated Surgically: Case Report
DOI:
https://doi.org/10.1532/HSF98.20041142Abstract
Noncompaction of the ventricular myocardium is a rare disorder that represents numerous prominent trabeculations and intratrabecular recesses in the ventricles. It is believed to represent not only an arrest in endomyocardial morphogenesis but also an unclassified cardiomyopathy. The pathology has been almost invariably associated with other congenital cardiac malformations. A female patient with noncompaction of the myocardium of both ventricles and congenitally corrected transposition of the great arteries (cTGA), situs inver-sus totalis, and atrial and ventricular septal defects is described. When she was 7 days old a permanent pacemaker was implanted because of complete heart block. Prazosin (Minipress), an a -receptor blocker, was administered, and the cardiac ejection fraction showed a striking increase from 20% to 42%. Despite careful and regular follow-up evaluations, the general condition of the patient slowly worsened. Five months after surgery she died of hepatorenal failure and low cardiac output. This case report is thought to be the first description of congenital complete heart block, cTGA, and situs inversus totalis with noncompaction of the myocardium of both ventricles.
References
Oechslin EN, Attenhofer Jost CH, Rojas JR, et al. 2000. Long-term follow-up of 34 adults with isolated left ventricular noncompaction: a distinct cardiomyopathy with poor prognosis. J Am Coll Cardiol 36:493-500.nOzkutlu S, Ayabakan C, Celiker A, Elshershari H. 2002. Noncompaction of ventricular myocardium: a study of twelve patients. J Am Soc Echocar-diogr 15:1523-8.nRichardson P, McKenna W, Bristow M, et al. 1996. Report of the 1995 World Health Organization/International Society and Federation of Cardiology Task Force on the Definition and Classification of Car-diomyopathies. Circulation 93:841-2.nRitter M, Oechslin E, Sutsch G, Attenhofer C, Schneider J, Jenni R. 1997. Isolated noncompaction of the myocardium in adults. Mayo Clin Proc 72:26-31.nToyono M, Kondo C, Nakajima Y, Nakazawa M, Momma K, Kusakabe K. 2001. Effects of carvedilol on left ventricular function, mass, and scintigraphic findings in isolated left ventricular non-compaction. Heart 86:4-7.nWilliams RR, Kent GB Jr, Edwards JE. 1951. Anomalous cardiac blood vessel communication with right ventricle: observations in case of pulmonary atresia with intact ventricular septum. Arch Pathol 52:480-7.nBleyl SB, Mumford BR, Brown-Harrison MC, et al. 1997. Xq28-linked noncompaction of the left ventricular myocardium: prenatal diagnosis and pathologic analysis of affected individuals. Am J Med Genet 72:257-65.nChenard J, Samson M, Beaulieu M. 1965. Embryonal sinusoids in the myocardium: report of a case successfully treated surgically. Can Med Assoc J 92:1356-9.nChin TK, Perloff JK, Williams RG, Jue K, Mohrmann R. 1990. Isolated noncompaction left ventricular myocardium: a study of eight cases. Circulation 82:507-13.nDusek J, Ostadal B, Duskova M. 1975. Postnatal persistence of spongy myocardium with embryonic blood supply. Arch Pathol 99:312-7.nFeldt RH, Rahimtoola SH, Davis GD. 1969. Anomalous ventricular myocardial patterns in a child with complex congenital heart disease. Am J Cardiol 23:732-74.nGrant RT. 1926. An unusual anomaly of the coronary vessels in the malformed heart of a child. Heart 13:273-83.nIchida F, Hamamichi Y, Miyawaki T, et al. 1999. Clinical features of isolated noncompaction of the ventricular myocardium. J Am Coll Cardiol 34:233-40.nJenni R, Goebel N, Tartini R, Schneider J, Arbenz U, Oelz O. 1986. Persisting myocardial sinusoids of both ventricles as an isolated anomaly: echocardiographic, angiographic, and pathologic anatomical findings. Cardiovasc Intervent Radiol 9:127-31.nLenox CC, Briner J. 1972. Absent proximal coronary arteries associated with pulmonary atresia. Am J Cardiol 30:666-9.nMitrani RD, Klein LS, Miles WM, et al. 1993. Regional cardiac sympathetic denervation in patients with ventricular tachycardia in the absence of coronary artery disease. J Am Coll Cardiol 22:1344-53.n
