Noncompaction of Ventricular Myocardium in a Patient with Congenitally Corrected Transposition of the Great Arteries Treated Surgically: Case Report
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.
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
How to Cite
Author Disclosure & Copyright Transfer Agreement
In order to publish the original work of another person(s), The Heart Surgery Forum® must receive an acknowledgment of the Author Agreement and Copyright Transfer Statement transferring to Forum Multimedia Publishing, L.L.C., a subsidiary of Carden Jennings Publishing Co., Ltd. the exclusive rights to print and distribute the author(s) work in all media forms. Failure to check Copyright Transfer agreement box below will delay publication of the manuscript.
A current form follows:
The author(s) hereby transfer(s), assign(s), or otherwise convey(s) all copyright ownership of the manuscript submitted to Forum Multimedia Publishing, LLC (Publisher). The copyright transfer covers the exclusive rights to reproduce and distribute the article and the material contained therein throughout the world in all languages and in all media of expression now known or later developed, including but not limited to reprints, photographic reproduction, microfilm, electronic data processing (including programming, storage, and transmission to other electronic data record(s), or any other reproductions of similar nature), and translations.
However, Publisher grants back to the author(s) the following:
- The right to make and distribute copies of all or part of this work for use of the author(s) in teaching;
- The right to use, after publication in The Heart Surgery Forum, all or part of the material from this work in a book by the author(s), or in a collection of work by the author(s);
- The royalty-free right to make copies of this work for internal distribution within the institution/company that employs the author(s) subject to the provisions below for a work-made-for-hire;
- The right to use figures and tables from this work, and up to 250 words of text, for any purpose;
- The right to make oral presentations of material from this work.
Publisher reserves the right to grant or refuse permission to third parties to republish all or part of the article or translations thereof. To republish, such third parties must obtain written permission from the Publisher. (This is in accordance with the Copyright Statute, United States Code, Title 17. Exception: If all authors were bona fide officers or employees of the U.S. Government at the time the paper was prepared, the work is a “work of the US Government” (prepared by an officer or employee of the US Government as part of official duties), and therefore is not subject to US copyright; such exception should be indicated on signature lines. If this work was prepared under US Government contract or grant, the US Government may reproduce, royalty-free, all or portions of this work and may authorize others to do so, for official US Government purposes only, if the US Government contract or grant so requires.
I have participated in the conception and design of this work and in the writing of the manuscript and take public responsibility for it. Neither this manuscript nor one with substantially similar content under my authorship has been published, has been submitted for publication elsewhere, or will be submitted for publication elsewhere while under consideration by The Heart Surgery Forum, except as described in an attachment. I have reviewed this manuscript (original version) and approve its submission. If I am listed above as corresponding author, I will provide all authors with information regarding this manuscript and will obtain their approval before submitting any revision. I attest to the validity, accuracy, and legitimacy of the content of the manuscript and understand that Publisher assumes no responsibility for the validity, accuracy, and legitimacy of its content. I warrant that this manuscript is original with me and that I have full power to make this Agreement. I warrant that it contains no matter that is libelous or otherwise unlawful or that invades individual privacy or infringes any copyright or other proprietary right. I agree to indemnify and hold Publisher harmless of and from any claim made against Publisher that relates to or arises out of the publication of the manuscript and agree that this indemnification shall include payment of all costs and expenses relating to the defense of any such claim, including all reasonable attorney’s fees.
I warrant that I have no financial interest in the drugs, devices, or procedures described in the manuscript (except as disclosed in the attached statement).
I state that the institutional Human Subjects Committee and/or the Ethics Committee approved the clinical protocol reported in this manuscript for the use of experimental techniques, drugs, or devices in human subjects and appropriate informed consent documents were utilized.
Furthermore, I state that any and all animals used for experimental purposes received humane care in USDA registered facilities in compliance with the “Principles of Laboratory Animal Care” formulated by the National Society for Medical Research and the “Guide for the Care and Use of Laboratory Animals” prepared by the Institute of Laboratory Animal Resources and published by the National Institutes of Health (NIH Publication No. 85-23, revised 1985).