Copyright © 2006, The European Society of Cardiology
Noncompaction of the left ventricle in a patient with dextroversion
Montefiore Medical Center, Albert Einstein College of Medicine, 111 East 210th Street, Echo Lab, Bronx, NY 10467, USA
Received 29 September 2005; received in revised form 2 December 2005; accepted after revision 15 December 2005.
* Corresponding author. Tel.: +1 718 920 4808; fax: +1 718 920 7709. dspevack{at}montefiore.org
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Abstract |
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Noncompaction of the left ventricle is a rare, congenital cardiomyopathy characterized by excessive trabeculation of the myocardium. Dextrocardia with situs solitus, commonly referred to as dextroversion, is associated with additional congenital heart disease. We report a case of noncompaction of the left ventricle in a patient with dextroversion, an association of which has not been previously described.
Keywords: Noncompaction; Dextroversion; Situs solitus; Dextrocardia
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Case presentation |
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A 46 year-old man with a history of dextrocardia, end-stage renal disease, hypertension and several prior cerebrovascular accidents was referred for evaluation. He reported having a left nephrectomy and splenectomy following a gunshot wound 15 years earlier. He denied having a history of chest pain, exertional dyspnea or arrhythmia.
On physical examination the vital signs were normal and the pulse was regular. The neck veins were normal and the lung fields were clear. Heart sounds were best heard in the right chest. A 2/6 holosystolic murmur was best heard in the right mid-axillary line. There was no peripheral edema.
The chest X-ray showed the heart's location in the right chest, with the descending aorta located to the left of midline, Fig. 1. The standard electrocardiogram showed normal sinus rhythm with a normal, leftward, P-wave axis, Fig. 2. The QRS axis was deviated superiorly to –90°. Large R-waves were seen in leads V1–V3 with progressively smaller R-waves in the lateral chest leads. An interventricular conduction delay was seen.
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Transthoracic echocardiography performed on the right chest showed that the morphologic right ventricle, which contained the moderator band and a tricuspid valve, pumped into a bifurcating pulmonary artery. This finding ruled out congenitally corrected transposition or double discordance. Excessive segmental thickening of the left ventricle was seen, with a noncompacted layer of spongiform trabeculae more than twice as thick as the underlying compacted layer, Fig. 3A. Color Doppler examination showed penetration of blood flow into the sinusoidal recesses of the ventricular myocardium, Fig. 3B. These findings were thought to be diagnostic of noncompaction of the left ventricle according to criteria proposed by Jenni et al.1 There was diffuse left ventricular hypokinesis with mildly reduced ventricular function. Moderate pulmonary hypertension was present.
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An MRI of the chest confirmed the normal location of the cardiac atria and great vessels, situs solitus, with the heart's apex rotated into the right chest, dextrocardia, Fig. 4. The finding of dextrocardia with situs solitus is often referred to as dextroversion. Pronounced trabeculation of the left ventricle was also observed mainly involving the apex and mid-ventricular postero-lateral walls.
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An angiotensin converting enzyme inhibitor was started due to left ventricular dysfunction, commonly associated with noncompaction.2,5,6 Anticoagulation was also initiated due to increased risk of embolization.2 Given the apparent increased risk of ventricular arrhythmias suggested by several small case series, the patient was referred for evaluation by an electrophysiologist for consideration of ICD implantation.2,6
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Discussion |
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Noncompaction of the left ventricle is a congenital cardiomyopathy characterized by a segmental thickened endocardial part of the myocardium with deep, blood-filled recesses and a normal epicardial layer.2 It is a rare disorder with an incidence estimated to be 1/2200 and is thought to be caused by the arrest of endomyocardial compaction in utero.3,4 Previous case series by Ichida and Oechslin have reported depressed systolic function in 48% and 82%, respectively.5,2 Other major complications include ventricular arrhythmias, with an increased incidence of sustained ventricular tachycardia.2,6 An increased incidence of thromboembolic events as high as 24% has been reported.2 Several diagnostic criteria have been proposed including a ratio
2 of wall thickness between the noncompacted, trabeculated, and the non-trabeculated, compacted layer of the left ventricular myocardium at end-systole, measured at the parasternal short-axis.1 Dextroversion is a rare congenital abnormality with an estimated incidence of 1/2800.7,8 This condition is different from dextrocardia with situs inversus, where a mirror-image reversal but preserved relationship exists between the heart, great vessels, and abdominal organs. The majority of patients with dextroversion have additional congenital heart disease including left to right shunts, decreased pulmonary blood flow and conotruncal abnormalities.8 To our knowledge, this is the first case report of a patient with both dextroversion and noncompaction of the left ventricle.
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References |
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- Jenni R., Oechslin E., Schneider J., Attenhofer Jost C., Kaufman P.A. Echocardiographic and pathoanatomical characteristics of isolated left ventricular non-compaction: a step towards classification as a distinct cardiomyopathy. Heart (2001) 86:666–671.
[Abstract/Free Full Text] - Oechslin E.N., Attenhofer Jost C.H., Rojas J.R., Kaufmann P.A., 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.
[Abstract/Free Full Text] - Ritter M., Oechslin R., Sutsch G., Attenhofer C., Schneider J., Jenni R. Isolated noncompaction of the myocardium in adults. Mayo Clin Proc (1997) 72:26–31.[Abstract]
- Engberding R., Bender F. Identification of a rare congenital anomaly of the myocardium by two dimensional echocardiography: persistence of isolated myocardial sinusoids. Am J Cardiol (1984) 53:1733–1734.[CrossRef][Web of Science][Medline]
- Ichida F., Hamamichi Y., Miyawaki T., Ono Y., Kamiya T., Akagi T., et al. Clinical features of isolated noncompaction of the ventricular myocardium. J Am Coll Cardiol (1999) 34:233–240.
[Abstract/Free Full Text] - Murphy R.T., Thaman R., Blanes J.G., Ward D., Sevdalis E., Papra E., et al. Natural history and familial characteristics of isolated left ventricular non-compaction. Eur Heart J (2005) 26:187–192.
[Abstract/Free Full Text] - Comstock C.H., Smith R., Lee W., Kirk J.S. Right fetal cardiac axis: clinical significance and associated findings. Obstet Gynecol (1998) 91:495–499.[CrossRef][Web of Science][Medline]
- Garg N., Agarwal B.L., Modi N., Radhakrishnan S., Sinha N. Dextrocardia: an analysis of cardiac structures in 125 patients. Int J Cardiol (2003) 88:143–155.[CrossRef][Web of Science][Medline]
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