Noncompaction of The Left Ventricle in A Patient With Dextroversion

70 M.A. Friedman et al.
10. Stollberger C, Finsterer J. Thrombi in left ventricular hyper- 12. Ascione L, Antonini-Canterin F, Macor F, Cervesato E,
trabeculation/noncompaction e review of the literature. Chiarella F, Giannuzzi P, et al. Relation between
Acta Cardiol 2004 Jun;59(3):341e4. early mitral regurgitation and left ventricular throm-
11. Ruvolo G, Fattouch K, Speziale G, Macrina F, Tonelli E, bus formation after acute myocardial infarction: re-
Marino B. Left ventricular thrombosis after blunt chest sults of the GISSI-3 echo substudy. Heart Aug 2002;
trauma. J Cardiovasc Surg (Torino) 2001 Apr;42(2):211e2. 88:131e6.
1525-2167/$32 ª 2005 The European Society of Cardiology. Published by Elsevier Ltd. All rights reserved.
10.1016/j.euje.2005.12.005
Noncompaction of the left ventricle in a patient

with dextroversion
Downloaded from http://ehjcimaging.oxfordjournals.org/ at Pennsylvania State University on March 6, 2014

Mark A. Friedman, Sampson Wiseman, Linda Haramati,
Garet M. Gordon, Daniel M. Spevack*
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 15 December 2005
Available online 28 February 2006
KEYWORDS Abstract Noncompaction of the left ventricle is a rare, congenital cardiomyopathy

Noncompaction; characterized by excessive trabeculation of the myocardium. Dextrocardia with situs
Dextroversion; solitus, commonly referred to as dextroversion, is associated with additional congen-
Situs solitus; ital heart disease. We report a case of noncompaction of the left ventricle in a patient
Dextrocardia with dextroversion, an association of which has not been previously described.
ª 2006 The European Society of Cardiology. Published by Elsevier Ltd. All rights
reserved.
Case presentation holosystolic murmur was best heard in the right

mid-axillary line. There was no peripheral edema.
A 46 year-old man with a history of dextrocardia, The chest X-ray showed the heart’s location in
end-stage renal disease, hypertension and several the right chest, with the descending aorta located
prior cerebrovascular accidents was referred for to the left of midline, Fig. 1. The standard electro-
evaluation. He reported having a left nephrectomy cardiogram showed normal sinus rhythm with a
and splenectomy following a gunshot wound 15 normal, leftward, P-wave axis, Fig. 2. The QRS axis
years earlier. He denied having a history of chest was deviated superiorly to 90 . Large R-waves
pain, exertional dyspnea or arrhythmia. were seen in leads V1eV3 with progressively smaller
On physical examination the vital signs were R-waves in the lateral chest leads. An interventricu-
normal and the pulse was regular. The neck veins lar conduction delay was seen.
were normal and the lung fields were clear. Heart Transthoracic echocardiography performed on
sounds were best heard in the right chest. A 2/6 the right chest showed that the morphologic right
ventricle, which contained the moderator band and
* Corresponding author. Tel.: þ1 718 920 4808; fax: þ1 718 920
a tricuspid valve, pumped into a bifurcating pulmo-
7709. nary artery. This finding ruled out congenitally
E-mail address: dspevack@montefiore.org (D.M. Spevack). corrected transposition or double discordance.
Noncompaction and dextroversion 71
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.
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 involv-
ing the apex and mid-ventricular postero-lateral
walls.

An angiotensin converting enzyme inhibitor was
started due to left ventricular dysfunction, com-
monly associated with noncompaction.2,5,6 Antico-
agulation was also initiated due to increased risk
of embolization.2 Given the apparent increased
risk of ventricular arrhythmias suggested by sev-
Figure 1 Chest X-ray showing the heart in the right
eral small case series, the patient was referred
chest with the descending aorta located to the left of
for evaluation by an electrophysiologist for
midline (arrow). Ao, aorta.
consideration of ICD implantation.2,6
Excessive segmental thickening of the left ventricle

was seen, with a noncompacted layer of spongiform Discussion
trabeculae more than twice as thick as the
underlying compacted layer, Fig. 3A. Color Doppler Noncompaction of the left ventricle is a congenital
examination showed penetration of blood flow cardiomyopathy characterized by a segmental
into the sinusoidal recesses of the ventricular myo- thickened endocardial part of the myocardium
cardium, Fig. 3B. These findings were thought to be with deep, blood-filled recesses and a normal
Figure 2 Typical left-sided electrocardiogram revealing a normal, leftward, P-wave axis. The QRS axis is deviated
superiorly to 90 . Large R-waves are seen in leads V1eV3 with progressively smaller R-waves in the lateral chest
leads. An interventricular conduction delay is seen.
72 M.A. Friedman et al.

Figure 3 Two-dimensional echocardiogram (apical four chamber view), depicting prominent trabeculations in the
left ventricle (arrow) (A). Color Doppler examination (B) shows penetration of blood flow into the sinusoidal recesses.
LV, left ventricle.
epicardial layer.2 It is a rare disorder with an inci- been proposed including a ratio 2 of wall thickness
dence estimated to be 1/2200 and is thought to be between the noncompacted, trabeculated, and
caused by the arrest of endomyocardial compac- the non-trabeculated, compacted layer of the
tion in utero.3,4 Previous case series by Ichida left ventricular myocardium at end-systole, measured
and Oechslin have reported depressed systolic at the parasternal short-axis.1
function in 48% and 82%, respectively.5,2 Other Dextroversion is a rare congenital abnormality
major complications include ventricular arrhyth- with an estimated incidence of 1/2800.7,8 This
mias, with an increased incidence of sustained condition is different from dextrocardia with situs
ventricular tachycardia.2,6 An increased incidence inversus, where a mirror-image reversal but pre-
of thromboembolic events as high as 24% has served relationship exists between the heart,
been reported.2 Several diagnostic criteria have great vessels, and abdominal organs. The majority
Figure 4 MRI long axis depicting normal location of the cardiac atria and great vessels, situs solitus, with the heart’s
apex rotated into the right chest, dextrocardia (A). The pulmonary artery is seen in the left chest, lateral to the aorta,
situs solitus (B). Ao, aorta; RA, right atrium; RV, right ventricle; LA, left atrium; LV, left ventricle; MPA, main pulmo-
nary artery; LMPA, left main pulmonary artery; RMPA, right main pulmonary artery.
Noncompaction and dextroversion 73
of patients with dextroversion have additional con- 3. Ritter M, Oechslin R, Sutsch G, Attenhofer C, Schneider J,
genital heart disease including left to right shunts, Jenni R. Isolated noncompaction of the myocardium in
adults. Mayo Clin Proc 1997;72:26e31.
decreased pulmonary blood flow and conotruncal 4. Engberding R, Bender F. Identification of a rare congenital
abnormalities.8 To our knowledge, this is the first anomaly of the myocardium by two dimensional echocardi-
case report of a patient with both dextroversion ography: persistence of isolated myocardial sinusoids. Am
and noncompaction of the left ventricle. J Cardiol 1984;53:1733e4.
5. 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;
References 34:233e40.
6. Murphy RT, Thaman R, Blanes JG, Ward D, Sevdalis E,
1. Jenni R, Oechslin E, Schneider J, Attenhofer Jost C, Papra E, et al. Natural history and familial characteristics
Kaufman PA. Echocardiographic and pathoanatomical charac- of isolated left ventricular non-compaction. Eur Heart J
teristics of isolated left ventricular non-compaction: a step to- 2005;26:187e92.
wards classification as a distinct cardiomyopathy. Heart 2001; 7. Comstock CH, Smith R, Lee W, Kirk JS. Right fetal cardiac
86:666e71. axis: clinical significance and associated findings. Obstet

2. Oechslin EN, Attenhofer Jost CH, Rojas JR, Kaufmann PA, Gynecol 1998;91:495e9.
Jenni R. Long-term follow-up of 34 adults with isolated left 8. Garg N, Agarwal BL, Modi N, Radhakrishnan S, Sinha N. Dex-
ventricular noncompaction: a distinct cardiomyopathy with trocardia: an analysis of cardiac structures in 125 patients.
poor prognosis. J Am Coll Cardiol 2000;36:493e500. Int J Cardiol 2003;88:143e55.
1525-2167/$32 ª 2006 The European Society of Cardiology. Published by Elsevier Ltd. All rights reserved.
10.1016/j.euje.2005.12.011

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70 M.A. Friedman et al.

Noncompaction of the left ventricle in a patient

Downloaded from http://ehjcimaging.oxfordjournals.org/ at Pennsylvania State University on March 6, 2014

KEYWORDS Abstract Noncompaction of the left ventricle is a rare, congenital cardiomyopathy

Case presentation holosystolic murmur was best heard in the right

diagnostic of noncompaction of the left ventricle

Downloaded from http://ehjcimaging.oxfordjournals.org/ at Pennsylvania State University on March 6, 2014

Excessive segmental thickening of the left ventricle

Downloaded from http://ehjcimaging.oxfordjournals.org/ at Pennsylvania State University on March 6, 2014

Downloaded from http://ehjcimaging.oxfordjournals.org/ at Pennsylvania State University on March 6, 2014

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