Download Noncompaction of the left ventricle in a patient with dextroversion

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10. Stollberger C, Finsterer J. Thrombi in left ventricular hypertrabeculation/noncompaction e review of the literature.
Acta Cardiol 2004 Jun;59(3):341e4.
11. Ruvolo G, Fattouch K, Speziale G, Macrina F, Tonelli E,
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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
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
KEYWORDS
Noncompaction;
Dextroversion;
Situs solitus;
Dextrocardia
Abstract 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.
ª 2006 The European Society of Cardiology. Published by Elsevier Ltd. All rights
reserved.
Case presentation
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
* Corresponding author. Tel.: þ1 718 920 4808; fax: þ1 718 920
7709.
E-mail address: [email protected] (D.M. Spevack).
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 V1eV3 with progressively smaller
R-waves in the lateral chest leads. An interventricular conduction delay was seen.
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.
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Received 29 September 2005; received in revised form 2 December 2005; accepted 15 December 2005
Available online 28 February 2006
Noncompaction and dextroversion
Figure 1 Chest X-ray showing the heart in the right
chest with the descending aorta located to the left of
midline (arrow). Ao, aorta.
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 involving the apex and mid-ventricular postero-lateral
walls.
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
Discussion
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
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.
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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
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M.A. Friedman et al.
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
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 pulmonary artery; LMPA, left main pulmonary artery; RMPA, right main pulmonary artery.
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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.
Noncompaction and dextroversion
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.
References
1. Jenni R, Oechslin E, Schneider J, Attenhofer Jost C,
Kaufman PA. Echocardiographic and pathoanatomical characteristics of isolated left ventricular non-compaction: a step towards classification as a distinct cardiomyopathy. Heart 2001;
86:666e71.
2. 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;36:493e500.
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3. Ritter M, Oechslin R, Sutsch G, Attenhofer C, Schneider J,
Jenni R. Isolated noncompaction of the myocardium in
adults. Mayo Clin Proc 1997;72:26e31.
4. Engberding R, Bender F. Identification of a rare congenital
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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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