![Show Menu](styles/mobile-menu.png)
![Page Background](./../common/page-substrates/page0050.png)
CARDIOVASCULAR JOURNAL OF AFRICA • Volume 27, No 1, January/February 2016
48
AFRICA
The multiple prominent trabeculations cause a restriction in
filling, an abnormal ventricular relaxation pattern and diastolic
dysfunction, with a generally poor eventual outcome for patients.
Other complications can include thrombus formation within the
recesses between trabeculae and subsequent thromboembolic
events.
Delayed enhancement in the myocardium has been shown to
increase under conditions of myocardial interstitial expansion
or fibrosis.
11
Previous histological studies have shown necrosis
and fibrosis in patients with LVNC.
12
These areas of fibrosis
may serve as a focus or as foci for future lethal ventricular
arrhythmias.
Cardiac MRI has proven very useful in identifying these
areas of fibrosis for characterisation and further management
(evaluation for heart transplant).
3
No such foci were found in our
patient (Fig. 1).
LVNC can be an isolated finding in the heart in the absence
of other cardiac abnormalities. However, associations with other
cardiac disorders, including coronary arterioventricular fistulae,
ventricular septal defects, patent ductus arteriosus, atrial septal
defects, a left coronary artery originating from the pulmonary
artery, and dextrocardia have all been reported.
3
Non-compaction of the myocardium can be either isolated
or in conjunction with other congenital heart diseases. LVNC
has been identified in relatively high association in patients
with Ebstein’s anomaly with a reported figure of up to 18% of
patients with Ebstein’s having non-compaction.
13
Other associations previously reported include mitochondrial
disorders, Barth syndrome, hypertrophic cardiomyopathy,
muscular dystrophy type 1, 1p36 deletion, Turner syndrome,
Ohtahara syndrome, distal 5q deletion, mosaic trisomy 22,
trisomy 13, Di George syndrome, and 1q43 deletion with
decreasing frequency, as well as Pierre-Robin syndrome.
6
Malfunctioning of a rho-associated kinase has been implicated
in the onset of the heterotaxy syndrome.
14
Karyotyping and
genetic testing have not been performed
in our patient to date.
CT angiography and/or MRI can be used in these patients
to identify vasculature and other cardiac abnormalities, and
associated congenital non-cardiac abnormalities.
Conclusion
We report on only the third known case of dextrocardia, situs
ambiguous with polysplenia, and left ventricular non-compaction
in an adult. All the characteristic morphological features could
easily be identified on imaging studies including but not limited
to echocardiography, CT angiography and MRI.
References
1.
Ichida F, Tsubata S, Bowles KR,
et al
. Novel gene mutations in patients
with left ventricular noncompaction or Barth syndrome.
Circulation
2001;
103
: 1256–1263. DOI: 10.1161/01.CIR.103.9.1256.
2.
Maldjian PD, Saric M. Approach to dextrocardia in adults: review.
Am J
Roentgenol
2007;
188
: S39–S49. DOI: 10.2214/AJR.06.1179
3.
Cho YH, Jin SJ, Je HC,
et al.
A case of noncompaction of the ventricu-
lar myocardium combined with situs ambiguous with polysplenia.
Yonsei Med J
2007;
48
: 1052–1055.
4.
Bartram U, Wirbelauer J, Speer CP. Heterotaxy syndrome: asplenia and
polysplenia as indicators of visceral malposition and complex congeni-
tal heart disease.
Biol Neonate
2005;
88
: 278–290.
5.
Ichida F, Hamamichi Y, Miyawaki T,
et al
. Clinical features of isolated
noncompaction of the ventricular myocardium: Long-term clinical
course, hemodynamic properties, and genetic background
. J Am Coll
Cardiol
1999;
34
: 233–240.
6.
Aypar E, Sert A, Gokmen Z, Aslan E, Odabas D. Isolated left ventricu-
lar noncompaction in a newborn with Pierre-Robin sequence.
Pediatr
Cardiol
2013;
34
: 452–454.
7.
Bellet S, Gouley BA. Congenital heart disease with multiple cardiac
anomalies: report of a case showing aortic atresia, fibrous scar in
myocardium and embryonal sinusoidal remains.
Am J Med Sci
1932;
183
: 458–465.
8.
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;
103
: 1256–1263.
9.
Wald R, Veldtman G, Golding F,
et al
. Determinants of outcome in
isolated ventricular noncompaction in childhood.
Am J Cardiol
2004;
94
: 1581–1584.
10. Jenni R, Wyss CA, Oechslin EN, Kaufman PA. Isolated ventricular
noncompaction is associated with coronary microcirculatory dysfunc-
tion.
J Am Coll Cardiol
2002;
39
: 450–454.
11. Moon JC, Mundy HR, Lee PJ, Mohiaddin RH, Pennell DJ. Images in
cardiovascular medicine. Myocardial fibrosis in glycogen storage disease
type III.
Circulation
2003;
107
: e47.
12. Oechslin EN, Attenhofer Jost CH, Rojas JR, Jaufman PA, Jenni R.
Long-term follow up of 34 adults with isolated left ventricular noncom-
paction: a distinct cardiomyopathy with poor prognosis.
J Am Coll
Cardiol
2000;
36
: 493–500.
13. Attenhofer Jost CH, Connolly HM, O’Leary PW, Warnes CA, Tajik AJ,
Seward JB. Left heart lesions in patients with Ebstein’s anomaly.
Mayo
Clin Proc
2005;
80
: 361–368.
14. Egashira T, Yuassa S, Kimura M,
et al
. Coexistence of two distinct
fascinating cardiovascular disorders: Heterotaxy syndrome with left
ventricular non-compaction and vasopspastic angina.
Int J Cardiol
2014;
174
: e54–e56.