Cardiovascular Journal of Africa: Vol 27 No 1 (January/February 2016)

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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).3No 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.
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