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CARDIOVASCULAR JOURNAL OF AFRICA • Volume 25, No 5, September/October 2014
AFRICA
e11
functional class I/II, the remaining had symptoms of congestive
heart failure. Ventricular arrhythmias were documented in
more than a third of the patients. In this study, the presence of
ventricular non-compaction in more than three segments was the
sign of a poor prognosis and was associated with a functional
class greater than II and ventricular arrhythmias.
7
In a nine-year echocardiographic study by Ritter
et al
.,
only 17 cases of INVM in adult subjects were identified.
8
They
also emphasised that diagnosis of non-compaction of the
LV myocardium in an adult population may be overlooked
because of similarities with other more frequently diagnosed
cardiomyopathies, and echocardiographic screening of first-
degree relatives was recommended due to its familial association.
9
Kahn
et al.
stated that end-stage congestive heart failure
should be managed with heart transplantation, and potential
life-threatening ventricular tachyarrhythmias with an ICD
because the main causes of death were severe heart failure and
sudden cardiac death.
10
In a study by Fazio
et al
. of 238 patients
affected by non-compaction, only 11 patients had documented
ventricular tachycardia.
11
In another study, it was stipulated that
INVM was often related to systolic dysfunction and ventricular
dilatation. Malignant ventricular arrhythmias were seen in
47% and sudden cardiac death in almost 50% of the patients.
12
In addition, other cases of INVM, presenting initially with
ventricular tachycardia, have been described in the literature.
13,14
Conclusion
A high index of suspicion for the diagnosis of INVM is
necessary because of the high incidence of heart failure and
other complications, such as malignant ventricular arrhythmias,
death and thromboembolic events. Early diagnosis is important
and may be life-saving, especially for patients with a family
history of sudden cardiac death, as seen in our case. Treatment
should be individualised and directed towards prevention and
management of heart failure, ventricular arrhythmias and
thromboembolic events.
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ECG shows basal atrial fibrillation and sustained monomorphic ventricular tachycardia, induced after programmed ventricu-
lar stimulation.