CARDIOVASCULAR JOURNAL OF AFRICA • Volume 25, No 1, January/February 2014
AFRICA
e5
Case Report
Tachycardia-induced cardiomyopathy due to repetitive
monomorphic ventricular ectopy in association with
isolated left ventricular non-compaction
Damirbek Osmonov, Kazim Serhan Özcan, Ahmet Ekmekçi, Bariş Güngör, Ahmet Taha Alper, Kadir Gürkan
Abstract
Isolated left ventricular non-compaction is a rare genetic
disorder manifesting mainly with heart failure, ventricular
arrhythmias and systemic embolism. Isolated ventricular
tachycardia originating from the right ventricular outflow
tract is an arrhythmia that can be treated medically and/or by
radiofrequency catheter ablation. Here, we report a case of an
asymptomatic 16-year-old boy with a new diagnosis of dilated
cardiomyopathy, left ventricular non-compaction and right
ventricular outflow tract tachycardia. Electrophysiological
studies and radiofrequency ablation of the right ventricular
outflow tract tachycardia resulted in normalisation of left
ventricular systolic function. This is the first case reporting
left ventricular non-compaction in association with tachycar-
dia-induced cardiomyopathy secondary to repetitive mono-
morphic right ventricular outflow tract tachycardia.
Keywords:
cardiomyopathy, dilated, left ventricular non-
compaction, heart failure, ventricular premature complexes
Submitted 21/4/12, accepted 8/11/13
Published online 2/12/13
Cardiovasc J Afr
2014;
25
: e5–e7
DOI: 10.5830/CVJA-2013-080
Isolated left ventricular non-compaction is a genetic disorder
that develops secondary to arrest of the compaction process
of the sponge-like embryonic myocardium to adult-type
myocardium during the first trimester. Heart failure, ventricular
arrhythmias and systemic embolism are manifestations of the
disease.
1
We present a case of left ventricular non-compaction
with tachycardia-induced cardiomyopathy related to repetitive
monomorphic premature ventricular contractions originating
from the right ventricular outflow tract.
Case report
A 16-year-old boy was referred to our cardiology department
because of repetitive premature ventricular contractions, which
were diagnosed incidentally during pre-operative evaluation for
plastic surgery. His history was unremarkable for any cardiac
disease and he did not have any symptoms, including palpitation
and dyspnoea, during assessment.
An electrocardiogram revealed normal sinus rhythm with
repetitive monomorphic couplets/triplets of premature ventricular
contractions with left bundle branch block morphology and
inferior QRS axis (Fig. 1). Twenty-four-hour rhythm Holter
monitoring revealed that 70% of the total heart beats consisted
of premature ventricular contractions.
Transthoracic echocardiography showed left ventricular
dilation and systolic dysfunction with an ejection fraction of
29%, and increase of the right ventricular outflow tract diameter.
The apicolateral site of the left ventricle had excessively
prominent trabeculations and deep inter-trabecular recesses with
a non-compacted:compacted myocardium ratio higher than 2.
Colour flow imaging revealed direct blood flow within the
deep inter-trabecular recesses, which was compatible with left
ventricular non-compaction. Cardiacmagnetic resonance imaging
confirmed the diagnosis of left ventricular non-compaction and
left ventricular cardiomyopathy (Fig. 2).
On electrophysiological study, the earliest ventricular activation
site was on the anteroseptal portion of the superior right ventricular
outflow tract. Pace mapping of this site revealed identical
electrocardiographicmorphologywith the 12-lead electrocardiogram
of the patient. The premature ventricular contractions disappeared
after radiofrequency ablation applied to this site.
The patient was discharged the day after the procedure.
Control echocardiography performed two months later revealed
normal left ventricular size and improved systolic function
with an ejection fraction of 53%, but persistant excessively
prominent trabeculations and deep inter-trabecular recesses with
a non-compacted:compacted myocardium ratio higher than 2. On
24-hour rhythm Holter monitoring, the rhythm was sinus without
any premature ventricular contractions.
Department of Cardiology, Almaty Sema Hospital, Almaty,
Kazakhstan
Damirbek Osmonov, MD
Department of Cardiology, Dr.Siyami Ersek Cardiovascular
and Thoracic Surgery Training and Research Hospital,
Istanbul, Turkey
Kazim Serhan Özcan, MD,
Ahmet Ekmekçi, MD
Bariş Güngör, MD
Ahmet Taha Alper, MD
Kadir Gürkan, MD
