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CARDIOVASCULAR JOURNAL OF AFRICA • Volume 25, No 5, September/October 2014
AFRICA
e9
Case Report
Sustained ventricular tachycardia in a patient with
isolated non-compaction cardiomyopathy
Yusuf Izzettin Alihanoglu, Ismail Dogu Kilic, Bekir Serhat Yildiz, Mustafa Kartin, Harun Evrengul
Abstract
Isolated non-compaction of the left ventricular myocardium
(INVM) was first described in 1984 as an unclassified cardio-
myopathy, not being dilated, hypertrophic or restrictive. It is
assumed to occur as a result of an arrest in endomyocardial
morphogenesis during normal development of the heart. The
disease is characterised by heart failure due to systolic and
diastolic left ventricular (LV) dysfunction, systemic emboli
and ventricular arrhythmias. Echocardiography has been
shown to be the method of choice in diagnosis. INVM is a
rare congenital cardiomyopathy and only a few cases of this
condition have been reported. It is characterised by prominent
and excessive trabeculation in a ventricular wall segment, with
deep inter-trabecular spaces perfused from the ventricular
cavity. We report a case of INVM with ventricular tachycar-
dia induced during electrophysiological study in a 24-year-old
female patient with a family history of sudden death.
Keywords:
isolated non-compaction cardiomyopathy, cardiomy-
opathy, ventricular tachycardia, family history, sudden cardiac
death
Submitted 7/6/14, accepted 25/6/14
Cardiovasc J Afr
2014;
25
: e9–e12
www.cvja.co.zaDOI: 10.5830/CVJA-2014-037
Non-compaction cardiomyopathy is a congenital pathological
disorder that can occur in association with other congenital
anomalies, such as pulmonary valve atresia or aortic atresia with
intact ventricular septum.
1
It is also an isolated disease without
other structural diseases of the heart.
2
The incidence of non-compaction cardiomyopathy is estimated
at 0.05% in adults. Echocardiography has been shown to be the
method of choice in the diagnosis of isolated non-compaction
of the left ventricular myocardium (INVM). The age of onset
of symptoms ranges from infancy to old age. INVM is the result
of an arrest in compaction of the myocardial fibres during intra-
uterine life. Although the most frequent sites involved are the left
ventricular (LV) apex and inferior wall, involvement of other LV
walls and the right ventricle has also been reported.
3
The prognosis of patients with INVM is determined by the
degree and progression of heart failure, and the presence of
thromboembolic events and arrhythmias. Symptoms of heart
failure lead to hospital admission in the majority of adult
patients with INVM. Arrhythmias include atrial arrhythmias,
ventricular tachycardia, and sudden cardiac death.
4
Case report
A 25-year-old female came to the out-patient cardiology
department with a history of palpitations, sweating and shortness
of breath over the previous two months. Her New York Heart
Association functional capacity was class II. She mentioned
that her mother had died suddenly at 25 years old without
any known reason. There was no history of any risk factor for
coronary artery disease or any other significant medical illness
in our patient.
On physical examination, her pulse rate was 110 beats per
minute and blood pressure was 100/60 mmHg. On auscultation,
the heart rate was dysrhythmic and tachycardic, and the heart
sounds were normal except for a systolic 2/6 ejection murmur
heard at the apex. She had mild bilateral pedal oedema. Other
examination findings were essentially normal. In addition,
laboratory results were within the normal range.
An electrocardiogram showed atrial fibrillation with a
rapid ventricular response and non-specific intraventricular
conduction delay. On transthoracic Doppler echocardiography,
the left ventricle was visualised as hypertrophied and dilated
with an end-diastolic diameter greater than 6 cm. LV wall
motions were globally hypokinetic and the ejection fraction was
calculated as 32% by the Simpson method. There was mild mitral
and tricuspid regurgitation.
The echocardiographic findings were suggestive of INVM,
with the end-systolic ratio of the non-compacted structure of the
left ventricle to the compacted layer greater than 2, prominent
trabeculations and deep inter-trabecular recesses, into which
blood from the ventricular cavity was flowing, established by
colour Doppler (Fig. 1D). The non-compacted segments were
Department of Cardiology, Medical Faculty, Pamukkale
University, Denizli, Turkey
Yusuf Izzettin Alihanoglu, MD,
aliizyu@mynet.comIsmail Dogu Kilic, MD
Bekir Serhat Yildiz, MD
Harun Evrengul, MD
Department of Cardiology, Nevsehir State Hospital,
Nevsehir, Turkey
Mustafa Kartin, MD