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Case Report Cardiomyopathy
Left Dominant Arrhythmogenic Cardiomyopathy Causing Sustained
Ventricular Tachycardia – A Case Report
Francesco Guglielmi, 1 Sergio Cannas, 1 Rocco Arancio, 1 Nicolò Martini 2 and Bortolo Martini 1
1.Cardiac Unit, Alto Vicentino Hospital, Santorso (VI), Italy; 2. Ferrara Medical School, Ferrara University, Ferrara, Italy
Abstract A 56-year-old white man was admitted to hospital because of palpitations and dyspnoea. He had no family history of cardiomyopathy
or sudden death, and the electrocardiograms (ECG) of his son and parents were normal. He had an history of palpitations, and in the
emergency department ECG showed sustained ventricular tachycardia-flutter with inferior axis right bundle branch block (RBBB) in V1
and heart rate 250 beats per minute. His standard ECG showed a complete RBBB pattern with minor ST-T segment changes. 2D echo
and coronary angiography were normal, while magnetic resonance imaging (MRI) revealed a reduction in left mid-lateral wall thickening
with hypokinetic movement and mild reduction of ejection fraction. An Extensive sub-epicardial area of conspicuous left ventricular
intra-myocardial fat and sub-epicardial left ventricular late enhancement in lateral wall, was also seen at MRI. No abnormalities of right
ventricular size, function or tissue characterisation were detected. A diagnosis of atypical left dominant arrhythmogenic cardiomyopathy
was made, based on clinical and images basis, as gene testing was not available.
Keywords Ventricular tachycardia, left dominant arrhythmogenic cardiomyopathy, desmoplakin
Disclosure: Francesco Guglielmi, Sergio Cannas, Rocco Arancio, Nicolò Martini, and Bortolo Martini have no conflicts of interest to declare in relation to this article. No
funding was received in the publication of the article.
Compliance with Ethical Guidance: All procedures were followed in accordance with the responsible committee on human experimentation and with the Helsinki
Declaration of 1975 and subsequent revisions, and informed consent was received from the patient involved in this case study.
Open Access: This article is published under the Creative Commons Attribution Noncommercial License, which permits any non-commercial use, distribution, adaptation
and reproduction provided the original author(s) and source are given appropriate credit.
Received: 26 January 2016 Accepted: 24 February 2016 Citation: European Journal of Arrhythmia & Electrophysiology, 2016;2(1):37–9
Correspondence: Bortolo Martini, MD, Via Gioberti 9, 36016 Thiene (VI), Italy. E: firstname.lastname@example.org
The purpose of this report is to describe an unusual case of
arrhythmogenic cardiomyopathy, involving the left ventricle (LV),
with an atypical standard electrocardiogram (ECG) pattern. Sustained
ventricular arrhythmias in a middle-aged subject are usually related
to ischemic heart disease. A latent or overt cardiomyopathy is
probably the second cause of a rhythm disturbance especially.
Idiopathic forms are rare at this age, but only a detailed investigation
can clarify the organic substrate, that was so unusual in the
A final diagnosis of left dominant arrhythmogenic cardiomyopathy
(LDAC) was reached and the patient was discharged on bisoprololo
10 mg twice per day with indication for very close follow-up and
restriction of physical activity. The choice of not implanting an
implantable cardioverter-defibrillator or to perform an ablation, was
decided after a discussion with the patient, who refused an invasive
procedure. After a 4-year follow-up, the patient is asymptomatic, and
no repetitive ventricular arrhythmias have been detected during Holter
monitoring and exercise stress test.
Limitations A 56-year-old white man was admitted to hospital because of
palpitations and dyspnoea. He had no family history of cardiomyopathy
or sudden death. He complained of palpitations starting in 2002, but
had not been to see a doctor. In the emergency department, the ECG
showed sustained ventricular tachycardia-flutter with apparently
inferior axis right bundle branch block (RBBB) in V1 and heart rate
250 beats per minute (Figure 1). The ventricular tachycardia was
associated with initial signs and symptoms of left heart failure and
the patient was referred to the Coronary Care Unit where sinus
rhythm was restored by direct current electric shock. Symptoms
promptly restored after the procedure. The results of the investigation
performed are listed in Table 1.
Genetic studies have not yet been performed because of high cost/
TOU CH MED ICA L MEDIA
Discussion After the description of Markus and Fontaine, 1 the arrhythmogenic
right ventricular dysplasia/cardiomyopathy (ARVD/C) has crossed
the borders of the right ventricle. LDAC has been recognised as
an associated or isolated entity also characterised pathologically
by fibro-adipose replacement of the LV, often occurring as a
circumferential band in the outer third of the myocardium and
the right side of the inter-ventricular septum. 2–5 First described at
post-mortem examination in sudden cardiac death (SCD) victims,