A rare presentation of spontaneous ventricular tachycardia in young adult

Ram Narayan; Mansoor C Abdulla; Jemshad Alungal

doi:10.4103/heartindia.heartindia_40_16

CASE REPORT

Year : 2017 | Volume : 5 | Issue : 1 | Page : 45-47

A rare presentation of spontaneous ventricular tachycardia in young adult

Ram Narayan, Mansoor C Abdulla, Jemshad Alungal
Department of General Medicine, MES Medical College, Perinthalmanna, Kerala, India

Date of Web Publication

8-Mar-2017

Correspondence Address:
Dr. Ram Narayan
Department of General Medicine, MES Medical College, Perinthalmanna - 679 338, Kerala
India

Source of Support: None, Conflict of Interest: None

Check

DOI: 10.4103/heartindia.heartindia_40_16

Abstract

Arrhythmogenic right ventricular dysplasia (ARVD) is a common cause of sudden cardiac death in young adults. It is a disease that has a wide spectrum of presentation. Early identification of the disorder is of paramount importance considering the fatal complications. Here, with this case, a rare sporadic presentation of ARVD, we try to discuss the various presentations, methods of diagnosis and options for treatment for this frequently masquerading and occasionally fatal disorder of cardiac myocytes.

Keywords: Arrhythmogenic right ventricular dysplasia, cardiac desmosones, epsilon waves, fibrofatty infiltration, right ventricular dilataion, sporadic

How to cite this article:
Narayan R, Abdulla MC, Alungal J. A rare presentation of spontaneous ventricular tachycardia in young adult. Heart India 2017;5:45-7

How to cite this URL:
Narayan R, Abdulla MC, Alungal J. A rare presentation of spontaneous ventricular tachycardia in young adult. Heart India [serial online] 2017 [cited 2023 Feb 2];5:45-7. Available from: https://www.heartindia.net/text.asp?2017/5/1/45/201743

Introduction

Arrhythmogenic right ventricular dysplasia (ARVD) is a common heritable disorder of cardiomyocytes characterized by disorder of desmosomes leading to fibrofatty infiltration of myocardium.^[1] Clinical features of the disease vary from mild occasional lightheadedness to life-threatening ventricular arrhythmia though a family history of the same is almost always present. Here, we present a rare case of ARVD which presented sporadically.

Case Report

A 33-year-old construction worker with no premorbidities and not on any regular medication presented to our emergency department with sudden onset of palpitations, lightheadedness, and chest pain for 20 min. He had similar complaints 5 days back which lasted for 10 min and improved spontaneously. He is hailing from a construction worker family where even women do heavy manual labor. There is no family history of unexplained sudden deaths, arrhythmias, or cardiac illness traceable up to three generations. His general physical examination was normal, pulse rate 160 beats/min, and blood pressure 90/60 mmHg. His cardiovascular system examination was normal.

His electrocardiogram (EKG) showed monomorphic ventricular tachycardia of right ventricular origin and was successfully cardioverted from emergency department. His hemoglobin was 13.6 g/dl, total leukocyte count 9800 cells/cumm, and platelet count 2.3 lakhs/cumm. Renal, liver, and thyroid function tests were normal. Complete electrolyte panel including sodium, potassium, calcium, magnesium, and phosphorous was normal. Arterial blood gas analysis showed no metabolic acidosis. Chest radiograph was normal.

Repeat EKG after cardioversion showed sinus pattern with a heart rate of 100 beats/min, normal axis, widening of qRs (160 ms) in V₁ to V₃ leads with T inversion and epsilon waves in V₁ to V₃ leads. His echocardiogram revealed right ventricular dilatation with thinning of right ventricular free wall, significantly reduced right ventricular ejection fraction and regional wall hypokinesis right ventricle with normal left ventricle. Cardiac magnetic resonance imaging demonstrated fibrofatty infiltration of right ventricular myocardium. He was diagnosed to have ARVD based on the International Task Force Criteria for the same [Figure 1].

Figure 1: (a) Initial electrocardiogram showing monomorphic ventricular tachycardia with ventricular ectopics and left bundle branch block pattern (block arrow in a) suggestive of right ventricular origin. (b) Repeat electrocardiogram showing Epsilon waves (block arrows in b) in leads V1 to V3. (c) Parasternal long axis view of patient's echocardiogram showing right ventricular dilatation (block arrow in c) with free wall thinning. (d) Cardiac magnetic resonance imaging demonstrating fibro-fatty infiltration of myocardium (block arrow in d)

Click here to view

He was admitted and was started on propranolol. After six uneventful days of hospitalization, he was discharged in good condition. He was advised to avoid exercises and strenuous manual labor. He was also planned for further management with implantable cardiac defibrillator if symptoms recur.

Discussion

ARVD is a genetic disorder that affects areas on surface of heart muscle cells which link the cells together, desmosomes.^[1] ARVD is inherited as an autosomal dominant disorder, and hallmark is replacement of normal myocardium with fibrofatty infiltrates that render it hypokinetic leading to a multitude of arrhythmias with right ventricular origin. Autosomal recessive variants are also seen (Naxos disease) with mutation in similar protein, plakoglobin.^[2] Rarely nongenetic diseases such as congenital anomalies and viral or inflammatory myocarditis may produce fibrofatty infiltration of myocardium.^[3] ARVD is a cause of sudden cardiac death in children and athletes and usually manifests before the age of forty.

Although ARVD is a genetic disease, genetic testing is not mandatory for the diagnosis of the same. The International Task Force Criteria has been established in 1995 and modified later with improved diagnostic accuracy.^[3],[4] Diagnosis may be made with two major, one major, and two minor or four minor criteria. Major criteria includes right ventricular dysfunction (severe dilatation and reduction of right ventricular ejection fraction with normal left ventricle), fibrofatty infiltration of myocardium in biopsy or magnetic resonance imaging, conduction abnormalities (epsilon waves in V₁ to V₃, localized prolongation >110 ms of qRs in V₁ to V₃), and family history of the same confirmed on autopsy or surgery. Minor criteria includes right ventricular dysfunction (mild global right ventricular dilatation, regional right ventricular hypokinesis), fibrofatty infiltration of myocardium, conduction abnormalities (inverted T waves in V₁ to V₃ in the absence of right bundle branch block, late potentials on signal averaged EKG, frequent premature ventricular complexes >1000 in 24 h), and family history of sudden cardiac death in family. Our patient satisfies three major criteria except family history as well as majority of minor criteria, and hence diagnosed to have ARVD.

Clinical features of ARVD vary from slight lightheadedness to sudden unexplained death. The attacks are mostly predisposed by exercises. The overall mortality rate of condition is 19%^[5] which make it mandatory to suspect the problem in a young adult with indolent symptoms such as lightheadedness. There are case reports of late presentation of ARVD, even in the seventies because of its occasional mild course.^[6] There are also reports of ARVD complicating pregnancy with shortness of breath and palpitations during second trimester.^[7]

As clinical presentations vary significantly, suspicion should be kept if there are unexplained T wave inversions in septal leads in a young adult. He should be followed up with an echocardiogram of heart and more importantly a cardiac magnetic resonance imaging. Cardiac magnetic resonance imaging in addition to fibrofatty infiltrations of myocardium can also pick up reduction of myocardial thickening, dilatation, and aneurismal changes with reduction in wall motion as well.^[8]

The goal of management ^[9] is to prevent sudden cardiac death. Beta-adrenergic blocking agents as well as amiodarone may be tried to prevent recurrence of arrhythmias. Implantable cardiac defibrillator is a tried out option among those patients who are at high risk. In those who are resistant to all these, radiofrequency ablation ^[10] may be tried. However, it is difficult as fibrofatty infiltration is patchy and segmental. All patients should be barred from strenuous labor and exercises, and relatives should be screened with EKG and echocardiogram and preferably cardiac magnetic resonance imaging.

Conclusion

ARVD is a cause of sudden cardiac death. As the clinical features and course of illness varies significantly, a huge amount of suspicion should be kept in mind if any young adult turns up with suggestive symptoms. We are presenting this case as a prototype of a fatal genetic disease to create awareness among practitioners and make them competent enough to offer a treatment even if it manifests with or without family history.

Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest.

References

1.	Fressart V, Duthoit G, Donal E, Probst V, Deharo JC, Chevalier P, et al. Desmosomal gene analysis in arrhythmogenic right ventricular dysplasia/cardiomyopathy: Spectrum of mutations and clinical impact in practice. Europace 2010;12:861-8.
2.	Antoniades L, Tsatsopoulou A, Anastasakis A, Syrris P, Asimaki A, Panagiotakos D, et al. Arrhythmogenic right ventricular cardiomyopathy caused by deletions in plakophilin-2 and plakoglobin (Naxos disease) in families from Greece and Cyprus: Genotype-phenotype relations, diagnostic features and prognosis. Eur Heart J 2006;27:2208-16.
3.	Marcus F, Basso C, Gear K, Sorrell VL. Pitfalls in the diagnosis of arrhythmogenic right ventricular cardiomyopathy/dysplasia. Am J Cardiol 2010;105:1036-9.
4.	Dalal D, Nasir K, Bomma C, Prakasa K, Tandri H, Piccini J, et al. Arrhythmogenic right ventricular dysplasia: A United States experience. Circulation 2005;112:3823-32.
5.	Hulot JS, Jouven X, Empana JP, Frank R, Fontaine G. Natural history and risk stratification of arrhythmogenic right ventricular dysplasia/cardiomyopathy. Circulation 2004;110:1879-84.
6.	Papaioannou GI, Apostolopoulos T, Stambola S, Zilidis A, Gialafos J. Late presentation of arrhythmogenic right ventricular cardiomyopathy: A case report. J Med Case Rep 2009;3:7235.
7.	Güdücü N, Kutay SS, Ozenç E, Ciftçi C, Yigiter AB, Isçi H. Management of a rare case of arrhythmogenic right ventricular dysplasia in pregnancy: A case report. J Med Case Rep 2011;5:300.
8.	Kayser HW, van der Wall EE, Sivananthan MU, Plein S, Bloomer TN, de Roos A. Diagnosis of arrhythmogenic right ventricular dysplasia: A review. Radiographics 2002;22:639-48.
9.	Buja G, Estes NA 3^rd, Wichter T, Corrado D, Marcus F, Thiene G. Arrhythmogenic right ventricular cardiomyopathy/dysplasia: Risk stratification and therapy. Prog Cardiovasc Dis 2008;50:282-93.
10.	Kottkamp H, Hindricks G. Catheter ablation of ventricular tachycardia in ARVC: Is curative treatment at the horizon? J Cardiovasc Electrophysiol 2006;17:477-9.