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| CASE REPORT |
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| Year : 2015 | Volume
: 12
| Issue : 1 | Page : 36-38 |
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Troublesome recurrent paroxysms of arrhythmia due to arrhythmogenic right ventricular dysplasia
Basil N Okeahialam
Department of Medicine, Cardiology Unit, Jos University Teaching Hospital, Jos, Nigeria
| Date of Web Publication | 5-Jan-2015 |
Correspondence Address:
Basil N Okeahialam
Department of Medicine, Cardiology Unit, Jos University Teaching Hospital, Jos
Nigeria
 Source of Support: None, Conflict of Interest: None  | Check |
DOI: 10.4103/0189-7969.148485
Arrhythmias could unsettle a clinician ill-equipped to define it and cause haemodynamic derangement in the patient. A young adult Nigerian female with no known family history of heart disease or sudden death is reported. She presented with recurrent palpitations which over time became more frequent and interfered with her life. She had no history of obvious heart disease or anything to explain the occurrence. In the course of evaluation her ECG suggested arrhythmogenic right ventricular dysplasia. It is being reported as it is said to be rare in Africa, and rarer still among females Keywords: Arrhythmogenic right ventricular dysplasia, Nigeria, female
How to cite this article:
Okeahialam BN. Troublesome recurrent paroxysms of arrhythmia due to arrhythmogenic right ventricular dysplasia. Nig J Cardiol 2015;12:36-8 |
| Introduction | |  |
Cardiac dysrrhythmias could present a devastating experience to the patient, and a nightmarish experience to the clinician ill equipped to confirm and manage such phenomena. As they have various causes, it is vital to define them so as to initiate appropriate action. In our environment deficient in modern diagnostic infrastructure, diagnosis most times is fortuitous; and requires a high level of alertness on the part of the clinician.
Recently a young lady presented with palpitations which over eight years got more frequent and started to interfere with her daily activities. In the course of evaluation electrocardiograms suggestive of Arrhythmogenic Right Ventricular Dysplasia (ARVD) were recorded. Not being commonly reported as yet Nigeria and in a female (a gender less commonly afflicted), the need to report this became imperative.
| Case report | | |
DA is a 34-year-old Nigerian woman who was first seen in February 2012 complaining of recurrent palpitations lasting for over 8 years. The frequency of the palpitations had increased over the years. There was no history suggestive of hypertension, diabetes mellitus, rheumatic heart disease, thyroid disease or heart failure. She took coffee as her regular beverage and indulged regularly in cola-based soft drinks. There was no history of sudden cardiac death or similar illness in the family.
She was naïve to oral contraceptive pills and on parity was 1 +1 with 1 alive having miscarried once. The live birth was by Cesarean section occasioned by abruptio placentae. The clinician who saw her first and referred her eventually, gave a short course of Amiodarone which seemed to help and requested an electrocardiogram (ECG), echocardiography and thyroid function test. The electrocardiogram revealed a junctional tachycardia [Figure 1]. Echocardiography was normal with no structural abnormality or ventricular dysfunction. Imaging modalities namely right ventricular angiography and cardiac magnetic resonance imaging that support diagnosis were not done as they were not available in our center. Thyroid function test returned normal as well as the electrolyte, urea, and creatinine. | Figure 1: Electrocardiogram showing junctional tachycardia. Note inverted p waves in leads II, III, aVf (arrowed)
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Physical examination on first encounter with the author revealed a normal cardiovascular status. An ECG done as part of this evaluation did not show the junctional tachycardia but features suggestive of arrhythmogenic right ventricular dysplasis with a prolonged QTc of 449 msec [Figure 2]. She was advised to avoid all stimulants and given Carvedilol 3.125 mg daily per oral. On this she was without the complaint of palpitations and stopped treatment on her own after a while. This resulted in a recurrence. | Figure 2: Electrocardiogram showing Localized prolonged QRS complex in right praecordial leads (120 msec) T wave inversion in V1 to V3 in the absence of right bundle branch block. QRS duration in V1 through V3 > V6 by greater than 25 msec (40 msec) Ratio of QRS duration in V1+V2+V3/V4+V5+V6 > 1.2 (1.2)
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On a second review, all was well on physical examination but for a low blood pressure of 94/64 mmHg. She was asked to resume Carvedilol but at a lower dose of 1.56 mg and return with a repeat ECG. This showed junctional tachycardia again similar to the first ECG shown in [Figure 1]. While on above dose of Carvedilol and waiting for her appointment, she had a 5-hour long episode of palpitations one night (after a long tiring day with domestic chores) during which she felt dizzy and faint. Home electronic blood pressure apparatus indicated "error" for most of the period; and after self-administered salt solution drink gave a 50/40 mmHg reading. She reported to hospital the next morning and had a pulse of 89/min with ectopics and blood pressure of 104/70 mmHg, sitting and 110/80 mmHg standing. Another ECG done on the spot showed features similar to [Figure 2] except that T wave inversion had extended to lead V4 and appeared deeper than the previous one in [Figure 2]. In addition the QTc was further prolonged at 474 msec.
At this point a 24-hour Holter monitoring was commenced, while she was still on the same low dose of Carvedilol. No palpitation occurred during the 24-hour period of Holter monitoring, hence no abnormality was recorded. She was allowed to go home on the same treatment. When she returned to clinic on due appointment, her pulse was 74/min good volume and regular and blood pressures were 84/70 mmHg sitting and 100/80 mmHg standing. Carvediolol was gradually withdrawn with Amiodarone re-introduced at 200 mg daily. On this, she reported no episode when she came for her next appointment. Carvedilol was dropped finally (after gradual withdrawal) and Amiodarone continued. Two weeks after this clinic visit, she had an episode of palpitation for which she rushed to an ECG centre for ECG test. This again showed a junctional tachycardia. With anxiety mounting and insomnia setting in, Flunitrazepam 10 mg nocte was added with good effect. It was gradually withdrawn and Amiodrone continued. Since then there has not been another long spell of palpitation.
| Comments | | |
As posited in an editorial, diagnosing ARVD can be a huge challenge and requires a high index of suspicion and repeat evaluations [1] as was the case here. This disease is said to occur in all races though reports from Africa had been sparse [2] before the publication from the South African Registry. [3] Although reported more in males in most series. [1],[3],[4] our patient was female who presented typically with palpitation, dizziness and faintness; common ways that such cases present. [1] There are four classical phases of presentation: The concealed phase (when individuals are asymptomatic but at risk of sudden death), overt electrical phase (manifest with effort-induced palpitations and syncope), diffuse right ventricular dysfunction (manifest with severe right heart failure with persevered left ventricular function) and biventricular failure or dilated cardiomyopathy (extension of dysplastic process to involve all ventricles. [5] This patient was in the second phase when palpitation and syncope follow effort. Although usually ascribed to exerting exercise, one of her episodes followed a long hectic day of domestic chores that exhausted her severely. It would appear that whatever exhausts such patients could trigger an electrical phenomenon capable of leading to sudden cardiac death. The normal echocardiogram would also suggest that she was yet to get to phases 3 and 4 when heart failure (right and bi-ventricular) is associated.
The major diagnostic criterion of ARVD evident here is localized prolongation of QRS complex in right praecordial, leads in excess of 110 m sec. [2] Here it was 120 m sec [Figure 2]. The other minor electrocardiographic criteria encountered were T wave invertion in V1 through V3 in the absence of right bundle branch block (RBBB) and above 12 years of age [Figure 2]; parietal block where QRS duration in V 1 through V 3 exceeds that in V 6 by greater than 25 msec (here it was 40 msec); ratio of QRS duration in leads V 1 + V 2 + V 3 /V 4 + V 5 + V 6 = 1.2. [6] Here it was 1.2. The patient denied family history. Although it is an inherited disease, expression and penetrance in family members is variable. Less than 4% of relatives of patients were found to have the disease in one positive evaluation study. [7]
In conclusion such cardiopathies as ARVD may really not be as rare as previously thought in our environment. The need for equipped electrophysiology laboratories to appropriately diagnose cases of palpitation and facilities for interventional cardiology become compelling. Intra-cardiac defibrillators are required when medical treatment becomes unsatisfactory in the background of recurrent symptoms.
| Acknowledgment | | |
I thank Prof. M A Araoye for his opinion on the illustrative electrocardiograms.
| References | | |
| 1. | Hendricks N, Watkins DA, Mayosi BM. Lessons from the first report of the arrhythmogenic right ventricular cardiomyopathy registry of South Africa. Cardiovasc J Afr 2011;21:129-30.  |
| 2. | Bode-Thomas F, John C. Arthymogenic right ventricular dysplasia in a 13 year old Nigeria boy. Jos J Med 2006;1:8-9. |
| 3. | Hendricks N, Mayosi BM, Okreglicki A. The south african arrhythmogenic right ventricular cardiomyopathy registry: A brief review and status report. SA Heart 2008;5:148-54. |
| 4. | Yerra L, Caskey D, Modi K, Reddy P. Arrhythmogenic right ventricular dysplasia/cardiomyopathy: Clinical profile of four patients and review. South Med J 2008;101:309-16. |
| 5. | Sen-Chowdry S, Lowe MD, Sparton SC, Mckenna WJ. Arrhythmogenic right ventricular cardiomyopathy: Clinical presentation, diagnosis and management. Am J Med 2004;117:685-95. |
| 6. | Nasir K, Bomma C, Tandri H, Roguin A, Dalal D, Prakesa K, et al. Electrocardiographic features of Arrhythmogenic Right Ventricular Dysplasia/Cardiomyopathy according to Disease Severity. A need to broaden diagnostic criteria. Circulation 2004;110:1527-34. |
| 7. | Hamid MS, Norman M, Quraishi A, Firoozi S, Thaman R, Gimeno JR, et al. Prospective evaluation of relatives for familial arrythmogenic right ventricular cardiomyopathy/dysplasia reveals a need to broaden diagnostic criteria. J Am Coll Cardiol 2002;40:1445-50. |
[Figure 1], [Figure 2]
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