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ARTICLE CONTRIBUTORS

David P Ripley

Syed A Ehsanullah

Simon A James

Andrew J Turley

Clinical Articles, Lead Article

Case report: ventricular tachycardia in mitral valve prolapse

Middlesbrough workers review the literature on ventricular tachycardia in mitral valve prolapse and describe a case of a 61-year old lady with recurrent VT associated with prolapse, who received successful medical and device treatment

Introduction

Mitral valve prolapse (MVP) is one of the most common causes of primary mitral regurgitation (MR) with an estimated prevalence in the western world of 4-5%.1,2 Many patients with this disorder present with symptomatic atrial or ventricular arrhythmias.3-7 Some studies report a prevalence of ventricular arrhythmias in MVP of over 30%.8 Patients with MR and MVP have more ventricular arrhythmias than those with MVP alone.9 Similarly, whilst other structural cardiac abnormalities are well recognised as a cause of ventricular tachycardia (VT), in 8-16% of patients with refractory VT the only cardiac abnormality found is MVP.3-5

Sudden cardiac death (SCD) is a major public health concern, accounting for 400,000 deaths per annum in the western world.10,11 An association has been observed between MVP and SCD.12-19 MVP is estimated to be the cause of 2% of SCD in competitive athletes12 and MVP is also a common observation in both autopsy studies and survivors of SCD.13-19 Whilst the incidence of SCD in MVP is not well established the literature suggests around 2 per 10,000  patients per year with the risk 50 to 100 fold higher if there is significant concomitant mitral regurgitation.3 The relationship between the MVP and SCD is thought to be due to ventricular arrhythmias.13

Case report

A 61-year old lady presented with syncope preceded by light-headedness and feeling hot. The event, which was witnessed by her husband, occurred after eating in a restaurant. She regained consciousness spontaneously and later described palpitations associated with chest discomfort for a few minutes afterwards. She described similar episodes for the last 6 months.  She had a past medical history of vitamin B12 deficiency, osteoarthritis and was under long term follow up for asymptomatic bileaflet mitral valve prolapse with moderate mitral regurgitation.  She was taking cod liver oil, glucosamine/chrondatin sulphate and vitamin B12 and had no known allergies. There was no family history of sudden cardiac death. She was a life long non-smoker and drank 12 units of alcohol per week.

On examination she was alert, and haemodynamically stable.  The only positive finding was an apical pan-systolic murmur. Her biochemical and haematological tests were normal. 12-lead ECG confirmed sinus rhythm with a ventricular rate of 72 bpm, normal PR interval and corrected QTc, normal cardiac axis, no ST/T-wave changes (figure 1a).

Figure 1a. 12 lead electrocardiogram - normal sinus rhythm.

Figure 1a. 12 lead electrocardiogram - normal sinus rhythm.

After 24hrs of observation and cardiac monitoring she was discharged home and ambulatory, however, electrocardiogram was fitted.  She had a recurrence of her symptoms with further syncope.  Her ambulatory electrocardiogram showed monomorphic ventricular tachycardia. She was admitted to the local coronary care unit where further VT was recorded (figure 1b).

Figure 1b.  12 lead electrocardiogram - monomorphic ventricular tachycardia

Figure 1b. 12 lead electrocardiogram - monomorphic ventricular tachycardia

She was commenced on a long acting β-blocker and transferred to the regional cardiothoracic centre for further evaluation and treatment. Left and right heart catheterisation demonstrated normal epicardial coronary arteries with normal right sided hameodynamic measurements. Transoesophageal echocardiography confirmed moderate mitral regurgitation along the length of the coaptation line due to excess mitral valve tissue causing prolapse of both the posterior leaflet (P1 and P2) as well as the anterior leaflet (A1 and A2) (figure 2). There was no flow reversal in the pulmonary veins. Left ventricular size and function was normal as were the other valves.

Figure 2.  Transoesophageal echocardiography showing bilateral mitral valve leaflet prolapse with moderate mitral regurgitation

Figure 2. Transoesophageal echocardiography showing bilateral mitral valve leaflet prolapse with moderate mitral regurgitation

Electrophysiology study and programmed VT stimulation was performed which demonstrated a haemodynamically unstable sustained monomorphic ventricular tachycardia, originating from the aorto-mitral continuity (figures 3a and 3b) which required prompt defibrillation.  The dose of bisoprolol was uptitrated to a maximum tolerated dose and she received an implantable cardiac defibrillator (ICD).

Figure 3a. Programmed VT stimulation inducing ventricular tachycardia

Figure 3a. Programmed VT stimulation inducing ventricular tachycardia

Figure 3b. Monomorphic VT with a cycle length of 217ms originating from the aorto mitral continuity

Figure 3b. Monomorphic VT with a cycle length of 217ms originating from the aorto mitral continuity

She was discharged home and on subsequent follow up remains well with no further palpitations and no ventricular arrhythmias on device interrogation.

Discussion

Ventricular arrhythmias associated with MVP have been reported in a number of patients with syncope and SCD. The underlying mechanism causing the ventricular arrhythmias in those with MVP is not fully understood.

One study of 58 consecutive patients with MVP found 34% had ventricular arrhythmias (defined as ventricular premature contractions (VPCs), VPC couplets and ventricular tachycardia)8. Multivariable logistic regression analysis has shown that the presence of moderate to severe mitral regurgitation was the only independent predictor of ventricular arrhythmias (RR 8.4, 95% CI:1.49-47.64, p=0.01).8 However, complex ventricular arrhythmias have been reported in those with mitral regurgitation of any cause9 with the severity of the mitral regurgitation again a predictor of arrhythmic events.20

There is undoubtedly a relationship between MVP and SCD although inconclusive evidence regarding a causative role. The incidence of SCD in MVP, however, is around 2 per 10,000  patients per year with the risk 50 to 100 fold higher if there is significant concomitant mitral regurgitation.3 Once again raising the question of the significance of mitral regurgitation in association with MVP.

Furthermore Nishimura et al21 identified a higher incidence of SCD in those patients with MVP and redundant MV leaflets defined as a thickness on M-mode as 5mm or more. This was supported by Zouridikas et al who showed that patients with thickened anterior mitral valve leaflets was associated with complex ventricular arrhythmias.22 Further independent predictors of SCD in MVP with a flail leaflet include NYHA class III/IV, LVEF < 50% and presence of atrial fibrillation.23 In our case the patient has normal thickness mitral valve leaflets and non-of the mentioned risk factors for SCD.

European Society of Cardiology guidelines for the management of patients with ventricular arrhythmias and prevention of sudden cardiac death recommend electrophysiology studies (EPS) for the induction of VT as a Class I indication in those presenting with syncope and structural heart disease (level of evidence B).24 The test is used to document the inducibility of VT. It is recognised, however, that induction of VT/VF with aggressive strategies is not specific.21 In our case the VT was not only easily induced but was haemodynamically unstable and required prompt defibrillation.  Our patient was managed with β-blockade and an ICD device.  An ICD is regarded as a class I indication, level of evidence B, in patients with documented VT and structural heart disease.24

Mitral valve repair or replacement is recommended in those with in severe symptomatic mitral regurgitation and EF > 30%.25 Surgical correction is also recommended in some groups of asymptomatic patients (those with dilated LV, LV systolic dysfunction, atrial fibrillation or pulmonary artery systolic pressure of >50mmHg).25. There is, however, currently no evidence for a surgical approach in those with preserved LV function and recurrent ventricular arrhythmias25 as in our patient.

Conflicts of Interest

None declared.

References

1. Markiewicz W, Stoner J, London E, Hunt SA, Popp RL. Mitral valve prolapse in one hundred presumably healthy young females. Circulation 1976;53:464.

2. Sbarbaro JA, Mehlman DJ, Wu L, Brooks HL. A prospective study of mitral valvular prolapse in young men. Chest 1979;75:555.

3. Kligfield P, Levy D, Devereux RB, Savage DD. Arrhythmias and sudden death in mitral valve prolapse. Am Heart J 1987;113:1298.

4. Schaal SF. Ventricular arrhythmias in patients with mitral valve prolapse. Cardiovasc Clin 1992;22:307.

5. Kligfield P, Devereux RB. Is the mitral valve prolapse patient at high risk of sudden death identifiable? Cardiovasc Clin 1990;21:143.

6. Babuty D, Cosnay P, Breuillac JC, Charniot JC, Delhomme C, Fauchier L, Fauchier JP. Ventricular arrhythmia factors in mitral valve prolapse. Pacing Clin Electrophysiol 1994;17:1090.

7. Zuppiroli A, Mori F, Favilli S, Barchielli A, Corti G, Montereggi A, Dolara A. Arrhythmias in mitral valve prolapse: relation to anterior mitral leaflet thickening, clinical variables, and color Doppler echocardiographic parameters. Am Heart J 1994;128:919.

8.Turker Y, Ozaydin M, Acar G, et al. Predictors of ventricular arrhythmias in patients with mitral valve prolapse. Int J Cardiovasc Imaging. 2010:26: 139–45

9. Kligfield P, Hochreiter C, Kramer H, Devereux RB, Niles N, Kramer-Fox R, Borer JS. Complex arrhythmias in mitral regurgitation with and without mitral valve prolapse: contrast to arrhythmias in mitral valve prolapse without mitral regurgitation. Am J Cardiol 1985;55:1545.

10. de Vreede-Swagemakers JJ, Gorgels AP, Dubois-Arbouw WI, van Ree JW, Daemen MJ, Houben LG, Wellens HJ. Out-of-hospital cardiac arrest in the 1990’s: a population-based study in the Maastricht area on incidence, characteristics and survival. J Am Coll Cardiol 1997;30:1500–5.

11. Zheng Z, Croft JB, Giles WH, Mensah GA. Sudden Cardiac Death in the United States, 1989 to 1998. Circulation. 2001;104:2158.

12. Maron BJ, Epstein SE, Roberts WC. Causes of sudden death in competitive athletes. J Am Coll Cardiol. 1989; 7: 204–14.

13. Boudoulas H, Schaal SF, Stang JM, Fontana ME, Kolibash AJ, Wooley CF. Mitral valve prolapse: cardiac arrest with long-term survival. Int J Cardiol 1990;26:37.

14. Vohra J, Sathe S, Warren R, Tatoulis J, Hunt D. Malignant ventricular arrhythmias in patients with mitral valve prolapse and mild mitral regurgitation. Pacing Clin Electrophysiol 1993;16:387.

15. Davies MJ, Moore BP, Braimbridge MV. The floppy mitral valve. Study of incidence, pathology, and complications in surgical, necropsy, and forensic material. Br Heart J 1978;40:468.

16. Chesler E, King RA, Edwards JE. The myxomatous mitral valve and sudden death. Circulation 1983;67:632.

17. Nishimura RA, McGoon MD, Shub C, Miller FA Jr, Ilstrup DM, Tajik AJ. Echocardiographically documented mitral-valve prolapse. Long-term follow-up of 237 patients. N Engl J Med 1985;313:1305.

18. Düren DR, Becker AE, Dunning AJ. Long-term follow-up of idiopathic mitral valve prolapse in 300 patients: a prospective study. J Am Coll Cardiol 1988; 11:42.

19. Anders S, Said S, Schulz F, Püschel K. Mitral valve prolapse syndrome as cause of sudden death in young adults. Forensic Sci Int 2007;171:127.

20. Martínez-Rubio A, Schwammenthal Y, Schwammenthal E, et al. Patients with valvular heart disease presenting with sustained ventricular tachycardia or syncope: results of programmed ventricular stimulation and long-term follow-up. Circulation. 1997;96: 500–8.

21. Nishimura RA, McGoon MD, Shub C, Miller FA Jr, Ilstrup DM, Tajik AJ. Echocardiographically documented mitral-valve prolapse. Long term follow-up of 237 patients, NEJM. 1985;313:1305–9.

22. Zouridakis EG, Parthenakis FI, Kochiadakis GE, Kanoupakis EM, Vardas PE. QT dispersion in patients with mitral valve prolapse is related to the echocardiographic degree of the prolapse and mitral leaflet thickness. Europace. 2001;3:292–8.

23. Grigioni F, Enriquez-Sarano M, Ling LH, Bailey KR, Seward JB, Tajik AJ, Frye RL. Sudden death in mitral regurgitation due to flail leaflet. JACC. 1999; 34:2078.

24. Zipes DP, Camm AJ, Borggrefe M, et al. ACC/AHA/ESC 2006 guidelines for management of patients with ventricular arrhythmias and the prevention of sudden cardiac death. Europace. 2006;8:746–837.

25. Vahanian A, Baumgartner H, Bax J, et al; Task Force on the Management of Valvular Hearth Disease of the European Society of Cardiology; ESC Committee for Practice Guidelines. Guidelines on the management of valvular heart disease. European Heart Journal 2007;28:230–68.

Authors

Dr David P Ripley
Cardiology Specialist Trainee
(david.ripley@nhs.net
)

Syed A Ehsanullah
Foundation Year Doctor

Simon A James
Consultant Cardiologist

Andrew J Turley
Consultant Cardiologist

James Cook University Hospital, Marton Road, Middlesbrough, TS4 3BW

Citation

Ripley DP, Ehsanullah SA, James SA, Turley AJ.  Ventricular tachycardia in mitral valve prolapse: a review of the literature and case illustration.  BJC Arrhythmia Watch 2012;Issue 22 (Mar)

Published on: March 9, 2012

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