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Letters to the Editor Mitral valve repair is an effective treatment for ventricular arrhythmias in mitral valve prolapse syndrome Deepika R. Abbadi a, , Rahul Purbey d , Indu G. Poornima b,c a Internal Medicine, Allegheny General Hospital, 320 E North Avenue, Pittsburgh, PA 15212, United States b Nuclear Cardiology, Allegheny General Hospital, United States c Cardiology Fellowship, Allegheny General Hospital, United States d Allegheny General Hospital, United States article info Article history: Received 7 June 2014 Accepted 27 July 2014 Available online xxxx Keywords: Mitral valve prolapse Mitral valve prolapse syndrome Ventricular arrhythmias Sudden cardiac death Mitral regurgitation The prognosis of ventricular arrhythmias associated with mitral valve prolapse (MVP) syndrome is unclear. We present a case of MVP that emphasizes the role of mitral valve repair in the treatment of ven- tricular arrhythmias resulting from this condition. The case also illus- trates the need for high vigilance in those with malignant features of MVP, to prevent sudden cardiac death. A 36-year-old Caucasian female with history of a benign murmur since adolescence, complained of sudden onset palpitations and dizzi- ness in her 4th postpartum month and suffered a cardiac arrest. Her pregnancy and delivery were uneventful except for mild pregnancy in- duced hypertension that resolved after delivery. Since delivery, she had three episodes of near syncope and an echocardiogram showed bileaet MVP with moderate mitral regurgitation and normal left ventricular systolic function. A 24-hour Holter monitor performed 2 weeks before presentation demonstrated frequent PVC's and she was started on met- oprolol 25 mg twice daily. After successful resuscitation from ventricu- lar brillation, physical examination revealed a holosystolic murmur in the apical area without evidence of congestive heart failure. EKG showed sinus tachycardia with frequent premature ventricular contrac- tions (PVC) in bigeminy and short runs of nonsustained ventricular tachycardia (NSVT). QTc and cardiac enzymes were normal. Intrave- nous amiodarone was initiated but due to persistent, recurrent runs of NSVT, lidocaine was added with decrease in frequency of arrhythmias. Transesophageal echocardiogram showed bi-leaet MVP, moderate to severe mitral regurgitation (See Fig. 2.), dilated mitral annulus, thick re- dundant mitral leaets, torn secondary chordae tendineae to the anteri- or mitral leaet and normal left ventricular end-diastolic and end- systolic diameter with left ventricular ejection fraction of 5560%. Coro- nary angiography to exclude spontaneous coronary dissection showed normal coronaries. Cardiac MRI did not reveal arrhythmogenic right ventricular dysplasia or hypertrophic cardiomyopathy but revealed some patchy brosis in the anteroseptal and posterior walls. A debril- lator was implanted for recurrent runs of VT. She was discharged home on aspirin and metoprolol. Persistent symptoms continued with palpitations, near syncope and fatigue. Interrogation of her debrillator showed multiple PVC's with runs of NSVT unresponsive to higher doses of metoprolol. The frequency of PVC's decreased after initiation of Dilantin 400 mg three times daily. This decreased her ectopic burden but she continued to be symptomatic with palpitations. Although she did not have any symptoms of volume overload and left ventricular dimensions did not meet surgical criteria for mitral valve repair, she was referred for mitral valve repair due to the persistent arrhythmias and symptoms. She underwent mitral valve repair with a #36 Edwards Physio Annuloplasty ring with no residual mi- tral regurgitation on the postoperative echo. No episodes of NSVT were noted in the postoperative period. After discharge, Dilantin has been ta- pered off and discontinued and the dose of metoprolol has been progres- sively decreased. Three years postoperatively, no ICD shocks have been reported and the patient does not report any palpitations. Mitral valve prolapse usually has a benign course, but can occasion- ally lead to serious complications [1]. Some of them are due to autonom- ic dysfunction causing malignant arrhythmias, syncope, pre-syncope, orthostatic hypotension and sudden cardiac death [2]. Bi-leaet MVP, female gender, biphasic or inverted T waves in the inferior leads, and frequent complex ventricular ectopic activity including PVC's of the out- ow tract alternating with PVCs of papillary or fascicular origin, as seen in our patient (See Fig. 1.), were collectively described as a malignantphenotype of MVP by Sriram et al. [3]. However this retrospective series did not examine the role of mitral valve surgery on the incidence of ven- tricular arrhythmias in survivors of cardiac arrest. The estimated rate of SCD in patients with MVP is 1640 per 10,000 (0.20.4%) per year and twice the incidence in the general population [5]. Univariable predictors of ventricular arrhythmias include International Journal of Cardiology xxx (2014) xxxxxx Corresponding author. Tel.: +1 4129994816 (Cell), +1 4123594970 (Ofce). E-mail address: [email protected] (D.R. Abbadi). IJCA-18493; No of Pages 3 http://dx.doi.org/10.1016/j.ijcard.2014.07.174 0167-5273/Published by Elsevier Ireland Ltd. Contents lists available at ScienceDirect International Journal of Cardiology journal homepage: www.elsevier.com/locate/ijcard Please cite this article as: Abbadi DR, et al, Mitral valve repair is an effective treatment for ventricular arrhythmias in mitral valve prolapse syndrome, Int J Cardiol (2014), http://dx.doi.org/10.1016/j.ijcard.2014.07.174

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Page 1: Mitral valve repair is an effective treatment for ventricular arrhythmias in mitral valve prolapse syndrome

International Journal of Cardiology xxx (2014) xxx–xxx

IJCA-18493; No of Pages 3

Contents lists available at ScienceDirect

International Journal of Cardiology

j ourna l homepage: www.e lsev ie r .com/ locate / i j ca rd

Letters to the Editor

Mitral valve repair is an effective treatment for ventricular arrhythmias inmitral valve prolapse syndrome

Deepika R. Abbadi a,⁎, Rahul Purbey d, Indu G. Poornima b,c

a Internal Medicine, Allegheny General Hospital, 320 E North Avenue, Pittsburgh, PA 15212, United Statesb Nuclear Cardiology, Allegheny General Hospital, United Statesc Cardiology Fellowship, Allegheny General Hospital, United Statesd Allegheny General Hospital, United States

⁎ Corresponding author. Tel.: +1 4129994816 (Cell), +E-mail address: [email protected] (D.R. Abbadi).

http://dx.doi.org/10.1016/j.ijcard.2014.07.1740167-5273/Published by Elsevier Ireland Ltd.

Please cite this article as: Abbadi DR, et al,syndrome, Int J Cardiol (2014), http://dx.doi

a r t i c l e i n f o

Article history:

Received 7 June 2014Accepted 27 July 2014Available online xxxx

Keywords:Mitral valve prolapseMitral valve prolapse syndromeVentricular arrhythmiasSudden cardiac deathMitral regurgitation

Transesophageal echocardiogram showed bi-leaflet MVP, moderate toseveremitral regurgitation (See Fig. 2.), dilatedmitral annulus, thick re-dundantmitral leaflets, torn secondary chordae tendineae to the anteri-or mitral leaflet and normal left ventricular end-diastolic and end-systolic diameter with left ventricular ejection fraction of 55–60%. Coro-nary angiography to exclude spontaneous coronary dissection showednormal coronaries. Cardiac MRI did not reveal arrhythmogenic rightventricular dysplasia or hypertrophic cardiomyopathy but revealedsome patchy fibrosis in the anteroseptal and posterior walls. A defibril-lator was implanted for recurrent runs of VT. She was discharged homeon aspirin and metoprolol.

The prognosis of ventricular arrhythmias associated with mitralvalve prolapse (MVP) syndrome is unclear. We present a case of MVPthat emphasizes the role of mitral valve repair in the treatment of ven-tricular arrhythmias resulting from this condition. The case also illus-trates the need for high vigilance in those with malignant features ofMVP, to prevent sudden cardiac death.

A 36-year-old Caucasian female with history of a benign murmursince adolescence, complained of sudden onset palpitations and dizzi-ness in her 4th postpartum month and suffered a cardiac arrest. Herpregnancy and delivery were uneventful except for mild pregnancy in-duced hypertension that resolved after delivery. Since delivery, she hadthree episodes of near syncope and an echocardiogram showed bileafletMVP with moderate mitral regurgitation and normal left ventricularsystolic function. A 24-hour Holter monitor performed 2 weeks beforepresentation demonstrated frequent PVC's and she was started onmet-oprolol 25 mg twice daily. After successful resuscitation from ventricu-lar fibrillation, physical examination revealed a holosystolic murmur inthe apical area without evidence of congestive heart failure. EKGshowed sinus tachycardia with frequent premature ventricular contrac-tions (PVC) in bigeminy and short runs of nonsustained ventriculartachycardia (NSVT). QTc and cardiac enzymes were normal. Intrave-nous amiodarone was initiated but due to persistent, recurrent runs of

1 4123594970 (Office).

Mitral valve repair is an effe.org/10.1016/j.ijcard.2014.07.

NSVT, lidocaine was added with decrease in frequency of arrhythmias.

Persistent symptoms continued with palpitations, near syncope andfatigue. Interrogation of her defibrillator showed multiple PVC's withruns of NSVT unresponsive to higher doses of metoprolol. The frequencyof PVC's decreased after initiation of Dilantin 400 mg three times daily.This decreased her ectopic burden but she continued to be symptomaticwith palpitations. Although she did not have any symptoms of volumeoverload and left ventricular dimensions did not meet surgical criteriafor mitral valve repair, she was referred for mitral valve repair due tothe persistent arrhythmias and symptoms. She underwent mitral valverepair with a #36 Edwards Physio Annuloplasty ringwith no residual mi-tral regurgitation on the postoperative echo. No episodes of NSVT werenoted in the postoperative period. After discharge, Dilantin has been ta-pered off and discontinued and the dose of metoprolol has been progres-sively decreased. Three years postoperatively, no ICD shocks have beenreported and the patient does not report any palpitations.

Mitral valve prolapse usually has a benign course, but can occasion-ally lead to serious complications [1]. Someof themare due to autonom-ic dysfunction causing malignant arrhythmias, syncope, pre-syncope,orthostatic hypotension and sudden cardiac death [2]. Bi-leaflet MVP,female gender, biphasic or inverted T waves in the inferior leads, andfrequent complex ventricular ectopic activity including PVC's of the out-flow tract alternating with PVCs of papillary or fascicular origin, as seenin our patient (See Fig. 1.), were collectively described as a ‘malignant’phenotype ofMVP by Sriram et al. [3]. However this retrospective seriesdid not examine the role ofmitral valve surgery on the incidence of ven-tricular arrhythmias in survivors of cardiac arrest.

The estimated rate of SCD in patients with MVP is 16–40 per 10,000(0.2–0.4%) per year and twice the incidence in the general population[5]. Univariable predictors of ventricular arrhythmias include

ctive treatment for ventricular arrhythmias in mitral valve prolapse174

Page 2: Mitral valve repair is an effective treatment for ventricular arrhythmias in mitral valve prolapse syndrome

Fig. 1. EKG rhythm strips showing multiple PVC's and NSVT.

e2 D.R. Abbadi et al. / International Journal of Cardiology xxx (2014) xxx–xxx

echocardiographically-determined isovolumetric relaxation time, LVdi-latation and dysfunction, mitral anterior leaflet length [4], thickness ofthe redundant leaflet [1,5], chordal rupture causing flail mitral leafletand the presence of moderate to severe mitral regurgitation [1,6].

Fig. 2. ECHO with color doppler show

Please cite this article as: Abbadi DR, et al, Mitral valve repair is an effesyndrome, Int J Cardiol (2014), http://dx.doi.org/10.1016/j.ijcard.2014.07.

Ackay et al. identified anteriormitral leaflet length alongwith enhancedQTc dispersion as independent predictors of VT in patients with classicMVP of N5 mm, as observed in our patient [1,6]. Gornick et al. showedthat papillary muscle traction in a canine heart model could lead to

ing severe mitral regurgitation.

ctive treatment for ventricular arrhythmias in mitral valve prolapse174

Page 3: Mitral valve repair is an effective treatment for ventricular arrhythmias in mitral valve prolapse syndrome

e3D.R. Abbadi et al. / International Journal of Cardiology xxx (2014) xxx–xxx

significant regional changes in ventricular refractoriness [7] and a simi-lar mechanism could be operational in humans. Delayed contrast en-hancement of the papillary muscles on cardiac MRI could representfibrosis of papillary muscles and a nidus for arrhythmias [8]. Guidelinesfor surgical repair ofmitral valve prolapse typically include symptoms ofvolume overload and left ventricular dilatation in the presence of severemitral regurgitation and MVP [9,10]. More recent studies advocate forearly surgical intervention especially when repair is feasible, even inasymptomatic patients [9,10]. However ventricular arrhythmias aretypically not a consideration for surgical intervention as there is not aclear association between the severity of mitral regurgitation and ar-rhythmias. In our patient with a malignant phenotype of MVP, surgicalintervention clearly reduced the incidence of ventricular arrhythmias.Future studies need to evaluate the value of early surgical interventionin patients with a similar phenotype.

Conflict of interest

The authors report no relationships that could be construed as a con-flict of interest.

Please cite this article as: Abbadi DR, et al, Mitral valve repair is an effesyndrome, Int J Cardiol (2014), http://dx.doi.org/10.1016/j.ijcard.2014.07.

References

[1] Hayek E, Gring CN, Griffin BP. Mitral valve prolapse. Lancet 2005;365(9458):507–18.

[2] Hu X, Zhao Q. Autonomic dysregulation as a novel underlying cause of mitral valveprolapse: a hypothesis. Med Sci Monit 2011;17(9):Hy27–31.

[3] Sriram CS, Syed FF, FergusonME, et al. Malignant bileaflet mitral valve prolapse syn-drome in patients with otherwise idiopathic out-of-hospital cardiac arrest. J Am CollCardiol 2013;62(3):222–30.

[4] AkcayM, YuceM, Pala S, et al. Anterior mitral valve length is associatedwith ventric-ular tachycardia in patients with classical mitral valve prolapse. Pacing ClinElectrophysiol 2010;33(10):1224–30.

[5] Nishimura RA, McGoon MD, Shub C, Miller Jr FA, Ilstrup DM, Tajik AJ.Echocardiographically documented mitral-valve prolapse. Long-term follow-up of237 patients. N Engl J Med 1985;313(21):1305–9.

[6] Marks AR, Choong CY, Sanfilippo AJ, Ferre M, Weyman AE. Identification of high-riskand low-risk subgroups of patients with mitral-valve prolapse. N Engl J Med 1989;320(16):1031–6.

[7] Gornick CC, Tobler HG, Pritzker MC, Tuna IC, Almquist A, Benditt DG. Electrophysio-logic effects of papillary muscle traction in the intact heart. Circulation 1986;73(5):1013–21.

[8] Van der Wall EE, Schalij MJ. Mitral valve prolapse: a source of arrhythmias? Int JCardiovasc Imaging 2010;26(2):147–9.

[9] Enriquez-Sarano M. Timing of mitral valve surgery. Heart 2002;87(1):79–85.[10] Enriquez-Sarano M, Sundt III TM. Early surgery is recommended for mitral regurgi-

tation. Circulation 2010;121(6):804–11 [discussion 12].

ctive treatment for ventricular arrhythmias in mitral valve prolapse174