Advertisement
Heart, Lung and Circulation

Surgical Cryoablation of Malignant Papillary Muscle Arrhythmias During Mitral Valve Prolapse Surgery – Putting a Freeze on Sudden Cardiac Death

      Mitral valve prolapse (MVP) is a common finding with a reported prevalence of approximately 2.4% in patients from a general population in the Framingham Heart Study [
      • Freed L.A.
      • Levy D.
      • Levine R.A.
      • Larson M.G.
      • Evans J.C.
      • Fuller D.L.
      • et al.
      Prevalence and clinical outcome of mitral-valve prolapse.
      ]. The underlying pathogenesis is myxomatous degeneration of the mitral valve that may lead to varying degrees of mitral regurgitation (MR) [
      • Guy T.S.
      • Hill A.C.
      Mitral valve prolapse.
      ,
      • Enriquez-Sarano M.
      • Akins C.W.
      • Vahanian A.
      Mitral regurgitation.
      ]. Many patients with MVP remain asymptomatic to their condition. Some, however, develop complications that may include symptomatic MR, infective endocarditis, heart failure, atrial and complex ventricular arrhythmias—the latter which may lead to sudden cardiac death (SCD) [
      • Basso C.
      • Perazzolo Marra M.
      • Rizzo S.
      • De Lazzari M.
      • Giorgi B.
      • Cipriani A.
      • et al.
      Arrhythmic mitral valve prolapse and sudden cardiac death.
      ]. In MVP patients, morphological changes to valve apparatus have been postulated to cause displacement of the attached papillary muscle (PM), affecting both its mechanical and electrophysiological function. One thought is that this may result in PM fibrosis, in combination with the extensive Purkinje network at the PM, establishes the substrate and triggers for malignant ventricular arrhythmias (VA), as seen on cardiac magnetic resonance imaging (CMR) and histopathological studies [
      • Basso C.
      • Iliceto S.
      • Thiene G.
      • Marra M.P.
      Mitral valve prolapse, ventricular arrhythmias, and sudden death.
      ,
      • Kitkungvan D.
      • Nabi F.
      • Kim R.J.
      • Bonow R.O.
      • Khan M.A.
      • Xu J.
      • et al.
      Myocardial fibrosis in patients with primary mitral regurgitation with and without prolapse.
      ,
      • Han H.-C.
      • Parsons S.A.
      • Curl C.L.
      • Teh A.W.
      • Raaijmakers A.J.A.
      • Koshy A.N.
      • et al.
      Systematic quantification of histologic ventricular fibrosis in isolated mitral valve prolapse and sudden cardiac death.
      ,
      • Bennett S.
      • Thamman R.
      • Griffiths T.
      • Oxley C.
      • Khan J.N.
      • Phan T.
      • et al.
      Mitral annular disjunction: o systematic review of the literature.
      ]. The “Padua” hypothesis indeed postulates mitral annular disjunction (MAD) contributes to regional left ventricle (LV) myocardial stretch/friction at the inferobasal LV and the PM, leading to hypertrophy/fibrosis of the inferobasal LV, involving the Purkinje fibres and PM, which leads to electrical instability and VA, namely Purkinje-triggered ventricular fibrillation (VF), and/or sustained monomorphic ventricular tachycardia (VT) from the inferobasal LV [
      • Basso C.
      • Iliceto S.
      • Thiene G.
      • Marra M.P.
      Mitral valve prolapse, ventricular arrhythmias, and sudden death.
      ]. Whilst initial associations between MVP and SCD were hypothesised nearly half a century ago, difficulties still remain in predicting precisely which patients are predisposed to SCD [
      • Shappell S.D.
      • Marshall C.E.
      • Brown R.E.
      • Bruce T.A.
      Sudden death and the familial occurrence of mid-systolic click, late systolic murmur syndrome.
      ]. In SCD victims of indeterminate aetiology, MVP was prevalent in up to 11.7% of patients [
      • Nalliah C.J.
      • Mahajan R.
      • Elliott A.D.
      • Haqqani H.
      • Lau D.H.
      • Vohra J.K.
      • et al.
      Mitral valve prolapse and sudden cardiac death: a systematic review and meta-analysis.
      ].

      Keywords

      To read this article in full you will need to make a payment

      Purchase one-time access:

      Academic & Personal: 24 hour online accessCorporate R&D Professionals: 24 hour online access
      One-time access price info
      • For academic or personal research use, select 'Academic and Personal'
      • For corporate R&D use, select 'Corporate R&D Professionals'

      Subscribe:

      Subscribe to Heart, Lung and Circulation
      Already a print subscriber? Claim online access
      Already an online subscriber? Sign in
      Institutional Access: Sign in to ScienceDirect

      References

        • Freed L.A.
        • Levy D.
        • Levine R.A.
        • Larson M.G.
        • Evans J.C.
        • Fuller D.L.
        • et al.
        Prevalence and clinical outcome of mitral-valve prolapse.
        N Engl J Med. 1999; 341: 1-7
        • Guy T.S.
        • Hill A.C.
        Mitral valve prolapse.
        Annu Rev Med. 2012; 63: 277-292
        • Enriquez-Sarano M.
        • Akins C.W.
        • Vahanian A.
        Mitral regurgitation.
        Lancet. 2009; 373: 1382-1394
        • Basso C.
        • Perazzolo Marra M.
        • Rizzo S.
        • De Lazzari M.
        • Giorgi B.
        • Cipriani A.
        • et al.
        Arrhythmic mitral valve prolapse and sudden cardiac death.
        Circulation. 2015; 132: 556-566
        • Basso C.
        • Iliceto S.
        • Thiene G.
        • Marra M.P.
        Mitral valve prolapse, ventricular arrhythmias, and sudden death.
        Circulation. 2019; 140: 952-964
        • Kitkungvan D.
        • Nabi F.
        • Kim R.J.
        • Bonow R.O.
        • Khan M.A.
        • Xu J.
        • et al.
        Myocardial fibrosis in patients with primary mitral regurgitation with and without prolapse.
        J Am Coll Cardiol. 2018; 72: 823-834
        • Han H.-C.
        • Parsons S.A.
        • Curl C.L.
        • Teh A.W.
        • Raaijmakers A.J.A.
        • Koshy A.N.
        • et al.
        Systematic quantification of histologic ventricular fibrosis in isolated mitral valve prolapse and sudden cardiac death.
        Heart Rhythm. 2021; 18: 570-576
        • Bennett S.
        • Thamman R.
        • Griffiths T.
        • Oxley C.
        • Khan J.N.
        • Phan T.
        • et al.
        Mitral annular disjunction: o systematic review of the literature.
        Echocardiography. 2019; 36: 1549-1558
        • Shappell S.D.
        • Marshall C.E.
        • Brown R.E.
        • Bruce T.A.
        Sudden death and the familial occurrence of mid-systolic click, late systolic murmur syndrome.
        Circulation. 1973; 48: 1128-1134
        • Nalliah C.J.
        • Mahajan R.
        • Elliott A.D.
        • Haqqani H.
        • Lau D.H.
        • Vohra J.K.
        • et al.
        Mitral valve prolapse and sudden cardiac death: a systematic review and meta-analysis.
        Heart. 2019; 105: 144-151
        • Cronin E.M.
        • Bogun F.M.
        • Maury P.
        • Peichl P.
        • Chen M.
        • Namboodiri N.
        • et al.
        2019 HRS/EHRA/APHRS/LAHRS expert consensus statement on catheter ablation of ventricular arrhythmias.
        Heart Rhythm. 2020; 17: e2-e154
        • Enriquez A.
        • Shirai Y.
        • Huang J.
        • Liang J.
        • Briceño D.
        • Hayashi T.
        • et al.
        Papillary muscle ventricular arrhythmias in patients with arrhythmic mitral valve prolapse: Electrophysiologic substrate and catheter ablation outcomes.
        J Cardiovasc Electrophysiol. 2019; 30: 827-835
        • Muthukumar L.
        • Jahangir A.
        • Jan M.F.
        • Perez Moreno A.C.
        • Khandheria B.K.
        • Tajik A.J.
        Association between malignant mitral valve prolapse and sudden cardiac death: a review.
        JAMA Cardiol. 2020; 5: 1053-1061
        • Nordhues B.D.
        • Siontis K.C.
        • Scott C.G.
        • Nkomo V.T.
        • Ackerman M.J.
        • Asirvatham S.J.
        • et al.
        Bileaflet mitral valve prolapse and risk of ventricular dysrhythmias and death.
        J Cardiovasc Electrophysiol. 2016; 27: 463-468
        • Naksuk N.
        • Syed F.F.
        • Krittanawong C.
        • Anderson M.J.
        • Ebrille E.
        • DeSimone C.V.
        • et al.
        The effect of mitral valve surgery on ventricular arrhythmia in patients with bileaflet mitral valve prolapse.
        Indian Pacing Electrophysiol J. 2016; 16: 187-191
        • Schron B.E.
        • Exner V.D.
        • Yao S.Q.
        • Jenkins S.L.
        • Steinberg R.J.
        • Cook P.J.
        • et al.
        Quality of life in the antiarrhythmics versus implantable defibrillators trial: impact of therapy and influence of adverse symptoms and defibrillator shocks.
        Circulation. 2002; 105: 589-594
        • Van Herendael H.
        • Dorian P.
        Amiodarone for the treatment and prevention of ventricular fibrillation and ventricular tachycardia.
        Vasc Health Risk Manag. 2010; 6: 465-472
        • Bokhari F.
        • Newman D.
        • Greene M.
        • Korley V.
        • Mangat I.
        • Dorian P.
        Long-term comparison of the implantable cardioverter defibrillator versus amiodarone.
        Circulation. 2004; 110: 112-116
        • Yamada T.
        • Doppalapudi H.
        • McElderry H.T.
        • Okada T.
        • Murakami Y.
        • Inden Y.
        • et al.
        Electrocardiographic and electrophysiological characteristics in idiopathic ventricular arrhythmias originating from the papillary muscles in the left ventricle.
        Circ Arrhythm Electrophysiol. 2010; 3: 324-331
        • Syed F.F.
        • Ackerman M.J.
        • McLeod C.J.
        • Kapa S.
        • Mulpuru S.K.
        • Sriram C.S.
        • et al.
        Sites of successful ventricular fibrillation ablation in bileaflet mitral valve prolapse syndrome.
        Circ Arrhythm Electrophysiol. 2016; 9e004005
        • Vohra J.
        • Morton J.B.
        • Morgan J.
        • Tatoulis J.
        Cryoablation of papillary muscles at surgery for malignant ventricular arrhythmias due to mitral valve prolapse.
        Heart Lung Circ. 2022; 31: 1285-1290
        • Dixit S.
        • Woo Y.J.
        134 - Surgery for Ventricular Arrhythmias.
        in: Zipes D.P. Jalife J. Stevenson W.G. Cardiac Electrophysiology: From Cell to Bedside. Seventh Edition 7th ed. Elsevier, 2018: 1307-1315
        • Kienzle M.G.
        • Miller J.
        • Falcone R.A.
        • Harken A.
        • Josephson M.E.
        Intraoperative endocardial mapping during sinus rhythm: relationship to site of origin of ventricular tachycardia.
        Circulation. 1984; 70: 957-965
        • Peichl P.
        • Baran J.
        • Wichterle D.
        • Čihák R.
        • Skála T.
        • Aldhoon B.
        • et al.
        The tip of the muscle is a dominant location of ventricular ectopy originating from papillary muscles in the left ventricle.
        J Cardiovasc Electrophysiol. 2018; 29: 64-70
        • Seiler J.
        • Lee J.C.
        • Roberts-Thomson K.C.
        • Stevenson W.G.
        Intracardiac echocardiography guided catheter ablation of incessant ventricular tachycardia from the posterior papillary muscle causing tachycardia mediated cardiomyopathy.
        Heart Rhythm. 2009; 6: 389-392
        • El-Eshmawi A.
        • Pandis D.
        • Miller M.A.
        • Boateng P.
        • Dukkipati S.R.
        • Reddy V.Y.
        • et al.
        Surgical cryoablation of papillary muscle pvcs during mitral valve surgery: therapeutic consideration for malignant MVP.
        J Am Coll Cardiol. 2020; 76: 3061-3062
        • Vahanian A.
        • Beyersdorf F.
        • Praz F.
        • Milojevic M.
        • Baldus S.
        • Bauersachs J.
        • et al.
        2021 ESC/EACTS Guidelines for the management of valvular heart disease: developed by the Task Force for the Management of Valvular Heart Disease of the European Society of Cardiology (ESC) and the European Association for Cardio-Thoracic Surgery (EACTS).
        Eur Heart J. 2021; 43: 561-632
        • 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: 387-393
        • Lee A.
        • Hamilton-Craig C.
        • Denman R.
        • Haqqani H.M.
        Catheter ablation of papillary muscle arrhythmias: implications of mitral valve prolapse and systolic dysfunction.
        Pacing Clin Electrophysiol. 2018; 41: 750-758

      Linked Article