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• W2631242570 abstract "Tabled 1KEY TEACHING POINTS•Evidence of myocardial damage on cardiac magnetic resonance images is found in a majority of patients with muscular dystrophies involving abnormal dystrophin synthesis (Becker and Duchenne muscular dystrophies).•Subepicardial fibrosis of the inferolateral wall of the left ventricle (LV) is a pathological hallmark of dystrophin-associated cardiomyopathies. Subendocardial myocardium is usually spared.•In our case, the area of abnormal electroanatomic substrate was confined entirely to the epicardial surface of the inferolateral LV, whereas endocardial LV mapping was normal. This case report highlights importance of epicardial ventricular tachycardia (VT) mapping in patients with dystrophin-associated cardiomyopathies.•Contrast-enhanced cardiac computed tomography can be potentially useful for localizing of abnormal myocardial substrate in patients with an implantable cardioverter defibrillator undergoing VT ablation. Open table in a new tab Becker muscular dystrophy (BMD) and Duchenne muscular dystrophy (DMD) are X-linked genetic disorders involving abnormal dystrophin synthesis.1Emery A.E. The muscular dystrophies.Lancet. 2002; 359: 687-695Abstract Full Text Full Text PDF PubMed Scopus (1163) Google Scholar Cardiac involvement is frequent in these disorders. Echocardiograms show abnormal systolic function of the left ventricle (LV) in 50%–70% of BMD patients, whereas evidence of myocardial damage detected by cardiac magnetic resonance (CMR) imaging can be found even in patients with normal echocardiograms.2Verhaert D. Richards K. Rafael-Fortney J.A. Raman S.V. Cardiac involvement in patients with muscular dystrophies: Magnetic resonance imaging phenotype and genotypic considerations.Circ Cardiovasc Imaging. 2011; 4: 67-76Crossref PubMed Scopus (145) Google Scholar, 3Silva M.C. Meira Z.M.A. Gurgel Giannetti J.G. da Silva M.M. Campos A.F.O. Barbosa M.M. Starling Filho G.M. Ferreira R.A. Zatz M. Rochitte C.E. Myocardial delayed enhancement by magnetic resonance imaging in patients with muscular dystrophy.J Am Coll Cardiol. 2007; 49: 1874-1879Abstract Full Text Full Text PDF PubMed Scopus (171) Google Scholar, 4Yilmaz A. Gdynia H.-J. Baccouche H. Mahrholdt H. Meinhardt G. Basso C. Thiene G. Sperfeld A.-D. Ludolph A.C. Sechtem U. Cardiac involvement in patients with Becker muscular dystrophy: new diagnostic and pathophysiological insights by a CMR approach.J Cardiovasc Mag Res. 2008; 10: 50Crossref PubMed Scopus (94) Google Scholar Some mutations in the dystrophin gene cause isolated cardiomyopathy without skeletal myopathy.5Feng J. Yan J.Y. Buzin C.H. Sommer S.S. Towbin J.A. Comprehensive mutation scanning of the dystrophin gene in patients with nonsyndromic X-linked dilated cardiomyopathy.J Am Coll Cardiol. 2002; 40: 1120-1124Abstract Full Text Full Text PDF PubMed Scopus (46) Google Scholar Although ventricular arrhythmias appear to be a common manifestation of dystrophin-associated cardiomyopathy, their mechanism and arrhythmogenic substrate are poorly characterized.2Verhaert D. Richards K. Rafael-Fortney J.A. Raman S.V. Cardiac involvement in patients with muscular dystrophies: Magnetic resonance imaging phenotype and genotypic considerations.Circ Cardiovasc Imaging. 2011; 4: 67-76Crossref PubMed Scopus (145) Google Scholar We report a case of recurrent drug-refractory ventricular tachycardia (VT) treated with epicardial ablation in a BMD patient with dilated cardiomyopathy. The patient is a 26-year-old male with a history of BMD due to deletion of exons 38–43 in the dystrophin gene. The disease was first diagnosed in 2005 when he presented to a pediatric neurology clinic with proximal muscle weakness and elevated creatinine kinase levels. A multigated acquisition scan, done as a part of his initial workup, revealed an LV ejection fraction (LVEF) of 36%. In 2011, in view of progressive worsening of his LV systolic function despite optimal medical therapy (LVEF of 20% by echocardiogram), he underwent placement of a primary prevention implantable cardioverter defibrillator (ICD). In June 2013, he received an ICD shock for syncopal monomorphic VT at a rate of 260 beats per minute (bpm). ICD interrogation also showed multiple logged nonsustained VTs. He was subsequently started on amiodarone. In November 2014, after a period of relative quiescence, the patient presented to an outside hospital with recurrent VT requiring 2 ICD shocks. The maintenance dosage of amiodarone was temporarily increased from 200 to 400 mg a day. In April 2015, he presented with another episode of syncope. ICD interrogation revealed 2 additional episodes of monomorphic VT (230–250 bpm) terminated by shocks. VT ablation was thus pursued. A preprocedure contrast-enhanced cardiac computed tomography (CT) image showed severely enlarged LV (LV-end diastolic volume and end systolic volume of 366 mL and 256 mL, respectively), LVEF of 30%, global hypokinesis, and akinesis of the inferolateral wall of the LV. The right ventricle was normal. First-pass contrast-enhanced images suggested loss of myocardium predominantly within the inferolateral wall of the LV (Figure 1A). Delayed enhancement was noted in these segments, with sparing of the subendocardium. An epicardial origin of his arrhythmia was suspected based on the latter finding as well as previously published postmortem and CMR series showing predominantly subepicardial involvement in dystrophin-associated cardiomyopathies (please see the Discussion section). The patient presented to the electrophysiology (EP) laboratory in sinus rhythm (SR) with intermittent atrial pacing at a rate of 65 bpm. Surface electrocardiogram (ECG) showed a right bundle branch block (RBBB) intrinsic QRS pattern with QRS duration of 190 milliseconds and PR interval of 250 milliseconds. The AH interval was 75 milliseconds and HV interval was 84 milliseconds. An intracardiac ultrasound probe (CARTO Sound, Biosense Webster, Diamond Bar, CA) and an electroanatomic mapping (EAM) system (CARTO 4, Biosense Webster) were then used to create endocardial geometries of the ventricles. After obtaining pericardial access, a high-density epicardial voltage map was obtained in SR using a decapolar catheter (DecaNav, Biosense Webster). The mapping catheter was placed into the pericardium via a steerable sheath used for better maneuverability and support (40 cm, Agilis, St Jude Medical, St Paul, MN). There was a large area of low-amplitude (<1 mV) fractionated electrograms (EGMs) in the inferolateral wall of the LV consistent with “scar” (Figure 1B).6Cano O. Hutchinson M. Lin D. Garcia F. Zado E. Bala R. Riley M. Cooper J. Dixit S. Gersenfeld E. Callans D. Marchlinski F.E. Electroanatomic substrate and ablation outcome for suspected epicardial ventricular tachycardia in left ventricular cardiomyopathy.J Am Coll Cardiol. 2009; 54: 799-808Abstract Full Text Full Text PDF PubMed Scopus (287) Google Scholar The “scar” area corresponded to the area of abnormal substrate on the CT images (Figures 1A and 1B). High-frequency (mostly multicomponent) potentials were noted predominantly at the border of the “scar” (Figure 1C). Following transseptal access, high-density SR endocardial voltage mapping of the LV was performed with a duodecapolar catheter (Pentaray, Biosense Webster). This mapping demonstrated normal voltage (>1.5 mV) and EGM morphology over the entire endocardial surface of the LV. A monomorphic VT with RBBB/superior-axis QRS morphology and a cycle length (CL) of 305 milliseconds was easily (double extrastimuli) and reproducibly induced with programmed ventricular stimulation (Figure 2A). There were no identifiable His potentials. Twelve-lead QRS morphology of the tachycardia suggested its epicardial origin (slurring in the initial portion of the QRS, inferior Q waves, pseudo delta wave of 95 milliseconds, and a maximum deflection index of 0.6 milliseconds).7Valles E. Bazan V. Marchlinski F.E. ECG criteria to identify epicardial ventricular tachycardia in nonischemic cardiomyopathy.Circ Arrhythm Electrophysiol. 2010; 3: 63-71Crossref PubMed Scopus (198) Google Scholar, 8Fernandez-Armenta J. Berruezo A. How to recognize epicardial origin of ventricular tachycardia.Cur Card Rev. 2014; 10: 246-256Crossref Scopus (16) Google Scholar Detailed activation and entrainment mapping was not feasible, because of rapid hemodynamic compromise and inconsistent ventricular capture at the maximum output over most of the attempted pacing sites within the epicardial “scar.” Thus, brief epicardial activation mapping with the decapolar catheter during VT was limited to the area exhibiting high-frequency potentials in SR. Sites showing sharp high-frequency mid- or continuous diastolic potentials were tagged on the map (Figures 1B and 2B). VT CL transiently increased from 305 to 330 milliseconds during mapping at these sites. Endocardial LV mapping during VT was not done. Coronary angiography of the left coronary system was then performed, and the angiogram was registered to the epicardial map using the UNIVU technology (Biosense Webster) to avoid ablating directly near the coronary arteries. High-output pacing was performed to mark the course of the phrenic nerve (PN) (Figure 1B). Initially, ablation was performed at the sites showing diastolic potentials during the tachycardia. Because the course of the PN was directly over the ablation target area, a peripheral angioplasty balloon catheter (Armada 35 PTA 14 × 6 cm, Abbott Vascular, Santa Clara, CA) was used to mechanically displace the parietal pericardium.9Buch E. Vaseghi M. Cesario D.A. Shivkumar K. A novel method for preventing phrenic nerve injury during catheter ablation.Heart Rhythm. 2007; 4: 95-98Abstract Full Text Full Text PDF PubMed Scopus (67) Google Scholar An externally irrigated–tip catheter (ThermoCool SmartTouch, Biosense Webster) was used for ablation. Before each radiofrequency application, high-output pacing was performed to ensure lack of PN capture. Following ablation at the tagged sites, the targeted VT was no longer inducible. Two other VT morphologies, which were suspected to be epicardial in origin, were then induced with a more aggressive pacing protocol (Figure 3). Additional ablation lesions were empirically delivered at the sites showing low-amplitude high-frequency potentials in SR. Because the patient was marginally stable at the end of the procedure, further VT induction attempts were deemed unsafe. A few hours after the procedure, he had 2 episodes of fast VT (320–330 bpm) thought to be triggered by a pericardial drain left in the area of the arrhythmogenic “scar.” The drain was subsequently removed. His previous antiarrhythmic regimen with amiodarone was resumed. He has had no further VT recurrences over the ensuing 6 months.Figure 3Twelve-lead electrocardiograms of ventricular tachycardias induced following initial ablation.View Large Image Figure ViewerDownload (PPT) To the best of our knowledge, this report is the first to describe arrhythmogenic electroanatomic substrate in a patient with BMD. The area of abnormal EGMs consistent with “scar” was confined entirely to the epicardial surface of the LV. Although detailed mapping was not feasible, QRS morphology, EP findings, and response to ablation of the initially induced VT strongly suggest its origin at the area of the epicardial “scar.” QRS morphologies of other induced VTs are also suspicious for their epicardial origin. However, mapping of these VTs was not done. EAM findings in the present case are consistent with the results of previously published pathology and CMR series in patients with DMD- and BMD-associated cardiomyopathies. Postmortem heart studies have shown that early cardiac involvement in DMD characteristically presents with fibrosis of subepicardial myocardium, predominantly in the inferolateral LV. In advanced cardiomyopathy, diffuse myocardial fibrosis of the LV and interventricular septum is usually seen. However, subendocardial myocardium is typically spared.10Frankel K.A. Rosser R.J. The pathology of the heart in progressive muscular dystrophy: epimyocardial fibrosis.Hum Pathol. 1976; 7: 375-386Abstract Full Text PDF PubMed Scopus (241) Google Scholar, 11Sanyal S.K. Johnson W.W. Thapar M.K. Pitner S.E. An ultrastructural basis for electrocardiographic alterations associated with Duchenne’s progressive muscular dystrophy.Circulation. 1978; 57: 1122-1129Crossref PubMed Scopus (117) Google Scholar More recently, CMR studies in patients with DMD and BMD have demonstrated characteristic subepicardial distribution patterns of late gadolinium enhancement primarily involving the inferolateral LV.3Silva M.C. Meira Z.M.A. Gurgel Giannetti J.G. da Silva M.M. Campos A.F.O. Barbosa M.M. Starling Filho G.M. Ferreira R.A. Zatz M. Rochitte C.E. Myocardial delayed enhancement by magnetic resonance imaging in patients with muscular dystrophy.J Am Coll Cardiol. 2007; 49: 1874-1879Abstract Full Text Full Text PDF PubMed Scopus (171) Google Scholar, 4Yilmaz A. Gdynia H.-J. Baccouche H. Mahrholdt H. Meinhardt G. Basso C. Thiene G. Sperfeld A.-D. Ludolph A.C. Sechtem U. Cardiac involvement in patients with Becker muscular dystrophy: new diagnostic and pathophysiological insights by a CMR approach.J Cardiovasc Mag Res. 2008; 10: 50Crossref PubMed Scopus (94) Google Scholar The exact mechanism of preferential damage of the subepicardial myocardium in the inferolateral LV wall in patients with dystrophin-associated cardiomyopathies remains unclear. A potential explanation is that this region might be exposed to higher mechanical stress than other regions of the heart and thus is more susceptible to damage in patients with dystrophin mutations or other conditions.2Verhaert D. Richards K. Rafael-Fortney J.A. Raman S.V. Cardiac involvement in patients with muscular dystrophies: Magnetic resonance imaging phenotype and genotypic considerations.Circ Cardiovasc Imaging. 2011; 4: 67-76Crossref PubMed Scopus (145) Google Scholar, 4Yilmaz A. Gdynia H.-J. Baccouche H. Mahrholdt H. Meinhardt G. Basso C. Thiene G. Sperfeld A.-D. Ludolph A.C. Sechtem U. Cardiac involvement in patients with Becker muscular dystrophy: new diagnostic and pathophysiological insights by a CMR approach.J Cardiovasc Mag Res. 2008; 10: 50Crossref PubMed Scopus (94) Google Scholar Interestingly, a similar pathological pattern is commonly observed in patients with viral myocarditis. It has been shown that dystrophin disruption is a key mechanism of myocardial damage in enterovirus-induced myocarditis.12Badorff C. Knowlton K.U. Dystrophin disruption in enterovirus-induced myocarditis and dilated cardiomyopathy: from bench to bedside.Med Microbiol Immunol. 2004; 193: 121-126Crossref PubMed Scopus (65) Google Scholar Bundle branch reentry (BBR) is another potential mechanism of VT in patients with dystrophin-associated cardiomyopathies due to common involvement of the intraventricular conduction system. A case of BBR VT has been previously reported in a patient with BMD.13Negri S.M. Cowan M.D. Becker muscular dystrophy with bundle branch reentry ventricular tachycardia.J Cardiovasc Electrophysiol. 1998; 9: 652-654Crossref Scopus (28) Google Scholar However, the prevalence of BBR VT in these patients is unknown. Preablation contrast-enhanced cardiac CT was used to identify abnormal myocardium prior to ablation. Although gadolinium-enhanced CMR remains the gold standard for characterizing myocardial pathology and has been shown to facilitate the decision about the need for pericardial access in cardiomyopathy patients undergoing VT ablation,14Andreu D. Ortiz-Perez J.T. Boussy T. Fernandez-Armenta J. de Caralt T.M. Perea R.J. Prat-Gonzalez S. Mont L. Brugada J. Berruezo A. Usefulness of contrast-enhanced cardiac magnetic resonance in identifying the ventricular arrhythmia substrate and the approach needed for ablation.Eur Heart J. 2014; 35: 1316-1326Crossref PubMed Scopus (100) Google Scholar it is generally contraindicated in patients with a non-CMR compatible ICD system. Contrast-enhanced cardiac CT has recently emerged as a possible alternative imaging technology to CMR in ICD patients.15Tian J. Jeudy J. Smith M.F. et al.Three-dimensional contrast-enhanced multidetector CT for anatomic, dynamic, and perfusion characterization of abnormal myocardium to guide ventricular tachycardia ablation.Circ Arrhythm Electrophysiol. 2010; 3: 496-504Crossref Scopus (83) Google Scholar This case report highlights the importance of epicardial VT mapping in patients with dystrophin-associated cardiomyopathies. Contrast-enhanced cardiac CT can be potentially useful for localizing abnormal myocardial substrate in ICD patients undergoing VT ablation." @default.
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• W2631242570 title "Epicardial ablation of ventricular tachycardia in a patient with Becker muscular dystrophy" @default.
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