A 16 year old female with hypertrophic cardiomyopathy was treated with alcohol ablation for
NYHA class III symptoms on medical therapy. Three months later, patient underwent
a second alcohol ablation procedure for continued symptoms. Follow-up, for 4 years now,
continues to show resolution of symptoms. (Cardiol J 2007; 14: 301-304

Gradus-Pizlo, Irmina

Rampurwala, Abbas Y.

English

Via Medica Journals

301www.cardiologyjournal.orgCASE REPORTCardiology Journal2007, Vol. 14, No. 3, pp. 301304Copyright ' 2007 Via MedicaISSN 18975993Address for correspondence: Irmina Gradus-Pizlo, MDDirector, Advanced Heart Care ProgramKrannert Institute of CardiologyIndiana University School of Medicine1801 North Senate Boulevard, MPC II, Suite D4081Indianapolis, IN 46202, USATel:  +1 317 962 0533, fax: +1 317 962 0116e-mail: igradus@iupui.eduReceived: 27.02.2007 Accepted: 3.04.2007Multiple alcohol septal ablations in a youngpatient with hypertrophic cardiomyopathyAbbas Y. Rampurwala and Irmina Gradus-PizloKrannert Institute of Cardiology, Indiana University School of Medicine, Indianapolis, USAAbstractA 16 year old female with hypertrophic cardiomyopathy was treated with alcohol ablation forNYHA class III symptoms on medical therapy. Three months later, patient underwenta second alcohol ablation procedure for continued symptoms. Follow-up, for 4 years now,continues to show resolution of symptoms. (Cardiol J 2007; 14: 301304)Key words: hypertrophic cardiomyopathy, alcohol ablation, surgical myectomyIntroductionHypertrophic cardiomyopathy (HCM) is a ge-netic disease characterized by hypertrophy of theleft ventricle and in a subset of patients, obstructionof the left ventricular outflow tract (LVOT) [1, 2]. Sig-nificant outflow tract obstruction is an independentpredictor of poor prognosis in patients with HCM [3].The incidence of LVOT obstruction has been dem-onstrated to be present in higher than previouslyreported percentage of patients when exercisestress testing was used to provoke a gradient [4].In the treatment of obstructive HCM non-pharma-cologic options include surgery and transcatheterethanol septal reduction. Dual chamber pacing hasonly a limited role. Patients with obstructive HCMare candidates for septal reduction therapy if theyare severely symptomatic (New York Heart Asso-ciation functional class III or IV) or have recurrentsyncope despite pharmacologic therapy.Case reportA 16 year old female with a family history ofhypertrophic cardiomyopathy presented with symp-toms of increasing fatigue and NYHA functionalclass III. She had a loud systolic ejection murmurat rest, which increased with Valsalva maneuver.Echocardiography showed septal hypertrophy withseptal thickness of 27 mm (Fig. 1), systolic anteri-or motion (SAM) of mitral valve leaflet, restingLVOT gradient of 70 mm Hg which increased to100 mm Hg with Valsalva maneuver. She had an ep-isode consistent with presyncope during physicalFigure 1. Pre-ablation parasternal long axis echocardio-graph showing thickened septum (A) and narrow leftventricular outflow tract.302Cardiology Journal 2007, Vol. 14, No. 3www.cardiologyjournal.orgactivity and a family history of ventricular arrhyth-mias. Patient underwent ICD implantation and wason medical therapy with beta blockers and calciumchannel blockers. Medications were very poorlytolerated because of hypotension and fatigue.Patient was no longer able to attend school. Optionsof surgical vs. transcatheter ethanol septal reduc-tion procedures were discussed with her family andher. A decision was made to proceed with ethanolseptal ablation. First septal perforator branch of theLAD was engaged with angioplasty catheter andslow infusion of ethanol was performed. Immedi-ate resolution of LVOT gradient was noted. Systo-lic ejection murmur was no longer present. Patientwas monitored in the intensive care unit, cardiactroponin peaked at 30 and ECG showed septal Qwaves. The next post-procedure day, physical ex-amination demonstrated recurrence of systolicmurmur at rest. Echocardiogram confirmed recur-rence of LVOT gradient and presence of SAM ofmitral valve leaflet. The hope was that recurrenceof gradient was in part due to increased edema ofinfracted proximal septal tissue and that with de-velopment of scar and left ventricular remodelingthere will be improvement in LVOT gradient. Clin-ically, patient reported symptomatic improvementin her fatigue and she was able to return to school.Serial echocardiograms showed development ofsmall scar in the proximal septum but persistenceof LVOT gradient and SAM. Within three monthspatient also had recurrence of symptoms. A deci-sion was made to repeat the transcatheter ethanolseptal reduction procedure with injection of etha-nol into the second and third septal perforatorbranches of LAD. Immediate results of this proce-dure were equally rewarding as the first one. Therewas immediate resolution of LVOT gradient in thecatheterization laboratory and disappearance ofsystolic murmur. This time troponin peaked at 50.We were disappointed when on the second post-procedure day the murmur returned with the sameintensity and again LVOT obstruction was demon-strated by echocardiography. But this time patientfelt significantly better and had objective improve-ment in physical activity tolerance.  She returnedto school full time and was able to finish high school.Serial echocardiograms demonstrated gradual thin-ning of the interventricular septum, disappearanceof LVOT obstruction and SAM by the third monthpost second procedure. Septal remodeling with pro-gressive septal thinning was observed to occur overperiod up to 1 year (Fig. 2). Patient was able to attendcollege and is now working full time as a paramedic.She has no limitations of physical activity and is ableto exercise regularly.DiscussionWith surgical myectomy, the proximal septumis approached through the aortic valve via an aortot-omy and 3 to 15 g of septal muscle is removed [58].Perioperative mortality is reported to be less than1% and centers with extensive experience reporteven lower mortality in uncomplicated cases. Com-plications of myectomy include a ventricular septaldefect due to excessive removal of septal muscle,ischemia of the hypertrophied muscle due to inad-equate intraoperative protection and resulting leftventricular dysfunction, aortic valve regurgitationdue to traction on the aortic valve to improve visu-alization of and access to the interventricular sep-tum, and left bundle branch block (LBBB) or com-plete heart block (CHB) requiring a permanentpacemaker in approximately 510% of patients.Reduction of LVOT gradient, improvement in func-tional class and long-term survival is excellent af-ter surgical myectomy at experienced centers [9].Transcatheter ethanol septal ablation consistsof infarction and thinning of the proximal interven-tricular septum via infusion of ethanol into the firstseptal perforating branch of the left anterior de-scending coronary artery through an angioplastycatheter.  Ethanol septal ablation reduces LV out-flow obstruction, improves symptoms, and in-creases exercise capacity. Long-term benefit re-sults from the creation of localized septal infarctionand scarring, which increase LV outflow diameteras a result of septal thinning and therapeuticremodeling [1013]. This procedure significantlyreduces the resting LVOT gradient, the induciblegradient and improves functional class [14]. A subsetof patients who do not show initial benefit can havelater improvement [15]. These late responders hadFigure 2. Post-ablation (2nd ablation) parasternal longaxis echocardiography showing septal scar (A) withresolution of left ventricular outflow tract obstruction.303Abbas Y. Rampurwala and Irmina Gradus-Pizlo, Multiple alcohol septal ablationswww.cardiologyjournal.orga similarly improved LVOT gradient at one yearcompared to those who improved immediately af-ter the procedure. Complete heart block (CHB)requiring a pacemaker occurs in 1425% of patientsafter ethanol septal ablation [16, 17].Both surgical myectomy and ethanol septalablation reduce LV outflow tract obstruction andimprove NYHA class in HCM. When efficacy of thetwo techniques was compared the resting LVOTgradient fell to a similar degree with both ethanolablation and surgical myectomy, there was similarreduction in septal thickness and improvement inexercise duration in both groups. The incidence ofCHB was higher with ethanol ablation, but surgerywas associated with a significant increase in mildaortic regurgitation [18]. A recent study showedconsistent anterior basal septal reduction with my-ectomy but not with alcohol ablation, as assessedby cardiac magnetic resonance imaging [19].The advantages of ethanol ablation includeavoidance of cardiopulmonary bypass, shorter hos-pital stay and recovery time and reduced expense.Advantages of surgical myectomy include moreimmediate and complete relief of resting and pro-voked obstruction and concomitant mitral regurgi-tation, lower incidence of CHB requiring pacemak-er insertion and proven long-term (> 20 years)efficacy; similar data with ethanol ablation are notyet available.In our patient, four year follow up shows satis-factory results with improved functional capacity,lack of LVOT gradient and preservation of left ven-tricular function without development of completeheart block. However the degree and extent of theleft ventricular septal thinning is concerning to us.In retrospect, we probably should have shown morerestrain and allowed more time for the remodelingbefore the second ablation procedure.References1. Wigle ED, Rakowski H, Kimball BP, Williams WG.Hypertrophic cardiomyopathy: Clinical spectrum andtreatment. Circulation, 1995; 92: 1680.2. Maron, BJ, McKenna, WJ, Danielson, GK et al.American College of Cardiology/European Society ofCardiology clinical expert consensus document onhypertrophic cardiomyopathy. A report of the Amer-ican College of Cardiology Foundation Task Forceon Clinical Expert Consensus Documents and theEuropean Society of Cardiology Committee for Prac-tice Guidelines. J Am Coll Cardiol, 2003; 42: 1687.3. Maron MS, Olivotto I, Betocchi S et al. Effect of leftventricular outflow tract obstruction on clinical out-come in hypertrophic cardiomyopathy. N Engl J Med,2003; 348: 295.4. Maron MS, Olivotto I, Zenovich AG et al. Hyper-trophic cardiomyopathy is predominantly disease ofleft ventricular outflow tract obstruction. Circulation,2006; 114: 2232.5. Morrow AG, Reitz BA, Epstein SE et al. Operativetreatment in hypertrophic subaortic stenosis: Tech-niques, and the results of pre and postoperative as-sessments in 83 patients. Circulation, 1975; 52: 88.6. Beahrs MM, Tajik AJ, Seward JB et al. Hypertrophicobstructive cardiomyopathy: Ten to 21-year follow-upafter partial septal myectomy. Am J Cardiol, 1983;51: 1160.7. Schulte HD, Bircks WH, Loesse B et al. Prognosis ofpatients with hypertrophic obstructive cardiomyopa-thy after transaortic myectomy. Late results up totwenty-five years. J Thorac Cardiovasc Surg, 1993;106: 709.8. Ommen SR, Maron BJ, Olivotto I et al. Long-termeffects of surgical septal myectomy on survival inpatients with obstructive hypertrophic cardiomyopa-thy. J Am Coll Cardiol, 2005; 46: 470.9. Flores-Ramirez R, Lakkis NM, Middleton KJ et al.Echocardiographic insights into the mechanisms ofrelief of left ventricular outflow tract obstruction af-ter nonsurgical septal reduction therapy in patientswith hypertrophic obstructive cardiomyopathy. J AmColl Cardiol, 2001; 37: 208.10. Kuhn H, Gietzen FH, Schafers M et al. Changes inthe left ventricular outflow tract after transcoronaryablation of septal hypertrophy (TASH) for hyper-trophic obstructive cardiomyopathy as assessed bytransoesophageal echocardiography and by measur-ing myocardial glucose utilization and perfusion. EurHeart J, 1999; 20: 1808.11. Mazur W, Nagueh SF, Lakkis NM et al. Regressionof left ventricular hypertrophy after nonsurgical sep-tal reduction therapy for hypertrophic obstructivecardiomyopathy. Circulation, 2001; 103: 1492.12. van Dockum WG, Beek AM, ten Cate FJ et al.Early onset and progression of left ventricularremodeling after alcohol septal ablation in hyper-trophic obstructive cardiomyopathy. Circulation,2005; 111: 2503.13. Faber L, Seggewiss H, Gleichmann U. Percutaneoustransluminal septal myocardial ablation in hyper-trophic obstructive cardiomyopathy: Results with re-spect to intraprocedural myocardial contrast echocar-diography. Circulation, 1998; 98: 2415.14. Yoerger DM, Picard MH, Palacios IF et al. Timecourse of pressure gradient response after first alco-hol septal ablation for obstructive hypertrophic car-diomyopathy. Am J Cardiol, 2006; 97: 1511.304Cardiology Journal 2007, Vol. 14, No. 3www.cardiologyjournal.org15. Chang SM, Nagueh SF, Spencer WH, 3rd, Lakkis NM.Complete heart block: determinants and clinical im-pact in patients with hypertrophic obstructive cardi-omyopathy undergoing nonsurgical septal reductiontherapy. J Am Coll Cardiol, 2003; 42: 296.16. Chen AA, Palacios IF, Mela T et al. Acute predictorsof subacute complete heart block after alcohol septalablation for obstructive hypertrophic cardiomyopa-thy. Am J Cardiol, 2006; 97: 264.17. Nagueh SF, Ommen SR, Lakkis NM et al. Comparisonof ethanol septal reduction therapy with surgical mye-ctomy for the treatment of hypertrophic obstructivecardiomyopathy. J Am Coll Cardiol, 2001; 38: 1701.18. Valeti US, Nishimura RA, Holmes DR et al. Compa-rison of surgical septal myectomy and alcohol septalablation with cardiac magnetic resonance imaging inpatients with hypertrophic obstructive cardiomyopa-thy. J Am Coll Cardiol, 2007; 49: 350357.

Multiple alcohol septal ablations in a young patient with hypertrophic cardiomyopathy

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Multiple alcohol septal ablations in a young patient with hypertrophic cardiomyopathy

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