Hypertrophic cardiomyopathy surgery: Difference between revisions

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(/* Selection of Patients for Heart Transplantation (DO NOT EDIT) {{cite journal |author=Gersh BJ, Maron BJ, Bonow RO, Dearani JA, Fifer MA, Link MS, Naidu SS, Nishimura RA, Ommen SR, Rakowski H, Seidman CE, Towbin JA, Udelson JE, Yancy CW |title=2...)
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{{Hypertrophic cardiomyopathy}}
{{Hypertrophic cardiomyopathy}}


'''Editors-In-Chief:''' C. Michael Gibson, M.S., M.D. [mailto:charlesmichaelgibson@gmail.com], Cafer Zorkun, M.D. [mailto:zorkun@perfuse.org], Caitlin J. Harrigan [mailto:charrigan@perfuse.org], Martin S. Maron, M.D., and Barry J. Maron, M.D.
'''Editors-In-Chief:''' C. Michael Gibson, M.S., M.D. [mailto:charlesmichaelgibson@gmail.com], Cafer Zorkun, M.D. [mailto:zorkun@perfuse.org], Caitlin J. Harrigan [mailto:charrigan@perfuse.org], Martin S. Maron, M.D., and Barry J. Maron, M.D. {{Soroush}}
 
==Overview==
[[Septal myectomy]] is a [[Cardiac surgery|surgical]] treatment for [[hypertrophic cardiomyopathy]] (HCM). Septal myectomies have been successfully performed for more than 25 years.
 
Cardiac transplantation can be performed in patients with HOCM and has been associated with better post-operative survival than those patients transplanted for ischemic cardiomyopathy.


==Cardiac Transplantation==
==Cardiac Transplantation==
Cardiac transplantation can be performed in patients with HOCM and has been associated with better post-operative survival than those patients transplanted for ischemic cardiomyopathy <ref> Martin S. Maron;  Benjamin M. Kalsmith;  James E. Udelson;  Wenjun Li and  David Denofrio.Survival Following Cardiac Transplantation in Patients with Hypertrophic Cardiomyopathy.doi: 10.1161/CIRCHEARTFAILURE.109.922872</ref>.
Cardiac transplantation can be performed in patients with HOCM and has been associated with better post-operative survival than those patients transplanted for ischemic cardiomyopathy. <ref> Martin S. Maron;  Benjamin M. Kalsmith;  James E. Udelson;  Wenjun Li and  David Denofrio.Survival Following Cardiac Transplantation in Patients with Hypertrophic Cardiomyopathy.doi: 10.1161/CIRCHEARTFAILURE.109.922872</ref>


In cases that are refractory to all other forms of treatment, [[heart transplant|cardiac transplantation]] is an option.
In cases that are refractory to all other forms of treatment, [[heart transplant|cardiac transplantation]] is an option.


== 2020 AHA/ACC Guideline for the Diagnosis and Treatment of Patients With Hypertrophic Cardiomyopathy A Report of the American College of Cardiology/American Heart  Association Joint Committee on Clinical Practice Guidelines<ref name="pmid36334952">{{cite journal| author=Writing Committee Members. Isselbacher EM, Preventza O, Hamilton Black Iii J, Augoustides JG, Beck AW | display-authors=etal| title=2022 ACC/AHA Guideline for the Diagnosis and Management of Aortic Disease: A Report of the American Heart Association/American College of Cardiology Joint Committee on Clinical Practice Guidelines. | journal=J Am Coll Cardiol | year= 2022 | volume= 80 | issue= 24 | pages= e223-e393 | pmid=36334952 | doi=10.1016/j.jacc.2022.08.004 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=36334952  }}</ref> ==
=== Recommendations for ICD Placement in High-Risk Patients With HCM Referenced studies that support the recommendations are summarized in the Online Data Supplement ===
{| class="wikitable"
|-
| colspan="1" style="text-align:center; background:LightGreen" |[[ACC AHA guidelines classification scheme#Classification of Recommendations|Class I]]
|-
| bgcolor="LightGreen" |1. In patients with HCM, application of individual clinical judgment is recommended when assessing the prognostic strength of conventional risk marker(s) within the clinical profile of the individual patient, as well as a thorough and balanced discussion of the evidence, benefits, and estimated risks to engage the fully informed patient’s active participation in ICD decision-making ''([[ACC AHA guidelines classification scheme#Level of Evidence|Level of Evidence: C-EO]])''
2. For patients with HCM, and previous documented cardiac arrest or sustained VT, ICD placement is recommended ''([[ACC AHA guidelines classification scheme#Level of Evidence|Level of Evidence: B-NR]])''
|}
{| class="wikitable"
|-
| colspan="1" style="text-align:center; background:LemonChiffon" |[[ACC AHA guidelines classification scheme#Classification of Recommendations|Class IIa]]
|-
| bgcolor="LemonChiffon" |3. For adult patients with HCM with ≥1 major risk factors for SCD, it is reasonable to offer an ICD. These major risk factors include2,3,7–21(Figure 3, Table 7):''([[ACC AHA guidelines classification scheme#Level of Evidence|Level of Evidence: B-NR]])''
a. Sudden death judged definitively or likely attributable to HCM in ≥1 first-degree or close relatives who are ≤50 years of age;
b. Massive LVH ≥30 mm in any LV segment;
c. ≥1 Recent episodes of syncope suspected by clinical history to be arrhythmic (ie, unlikely to be of neurocardiogenic [vasovagal] etiology, or related to LVOTO);
d. LV apical aneurysm, independent of size;
e. LV systolic dysfunction (EF <50%).
4. For children with HCM who have ≥1 conventional risk factors, including unexplained syncope, massive LVH, NSVT, or family his-tory of early HCM-related SCD, ICD placement is reasonable after considering the relatively high complication rates of long-term ICD placement in younger patients''([[ACC AHA guidelines classification scheme#Level of Evidence|Level of Evidence: B-NR]])''
5. For patients ≥16 years of age with HCM and with ≥1 major SCD risk factors, discussion of the estimated 5-year sudden death risk and mortality rates can be useful during the shared decision-making process for ICD placemen''([[ACC AHA guidelines classification scheme#Level of Evidence|Level of Evidence: B-NR]])''
|}
{| class="wikitable"
|-
| colspan="1" style="text-align:center; background:LemonChiffon" |[[ACC AHA guidelines classification scheme#Classification of Recommendations|Class IIb]]
|-
| bgcolor="LemonChiffon" |6. In select adult patients with HCM and without major SCD risk factors after clinical assessment, or in whom the decision to proceed with ICD placement remains otherwise uncertain, ICD may be considered in patients with extensive LGE by contrast-enhanced CMR imaging or NSVT present on ambulatory monitoring''([[ACC AHA guidelines classification scheme#Level of Evidence|Level of Evidence: B-NR]])''
7. In select pediatric patients with HCM in whom risk stratification is otherwise less certain, it may be useful to consider additional factors such as extensive LGE on contrast-enhanced CMR imaging and systolic dysfunction in risk stratification''([[ACC AHA guidelines classification scheme#Level of Evidence|Level of Evidence: C-LD]])''
|}
=== Recommendations for Invasive Treatment of Symptomatic Patients With Obstructive HCM Referenced studies that support the recommendations are summarized in the Online Data Supplement ===
{| class="wikitable"
|-
| colspan="1" style="text-align:center; background:LightGreen" |[[ACC AHA guidelines classification scheme#Classification of Recommendations|Class I]]
|-
| bgcolor="LightGreen" |2. In symptomatic patients with obstructive HCM who have associated cardiac disease requiring surgical treatment (eg, associated anomalous papillary muscle, markedly elongated anterior mitral leaflet, intrinsic mitral valve disease, multivessel CAD, valvular aortic stenosis), surgical myectomy, performed at experienced centers,† is recommended''([[ACC AHA guidelines classification scheme#Level of Evidence|Level of Evidence: B-NR]])''
|}
{| class="wikitable"
|-
| colspan="1" style="text-align:center; background:LemonChiffon" |[[ACC AHA guidelines classification scheme#Classification of Recommendations|Class IIb]]
|-
| bgcolor="LemonChiffon" |4. In patients with obstructive HCM, earlier (NYHA class II) surgical myectomy performed at comprehensive HCM centers (Table 3 and Table 4) may be reasonable in the presence of additional clinical factors, including3,11–22:''([[ACC AHA guidelines classification scheme#Level of Evidence|Level of Evidence: B-NR]])''
a.Severe and progressive pulmonary hyper-tension thought to be attributable to LVOTO or associated MR.
b.Left atrial enlargement with ≥1 episodes  of symptomatic AF
c.Poor functional capacity attributable to LVOTO as documented on treadmill exercise testing.
d.Children and young adults with very high resting LVOT gradients (>100 mm Hg).
5. For severely symptomatic patients with obstructive HCM, SRT in eligible patients,* performed at experienced centers† (Table 3 and Table 4), may be considered as an alter-native to escalation of medical therapy after shared decision-making including risks and benefits of all treatment options.''([[ACC AHA guidelines classification scheme#Level of Evidence|Level of Evidence: C-LD]])''
|}
'''Recommendations for Management of Patients With Nonobstructive HCM With Preserved EF Referenced studies that support the recommendations are summarized in the Online Data Supplement'''
{| class="wikitable"
|-
| colspan="1" style="text-align:center; background:LemonChiffon" |[[ACC AHA guidelines classification scheme#Classification of Recommendations|Class IIb]]
|-
| bgcolor="LemonChiffon" |4. In highly selected patients with apical HCM with severe dyspnea or angina (NYHA class III or class IV) despite maximal medical therapy, and with preserved EF and small LV cavity size (LV end-diastolic volume <50 mL/m2 and LV stroke volume <30 mL/m2), apical myectomy by experienced surgeons at comprehensive centers may be considered to reduce symptoms''([[ACC AHA guidelines classification scheme#Level of Evidence|Level of Evidence: C-LD]])''
|}
=== Recommendations for Management of Atrial Fibrillation Referenced studies that support the recommendations are summarized in the Online Data Supplement ===
{| class="wikitable"
|-
| colspan="1" style="text-align:center; background:LemonChiffon" |[[ACC AHA guidelines classification scheme#Classification of Recommendations|Class IIa]]
|-
| bgcolor="LemonChiffon" |7. In patients with HCM and AF who require surgical myectomy, concomitant surgical AF ablation procedure can be beneficial for AF rhythm control''([[ACC AHA guidelines classification scheme#Level of Evidence|Level of Evidence: B-NR]])''
|}
=== Recommendations for Patients With HCM and Advanced HF Referenced studies that support the recommendations are summarized in the Online Data Supplement ===
{| class="wikitable"
|-
| colspan="1" style="text-align:center; background:LightGreen" |[[ACC AHA guidelines classification scheme#Classification of Recommendations|Class I]]
|-
| bgcolor="LightGreen" |4. In patients with nonobstructive HCM and advanced HF (NYHA class III to class IV despite guideline-directed therapy) or with life-threatening ventricular arrhythmias refractory to maximal guideline-directed therapy, assessment for heart transplantation in accordance with current listing criteria is recommended''([[ACC AHA guidelines classification scheme#Level of Evidence|Level of Evidence: B-NR]])''
|}
{| class="wikitable"
|-
| colspan="1" style="text-align:center; background:LemonChiffon" |[[ACC AHA guidelines classification scheme#Classification of Recommendations|Class IIa]]
|-
| bgcolor="LemonChiffon" |6. In patients with nonobstructive HCM and advanced HF (NYHA functional class III to class IV despite GDMT) who are candidates for heart transplantation, continuous-flow LVAD therapy is reasonable as a bridge to heart transplantation''([[ACC AHA guidelines classification scheme#Level of Evidence|Level of Evidence: B-NR]])''
7. In patients with HCM and LVEF <50%, ICD placement can be beneficial''([[ACC AHA guidelines classification scheme#Level of Evidence|Level of Evidence: C-LD]])''
8. In patients with HCM and LVEF <50%, NYHA functional class II to class IV symptoms despite guideline-directed therapy, and LBBB, CRT can be beneficial to improve symptoms''([[ACC AHA guidelines classification scheme#Level of Evidence|Level of Evidence: C-LD]])''
|}
==2011 ACCF/AHA Guideline for the Diagnosis and Treatment of Hypertrophic Cardiomyopathy (DO NOT EDIT)<ref name="pmid22075469">{{cite journal |author=Gersh BJ, Maron BJ, Bonow RO, Dearani JA, Fifer MA, Link MS, Naidu SS, Nishimura RA, Ommen SR, Rakowski H, Seidman CE, Towbin JA, Udelson JE, Yancy CW |title=2011 ACCF/AHA Guideline for the Diagnosis and Treatment of Hypertrophic Cardiomyopathy A Report of the American College of Cardiology Foundation/American Heart Association Task Force on Practice Guidelines Developed in Collaboration With the American Association for Thoracic Surgery, American Society of Echocardiography, American Society of Nuclear Cardiology, Heart Failure Society of America, Heart Rhythm Society, Society for Cardiovascular Angiography and Interventions, and Society of Thoracic Surgeons |journal=[[Journal of the American College of Cardiology]] |volume=58 |issue=25 |pages=e212–60 |year=2011 |month=December |pmid=22075469 |doi=10.1016/j.jacc.2011.06.011 |url=http://linkinghub.elsevier.com/retrieve/pii/S0735-1097(11)02275-3 |accessdate=2011-12-19}}</ref>==
==2011 ACCF/AHA Guideline for the Diagnosis and Treatment of Hypertrophic Cardiomyopathy (DO NOT EDIT)<ref name="pmid22075469">{{cite journal |author=Gersh BJ, Maron BJ, Bonow RO, Dearani JA, Fifer MA, Link MS, Naidu SS, Nishimura RA, Ommen SR, Rakowski H, Seidman CE, Towbin JA, Udelson JE, Yancy CW |title=2011 ACCF/AHA Guideline for the Diagnosis and Treatment of Hypertrophic Cardiomyopathy A Report of the American College of Cardiology Foundation/American Heart Association Task Force on Practice Guidelines Developed in Collaboration With the American Association for Thoracic Surgery, American Society of Echocardiography, American Society of Nuclear Cardiology, Heart Failure Society of America, Heart Rhythm Society, Society for Cardiovascular Angiography and Interventions, and Society of Thoracic Surgeons |journal=[[Journal of the American College of Cardiology]] |volume=58 |issue=25 |pages=e212–60 |year=2011 |month=December |pmid=22075469 |doi=10.1016/j.jacc.2011.06.011 |url=http://linkinghub.elsevier.com/retrieve/pii/S0735-1097(11)02275-3 |accessdate=2011-12-19}}</ref>==


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|-
|-
| bgcolor="LightGreen"|<nowiki>"</nowiki>'''1.''' Patients with advanced [[heart failure]] (end stage) and nonobstructive HCM not otherwise amenable to other treatment interventions, with [[EF]] less than or equal to 50% (or occasionally with preserved EF), should be considered for heart transplantation<ref name="pmid16831987">{{cite journal |author=Harris KM, Spirito P, Maron MS, ''et al.'' |title=Prevalence, clinical profile, and significance of left ventricular remodeling in the end-stage phase of hypertrophic cardiomyopathy |journal=Circulation |volume=114 |issue=3 |pages=216–25 |year=2006 |month=July |pmid=16831987 |doi=10.1161/CIRCULATIONAHA.105.583500 |url=}}</ref><ref name="pmid18237606">{{cite journal |author=Biagini E, Spirito P, Leone O, ''et al.'' |title=Heart transplantation in hypertrophic cardiomyopathy |journal=Am. J. Cardiol. |volume=101 |issue=3 |pages=387–92 |year=2008 |month=February |pmid=18237606 |doi=10.1016/j.amjcard.2007.09.085 |url=}}</ref>. ''([[ACC AHA guidelines classification scheme#Level of Evidence|Level of Evidence: B]])'' <nowiki>"</nowiki>
| bgcolor="LightGreen"|<nowiki>"</nowiki>'''1.''' Patients with advanced [[heart failure]] (end stage) and nonobstructive [[HCM]] not otherwise amenable to other treatment interventions, with [[EF]] less than or equal to 50% (or occasionally with preserved EF), should be considered for heart transplantation.<ref name="pmid16831987">{{cite journal |author=Harris KM, Spirito P, Maron MS, ''et al.'' |title=Prevalence, clinical profile, and significance of left ventricular remodeling in the end-stage phase of hypertrophic cardiomyopathy |journal=Circulation |volume=114 |issue=3 |pages=216–25 |year=2006 |month=July |pmid=16831987 |doi=10.1161/CIRCULATIONAHA.105.583500 |url=}}</ref><ref name="pmid18237606">{{cite journal |author=Biagini E, Spirito P, Leone O, ''et al.'' |title=Heart transplantation in hypertrophic cardiomyopathy |journal=Am. J. Cardiol. |volume=101 |issue=3 |pages=387–92 |year=2008 |month=February |pmid=18237606 |doi=10.1016/j.amjcard.2007.09.085 |url=}}</ref> ''([[ACC AHA guidelines classification scheme#Level of Evidence|Level of Evidence: B]])'' <nowiki>"</nowiki>


|-
|-
| bgcolor="LightGreen"|<nowiki>"</nowiki>'''2.''' Symptomatic children with HCM with restrictive physiology who are not responsive to or appropriate candidates for other therapeutic interventions should be considered for heart transplantation<ref name="pmid19782603">{{cite journal |author=Gajarski R, Naftel DC, Pahl E, ''et al.'' |title=Outcomes of pediatric patients with hypertrophic cardiomyopathy listed for transplant |journal=J. Heart Lung Transplant. |volume=28 |issue=12 |pages=1329–34 |year=2009 |month=December |pmid=19782603 |doi=10.1016/j.healun.2009.05.028 |url=}}</ref><ref name="pmid12015478">{{cite journal |author=Towbin JA |title=Cardiomyopathy and heart transplantation in children |journal=Curr. Opin. Cardiol. |volume=17 |issue=3 |pages=274–9 |year=2002 |month=May |pmid=12015478 |doi= |url=}}</ref>. ''([[ACC AHA guidelines classification scheme#Level of Evidence|Level of Evidence: C]])'' <nowiki>"</nowiki>
| bgcolor="LightGreen"|<nowiki>"</nowiki>'''2.''' Symptomatic children with [[HCM]] with restrictive physiology who are not responsive to or appropriate candidates for other therapeutic interventions should be considered for heart transplantation.<ref name="pmid19782603">{{cite journal |author=Gajarski R, Naftel DC, Pahl E, ''et al.'' |title=Outcomes of pediatric patients with hypertrophic cardiomyopathy listed for transplant |journal=J. Heart Lung Transplant. |volume=28 |issue=12 |pages=1329–34 |year=2009 |month=December |pmid=19782603 |doi=10.1016/j.healun.2009.05.028 |url=}}</ref><ref name="pmid12015478">{{cite journal |author=Towbin JA |title=Cardiomyopathy and heart transplantation in children |journal=Curr. Opin. Cardiol. |volume=17 |issue=3 |pages=274–9 |year=2002 |month=May |pmid=12015478 |doi= |url=}}</ref> ''([[ACC AHA guidelines classification scheme#Level of Evidence|Level of Evidence: C]])'' <nowiki>"</nowiki>
|}
|}


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|colspan="1" style="text-align:center; background:LightCoral"|[[ACC AHA guidelines classification scheme#Classification of Recommendations|Class III]] (Harm)
|colspan="1" style="text-align:center; background:LightCoral"|[[ACC AHA guidelines classification scheme#Classification of Recommendations|Class III]] (Harm)
|-
|-
|bgcolor="LightCoral"| <nowiki>"</nowiki>'''1.''' Heart transplantation should not be performed in mildly symptomatic patients of any age with HCM. ''([[ACC AHA guidelines classification scheme#Level of Evidence|Level of Evidence: C]])'' <nowiki>"</nowiki>
|bgcolor="LightCoral"| <nowiki>"</nowiki>'''1.''' Heart transplantation should not be performed in mildly symptomatic patients of any age with [[HCM]]. ''([[ACC AHA guidelines classification scheme#Level of Evidence|Level of Evidence: C]])'' <nowiki>"</nowiki>


|}
|}
==Septal Reduction Guidline==
==2011 ACCF/AHA Guideline for the Diagnosis and Treatment of Hypertrophic Cardiomyopathy (DO NOT EDIT)<ref name="pmid22075469">{{cite journal |author=Gersh BJ, Maron BJ, Bonow RO, Dearani JA, Fifer MA, Link MS, Naidu SS, Nishimura RA, Ommen SR, Rakowski H, Seidman CE, Towbin JA, Udelson JE, Yancy CW |title=2011 ACCF/AHA Guideline for the Diagnosis and Treatment of Hypertrophic Cardiomyopathy A Report of the American College of Cardiology Foundation/American Heart Association Task Force on Practice Guidelines Developed in Collaboration With the American Association for Thoracic Surgery, American Society of Echocardiography, American Society of Nuclear Cardiology, Heart Failure Society of America, Heart Rhythm Society, Society for Cardiovascular Angiography and Interventions, and Society of Thoracic Surgeons |journal=[[Journal of the American College of Cardiology]] |volume=58 |issue=25 |pages=e212–60 |year=2011 |month=December |pmid=22075469 |doi=10.1016/j.jacc.2011.06.011 |url=http://linkinghub.elsevier.com/retrieve/pii/S0735-1097(11)02275-3 |accessdate=2011-12-19}}</ref>==
===Septal Reduction Therapy (DO NOT EDIT)<ref name="pmid22075469">{{cite journal |author=Gersh BJ, Maron BJ, Bonow RO, Dearani JA, Fifer MA, Link MS, Naidu SS, Nishimura RA, Ommen SR, Rakowski H, Seidman CE, Towbin JA, Udelson JE, Yancy CW |title=2011 ACCF/AHA Guideline for the Diagnosis and Treatment of Hypertrophic Cardiomyopathy A Report of the American College of Cardiology Foundation/American Heart Association Task Force on Practice Guidelines Developed in Collaboration With the American Association for Thoracic Surgery, American Society of Echocardiography, American Society of Nuclear Cardiology, Heart Failure Society of America, Heart Rhythm Society, Society for Cardiovascular Angiography and Interventions, and Society of Thoracic Surgeons |journal=[[Journal of the American College of Cardiology]] |volume=58 |issue=25 |pages=e212–60 |year=2011 |month=December |pmid=22075469 |doi=10.1016/j.jacc.2011.06.011 |url=http://linkinghub.elsevier.com/retrieve/pii/S0735-1097(11)02275-3 |accessdate=2011-12-19}}</ref>===
{|class="wikitable"
|-
| colspan="1" style="text-align:center; background:LightGreen"|[[ACC AHA guidelines classification scheme#Classification of Recommendations|Class I]]
|-
| bgcolor="LightGreen"|<nowiki>"</nowiki>'''1.''' Septal reduction therapy should be performed only by experienced operators in the context of a comprehensive [[HCM]] clinical program and only for the treatment of eligible patients with severe [[Hypertrophic cardiomyopathy medical treatment|drug-refractory symptoms]] and LVOT obstruction.<ref name="pmid18435964">{{cite journal |author=van der Lee C, Scholzel B, ten Berg JM, ''et al.'' |title=Usefulness of clinical, echocardiographic, and procedural characteristics to predict outcome after percutaneous transluminal septal myocardial ablation |journal=Am. J. Cardiol. |volume=101 |issue=9 |pages=1315–20 |year=2008 |month=May |pmid=18435964 |doi=10.1016/j.amjcard.2008.01.003 |url=}}</ref> ''([[ACC AHA guidelines classification scheme#Level of Evidence|Level of Evidence: C]])''<nowiki>"</nowiki>
|}
{|class="wikitable"
|-
|colspan="1" style="text-align:center; background:LightCoral"|[[ACC AHA guidelines classification scheme#Classification of Recommendations|Class III]] (Harm)
|-
|bgcolor="LightCoral"| <nowiki>"</nowiki>'''1.''' Septal reduction therapy should not be done for adult patients with [[HCM]] who are asymptomatic with normal exercise tolerance or whose symptoms are controlled or minimized on [[Hypertrophic cardiomyopathy medical treatment|optimal medical therapy]]. ''([[ACC AHA guidelines classification scheme#Level of Evidence|Level of Evidence: C]])'' <nowiki>"</nowiki>
|-
|bgcolor="LightCoral"| <nowiki>"</nowiki>'''2.''' Septal reduction therapy should not be done unless performed as part of a program dedicated to the longitudinal and multidisciplinary care of patients with [[HCM]]. ''([[ACC AHA guidelines classification scheme#Level of Evidence|Level of Evidence: C]])'' <nowiki>"</nowiki>
|-
|bgcolor="LightCoral"| <nowiki>"</nowiki>'''3.''' [[Mitral valve replacement]] for relief of LVOT obstruction should not be performed in patients with [[HCM]] in whom septal reduction therapy is an option. ''([[ACC AHA guidelines classification scheme#Level of Evidence|Level of Evidence: C]])'' <nowiki>"</nowiki>
|}
==Sources==
*[http://circ.ahajournals.org/content/117/21/e350.full.pdf ACC/AHA/HRS 2008 Guidelines for Device-Based Therapy of Cardiac Rhythm Abnormalities] <ref name="Epstein"> Epstein AE, DiMarco JP, Ellenbogen KA, Estes NAM III, Freedman RA, Gettes LS, Gillinov AM, Gregoratos G, Hammill SC, Hayes DL, Hlatky MA, Newby LK, Page RL, Schoenfeld MH, Silka MJ, Stevenson LW, Sweeney MO. ACC/AHA/HRS 2008 guidelines for device-based therapy of cardiac rhythm abnormalities: executive summary: a report of the American College of Cardiology/American Heart Association Task Force on Practice Guidelines (Writing Committee to Revise the ACC/AHA/NASPE 2002 Guideline Update for Implantation of Cardiac Pacemakers and Antiarrhythmia Devices). Circulation. 2008; 117: 2820–2840. PMID 18483207 </ref>
*[http://europace.oxfordjournals.org/content/9/10/959.full.pdf The Task Force for Cardiac Pacing and Cardiac Resynchronization Therapy of the European Society of Cardiology. Developed in Collaboration with the European Heart Rhythm Association] <ref name="pmid17726043">{{cite journal| author=Vardas PE, Auricchio A, Blanc JJ, Daubert JC, Drexler H, Ector H et al.| title=Guidelines for cardiac pacing and cardiac resynchronization therapy. The Task Force for Cardiac Pacing and Cardiac Resynchronization Therapy of the European Society of Cardiology. Developed in collaboration with the European Heart Rhythm Association. | journal=Europace | year= 2007 | volume= 9 | issue= 10 | pages= 959-98 | pmid=17726043 | doi=10.1093/europace/eum189 | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=17726043  }} </ref>
*[http://content.onlinejacc.org/cgi/reprint/58/25/e212.pdf 2011 ACCF/AHA Guideline for the Diagnosis and Treatment of Hypertrophic Cardiomyopathy] <ref name="pmid22075468">{{cite journal |author=Gersh BJ, Maron BJ, Bonow RO, Dearani JA, Fifer MA, Link MS, Naidu SS, Nishimura RA, Ommen SR, Rakowski H, Seidman CE, Towbin JA, Udelson JE, Yancy CW |title=2011 ACCF/AHA Guideline for the Diagnosis and Treatment of Hypertrophic Cardiomyopathy: Executive Summary A Report of the American College of Cardiology Foundation/American Heart Association Task Force on Practice Guidelines Developed in Collaboration With the American Association for Thoracic Surgery, American Society of Echocardiography, American Society of Nuclear Cardiology, Heart Failure Society of America, Heart Rhythm Society, Society for Cardiovascular Angiography and Interventions, and Society of Thoracic Surgeons |journal=[[Journal of the American College of Cardiology]] |volume=58 |issue=25 |pages=2703–38 |year=2011 |month=December |pmid=22075468 |doi=10.1016/j.jacc.2011.10.825 |url=http://linkinghub.elsevier.com/retrieve/pii/S0735-1097(11)04383-X |accessdate=2011-12-19}}</ref><ref name="pmid22075469">{{cite journal |author=Gersh BJ, Maron BJ, Bonow RO, Dearani JA, Fifer MA, Link MS, Naidu SS, Nishimura RA, Ommen SR, Rakowski H, Seidman CE, Towbin JA, Udelson JE, Yancy CW |title=2011 ACCF/AHA Guideline for the Diagnosis and Treatment of Hypertrophic Cardiomyopathy A Report of the American College of Cardiology Foundation/American Heart Association Task Force on Practice Guidelines Developed in Collaboration With the American Association for Thoracic Surgery, American Society of Echocardiography, American Society of Nuclear Cardiology, Heart Failure Society of America, Heart Rhythm Society, Society for Cardiovascular Angiography and Interventions, and Society of Thoracic Surgeons |journal=[[Journal of the American College of Cardiology]] |volume=58 |issue=25 |pages=e212–60 |year=2011 |month=December |pmid=22075469 |doi=10.1016/j.jacc.2011.06.011 |url=http://linkinghub.elsevier.com/retrieve/pii/S0735-1097(11)02275-3 |accessdate=2011-12-19}}</ref>
==Septal Myectomy==
[[Septal myectomy]] is a [[cardiac surgery|surgical]] treatment for [[hypertrophic cardiomyopathy]] (HCM). Septal myectomies have been successfully performed for more than 25 years.
==History==
It has been performed successfully for more than 25 years.
==Indications==
Surgical [[septal myectomy]] is the gold standard for relief of symptoms for patients who do not experience relief of symptoms from medications.<ref name="pmid11886323">{{cite journal| author=Maron BJ| title=Hypertrophic cardiomyopathy: a systematic review. | journal=JAMA | year= 2002 | volume= 287 | issue= 10 | pages= 1308-20 | pmid=11886323 | doi= | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=11886323  }} </ref> <ref name="pmid12607696">{{cite journal| author=Sherrid MV, Chaudhry FA, Swistel DG| title=Obstructive hypertrophic cardiomyopathy: echocardiography, pathophysiology, and the continuing evolution of surgery for obstruction. | journal=Ann Thorac Surg | year= 2003 | volume= 75 | issue= 2 | pages= 620-32 | pmid=12607696 | doi= | pmc= | url= }} </ref> <ref name="pmid7671349">{{cite journal| author=Wigle ED, Rakowski H, Kimball BP, Williams WG| title=Hypertrophic cardiomyopathy. Clinical spectrum and treatment. | journal=Circulation | year= 1995 | volume= 92 | issue= 7 | pages= 1680-92 | pmid=7671349 | doi= | pmc= | url= }} </ref> <ref name="pmid14607462">{{cite journal| author=Maron BJ, McKenna WJ, Danielson GK, Kappenberger LJ, Kuhn HJ, Seidman CE et al.| title=American College of Cardiology/European Society of Cardiology clinical expert consensus document on hypertrophic cardiomyopathy. A report of the American College of Cardiology Foundation Task Force on Clinical Expert Consensus Documents and the European Society of Cardiology Committee for Practice Guidelines. | journal=J Am Coll Cardiol | year= 2003 | volume= 42 | issue= 9 | pages= 1687-713 | pmid=14607462 | doi= | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=14607462  }} </ref> <ref name="pmid15837258">{{cite journal| author=Sherrid MV, Barac I, McKenna WJ, Elliott PM, Dickie S, Chojnowska L et al.| title=Multicenter study of the efficacy and safety of disopyramide in obstructive hypertrophic cardiomyopathy. | journal=J Am Coll Cardiol | year= 2005 | volume= 45 | issue= 8 | pages= 1251-8 | pmid=15837258 | doi=10.1016/j.jacc.2005.01.012 | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=15837258  }} </ref> <ref name="pmid581298">{{cite journal| author=Morrow AG| title=Hypertrophic subaortic stenosis. Operative methods utilized to relieve left ventricular outflow obstruction. | journal=J Thorac Cardiovasc Surg | year= 1978 | volume= 76 | issue= 4 | pages= 423-30 | pmid=581298 | doi= | pmc= | url= }} </ref>
==Technique==
*It involves a midline thoracotomy (general anesthesia, opening the chest, and cardiopulmonary bypass) and removing a portion of the interventricular septum.<ref name="pmid11886323">{{cite journal| author=Maron BJ| title=Hypertrophic cardiomyopathy: a systematic review. | journal=JAMA | year= 2002 | volume= 287 | issue= 10 | pages= 1308-20 | pmid=11886323 | doi= | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=11886323  }} </ref>
*A modification of the Morrow myectomy termed extended myectomy, mobilization and partial excision of the papillary muscles has become the excision of choice. <ref name="pmid12607696">{{cite journal| author=Sherrid MV, Chaudhry FA, Swistel DG| title=Obstructive hypertrophic cardiomyopathy: echocardiography, pathophysiology, and the continuing evolution of surgery for obstruction. | journal=Ann Thorac Surg | year= 2003 | volume= 75 | issue= 2 | pages= 620-32 | pmid=12607696 | doi= | pmc= | url= }} </ref><ref name="pmid8067875">{{cite journal| author=Messmer BJ| title=Extended myectomy for hypertrophic obstructive cardiomyopathy. | journal=Ann Thorac Surg | year= 1994 | volume= 58 | issue= 2 | pages= 575-7 | pmid=8067875 | doi= | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=8067875  }} </ref><ref name="pmid7586394">{{cite journal| author=Schoendube FA, Klues HG, Reith S, Flachskampf FA, Hanrath P, Messmer BJ| title=Long-term clinical and echocardiographic follow-up after surgical correction of hypertrophic obstructive cardiomyopathy with extended myectomy and reconstruction of the subvalvular mitral apparatus. | journal=Circulation | year= 1995 | volume= 92 | issue= 9 Suppl | pages= II122-7 | pmid=7586394 | doi= | pmc= | url= }} </ref><ref name="Balaram, Sherrid et al 2005">Balaram SK, Sherrid MV, DeRose JJ, Hillel Z, Winson G, Swistel DG. Beyond extended myectomy for hypertrophic cardiomyopathy: The RPR (Resection–Plication–Release) Repair. ''Annals of Thoracic Surgery'' 2005; '''80''':217–23</ref>
*In selected patients with particularly large redundant mitral valves, anterior leaflet plication may be added to complete separation of the mitral valve and outflow.<ref name="Balaram, Sherrid et al 2005" /><ref name="McIntosh, Maron et al 1992">McIntosh CL, Maron BJ, Cannon RO, Klues H. Initial results of combined anterior mitral valve plication and ventricular septal myotomy–myectomy for relief of left ventricular outflow obstruction in patients with hypertrophic cardiomyopathy. ''[[Circulation (journal)|Circulation]]'' 1992; '''86''':II 60–7</ref>
==Efficacy and Procedural Success==
Surgical septal myectomy uniformly decreases left ventricular outflow tract obstruction and improves symptoms, and in experienced centers has a surgical mortality of 1%.
Surgical myectomy resection focused just on the subaortic septum, to increase the size of the outflow tract to reduce Venturi forces may be inadequate to abolish systolic anterior motion (SAM) of the anterior leaflet of the mitral valve. With this limited sort of resection the residual mid-septal bulge still redirects flow posteriorly: SAM persists because flow still gets behind the mitral valve. It is only when the deeper portion of the septal bulge is resected that flow is redirected anteriorly away from the mitral valve, abolishing SAM.<ref name="pmid12607696">{{cite journal| author=Sherrid MV, Chaudhry FA, Swistel DG| title=Obstructive hypertrophic cardiomyopathy: echocardiography, pathophysiology, and the continuing evolution of surgery for obstruction. | journal=Ann Thorac Surg | year= 2003 | volume= 75 | issue= 2 | pages= 620-32 | pmid=12607696 | doi= | pmc= | url= }} </ref><ref name="pmid8579024">{{cite journal| author=Nakatani S, Schwammenthal E, Lever HM, Levine RA, Lytle BW, Thomas JD| title=New insights into the reduction of mitral valve systolic anterior motion after ventricular septal myectomy in hypertrophic obstructive cardiomyopathy. | journal=Am Heart J | year= 1996 | volume= 131 | issue= 2 | pages= 294-300 | pmid=8579024 | doi= | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=8579024  }} </ref>
==Outcomes==
Septal myectomy is associated with a low perioperative mortality and a high late survival rate.  A study at the [[Mayo Clinic]] found surgical myectomy performed to relieve outflow obstruction and severe symptoms in HCM was associated with long-term survival equivalent to that of the general population, and superior to obstructive HCM without operation.  The results are shown below:<ref name="Ommen 2005">{{cite journal | author = Ommen S, Maron B, Olivotto I, Maron M, Cecchi F, Betocchi S, Gersh B, Ackerman M, McCully R, Dearani J, Schaff H, Danielson G, Tajik A, Nishimura R | title = Long-term effects of surgical septal myectomy on survival in patients with obstructive hypertrophic cardiomyopathy | journal = J Am Coll Cardiol | volume = 46 | issue = 3 | pages = 470-6 | year = 2005 | id = PMID 16053960}}</ref>
<center>
{| border="0"
|
  {| class="wikitable"
  |+ Survival (all-cause mortality) *
  |-
  ! Years
  ! With surgery
  ! Without surgery
  |-
  | 1
  | 98%
  | 90%
  |-
  | 5
  | 96%
  | 79%
  |-
  | 10
  | 83%
  | 61%
  |}
|
  {| class="wikitable"
  |+ Survival (HCM-related death)
  |-
  ! Years
  ! With surgery
  ! Without surgery
  |-
  | 1
  | 99%
  | 94%
  |-
  | 5
  | 98%
  | 89%
  |-
  | 10
  | 95%
  | 73%
  |}
|
  {| class="wikitable"
  |+ Survival (sudden cardiac death)
  |-
  ! Years
  ! With surgery
  ! Without surgery
  |-
  | 1
  | 100%
  | 97%
  |-
  | 5
  | 99%
  | 93%
  |-
  | 10
  | 99%
  | 89%
  |}
|}
<nowiki>*</nowiki> Includes 0.8% operative mortality.
</center>
==Comparison with alcohol ablation==
Either [[alcohol septal ablation]] or myectomy offers substantial clinical improvement for patients with [[hypertrophic obstructive cardiomyopathy]].
Hemodynamic resolution of the obstruction and its sequelae is more complete with myectomy.<ref name="Ralph-Edwards 2005">{{cite journal | author = Ralph-Edwards A, Woo A, McCrindle B, Shapero J, Schwartz L, Rakowski H, Wigle E, Williams W | title = Hypertrophic obstructive cardiomyopathy: comparison of outcomes after myectomy or alcohol ablation adjusted by propensity score | journal = J Thorac Cardiovasc Surg | volume = 129 | issue = 2 | pages = 351-8 | year = 2005 | id = PMID 15678046}}</ref>
==2011 ACCF/AHA Guideline for the Diagnosis and Treatment of Hypertrophic Cardiomyopathy (DO NOT EDIT)<ref name="pmid22075469">{{cite journal |author=Gersh BJ, Maron BJ, Bonow RO, Dearani JA, Fifer MA, Link MS, Naidu SS, Nishimura RA, Ommen SR, Rakowski H, Seidman CE, Towbin JA, Udelson JE, Yancy CW |title=2011 ACCF/AHA Guideline for the Diagnosis and Treatment of Hypertrophic Cardiomyopathy A Report of the American College of Cardiology Foundation/American Heart Association Task Force on Practice Guidelines Developed in Collaboration With the American Association for Thoracic Surgery, American Society of Echocardiography, American Society of Nuclear Cardiology, Heart Failure Society of America, Heart Rhythm Society, Society for Cardiovascular Angiography and Interventions, and Society of Thoracic Surgeons |journal=[[Journal of the American College of Cardiology]] |volume=58 |issue=25 |pages=e212–60 |year=2011 |month=December |pmid=22075469 |doi=10.1016/j.jacc.2011.06.011 |url=http://linkinghub.elsevier.com/retrieve/pii/S0735-1097(11)02275-3 |accessdate=2011-12-19}}</ref>==
===Septal Myectomy  (DO NOT EDIT)<ref name="pmid22075469">{{cite journal |author=Gersh BJ, Maron BJ, Bonow RO, Dearani JA, Fifer MA, Link MS, Naidu SS, Nishimura RA, Ommen SR, Rakowski H, Seidman CE, Towbin JA, Udelson JE, Yancy CW |title=2011 ACCF/AHA Guideline for the Diagnosis and Treatment of Hypertrophic Cardiomyopathy A Report of the American College of Cardiology Foundation/American Heart Association Task Force on Practice Guidelines Developed in Collaboration With the American Association for Thoracic Surgery, American Society of Echocardiography, American Society of Nuclear Cardiology, Heart Failure Society of America, Heart Rhythm Society, Society for Cardiovascular Angiography and Interventions, and Society of Thoracic Surgeons |journal=[[Journal of the American College of Cardiology]] |volume=58 |issue=25 |pages=e212–60 |year=2011 |month=December |pmid=22075469 |doi=10.1016/j.jacc.2011.06.011 |url=http://linkinghub.elsevier.com/retrieve/pii/S0735-1097(11)02275-3 |accessdate=2011-12-19}}</ref>===
{|class="wikitable"
|-
| colspan="1" style="text-align:center; background:LemonChiffon"|[[ACC AHA guidelines classification scheme#Classification of Recommendations|Class IIa]]
|-
|bgcolor="LemonChiffon"|<nowiki>"</nowiki>'''1.''' Consultation with centers experienced in performing both surgical [[septal myectomy]] and alcohol septal ablation is reasonable when discussing treatment options for eligible patients with HCM with severe drug-refractory symptoms and LVOT obstruction. ''([[ACC AHA guidelines classification scheme#Level of Evidence|Level of Evidence: C]])''<nowiki>"</nowiki>
|-
|bgcolor="LemonChiffon"|<nowiki>"</nowiki>'''2.''' [[septal myectomy|Surgical septal myectomy]], when performed in experienced centers, can be beneficial and is the first consideration for the majority of eligible patients with [[HCM]] with severe [[Hypertrophic cardiomyopathy medical treatment|drug-refractory symptoms]] and LVOT obstruction. ''([[ACC AHA guidelines classification scheme#Level of Evidence|Level of Evidence: B]])''<nowiki>"</nowiki>
|-
|bgcolor="LemonChiffon"|<nowiki>"</nowiki>'''3.''' [[septal myectomy|Surgical septal myectomy]], when performed at experienced centers, can be beneficial in symptomatic children with [[HCM]] and severe resting obstruction (>50 mm Hg) for whom standard medical therapy has failed. ''([[ACC AHA guidelines classification scheme#Level of Evidence|Level of Evidence: C]])''<nowiki>"</nowiki>
|}
==Sources==
*[http://circ.ahajournals.org/content/117/21/e350.full.pdf ACC/AHA/HRS 2008 Guidelines for Device-Based Therapy of Cardiac Rhythm Abnormalities] <ref name="Epstein"> Epstein AE, DiMarco JP, Ellenbogen KA, Estes NAM III, Freedman RA, Gettes LS, Gillinov AM, Gregoratos G, Hammill SC, Hayes DL, Hlatky MA, Newby LK, Page RL, Schoenfeld MH, Silka MJ, Stevenson LW, Sweeney MO. ACC/AHA/HRS 2008 guidelines for device-based therapy of cardiac rhythm abnormalities: executive summary: a report of the American College of Cardiology/American Heart Association Task Force on Practice Guidelines (Writing Committee to Revise the ACC/AHA/NASPE 2002 Guideline Update for Implantation of Cardiac Pacemakers and Antiarrhythmia Devices). Circulation. 2008; 117: 2820–2840. PMID 18483207 </ref>
*[http://europace.oxfordjournals.org/content/9/10/959.full.pdf The Task Force for Cardiac Pacing and Cardiac Resynchronization Therapy of the European Society of Cardiology. Developed in Collaboration with the European Heart Rhythm Association] <ref name="pmid17726043">{{cite journal| author=Vardas PE, Auricchio A, Blanc JJ, Daubert JC, Drexler H, Ector H et al.| title=Guidelines for cardiac pacing and cardiac resynchronization therapy. The Task Force for Cardiac Pacing and Cardiac Resynchronization Therapy of the European Society of Cardiology. Developed in collaboration with the European Heart Rhythm Association. | journal=Europace | year= 2007 | volume= 9 | issue= 10 | pages= 959-98 | pmid=17726043 | doi=10.1093/europace/eum189 | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=17726043  }} </ref>
*[http://content.onlinejacc.org/cgi/reprint/58/25/e212.pdf 2011 ACCF/AHA Guideline for the Diagnosis and Treatment of Hypertrophic Cardiomyopathy] <ref name="pmid22075468">{{cite journal |author=Gersh BJ, Maron BJ, Bonow RO, Dearani JA, Fifer MA, Link MS, Naidu SS, Nishimura RA, Ommen SR, Rakowski H, Seidman CE, Towbin JA, Udelson JE, Yancy CW |title=2011 ACCF/AHA Guideline for the Diagnosis and Treatment of Hypertrophic Cardiomyopathy: Executive Summary A Report of the American College of Cardiology Foundation/American Heart Association Task Force on Practice Guidelines Developed in Collaboration With the American Association for Thoracic Surgery, American Society of Echocardiography, American Society of Nuclear Cardiology, Heart Failure Society of America, Heart Rhythm Society, Society for Cardiovascular Angiography and Interventions, and Society of Thoracic Surgeons |journal=[[Journal of the American College of Cardiology]] |volume=58 |issue=25 |pages=2703–38 |year=2011 |month=December |pmid=22075468 |doi=10.1016/j.jacc.2011.10.825 |url=http://linkinghub.elsevier.com/retrieve/pii/S0735-1097(11)04383-X |accessdate=2011-12-19}}</ref><ref name="pmid22075469">{{cite journal |author=Gersh BJ, Maron BJ, Bonow RO, Dearani JA, Fifer MA, Link MS, Naidu SS, Nishimura RA, Ommen SR, Rakowski H, Seidman CE, Towbin JA, Udelson JE, Yancy CW |title=2011 ACCF/AHA Guideline for the Diagnosis and Treatment of Hypertrophic Cardiomyopathy A Report of the American College of Cardiology Foundation/American Heart Association Task Force on Practice Guidelines Developed in Collaboration With the American Association for Thoracic Surgery, American Society of Echocardiography, American Society of Nuclear Cardiology, Heart Failure Society of America, Heart Rhythm Society, Society for Cardiovascular Angiography and Interventions, and Society of Thoracic Surgeons |journal=[[Journal of the American College of Cardiology]] |volume=58 |issue=25 |pages=e212–60 |year=2011 |month=December |pmid=22075469 |doi=10.1016/j.jacc.2011.06.011 |url=http://linkinghub.elsevier.com/retrieve/pii/S0735-1097(11)02275-3 |accessdate=2011-12-19}}</ref>
==Related Chapters==
* [[Alcohol septal ablation]]


==References==
==References==
{{reflist|2}}
{{reflist|2}}


[[Category:Cardiomyopathy]]
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[[Category:Cardiomyopathy]]
 
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Latest revision as of 19:08, 10 December 2022

Hypertrophic Cardiomyopathy Microchapters

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Editors-In-Chief: C. Michael Gibson, M.S., M.D. [1], Cafer Zorkun, M.D. [2], Caitlin J. Harrigan [3], Martin S. Maron, M.D., and Barry J. Maron, M.D. Soroush Seifirad, M.D.[4]

Overview

Septal myectomy is a surgical treatment for hypertrophic cardiomyopathy (HCM). Septal myectomies have been successfully performed for more than 25 years.

Cardiac transplantation can be performed in patients with HOCM and has been associated with better post-operative survival than those patients transplanted for ischemic cardiomyopathy.

Cardiac Transplantation

Cardiac transplantation can be performed in patients with HOCM and has been associated with better post-operative survival than those patients transplanted for ischemic cardiomyopathy. [1]

In cases that are refractory to all other forms of treatment, cardiac transplantation is an option.


2020 AHA/ACC Guideline for the Diagnosis and Treatment of Patients With Hypertrophic Cardiomyopathy A Report of the American College of Cardiology/American Heart  Association Joint Committee on Clinical Practice Guidelines[2]

Recommendations for ICD Placement in High-Risk Patients With HCM Referenced studies that support the recommendations are summarized in the Online Data Supplement

Class I
1. In patients with HCM, application of individual clinical judgment is recommended when assessing the prognostic strength of conventional risk marker(s) within the clinical profile of the individual patient, as well as a thorough and balanced discussion of the evidence, benefits, and estimated risks to engage the fully informed patient’s active participation in ICD decision-making (Level of Evidence: C-EO)

2. For patients with HCM, and previous documented cardiac arrest or sustained VT, ICD placement is recommended (Level of Evidence: B-NR)


Class IIa
3. For adult patients with HCM with ≥1 major risk factors for SCD, it is reasonable to offer an ICD. These major risk factors include2,3,7–21(Figure 3, Table 7):(Level of Evidence: B-NR)

a. Sudden death judged definitively or likely attributable to HCM in ≥1 first-degree or close relatives who are ≤50 years of age; b. Massive LVH ≥30 mm in any LV segment; c. ≥1 Recent episodes of syncope suspected by clinical history to be arrhythmic (ie, unlikely to be of neurocardiogenic [vasovagal] etiology, or related to LVOTO); d. LV apical aneurysm, independent of size; e. LV systolic dysfunction (EF <50%). 4. For children with HCM who have ≥1 conventional risk factors, including unexplained syncope, massive LVH, NSVT, or family his-tory of early HCM-related SCD, ICD placement is reasonable after considering the relatively high complication rates of long-term ICD placement in younger patients(Level of Evidence: B-NR)

5. For patients ≥16 years of age with HCM and with ≥1 major SCD risk factors, discussion of the estimated 5-year sudden death risk and mortality rates can be useful during the shared decision-making process for ICD placemen(Level of Evidence: B-NR)

Class IIb
6. In select adult patients with HCM and without major SCD risk factors after clinical assessment, or in whom the decision to proceed with ICD placement remains otherwise uncertain, ICD may be considered in patients with extensive LGE by contrast-enhanced CMR imaging or NSVT present on ambulatory monitoring(Level of Evidence: B-NR)

7. In select pediatric patients with HCM in whom risk stratification is otherwise less certain, it may be useful to consider additional factors such as extensive LGE on contrast-enhanced CMR imaging and systolic dysfunction in risk stratification(Level of Evidence: C-LD)

Recommendations for Invasive Treatment of Symptomatic Patients With Obstructive HCM Referenced studies that support the recommendations are summarized in the Online Data Supplement

Class I
2. In symptomatic patients with obstructive HCM who have associated cardiac disease requiring surgical treatment (eg, associated anomalous papillary muscle, markedly elongated anterior mitral leaflet, intrinsic mitral valve disease, multivessel CAD, valvular aortic stenosis), surgical myectomy, performed at experienced centers,† is recommended(Level of Evidence: B-NR)
Class IIb
4. In patients with obstructive HCM, earlier (NYHA class II) surgical myectomy performed at comprehensive HCM centers (Table 3 and Table 4) may be reasonable in the presence of additional clinical factors, including3,11–22:(Level of Evidence: B-NR)

a.Severe and progressive pulmonary hyper-tension thought to be attributable to LVOTO or associated MR. b.Left atrial enlargement with ≥1 episodes  of symptomatic AF c.Poor functional capacity attributable to LVOTO as documented on treadmill exercise testing. d.Children and young adults with very high resting LVOT gradients (>100 mm Hg). 5. For severely symptomatic patients with obstructive HCM, SRT in eligible patients,* performed at experienced centers† (Table 3 and Table 4), may be considered as an alter-native to escalation of medical therapy after shared decision-making including risks and benefits of all treatment options.(Level of Evidence: C-LD)

Recommendations for Management of Patients With Nonobstructive HCM With Preserved EF Referenced studies that support the recommendations are summarized in the Online Data Supplement

Class IIb
4. In highly selected patients with apical HCM with severe dyspnea or angina (NYHA class III or class IV) despite maximal medical therapy, and with preserved EF and small LV cavity size (LV end-diastolic volume <50 mL/m2 and LV stroke volume <30 mL/m2), apical myectomy by experienced surgeons at comprehensive centers may be considered to reduce symptoms(Level of Evidence: C-LD)

Recommendations for Management of Atrial Fibrillation Referenced studies that support the recommendations are summarized in the Online Data Supplement

Class IIa
7. In patients with HCM and AF who require surgical myectomy, concomitant surgical AF ablation procedure can be beneficial for AF rhythm control(Level of Evidence: B-NR)

Recommendations for Patients With HCM and Advanced HF Referenced studies that support the recommendations are summarized in the Online Data Supplement

Class I
4. In patients with nonobstructive HCM and advanced HF (NYHA class III to class IV despite guideline-directed therapy) or with life-threatening ventricular arrhythmias refractory to maximal guideline-directed therapy, assessment for heart transplantation in accordance with current listing criteria is recommended(Level of Evidence: B-NR)
Class IIa
6. In patients with nonobstructive HCM and advanced HF (NYHA functional class III to class IV despite GDMT) who are candidates for heart transplantation, continuous-flow LVAD therapy is reasonable as a bridge to heart transplantation(Level of Evidence: B-NR)

7. In patients with HCM and LVEF <50%, ICD placement can be beneficial(Level of Evidence: C-LD) 8. In patients with HCM and LVEF <50%, NYHA functional class II to class IV symptoms despite guideline-directed therapy, and LBBB, CRT can be beneficial to improve symptoms(Level of Evidence: C-LD)

2011 ACCF/AHA Guideline for the Diagnosis and Treatment of Hypertrophic Cardiomyopathy (DO NOT EDIT)[3]

Selection of Patients for Heart Transplantation (DO NOT EDIT) [3]

Class I
"1. Patients with advanced heart failure (end stage) and nonobstructive HCM not otherwise amenable to other treatment interventions, with EF less than or equal to 50% (or occasionally with preserved EF), should be considered for heart transplantation.[4][5] (Level of Evidence: B) "
"2. Symptomatic children with HCM with restrictive physiology who are not responsive to or appropriate candidates for other therapeutic interventions should be considered for heart transplantation.[6][7] (Level of Evidence: C) "
Class III (Harm)
"1. Heart transplantation should not be performed in mildly symptomatic patients of any age with HCM. (Level of Evidence: C) "

Septal Reduction Guidline

2011 ACCF/AHA Guideline for the Diagnosis and Treatment of Hypertrophic Cardiomyopathy (DO NOT EDIT)[3]

Septal Reduction Therapy (DO NOT EDIT)[3]

Class I
"1. Septal reduction therapy should be performed only by experienced operators in the context of a comprehensive HCM clinical program and only for the treatment of eligible patients with severe drug-refractory symptoms and LVOT obstruction.[8] (Level of Evidence: C)"
Class III (Harm)
"1. Septal reduction therapy should not be done for adult patients with HCM who are asymptomatic with normal exercise tolerance or whose symptoms are controlled or minimized on optimal medical therapy. (Level of Evidence: C) "
"2. Septal reduction therapy should not be done unless performed as part of a program dedicated to the longitudinal and multidisciplinary care of patients with HCM. (Level of Evidence: C) "
"3. Mitral valve replacement for relief of LVOT obstruction should not be performed in patients with HCM in whom septal reduction therapy is an option. (Level of Evidence: C) "

Sources


Septal Myectomy

Septal myectomy is a surgical treatment for hypertrophic cardiomyopathy (HCM). Septal myectomies have been successfully performed for more than 25 years.

History

It has been performed successfully for more than 25 years.

Indications

Surgical septal myectomy is the gold standard for relief of symptoms for patients who do not experience relief of symptoms from medications.[12] [13] [14] [15] [16] [17]

Technique

  • It involves a midline thoracotomy (general anesthesia, opening the chest, and cardiopulmonary bypass) and removing a portion of the interventricular septum.[12]
  • A modification of the Morrow myectomy termed extended myectomy, mobilization and partial excision of the papillary muscles has become the excision of choice. [13][18][19][20]
  • In selected patients with particularly large redundant mitral valves, anterior leaflet plication may be added to complete separation of the mitral valve and outflow.[20][21]

Efficacy and Procedural Success

Surgical septal myectomy uniformly decreases left ventricular outflow tract obstruction and improves symptoms, and in experienced centers has a surgical mortality of 1%.

Surgical myectomy resection focused just on the subaortic septum, to increase the size of the outflow tract to reduce Venturi forces may be inadequate to abolish systolic anterior motion (SAM) of the anterior leaflet of the mitral valve. With this limited sort of resection the residual mid-septal bulge still redirects flow posteriorly: SAM persists because flow still gets behind the mitral valve. It is only when the deeper portion of the septal bulge is resected that flow is redirected anteriorly away from the mitral valve, abolishing SAM.[13][22]

Outcomes

Septal myectomy is associated with a low perioperative mortality and a high late survival rate. A study at the Mayo Clinic found surgical myectomy performed to relieve outflow obstruction and severe symptoms in HCM was associated with long-term survival equivalent to that of the general population, and superior to obstructive HCM without operation. The results are shown below:[23]

Survival (all-cause mortality) *
Years With surgery Without surgery
1 98% 90%
5 96% 79%
10 83% 61%
Survival (HCM-related death)
Years With surgery Without surgery
1 99% 94%
5 98% 89%
10 95% 73%
Survival (sudden cardiac death)
Years With surgery Without surgery
1 100% 97%
5 99% 93%
10 99% 89%

* Includes 0.8% operative mortality.

Comparison with alcohol ablation

Either alcohol septal ablation or myectomy offers substantial clinical improvement for patients with hypertrophic obstructive cardiomyopathy.

Hemodynamic resolution of the obstruction and its sequelae is more complete with myectomy.[24]

2011 ACCF/AHA Guideline for the Diagnosis and Treatment of Hypertrophic Cardiomyopathy (DO NOT EDIT)[3]

Septal Myectomy (DO NOT EDIT)[3]

Class IIa
"1. Consultation with centers experienced in performing both surgical septal myectomy and alcohol septal ablation is reasonable when discussing treatment options for eligible patients with HCM with severe drug-refractory symptoms and LVOT obstruction. (Level of Evidence: C)"
"2. Surgical septal myectomy, when performed in experienced centers, can be beneficial and is the first consideration for the majority of eligible patients with HCM with severe drug-refractory symptoms and LVOT obstruction. (Level of Evidence: B)"
"3. Surgical septal myectomy, when performed at experienced centers, can be beneficial in symptomatic children with HCM and severe resting obstruction (>50 mm Hg) for whom standard medical therapy has failed. (Level of Evidence: C)"

Sources

Related Chapters

References

  1. ↑ Martin S. Maron; Benjamin M. Kalsmith; James E. Udelson; Wenjun Li and David Denofrio.Survival Following Cardiac Transplantation in Patients with Hypertrophic Cardiomyopathy.doi: 10.1161/CIRCHEARTFAILURE.109.922872
  2. ↑ Writing Committee Members. Isselbacher EM, Preventza O, Hamilton Black Iii J, Augoustides JG, Beck AW; et al. (2022). "2022 ACC/AHA Guideline for the Diagnosis and Management of Aortic Disease: A Report of the American Heart Association/American College of Cardiology Joint Committee on Clinical Practice Guidelines". J Am Coll Cardiol. 80 (24): e223–e393. doi:10.1016/j.jacc.2022.08.004. PMID 36334952 Check |pmid= value (help).
  3. ↑ 3.0 3.1 3.2 3.3 3.4 3.5 3.6 3.7 Gersh BJ, Maron BJ, Bonow RO, Dearani JA, Fifer MA, Link MS, Naidu SS, Nishimura RA, Ommen SR, Rakowski H, Seidman CE, Towbin JA, Udelson JE, Yancy CW (2011). "2011 ACCF/AHA Guideline for the Diagnosis and Treatment of Hypertrophic Cardiomyopathy A Report of the American College of Cardiology Foundation/American Heart Association Task Force on Practice Guidelines Developed in Collaboration With the American Association for Thoracic Surgery, American Society of Echocardiography, American Society of Nuclear Cardiology, Heart Failure Society of America, Heart Rhythm Society, Society for Cardiovascular Angiography and Interventions, and Society of Thoracic Surgeons". Journal of the American College of Cardiology. 58 (25): e212–60. doi:10.1016/j.jacc.2011.06.011. PMID 22075469. Retrieved 2011-12-19. Unknown parameter |month= ignored (help)
  4. ↑ Harris KM, Spirito P, Maron MS; et al. (2006). "Prevalence, clinical profile, and significance of left ventricular remodeling in the end-stage phase of hypertrophic cardiomyopathy". Circulation. 114 (3): 216–25. doi:10.1161/CIRCULATIONAHA.105.583500. PMID 16831987. Unknown parameter |month= ignored (help)
  5. ↑ Biagini E, Spirito P, Leone O; et al. (2008). "Heart transplantation in hypertrophic cardiomyopathy". Am. J. Cardiol. 101 (3): 387–92. doi:10.1016/j.amjcard.2007.09.085. PMID 18237606. Unknown parameter |month= ignored (help)
  6. ↑ Gajarski R, Naftel DC, Pahl E; et al. (2009). "Outcomes of pediatric patients with hypertrophic cardiomyopathy listed for transplant". J. Heart Lung Transplant. 28 (12): 1329–34. doi:10.1016/j.healun.2009.05.028. PMID 19782603. Unknown parameter |month= ignored (help)
  7. ↑ Towbin JA (2002). "Cardiomyopathy and heart transplantation in children". Curr. Opin. Cardiol. 17 (3): 274–9. PMID 12015478. Unknown parameter |month= ignored (help)
  8. ↑ van der Lee C, Scholzel B, ten Berg JM; et al. (2008). "Usefulness of clinical, echocardiographic, and procedural characteristics to predict outcome after percutaneous transluminal septal myocardial ablation". Am. J. Cardiol. 101 (9): 1315–20. doi:10.1016/j.amjcard.2008.01.003. PMID 18435964. Unknown parameter |month= ignored (help)
  9. ↑ 9.0 9.1 Epstein AE, DiMarco JP, Ellenbogen KA, Estes NAM III, Freedman RA, Gettes LS, Gillinov AM, Gregoratos G, Hammill SC, Hayes DL, Hlatky MA, Newby LK, Page RL, Schoenfeld MH, Silka MJ, Stevenson LW, Sweeney MO. ACC/AHA/HRS 2008 guidelines for device-based therapy of cardiac rhythm abnormalities: executive summary: a report of the American College of Cardiology/American Heart Association Task Force on Practice Guidelines (Writing Committee to Revise the ACC/AHA/NASPE 2002 Guideline Update for Implantation of Cardiac Pacemakers and Antiarrhythmia Devices). Circulation. 2008; 117: 2820–2840. PMID 18483207
  10. ↑ 10.0 10.1 Vardas PE, Auricchio A, Blanc JJ, Daubert JC, Drexler H, Ector H; et al. (2007). "Guidelines for cardiac pacing and cardiac resynchronization therapy. The Task Force for Cardiac Pacing and Cardiac Resynchronization Therapy of the European Society of Cardiology. Developed in collaboration with the European Heart Rhythm Association". Europace. 9 (10): 959–98. doi:10.1093/europace/eum189. PMID 17726043.
  11. ↑ 11.0 11.1 Gersh BJ, Maron BJ, Bonow RO, Dearani JA, Fifer MA, Link MS, Naidu SS, Nishimura RA, Ommen SR, Rakowski H, Seidman CE, Towbin JA, Udelson JE, Yancy CW (2011). "2011 ACCF/AHA Guideline for the Diagnosis and Treatment of Hypertrophic Cardiomyopathy: Executive Summary A Report of the American College of Cardiology Foundation/American Heart Association Task Force on Practice Guidelines Developed in Collaboration With the American Association for Thoracic Surgery, American Society of Echocardiography, American Society of Nuclear Cardiology, Heart Failure Society of America, Heart Rhythm Society, Society for Cardiovascular Angiography and Interventions, and Society of Thoracic Surgeons". Journal of the American College of Cardiology. 58 (25): 2703–38. doi:10.1016/j.jacc.2011.10.825. PMID 22075468. Retrieved 2011-12-19. Unknown parameter |month= ignored (help)
  12. ↑ 12.0 12.1 Maron BJ (2002). "Hypertrophic cardiomyopathy: a systematic review". JAMA. 287 (10): 1308–20. PMID 11886323.
  13. ↑ 13.0 13.1 13.2 Sherrid MV, Chaudhry FA, Swistel DG (2003). "Obstructive hypertrophic cardiomyopathy: echocardiography, pathophysiology, and the continuing evolution of surgery for obstruction". Ann Thorac Surg. 75 (2): 620–32. PMID 12607696.
  14. ↑ Wigle ED, Rakowski H, Kimball BP, Williams WG (1995). "Hypertrophic cardiomyopathy. Clinical spectrum and treatment". Circulation. 92 (7): 1680–92. PMID 7671349.
  15. ↑ Maron BJ, McKenna WJ, Danielson GK, Kappenberger LJ, Kuhn HJ, Seidman CE; et al. (2003). "American College of Cardiology/European Society of Cardiology clinical expert consensus document on hypertrophic cardiomyopathy. A report of the American College of Cardiology Foundation Task Force on Clinical Expert Consensus Documents and the European Society of Cardiology Committee for Practice Guidelines". J Am Coll Cardiol. 42 (9): 1687–713. PMID 14607462.
  16. ↑ Sherrid MV, Barac I, McKenna WJ, Elliott PM, Dickie S, Chojnowska L; et al. (2005). "Multicenter study of the efficacy and safety of disopyramide in obstructive hypertrophic cardiomyopathy". J Am Coll Cardiol. 45 (8): 1251–8. doi:10.1016/j.jacc.2005.01.012. PMID 15837258.
  17. ↑ Morrow AG (1978). "Hypertrophic subaortic stenosis. Operative methods utilized to relieve left ventricular outflow obstruction". J Thorac Cardiovasc Surg. 76 (4): 423–30. PMID 581298.
  18. ↑ Messmer BJ (1994). "Extended myectomy for hypertrophic obstructive cardiomyopathy". Ann Thorac Surg. 58 (2): 575–7. PMID 8067875.
  19. ↑ Schoendube FA, Klues HG, Reith S, Flachskampf FA, Hanrath P, Messmer BJ (1995). "Long-term clinical and echocardiographic follow-up after surgical correction of hypertrophic obstructive cardiomyopathy with extended myectomy and reconstruction of the subvalvular mitral apparatus". Circulation. 92 (9 Suppl): II122–7. PMID 7586394.
  20. ↑ 20.0 20.1 Balaram SK, Sherrid MV, DeRose JJ, Hillel Z, Winson G, Swistel DG. Beyond extended myectomy for hypertrophic cardiomyopathy: The RPR (Resection–Plication–Release) Repair. Annals of Thoracic Surgery 2005; 80:217–23
  21. ↑ McIntosh CL, Maron BJ, Cannon RO, Klues H. Initial results of combined anterior mitral valve plication and ventricular septal myotomy–myectomy for relief of left ventricular outflow obstruction in patients with hypertrophic cardiomyopathy. Circulation 1992; 86:II 60–7
  22. ↑ Nakatani S, Schwammenthal E, Lever HM, Levine RA, Lytle BW, Thomas JD (1996). "New insights into the reduction of mitral valve systolic anterior motion after ventricular septal myectomy in hypertrophic obstructive cardiomyopathy". Am Heart J. 131 (2): 294–300. PMID 8579024.
  23. ↑ Ommen S, Maron B, Olivotto I, Maron M, Cecchi F, Betocchi S, Gersh B, Ackerman M, McCully R, Dearani J, Schaff H, Danielson G, Tajik A, Nishimura R (2005). "Long-term effects of surgical septal myectomy on survival in patients with obstructive hypertrophic cardiomyopathy". J Am Coll Cardiol. 46 (3): 470–6. PMID 16053960.
  24. ↑ Ralph-Edwards A, Woo A, McCrindle B, Shapero J, Schwartz L, Rakowski H, Wigle E, Williams W (2005). "Hypertrophic obstructive cardiomyopathy: comparison of outcomes after myectomy or alcohol ablation adjusted by propensity score". J Thorac Cardiovasc Surg. 129 (2): 351–8. PMID 15678046.


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