View source for Hypertrophic cardiomyopathy in special clinical scenarios

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{{Hypertrophic cardiomyopathy}}

'''Editor-In-Chief:''' [[C. Michael Gibson, M.S., M.D.]] [mailto:charlesmichaelgibson@gmail.com];{{AOEIC}} {{LG}} 

==Overview==

==Management of HCM in presence of Hypotension and Cardiovascular Collapse==
The first patient [[C. Michael Gibson, M.S., M.D.|I (C. Michael Gibson, M.D.)]] treated as a medical student was an 18 year old woman who had [[HOCM]]. She had just entered college and had partied throughout the night. She was [[vomiting]], developed new [[atrial fibrillation]] at a rate of 180 beats per minute. She had a [[syncope|syncopal episode]] and had a [[systolic blood pressure]] of 60 mm Hg. This young lady had sustained hemodynamic collapse as a result of volume depletion and [[tachycardia]].

====Precipitants of Hemodynamic Collapse====
*Volume depletion or dehydration which can be due to:
:*Vomiting
:*Diuretics
:*Hemorrhage
*Reduced pre-load which can be due to:
:*Sepsis
:*Venodilators such as nitrates
:*Following epidural blockade
*[[Tachycardia]]
*Withdrawal of [[beta-blockers]] or [[calcium channel blockers]]
*Decreased afterload due to:
:*Vasodilator therapy
:*Sepsis

====Physical examination Findings in Hemodynamic Collapse====
A rapid, weak pulse is present in the patient who is hypotensive. The [[JVP]] is flat. A systolic murmur is present.

====Echocardiographic Findings in Hemodynamic Collapse====
*A small hypercontractile left ventricle is present

*Prolonged systolic anterior motion of the mitral valve is present

*Mitral regurgitation with a posterior directed jet

====Treatment of Hemodynamic Collapse====
Initial treatment includes the following:
*<u>'''''Avoid nitrates'''''</u> even though it appears the patient is in heart failure!
*<u>'''''Avoid vasodilators'''''</u> again even though it appears the patient is in heart failure! Both these agents could cause further hemodynamic compromise.
*Administer beta-blockers to slow the heart rate and fluids to raise the left ventricular filling pressures.
*Elevate the legs to increase venous return and raise the preload

If the patient does not respond to these measures, then the following can also be administered:
*Intravenous phenylephrine at a rate of 100 to 180 µg/min, which is then reduced to 2 to 3 mL/min (40 to 60 drops/min).
:*How to mix the phenylephrine: Make a solution that contains 10 mg (1 mL of 1 percent phenylephrine) of phenylephrine diluted in 500 mL of D5W. Administer at a rate of 5 to 9 mL/min (i.e. 100 to 180 drops/min assuming there are 20 drops/mL). This solution provides a phenylephrine drip of 100 to 180 µg/min.
*Outside of the US, intravenous [[disopyramide]] at a dose of 50 mg over one to five minutes can be administered.

==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>==

=== Asymptomatic Patients (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.''' For patients with [[HCM]], it is recommended that comorbidities that may contribute to cardiovascular disease (e.g., [[hypertension]], [[diabetes]], [[hyperlipidemia]], [[obesity]]) be treated in compliance with relevant existing guidelines.<ref name="pmid19778679">{{cite journal |author=Redberg RF, Benjamin EJ, Bittner V, Braun LT, Goff DC, Havas S, Labarthe DR, Limacher MC, Lloyd-Jones DM, Mora S, Pearson TA, Radford MJ, Smetana GW, Spertus JA, Swegler EW |title=ACCF/AHA 2009 performance measures for primary prevention of cardiovascular disease in adults: a report of the American College of Cardiology Foundation/American Heart Association Task Force on Performance Measures (Writing Committee to Develop Performance Measures for Primary Prevention of Cardiovascular Disease) developed in collaboration with the American Academy of Family Physicians; American Association of Cardiovascular and Pulmonary Rehabilitation; and Preventive Cardiovascular Nurses Association: endorsed by the American College of Preventive Medicine, American College of Sports Medicine, and Society for Women's Health Research |journal=[[Journal of the American College of Cardiology]] |volume=54 |issue=14 |pages=1364–405 |year=2009 |month=September |pmid=19778679 |doi=10.1016/j.jacc.2009.08.005 |url=http://linkinghub.elsevier.com/retrieve/pii/S0735-1097(09)02609-6 |accessdate=2012-01-12}}</ref> ''([[ACC AHA guidelines classification scheme#Level of Evidence|Level of Evidence: C]])'' <nowiki>"</nowiki>
|}

{| 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.''' Low-intensity aerobic exercise is reasonable as part of a healthy lifestyle for patients with [[HCM]].<ref name="pmid15184297">{{cite journal |author=Maron BJ, Chaitman BR, Ackerman MJ, Bayés de Luna A, Corrado D, Crosson JE, Deal BJ, Driscoll DJ, Estes NA, Araújo CG, Liang DH, Mitten MJ, Myerburg RJ, Pelliccia A, Thompson PD, Towbin JA, Van Camp SP |title=Recommendations for physical activity and recreational sports participation for young patients with genetic cardiovascular diseases |journal=[[Circulation]] |volume=109 |issue=22 |pages=2807–16 |year=2004 |month=June |pmid=15184297 |doi=10.1161/01.CIR.0000128363.85581.E1 |url=http://circ.ahajournals.org/cgi/pmidlookup?view=long&pmid=15184297 |accessdate=2012-01-12}}</ref><ref name="pmid14607462">{{cite journal |author=Maron BJ, McKenna WJ, Danielson GK, Kappenberger LJ, Kuhn HJ, Seidman CE, Shah PM, Spencer WH, Spirito P, Ten Cate FJ, Wigle ED |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=[[Journal of the American College of Cardiology]] |volume=42 |issue=9 |pages=1687–713 |year=2003 |month=November |pmid=14607462 |doi= |url=http://linkinghub.elsevier.com/retrieve/pii/S0735109703009410 |accessdate=2012-01-12}}</ref> ''([[ACC AHA guidelines classification scheme#Level of Evidence|Level of Evidence: C]])'' <nowiki>"</nowiki>

|}

{| class="wikitable"
|-
| colspan="1" style="text-align:center; background:LemonChiffon"| [[ACC AHA guidelines classification scheme#Classification of Recommendations|Class IIb]]
|-
| bgcolor="LemonChiffon"|<nowiki>"</nowiki>'''1.''' The usefulness of [[Beta blockers|beta blockade]] and [[Calcium channel blocker|calcium channel blockers]] to alter clinical outcome is not well established for the management of asymptomatic patients with [[HCM]] with or without obstruction.<ref name="pmid14607462">{{cite journal |author=Maron BJ, McKenna WJ, Danielson GK, Kappenberger LJ, Kuhn HJ, Seidman CE, Shah PM, Spencer WH, Spirito P, Ten Cate FJ, Wigle ED |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=[[Journal of the American College of Cardiology]] |volume=42 |issue=9 |pages=1687–713 |year=2003 |month=November |pmid=14607462 |doi= |url=http://linkinghub.elsevier.com/retrieve/pii/S0735109703009410 |accessdate=2012-01-12}}</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 performed for asymptomatic adult and pediatric patients with [[HCM]] with normal effort tolerance regardless of the severity of obstruction.<ref name="pmid11886323">{{cite journal |author=Maron BJ |title=Hypertrophic cardiomyopathy: a systematic review |journal=[[JAMA : the Journal of the American Medical Association]] |volume=287 |issue=10 |pages=1308–20 |year=2002 |month=March |pmid=11886323 |doi= |url=http://jama.ama-assn.org/cgi/pmidlookup?view=long&pmid=11886323 |accessdate=2012-01-12}}</ref><ref name="pmid14607462">{{cite journal |author=Maron BJ, McKenna WJ, Danielson GK, Kappenberger LJ, Kuhn HJ, Seidman CE, Shah PM, Spencer WH, Spirito P, Ten Cate FJ, Wigle ED |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=[[Journal of the American College of Cardiology]] |volume=42 |issue=9 |pages=1687–713 |year=2003 |month=November |pmid=14607462 |doi= |url=http://linkinghub.elsevier.com/retrieve/pii/S0735109703009410 |accessdate=2012-01-12}}</ref> ''([[ACC AHA guidelines classification scheme#Level of Evidence|Level of Evidence: C]])'' <nowiki>"</nowiki>
|-
| bgcolor="LightCoral"|<nowiki>"</nowiki>'''2.''' In patients with [[HCM]] with resting or provocable outﬂow tract obstruction, regardless of symptom status, pure [[vasodilators]] and [[diuretics|high-dose diuretics]] are potentially harmful.<ref name="pmid11886323">{{cite journal |author=Maron BJ |title=Hypertrophic cardiomyopathy: a systematic review |journal=[[JAMA : the Journal of the American Medical Association]] |volume=287 |issue=10 |pages=1308–20 |year=2002 |month=March |pmid=11886323 |doi= |url=http://jama.ama-assn.org/cgi/pmidlookup?view=long&pmid=11886323 |accessdate=2012-01-12}}</ref><ref name="pmid14227306">{{cite journal |author=BRAUNWALD E, LAMBREW CT, ROCKOFF SD, ROSS J, MORROW AG |title=IDIOPATHIC HYPERTROPHIC SUBAORTIC STENOSIS. I. A DESCRIPTION OF THE DISEASE BASED UPON AN ANALYSIS OF 64 PATIENTS |journal=[[Circulation]] |volume=30 |issue= |pages=SUPPL 4:3–119 |year=1964 |month=November |pmid=14227306 |doi= |url= |accessdate=2012-01-12}}</ref> ''([[ACC AHA guidelines classification scheme#Level of Evidence|Level of Evidence: C]])'' <nowiki>"</nowiki>
|}

===Patients With LV Systolic Dysfunction (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.''' Patients with non-obstructive [[HCM]] who develop [[systolic dysfunction]] with an [[EF|EF less than or equal to 50%]] should be treated according to evidence-based medical therapy for adults with other forms of heart failure with reduced EF, including [[ACEIs|angiotensin-converting enzyme inhibitors]], [[angiotensin receptor blockers]], [[beta blockers]], and other indicated drugs<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="pmid9631972">{{cite journal |author=Maron BJ, Spirito P |title=Implications of left ventricular remodeling in hypertrophic cardiomyopathy |journal=Am. J. Cardiol. |volume=81 |issue=11 |pages=1339–44 |year=1998 |month=June |pmid=9631972 |doi= |url=}}</ref>. ''([[ACC AHA guidelines classification scheme#Level of Evidence|Level of Evidence: B]])'' <nowiki>"</nowiki>
|-
| bgcolor="LightGreen"|<nowiki>"</nowiki>'''2.''' Other concomitant causes of [[systolic dysfunction]] (such as [[CAD]]) should be considered as potential contributors to [[systolic dysfunction]] in patients with [[HCM]]. ''([[ACC AHA guidelines classification scheme#Level of Evidence|Level of Evidence: C]])'' <nowiki>"</nowiki>
|}

{|class="wikitable"
|-
| colspan="1" style="text-align:center; background:LemonChiffon"|[[ACC AHA guidelines classification scheme#Classification of Recommendations|Class IIb]]

|-
|bgcolor="LemonChiffon"|<nowiki>"</nowiki>'''1.''' [[Hypertrophic cardiomyopathy interventional cardiology#Automatic Implantable Cardiac Defibrillator (AICD) placement|ICD therapy]] may be considered in adult patients with advanced (as deﬁned by [[NYHA classification|NYHA functional class III or IV heart failure]]) non-obstructive [[HCM]], on maximal medical therapy, and [[EF|EF less than or equal to 50%]], who do not otherwise have an indication for an ICD<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>. ''([[ACC AHA guidelines classification scheme#Level of Evidence|Level of Evidence: C]])'' <nowiki>"</nowiki>

|-
|bgcolor="LemonChiffon"|<nowiki>"</nowiki>'''2.''' For patients with [[HCM]] who develop [[systolic dysfunction]], it may be reasonable to reassess the use of negative inotropic agents previously indicated, for example, [[verapamil]], [[diltiazem]], or [[disopyramide]], and to consider discontinuing those therapies. ''([[ACC AHA guidelines classification scheme#Level of Evidence|Level of Evidence: C]])'' <nowiki>"</nowiki>
|}

==Sources==
[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>

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

[[Category:Cardiomyopathy]]
[[Category:Cardiology]]

[[Category:Disease]]
[[Category:Overview complete]]
[[Category:Template complete]]

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