Hypertrophic cardiomyopathy in special clinical scenarios
Hypertrophic Cardiomyopathy Microchapters
Hypertrophic cardiomyopathy in special clinical scenarios On the Web
Management of HCM in presence of Hypotension and Cardiovascular Collapse
The first patient 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 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:
- Reduced pre-load which can be due to:
- Venodilators such as nitrates
- Following epidural blockade
- Vasodilator therapy
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:
- Avoid nitrates even though it appears the patient is in heart failure!
- Avoid vasodilators 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)
Asymptomatic Patients (DO NOT EDIT) 
|"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. (Level of Evidence: C) "|
|Class III (Harm)|
|"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. (Level of Evidence: C) "|
|"2. In patients with HCM with resting or provocable outﬂow tract obstruction, regardless of symptom status, pure vasodilators and high-dose diuretics are potentially harmful. (Level of Evidence: C) "|
|"1. Low-intensity aerobic exercise is reasonable as part of a healthy lifestyle for patients with HCM. (Level of Evidence: C) "|
|"1. The usefulness of beta blockade and calcium channel blockers to alter clinical outcome is not well established for the management of asymptomatic patients with HCM with or without obstruction. (Level of Evidence: C) "|
Patients With LV Systolic Dysfunction (DO NOT EDIT)
|"1. Patients with non-obstructive HCM who develop systolic dysfunction with an 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 angiotensin-converting enzyme inhibitors, angiotensin receptor blockers, beta blockers, and other indicated drugs. (Level of Evidence: B) "|
|"2. Other concomitant causes of systolic dysfunction (such as CAD) should be considered as potential contributors to systolic dysfunction in patients with HCM. (Level of Evidence: C) "|
|"1. ICD therapy may be considered in adult patients with advanced (as deﬁned by NYHA functional class III or IV heart failure) non-obstructive HCM, on maximal medical therapy, and EF less than or equal to 50%, who do not otherwise have an indication for an ICD. (Level of Evidence: C) "|
|"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. (Level of Evidence: C) "|
- ↑ 1.0 1.1 1.2 1.3 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 (December 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 on 2011-12-19.
- ↑ 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 (September 2009). "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 of the American College of Cardiology 54 (14): 1364–405. doi:10.1016/j.jacc.2009.08.005. PMID 19778679. Retrieved on 2012-01-12.
- ↑ 3.0 3.1 Maron BJ (March 2002). "Hypertrophic cardiomyopathy: a systematic review". JAMA : the Journal of the American Medical Association 287 (10): 1308–20. PMID 11886323. Retrieved on 2012-01-12.
- ↑ 4.0 4.1 4.2 Maron BJ, McKenna WJ, Danielson GK, Kappenberger LJ, Kuhn HJ, Seidman CE, Shah PM, Spencer WH, Spirito P, Ten Cate FJ, Wigle ED (November 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". Journal of the American College of Cardiology 42 (9): 1687–713. PMID 14607462. Retrieved on 2012-01-12.
- ↑ BRAUNWALD E, LAMBREW CT, ROCKOFF SD, ROSS J, MORROW AG (November 1964). "IDIOPATHIC HYPERTROPHIC SUBAORTIC STENOSIS. I. A DESCRIPTION OF THE DISEASE BASED UPON AN ANALYSIS OF 64 PATIENTS". Circulation 30: SUPPL 4:3–119. PMID 14227306. Retrieved on 2012-01-12.
- ↑ 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 (June 2004). "Recommendations for physical activity and recreational sports participation for young patients with genetic cardiovascular diseases". Circulation 109 (22): 2807–16. doi:10.1161/01.CIR.0000128363.85581.E1. PMID 15184297. Retrieved on 2012-01-12.
- ↑ 7.0 7.1 Harris KM, Spirito P, Maron MS, et al. (July 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.
- ↑ Maron BJ, Spirito P (June 1998). "Implications of left ventricular remodeling in hypertrophic cardiomyopathy". Am. J. Cardiol. 81 (11): 1339–44. PMID 9631972.
- ↑ 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 (December 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 on 2011-12-19.
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