Hypertrophic cardiomyopathy in special clinical scenarios: Difference between revisions

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(New page: {{SI}} '''Editors-In-Chief:''' C. Michael Gibson, M.S., M.D. [mailto:mgibson@perfuse.org], Cafer Zorkun, M.D. [mailto:zorkun@perfuse.org], Caitlin J. Harrigan [mailto:charrigan@perfuse.or...)
 
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*Outside of the US, intravenous [[disopyramide]] at a dose of 50 mg over one to five minutes can be administered.
*Outside of the US, intravenous [[disopyramide]] at a dose of 50 mg over one to five minutes can be administered.


==Management of the HCM Patient During Pregnancy==
Among HCM patients who chronically have mild symptoms, pregnancy is generally well tolerated <ref name="pmid572730">{{cite journal | author = Oakley GD, McGarry K, Limb DG, Oakley CM | title = Management of pregnancy in patients with hypertrophic cardiomyopathy | journal = [[British Medical Journal]] | volume = 1 | issue = 6180 | pages = 1749–50 | year = 1979 | month = June | pmid = 572730 | pmc = 1599373 | doi = | url = | issn = }}</ref><ref name="pmid12446072">{{cite journal | author = Autore C, Conte MR, Piccininno M, Bernabò P, Bonfiglio G, Bruzzi P, Spirito P | title = Risk associated with pregnancy in hypertrophic cardiomyopathy | journal = [[Journal of the American College of Cardiology]] | volume = 40 | issue = 10 | pages = 1864–9 | year = 2002 | month = November | pmid = 12446072 | doi = | url = http://linkinghub.elsevier.com/retrieve/pii/S0735109702024956 | issn = }}</ref>. Although pregnancy causes vasodilation which should exacerbate the outflow gradient, pregnancy also causes fluid retention and an increase in plasma volume which increases preload and offsets the reduction in afterload. In a series of 100 HCM patients, only one of 28 asymptomatic patients developed NYHA Class III or IV [[heart failure]]. Among 12 previously symptomatic patients, 5 patients developed NYHA Class III or IV [[heart failure]]. It is notable that two [[sudden deaths]] occurred in this series of 100 patients. One of the two patients had a resting gradient of 115 mm Hg. The other patient had a markedly positive family history with 8 family members sustaining any early death, 5 of which were sudden death <ref name="pmid12446072">{{cite journal | author = Autore C, Conte MR, Piccininno M, Bernabò P, Bonfiglio G, Bruzzi P, Spirito P | title = Risk associated with pregnancy in hypertrophic cardiomyopathy | journal = [[Journal of the American College of Cardiology]] | volume = 40 | issue = 10 | pages = 1864–9 | year = 2002 | month = November | pmid = 12446072 | doi = | url = http://linkinghub.elsevier.com/retrieve/pii/S0735109702024956 | issn = }}</ref>.


Although beta blockers and verapamil may improve symptoms in the mother, the dosing should be limited to minimize the risk of fetal [[bradycardia]], growth retardation and [[hypoglycemia]], and growth retardation. There is more experience with the use beta blockers during pregnancy.
Due to the potential for venous pooling, <u>'''''epidural anesthesia should be avoided.'''''</u>. Blood should be crossed and typed in case a transfusion is needed for bleeding, which can exacerbate outflow obstruction. Home delivery without IV access is not preferred. Vaginal delivery is usually successful.


==References==
==References==

Revision as of 02:59, 8 August 2011

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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.

Please Take Over This Page and Apply to be Editor-In-Chief for this topic: There can be one or more than one Editor-In-Chief. You may also apply to be an Associate Editor-In-Chief of one of the subtopics below. Please mail us [4] to indicate your interest in serving either as an Editor-In-Chief of the entire topic or as an Associate Editor-In-Chief for a subtopic. Please be sure to attach your CV and or biographical sketch.

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:
  • Vomiting
  • Diuretics
  • Hemorrhage
  • Reduced pre-load which can be due to:
  • Sepsis
  • Venodilators such as nitrates
  • Following epidural blockade
  • 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:

  • 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.


References

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