Hypertrophic cardiomyopathy - Revision history

Danbot: Add MedicationDose entry (phenylephrine) with SMW annotations

2026-03-20T18:50:35Z

Add MedicationDose entry (phenylephrine) with SMW annotations

@@ Línea 101: / Línea 101: @@
 **''Avoid'' disopyramide alone with out BB or CCB
 **'''Avoid positive inotropic vasopressors''' (dopamine, dobutamine, norepinephrine, epi)
 ==Disposition==

Rossdonaldson1 at 21:35, 7 June 2022

2022-06-07T21:35:45Z

← Revisión anterior		Revisión del 21:35 7 jun 2022
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	==Background==		==Background==
			[[File:Hypertrophic_cardiomyopathy.png\|thumb\|HOCM]]
	*Genetically-linked (AD) hypertrophy of cardiac muscle - can but does not always cause outflow obstruction		*Genetically-linked (AD) hypertrophy of cardiac muscle - can but does not always cause outflow obstruction
	*Up to 30% of patients will have no family history<ref>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 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 of Practice.</ref>		*Up to 30% of patients will have no family history<ref>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 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 of Practice.</ref>
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	*Yamaguchi syndrome, an atypical HCOM, in which only 1% are non-Japanese		*Yamaguchi syndrome, an atypical HCOM, in which only 1% are non-Japanese

	~~[[File:Hypertrophic_cardiomyopathy.png\|thumb\|HOCM]]~~		{{Cardiomyopathy DDX}}

	==Clinical Features==		==Clinical Features==
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	==Differential Diagnosis==		==Differential Diagnosis==
	{{DDX syncope peds}}		{{DDX syncope peds}}
	~~{{Cardiomyopathy DDX}}~~
	{{Chest Pain DDX}}		{{Chest Pain DDX}}

Rossdonaldson1: /* Differential Diagnosis */

2022-05-14T12:30:14Z

Differential Diagnosis

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	==Differential Diagnosis==		==Differential Diagnosis==
			{{DDX syncope peds}}
	{{Cardiomyopathy DDX}}		{{Cardiomyopathy DDX}}

	{{Chest Pain DDX}}		{{Chest Pain DDX}}

Rossdonaldson1: /* Clinical Features */

2022-05-14T11:31:56Z

Clinical Features

← Revisión anterior		Revisión del 11:31 14 may 2022
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	==Clinical Features==		==Clinical Features==
	*[[Syncope]], [[chest pain]], [[dyspnea]]		===History===
			*[[Syncope]], [[chest pain]], [[dyspnea]], dizziness
	*Symptoms made worse by exertion		*Symptoms made worse by exertion
	**Increasing ventricular contractility further narrows outflow as volume is lost		**Increasing ventricular contractility further narrows outflow as volume is lost

			===Physical===
	*Harsh midsystolic crescendo-decrescendo [[murmur]]		*Harsh midsystolic crescendo-decrescendo [[murmur]]
	**Increases in intensity with valsalva and standing up		**Increases in intensity with valsalva and standing up
	***Due to decreased blood return to the heart		***Due to decreased blood return to the heart
	**Decreases with squatting and ~~trandelenburg~~		**Decreases with squatting and Trendelenburg
	***Due to increased peripheral resistance increases aorta and reduces obstruction		***Due to increased peripheral resistance increases aorta and reduces obstruction
	*Symptoms: Syncope or sudden death most common
	**Also exertional dyspnea, chest pain, syncope, dizziness, palpitations, or [[CHF]]

	==Differential Diagnosis==		==Differential Diagnosis==

Rossdonaldson1: /* Diagnosis */

2022-05-14T11:30:11Z

Diagnosis

← Revisión anterior		Revisión del 11:30 14 may 2022
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	===Diagnosis===		===Diagnosis===
	*~~Systolic murmur that increases with maneuvers that decrease preload (e.g. valsalva, transitioning from squatting to standing, etc.).~~		*Typically via echo
	*[[ECG]]
	**Nonspecific/normal.
	**Or, high voltage/LVH, deep narrow Q waves in 1, avL, V5, V6 = "daggers of death"

	==Management==		==Management==

Rossdonaldson1 at 11:29, 14 May 2022

2022-05-14T11:29:13Z

← Revisión anterior		Revisión del 11:29 14 may 2022
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	**Decreases with squatting and trandelenburg		**Decreases with squatting and trandelenburg
	***Due to increased peripheral resistance increases aorta and reduces obstruction		***Due to increased peripheral resistance increases aorta and reduces obstruction
			*Symptoms: Syncope or sudden death most common
			**Also exertional dyspnea, chest pain, syncope, dizziness, palpitations, or [[CHF]]

	==Differential Diagnosis==		==Differential Diagnosis==
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	==Evaluation==		==Evaluation==
	*Symptoms: Syncope or sudden death most common
	**Also exertional dyspnea, chest pain, syncope, dizziness, palpitations, or [[CHF]]
	*Systolic murmur that increases with maneuvers that decrease preload (e.g. valsalva, transitioning from squatting to standing, etc.).
	*[[ECG]]
	**Nonspecific/normal.
	**Or, high voltage/LVH, deep narrow Q waves in 1, avL, V5, V6 = "daggers of death"
	[[File:Needle-like q-waves.JPG\|thumbnail\|Needle-like Q-waves]]		[[File:Needle-like q-waves.JPG\|thumbnail\|Needle-like Q-waves]]
	[[File:HOCM Subramaniam.gif\|thumbnail\|HOCM<ref>http://www.thepocusatlas.com/pediatrics/</ref>]]		[[File:HOCM Subramaniam.gif\|thumbnail\|HOCM<ref>http://www.thepocusatlas.com/pediatrics/</ref>]]

	===Work-Up===		===Work-Up===
	*[[ECG]] abnormalities in ~90%, but mostly nonspecific		*[[ECG]] abnormalities in ~90%, but mostly nonspecific
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	**EF may be preserved until acute decompensation		**EF may be preserved until acute decompensation
	**Formal echo should include tissue Doppler imaging		**Formal echo should include tissue Doppler imaging

			===Diagnosis===
			*Systolic murmur that increases with maneuvers that decrease preload (e.g. valsalva, transitioning from squatting to standing, etc.).
			*[[ECG]]
			**Nonspecific/normal.
			**Or, high voltage/LVH, deep narrow Q waves in 1, avL, V5, V6 = "daggers of death"

	==Management==		==Management==

Elcatracho: /* Pharmacologic Management */

2021-03-06T03:56:08Z

Pharmacologic Management

← Revisión anterior		Revisión del 03:56 6 mar 2021
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	**Reasonable to add oral [[diuretics]] in nonobstructive HCM when symptoms persist despite BB or CCB		**Reasonable to add oral [[diuretics]] in nonobstructive HCM when symptoms persist despite BB or CCB
	*Class III (harm)		*Class III (harm)
	**''Avoid'' nifedipine and other dihydropyridine CCB		**''Avoid'' [[nifedipine]] and other dihydropyridine CCB
	**''Avoid'' ~~digitalis~~		**''Avoid'' [[digoxin]]
	**''Avoid'' disopyramide alone with out BB or CCB		**''Avoid'' disopyramide alone with out BB or CCB
	**'''Avoid positive inotropic vasopressors''' (dopamine, dobutamine, norepinephrine, epi)		**'''Avoid positive inotropic vasopressors''' (dopamine, dobutamine, norepinephrine, epi)

Elcatracho: /* Disposition */

2021-03-06T03:55:04Z

Disposition

← Revisión anterior		Revisión del 03:55 6 mar 2021
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	==Disposition==		==Disposition==
	*Admit for echo and cardiology evaluation		*Admit for echo and cardiology evaluation
	*If unable to admit, home on β-blocker, instructions for no exertion, follow up echo and cardiology ~~outpt~~		*If unable to admit, home on β-blocker, instructions for no exertion, follow up echo and cardiology outpatient

	==See Also==		==See Also==

NadavNahumi: /* Clinical Features */

2020-03-19T01:23:22Z

Clinical Features

← Revisión anterior		Revisión del 01:23 19 mar 2020
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	*Symptoms made worse by exertion		*Symptoms made worse by exertion
	**Increasing ventricular contractility further narrows outflow as volume is lost		**Increasing ventricular contractility further narrows outflow as volume is lost
	*~~Systolic~~ [[murmur]] ~~increases~~ with valsalva		*Harsh midsystolic crescendo-decrescendo [[murmur]]
	**~~Decreased~~ blood return to heart		**Increases in intensity with valsalva and standing up
	**Decreases with ~~squat/fist clench~~		***Due to decreased blood return to the heart
	***~~Increased~~ peripheral resistance increases aorta and reduces obstruction		**Decreases with squatting and trandelenburg
			***Due to increased peripheral resistance increases aorta and reduces obstruction

	==Differential Diagnosis==		==Differential Diagnosis==

ClaireLewis at 15:22, 25 September 2019

2019-09-25T15:22:40Z

← Revisión anterior		Revisión del 15:22 25 sep 2019
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	*Up to 30% of patients will have no family history<ref>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 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 of Practice.</ref>		*Up to 30% of patients will have no family history<ref>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 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 of Practice.</ref>
	*Average age of diagnosis between 30-40 years of age		*Average age of diagnosis between 30-40 years of age
	*Diastolic heart failure		*Diastolic [[heart failure]]
	**Abnormal LV function due to decreased compliance		**Abnormal LV function due to decreased compliance
	*Historically, obstructive forms known as:		*Historically, obstructive forms known as:
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	==Clinical Features==		==Clinical Features==
	*Syncope, chest pain, dyspnea		*[[Syncope]], [[chest pain]], [[dyspnea]]
	*Symptoms made worse by exertion		*Symptoms made worse by exertion
	**Increasing ventricular contractility further narrows outflow as volume is lost		**Increasing ventricular contractility further narrows outflow as volume is lost
	*Systolic murmur increases with valsalva		*Systolic [[murmur]] increases with valsalva
	**Decreased blood return to heart		**Decreased blood return to heart
	*~~Systolic murmur decreases~~ with squat/fist clench		**Decreases with squat/fist clench
	**Increased peripheral resistance increases aorta and reduces obstruction		***Increased peripheral resistance increases aorta and reduces obstruction

	==Differential Diagnosis==		==Differential Diagnosis==
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	==Management==		==Management==
	*Avoid exertion		*Avoid exertion
	*ICD +/- pacer for ventricular ~~arrhythmias~~		*ICD +/- pacer for [[ventricular dysrhythmias]]
	*Definitive treatment is myomectomy		*Definitive treatment is myomectomy

	===Decompensated===		===Decompensated===
	*Consider primary insult, which usually is preload issue (fluid depletion, bleed, etc.)		*Consider primary insult, which usually is preload issue (fluid depletion, bleed, etc.)
	*If decompensated presents as hypotensive CHF		*If decompensated presents as [[cardiogenic shock\|hypotensive CHF]]
	**Preserve preload		**Preserve preload
	***Careful hydration		***Careful hydration
	***Avoid high airway pressure if intubate		***Avoid high airway pressure if intubate
	**Limit tachycardia		**Limit tachycardia
	***Beta blockers		***[[Beta blockers]]
	**Avoid vasodilators (no nitrates)		**Avoid vasodilators (''no'' nitrates)
	**Maintain sinus ~~rythm~~ (i.e. cardiovert A. fib)		**Maintain sinus rhythm (i.e. cardiovert A. fib)
	**Increase afterload (hypotensive only)		**Increase afterload (hypotensive only)
	***Phenylephrine, max dose range depending on source		***[[Phenylephrine]], max dose range depending on source
	****Start at 100 mcg/min (~1.5 mcg/kg/min for average adult)		****Start at 100 mcg/min (~1.5 mcg/kg/min for average adult)
	****Range usually 0.5 - 6.0 mcg/kg/min, with efficacy minimal beyond 6 mcg/kg/min)<ref>Global RPH in reference to Micromedex. 12/2014. http://www.globalrph.com/phenylephrine_dilution.htm</ref>		****Range usually 0.5 - 6.0 mcg/kg/min, with efficacy minimal beyond 6 mcg/kg/min)<ref>Global RPH in reference to Micromedex. 12/2014. http://www.globalrph.com/phenylephrine_dilution.htm</ref>
	****Max phenylephrine infusion before other pressors		****Max phenylephrine infusion before other pressors
	****Fix acid-base disorder as acidosis may prevent pressor efficacy		****Fix acid-base disorder as acidosis may prevent pressor efficacy
	****Consider maxing vasopressin next		****Consider maxing [[vasopressin]] next
	*****Pressor effects more preserved in hypoxia and acidosis<ref>Overgaard CB and Dzavik V. Contemporary Reviews in Cardiovascular Medicine: Inotropes and Vasopressors - Review of Physiology and Clinical Use in Cardiovascular Disease. Circulation. 2008; 118: 1047-1056.</ref>		*****Pressor effects more preserved in hypoxia and acidosis<ref>Overgaard CB and Dzavik V. Contemporary Reviews in Cardiovascular Medicine: Inotropes and Vasopressors - Review of Physiology and Clinical Use in Cardiovascular Disease. Circulation. 2008; 118: 1047-1056.</ref>
	*****Preferred over pressors with beta agonism		*****Preferred over pressors with beta agonism
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	*Per Amer Coll of Cardiology 2011 recommendations<ref>American College of Cardiology. 2011. http://content.onlinejacc.org/article.aspx?articleid=1147838</ref>		*Per Amer Coll of Cardiology 2011 recommendations<ref>American College of Cardiology. 2011. http://content.onlinejacc.org/article.aspx?articleid=1147838</ref>
	*Class I		*Class I
	**beta-blockers for angina or dyspnea in adults in HCM regardless of obstructive physiology - use with caution in sinus brady or conduction abnormality		**[[beta-blockers]] for angina or dyspnea in adults in HCM regardless of obstructive physiology - use with caution in sinus brady or conduction abnormality
	**Titrate BB dose to symptoms, may increase BB dose to resting HR to 60 bpm		**Titrate BB dose to symptoms, may increase BB dose to resting HR to 60 bpm
	**PO ~~verapamil~~ titrated up to 480mg/d if patient unresponsive or cannot tolerate beta-blockers - caution in advanced HF, hypotension, sinus brady, high LVOT gradients		**[[Verapamil]] PO titrated up to 480mg/d if patient unresponsive or cannot tolerate beta-blockers - caution in advanced HF, hypotension, sinus brady, high LVOT gradients
	**'''IV phenylephrine''' for acute hypotension unresponsive to fluids		**'''IV [[phenylephrine]]''' for acute hypotension unresponsive to fluids
	*Class IIa		*Class IIa
	**Reasonable to add disopyramide with BB or verapamil if unresponsive to BB or CCB alone in obstructive HCM		**Reasonable to add disopyramide with BB or verapamil if unresponsive to BB or CCB alone in obstructive HCM
	**Reasonable to add oral diuretics in nonobstructive HCM when symptoms persist despite BB or CCB		**Reasonable to add oral [[diuretics]] in nonobstructive HCM when symptoms persist despite BB or CCB
	*Class III (harm)		*Class III (harm)
	**Avoid nifedipine and other dihydropyridine CCB		**''Avoid'' nifedipine and other dihydropyridine CCB
	**Avoid digitalis		**''Avoid'' digitalis
	**Avoid disopyramide alone with out BB or CCB		**''Avoid'' disopyramide alone with out BB or CCB
	**'''Avoid positive inotropic vasopressors''' (dopamine, dobutamine, norepinephrine, epi)		**'''Avoid positive inotropic vasopressors''' (dopamine, dobutamine, norepinephrine, epi)