Hypertrophic cardiomyopathy: Difference between revisions
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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 | ||
* | *Harsh midsystolic crescendo-decrescendo [[murmur]] | ||
** | **Increases in intensity with valsalva and standing up | ||
* | ***Due to decreased blood return to the heart | ||
** | **Decreases with squatting and trandelenburg | ||
***Due to increased peripheral resistance increases aorta and reduces obstruction | |||
==Differential Diagnosis== | ==Differential Diagnosis== | ||
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**Also exertional dyspnea, chest pain, syncope, dizziness, palpitations, or [[CHF]] | **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.). | *Systolic murmur that increases with maneuvers that decrease preload (e.g. valsalva, transitioning from squatting to standing, etc.). | ||
*ECG | *[[ECG]] | ||
**Nonspecific/normal. | **Nonspecific/normal. | ||
**Or, high voltage/LVH, deep narrow Q waves in 1, avL, V5, V6 = "daggers of death" | **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: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 | ||
**High voltage | **High voltage ECG (which may produce abnormal T-waves) | ||
**[[LVH]] | **[[LVH]] | ||
**[[Left atrial enlargement]] | **[[Left atrial enlargement]] | ||
**Tall R-wave in V1, mimicking posterior MI | **Tall R-wave in V1, mimicking posterior MI | ||
**"Needle-like" Q waves, especially in lateral leads, mimicking infarction Q waves | **"Needle-like" Q waves, especially in lateral leads, mimicking infarction Q waves | ||
*CXR may be normal as LV is non-dilated | *[[CXR]] may be normal as LV is non-dilated | ||
*Echo<ref>Mitevksa IP. Focus on echocardiography in hypertrophic cardiomyopathy - fourth in series. ESC Council for Cardiology Practice. Vol.13,N°20 - 14 Apr 2015.</ref> | *Echo<ref>Mitevksa IP. Focus on echocardiography in hypertrophic cardiomyopathy - fourth in series. ESC Council for Cardiology Practice. Vol.13,N°20 - 14 Apr 2015.</ref> | ||
**Unexplained wall thickness > 15 mm in any myocardial segment | **Unexplained wall thickness > 15 mm in any myocardial segment | ||
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==Management== | ==Management== | ||
*Avoid exertion | *Avoid exertion | ||
*ICD +/- pacer for ventricular | *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 | **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 | ||
Line 86: | Line 88: | ||
*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]] 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 | **''Avoid'' [[digoxin]] | ||
**Avoid disopyramide alone with out BB or CCB | **''Avoid'' disopyramide alone with out BB or CCB | ||
**'''Avoid positive inotropic vasopressors''' (dopamine, dobutamine, | **'''Avoid positive inotropic vasopressors''' (dopamine, dobutamine, norepinephrine, epi) | ||
==Disposition== | ==Disposition== | ||
*Admit | *Admit for echo and cardiology evaluation | ||
*If unable to admit, home on β-blocker, instructions for no exertion, follow up echo and cardiology outpatient | |||
==See Also== | ==See Also== |
Revision as of 03:56, 6 March 2021
Background
- 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[1]
- Average age of diagnosis between 30-40 years of age
- Diastolic heart failure
- Abnormal LV function due to decreased compliance
- Historically, obstructive forms known as:
- Idiopathic hypertrophic subaortic stenosis (IHSS)
- Asymmetric septal hypertrophy (ASH)
- Hypertrophic obstructive cardiomyopathy (HOCM)
- Yamaguchi syndrome, an atypical HCOM, in which only 1% are non-Japanese
Clinical Features
- Syncope, chest pain, dyspnea
- Symptoms made worse by exertion
- Increasing ventricular contractility further narrows outflow as volume is lost
- Harsh midsystolic crescendo-decrescendo murmur
- Increases in intensity with valsalva and standing up
- Due to decreased blood return to the heart
- Decreases with squatting and trandelenburg
- Due to increased peripheral resistance increases aorta and reduces obstruction
- Increases in intensity with valsalva and standing up
Differential Diagnosis
Cardiomyopathy
- Dilated cardiomyopathy
- Hypertrophic cardiomyopathy
- Restrictive cardiomyopathy
- Peripartum cardiomyopathy
- Takotsubo cardiomyopathy
- Arrhythmogenic right ventricular dysplasia
Chest pain
Critical
- Acute coronary syndromes (ACS)
- Aortic dissection
- Cardiac tamponade
- Coronary artery dissection
- Esophageal perforation (Boerhhaave's syndrome)
- Pulmonary embolism
- Tension pneumothorax
Emergent
- Cholecystitis
- Cocaine-associated chest pain
- Mediastinitis
- Myocardial rupture
- Myocarditis
- Pancreatitis
- Pericarditis
- Pneumothorax
Nonemergent
- Aortic stenosis
- Arthritis
- Asthma exacerbation
- Biliary colic
- Costochondritis
- Esophageal spasm
- Gastroesophageal reflux disease
- Herpes zoster / Postherpetic Neuralgia
- Hypertrophic cardiomyopathy
- Hyperventilation
- Mitral valve prolapse
- Panic attack
- Peptic ulcer disease
- Pleuritis
- Pneumomediastinum
- Pneumonia
- Rib fracture
- Stable angina
- Thoracic outlet syndrome
- Valvular heart disease
- Muscle sprain
- Psychologic / Somatic Chest Pain
- Spinal Root Compression
- Tumor
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"
Work-Up
- ECG abnormalities in ~90%, but mostly nonspecific
- High voltage ECG (which may produce abnormal T-waves)
- LVH
- Left atrial enlargement
- Tall R-wave in V1, mimicking posterior MI
- "Needle-like" Q waves, especially in lateral leads, mimicking infarction Q waves
- CXR may be normal as LV is non-dilated
- Echo[3]
- Unexplained wall thickness > 15 mm in any myocardial segment
- Septal/posterior wall thickness ratio:
- >1.3 in normotensive pts
- >1.5 in hypertensive pts
- Systolic anterior motion by M mode in PSL window, worsened by Valsalva
- Mitral regurgitation
- EF may be preserved until acute decompensation
- Formal echo should include tissue Doppler imaging
Management
- Avoid exertion
- ICD +/- pacer for ventricular dysrhythmias
- Definitive treatment is myomectomy
Decompensated
- Consider primary insult, which usually is preload issue (fluid depletion, bleed, etc.)
- If decompensated presents as hypotensive CHF
- Preserve preload
- Careful hydration
- Avoid high airway pressure if intubate
- Limit tachycardia
- Avoid vasodilators (no nitrates)
- Maintain sinus rhythm (i.e. cardiovert A. fib)
- Increase afterload (hypotensive only)
- Phenylephrine, max dose range depending on source
- 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)[4]
- Max phenylephrine infusion before other pressors
- Fix acid-base disorder as acidosis may prevent pressor efficacy
- Consider maxing vasopressin next
- Pressor effects more preserved in hypoxia and acidosis[5]
- Preferred over pressors with beta agonism
- Push dose pressor while waiting for IV drip:
- Place 1mL of 10mg/mL phenylephrine in 100mL NS
- Final concentration 100mcg/mL
- Use 1-2mL q2-5min (100-200mcg) in 10mL syringe
- Onset 1min; duration 20min
- Phenylephrine, max dose range depending on source
- Preserve preload
Pharmacologic Management
- Per Amer Coll of Cardiology 2011 recommendations[6]
- 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
- Titrate BB dose to symptoms, may increase BB dose to resting HR to 60 bpm
- 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
- Class IIa
- 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
- Class III (harm)
- Avoid nifedipine and other dihydropyridine CCB
- Avoid digoxin
- Avoid disopyramide alone with out BB or CCB
- Avoid positive inotropic vasopressors (dopamine, dobutamine, norepinephrine, epi)
Disposition
- Admit for echo and cardiology evaluation
- If unable to admit, home on β-blocker, instructions for no exertion, follow up echo and cardiology outpatient
See Also
References
- ↑ 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.
- ↑ http://www.thepocusatlas.com/pediatrics/
- ↑ Mitevksa IP. Focus on echocardiography in hypertrophic cardiomyopathy - fourth in series. ESC Council for Cardiology Practice. Vol.13,N°20 - 14 Apr 2015.
- ↑ Global RPH in reference to Micromedex. 12/2014. http://www.globalrph.com/phenylephrine_dilution.htm
- ↑ 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.
- ↑ American College of Cardiology. 2011. http://content.onlinejacc.org/article.aspx?articleid=1147838