QuestionSBA
QuestionChoice
There is no indication of ST-elevation on this ECG. A STEMI will cause ST elevation acutely, but it is important to note that the ECG changes will evolve over time
31759
b
ST-elevation myocardial infarction (STEMI)
QuestionChoice
Acute pericarditis is associated with global concave ST elevation and PR depression, which is not seen on this ECG
31760
c
Acute pericarditis
QuestionChoice
This ECG shows left ventricular hypertrophy (increased amplitude of the QRS complex, particularly noticeable in V1-V4) and non-specific T-wave inversion. In an otherwise young, healthy person, the most likely cause of left ventricular hypertrophy is HCM. This would also explain his symptoms of chest pain and shortness of breath on exertion. He should be referred for an urgent cardiology review for consideration of an implantable cardioverter-defibrillator (ICD)
31758
a
Hypertrophic cardiomyopathy (HCM)
QuestionChoice
Hyperkalaemia is classically associated with "tall tented T waves" on an ECG, which are not seen on this ECG
31761
d
Hyperkalaemia
QuestionChoice
RBBB is associated with a QRS duration of >120ms and an RSR' pattern in V1-V3 ("M-shaped" as in "WiLLiaM MaRRoW"), which is not seen on this ECG
31762
e
Right bundle branch block (RBBB)
QuestionComment
can't see all of the ecg

12476
User
subscriber
Bonello's Bad Boys
QuestionComment
Doesnt seem like an LVH on ecg
53414
QuestionComment
agreed - should be deep S waves in V1/V2 and tall R waves in V5/V6
53492
User
subscriber
Lymph Neoplasia
User
subscriber
Supine Bladder
Concept
Chapter
# Summary

Hypertrophic cardiomyopathy (HCM) is an autosomal dominant condition characterised by left ventricular hypertrophy without an apparent cause. It is a common inherited cardiac condition and a significant cause of sudden cardiac death in young adults. HCM is attributed to mutations in genes encoding sarcomere proteins leading to chaotic and disorganized myocytes. HCM may be asymptomatic but can also lead to exertional syncope, dyspnoea, chest pain and heart failure. Definitive diagnosis is made via echocardiogram. Management involves lifestyle modifications, medication for symptom control, surgical or interventional procedures for severe cases, and consideration of implantable cardioverter-defibrillators in those at risk of sudden cardiac death. Prognosis varies, with an annual mortality rate of 1.1%, and a 20-year survival rate estimated at 81% after diagnosis.

# Definition 

Hypetrophic cardiomyopathy (HCM) is an autosomal dominant condition characterised by asymmetrical septal hypertrophy leading to left ventricular hypetrophy and diastolic dysfunction in the absence of an obvious cause (e.g. hypertension). 

# Epidemiology 

HCM is estimated to impact 1 in 500 people. It is the most common inherited cardiac condition. Inheritance of HCM is in an autosomal dominant fashion. However, 50% of cases result due to sporadic mutations. Most of the hypertrophy develops during childhood and adolescent, however, genetic variation means late-onset disease can occur.

# Pathophysiology

HCM is attributed to mutations in one or a number of genes that encode for sarcomere proteins. A common mutation is in the beta-myosin heavy chain gene leading to myocyte hypetrophy with chaotic and disorganised myocytes.  

# Symptoms

* May be asymptomatic 
* Or can present with symptoms of left ventricular outflow obstruction, pulmonary congestion or heart failure: 
	* Exertional syncope 
	* Pre-syncope/syncope
	* Sudden cardiac death 
	* Exertional dyspnoea 
	* Fatigue
	* Chest pain: may be anginal (due to decreased blood flow through the coronary arteries) or atypical. 

# Signs

Physical examination can often be normal or non-specific, however typical findings may include:

* "Jerky" pulse
* Double apex beat
* Harsh ejection systolic murmur
* Apical thrill
* A wave in JVP 

# Differential Diagnoses

* Aortic Stenosis 
	* Similarities: both may have an ejection systolic murmur and present similarly with chest pain, exertional syncope and dyspnoea. 
	* Differences: can be difficult to delineate clinically, but demographics may be very different. Aortic stenosis tends to affect older patients, whilst HOCM is likely to be found in younger patients. 

* Hypertensive Disease 
	* Similarities: chronic high blood pressure can lead to left ventricular hypertrophy. 
	* Differences: echocardiogram reveals asymmetrical left ventricular hypertrophy in HOCM. Family history and patient demographics may also suggest HOCM over hypertensive disease. 

* Supravalvular Aortic Stenosis: 
	* Similarities: both may have an ejection systolic murmur. 
	* Differences: supravalvular aortic stenosis is a congenital cardiac condition whereas HOCM tends to develop over time. Supravalvular  stenosis can be distinguished from HOCM on echocardiography due to the level of obstruction either above or below the aortic valve respectively. 

# Investigations

**ECG** typically demonstrates: 

* Abnormal Q waves
* Deep T wave inversion 
* LVH 

**Echocardiogram: definitive diagnosis** 

* HCM is reliably diagnosed on echocardiogram. 
* Findings of HOCM on echocardiogram can be remembered with the mnemonic 'MR SAM ASH'. 
	* **M**itral **R**egurgitation
	* **S**ystolic **A**nterior **M**otion of the mitral valve leaflets 
	* **A**symmetrical **S**eptal **H**ypertrophy. 

**Genetic testing:** there is a role for genetic testing in HCM in families where there are known cases of HCM. 

# Management

## Conservative 

Many patients with HCM do not have any symptoms and have a normal life expectancy. They are often counselled on not undertaking particularly stressful exercise or competitive athletics.  

## Medical 

Medical treatment of HCM remains symptoms control. 
**1st line: beta-blockers** to reduce palpitations symptoms, ectopic beats and are anti-anginal. 

Other medications that can be used are: non-dihydropyridine calcium channel blockers (verapamil), anti-arrhythmics (amiodarone) and anticoagulation (for AF). 

## Surgical and Interventional

Management of the septal hypetrophy in those with severe left ventricular outflow tract obstruction (LVOTO) or symptoms that are refractory to medical management. 

* Surgical septal myectomy
* Alcohol septal ablation

For those at significant risk of sudden cardiac death an ICD may be inserted to mitigate this risk. 

# Complications

The abnormal morphology of the left ventricle can cause severe consequences for heart function: 

* Left ventricular outflow tract obstruction (LVOTO)
* Diastolic dysfunction: HFpEF
* Ischaemia 
* Mitral regurgitation: MR SAM ASH on echocardiogram 

# Prognosis 

The annual mortality rate for HCM is 1.1% of people per year and the HCM survival rate is estimated to be 81% at 20 years after diagnosis. 

# NICE Guidelines

[NICE Guidelines on Non-Surgical Reduction of the Myocardium in HCM](<https://www.nice.org.uk/guidance/ipg40>)

# References

[Patient UK HCM Summary](<https://patient.info/doctor/hypertrophic-cardiomyopathy-pro>)

[American Heart Association Article on HCM](<https://www.ahajournals.org/doi/full/10.1161/circresaha.116.309348>)
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640
Hypertrophic cardiomyopathy
Topic
35
Cardiology
6352
Hypertrophic obstructive cardiomyopathy (HOCM) often presents on ECG with left ventricular hypertrophy (increased amplitude of the QRS complex, especially in leads V1-V4) and non-specific T-wave inversion, indicating the presence of abnormal heart muscle.
Picture
355
HOCM ECG.jpg
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dkgCBICUh3eIh3iAh4mahc+H+A==
Topic
35
Cardiology
An 18 year old man attends to his GP with chest pain and shortness of breath on exertion. He has no significant past medical history. An ECG is performed:

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Which of the following is the most likely to account for the ECG findings?
a