Hypertrophic Cardiomyopathy HCM

Question 1

What is hypertrophic cardiomyopathy (HCM)?

Answer 1

hypertrophic cardiomyopathy is a genetic condition characterised by abnormal thickening (hypertrophy) of the myocardium, often involving the interventricular septum.

Question 2

What is the primary cause of hypertrophic cardiomyopathy?

Answer 2

hypertrophic cardiomyopathy is caused by mutations in genes encoding sarcomeric proteins, inherited in an autosomal dominant pattern.

Question 3

How does hypertrophic cardiomyopathy affect cardiac function?

Answer 3

The hypertrophied myocardium leads to diastolic dysfunction, left ventricular outflow tract (LVOT) obstruction, and increased risk of arrhythmias.

Question 4

What are the typical symptoms of hypertrophic cardiomyopathy?

Answer 4

Symptoms include chest pain, exertional dyspnoea, palpitations, syncope, and, in some cases, sudden cardiac death.

Question 5

What are the risk factors for hypertrophic cardiomyopathy?

Answer 5

Family history of hypertrophic cardiomyopathy, genetic predisposition, and certain mutations in sarcomeric protein genes.

Question 6

What is the pathophysiology of LVOT obstruction in hypertrophic cardiomyopathy?

Answer 6

LVOT obstruction occurs due to systolic anterior motion of the mitral valve, which impinges on the hypertrophied septum, obstructing blood flow.

Question 7

How is hypertrophic cardiomyopathy diagnosed?

Answer 7

Diagnosis is based on clinical features, echocardiography, ECG, and genetic testing in some cases.

Question 8

What are the common ECG findings in hypertrophic cardiomyopathy?

Answer 8

ECG may show left ventricular hypertrophy, deep T-wave inversions, ST-segment changes, and abnormal Q waves.

Question 9

What is the role of echocardiography in hypertrophic cardiomyopathy?

Answer 9

Echocardiography assesses the degree and distribution of hypertrophy, presence of LVOT obstruction, and diastolic dysfunction.

Question 10

What are the potential complications of hypertrophic cardiomyopathy?

Answer 10

Complications include sudden cardiac death, arrhythmias, heart failure, and thromboembolism.

Question 11

What is the prevalence of hypertrophic cardiomyopathy?

Answer 11

hypertrophic cardiomyopathy is relatively common, affecting approximately 1 in 500 people in the general population.

Question 12

How does hypertrophic cardiomyopathy increase the risk of sudden cardiac death (SCD)?

Answer 12

Sudden Cardiac Death in hypertrophic cardiomyopathy is often caused by ventricular arrhythmias or sudden haemodynamic collapse due to LVOT obstruction.

Question 13

What are the red flags for Sudden Cardiac Death in patients with hypertrophic cardiomyopathy?

Answer 13

Family history of Sudden Cardiac Death, unexplained syncope, severe hypertrophy (>30 mm), and non-sustained ventricular tachycardia on Holter monitoring.

Question 14

What are the differential diagnoses for hypertrophic cardiomyopathy?

Answer 14

Differential diagnoses include athletic heart syndrome, hypertensive heart disease, and aortic stenosis.

Question 15

What are the medical management options for hypertrophic cardiomyopathy?

Answer 15

Medications include beta-blockers, calcium channel blockers (e.g., verapamil), and disopyramide to reduce symptoms and outflow obstruction.

Question 16

What lifestyle modifications are recommended for hypertrophic cardiomyopathy patients?

Answer 16

Avoid strenuous exercise, maintain hydration, and avoid dehydration or vasodilators that can worsen LVOT obstruction.

Question 17

What is the role of implantable cardioverter-defibrillators (ICDs) in hypertrophic cardiomyopathy?

Answer 17

ICDs are used for primary or secondary prevention of sudden cardiac death in high-risk patients.

Question 18

When is septal myectomy indicated in hypertrophic cardiomyopathy?

Answer 18

Septal myectomy is considered in patients with severe left ventricular outflow tract (LVOT) obstruction and refractory symptoms despite medical therapy.

Question 19

What is alcohol septal ablation, and when is it used?

Answer 19

Alcohol septal ablation is a minimally invasive procedure to reduce septal thickness, used as an alternative to surgical myectomy in some patients.

Question 20

What are the key findings on physical examination in hypertrophic cardiomyopathy?

Answer 20

A harsh crescendo-decrescendo systolic murmur, best heard at the left sternal edge, which may increase with Valsalva manoeuvre.

Question 21

How is the murmur of hypertrophic cardiomyopathy distinguished from aortic stenosis?

Answer 21

The murmur of hypertrophic cardiomyopathy increases with Valsalva or standing and decreases with squatting, while the murmur of aortic stenosis does not change similarly.

Question 22

What are the recommendations for family screening in hypertrophic cardiomyopathy?

Answer 22

First-degree relatives should undergo genetic testing and/or echocardiography and ECG to screen for the condition.

Question 23

How is diastolic dysfunction managed in hypertrophic cardiomyopathy?

Answer 23

Diastolic dysfunction is managed with medications that slow heart rate, such as beta-blockers or calcium channel blockers, to improve ventricular filling.

Question 24

Why is early diagnosis of hypertrophic cardiomyopathy important?

Answer 24

Early diagnosis allows for risk stratification, lifestyle modifications, and appropriate interventions to prevent complications like Sudden Cardiac Death.

Question 25

What is the prognosis of hypertrophic cardiomyopathy?

Answer 25

With appropriate management, many patients have a good prognosis, though the risk of complications like Sudden Cardiac Death requires careful monitoring.