Cardiomyopathies

Question 1

What is hypertrophic cardiomyopathy?

Answer 1

A condition in which the heart muscle becomes thickened

The thickened heart muscle can make it harder for the heart to pump blood

The parts of the heart most commonly affected are the interventricular septum and the ventricles

Question 2

What is the clinical presentation of hypertrophic cardiomyopathy?

Answer 2

Fatigue
Peripheral oedema 
Shortness of breath
Syncope
Angina

Question 3

What causes hypertrophic cardiomyopathy?

Answer 3

1. Familial hypertrophic cardiomyopathy is inherited as an autosomal dominant trait
2. Heart failure

Question 4

How would you diagnose hypertrophic cardiomyopathy?

Answer 4

1. Clinical features

2. ECG, echocardiogram

Question 5

How would you manage hypertrophic cardiomyopathy?

Answer 5

Asymptomatic people will remain untreated

The primary goal of medications is to relieve symptoms such as chest pain, shortness of breath, and palpitations: beta blockers first line, then CCB

Question 6

What is dilated cardiomyopathy?

Answer 6

A condition in which the heart becomes enlarged and cannot pump blood effectively

Question 7

What are the signs and symptoms of dilated cardiomyopathy?

Answer 7

Shortness of breath
Syncope
Angina
Pulmonary oedema
Raised JVP

Signs and symptoms follow that of heart failure

Question 8

What is the pathophysiology of dilated cardiomyopathy?

Answer 8

The progression of heart failure is associated with left ventricular remodelling, which manifests as:

1. gradual increases in left ventricular end-diastolic and end-systolic volumes
2. wall thinning
3. A change in chamber geometry to a more spherical, less elongated shape

This process is usually associated with a continuous decline in ejection fraction

Question 9

What compensatory measures are employed to preserve CO in dilated cardiomyopathy?

Answer 9

1. Neurohormonal feedback, via activation of the sympathetic nervous system and the renin-angiotensin system.
2. Frank-Starling law

Question 10

How is dilated cardiomyopathy diagnosed?

Answer 10

Generalizsd enlargement of the heart is seen upon normal chest X-ray.

Pleural effusion may also be noticed, which is due to pulmonary venous hypertension.

ECG shows sinus tachycardia or atrial fibrillation, ventricular arrhythmias, left atrial enlargement

Question 11

How would you manage dilated cardiomyopathy?

Answer 11

Drug therapy can slow down progression and in some cases even improve the heart condition.

Standard therapy may include salt restriction, ACE inhibitors, diuretics, and beta blockers