Congenital heart defects & inherited cardiac diseases Flashcards Preview

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Flashcards in Congenital heart defects & inherited cardiac diseases Deck (41)
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1

What is hypertrophic cardiomyopathy?

An unexplained and often substantial thickening of the myocardium of the ventricular wall

2

How much a patient with hypertrophic cardiomyopathy present?

Asymptomatic
OR
Dyspnoea, chest pain, palpitations, lightheadedness, fatigue, syncope, sudden cardiac death

3

Why must you establish whether a patient is suffering from hypertrophic cardiomyopathy or congestive heart failure?

The symptoms of hypertrophic cardiomyopathy mimic those of congestive heart failure, but treatment with diuretics will worsen symptoms (due to decreased ventricular volume and increased outflow resistance)

4

Is hypertrophic cardiomyopathy inherited in an autosomal dominant or autosomal recessive pattern?

Dominant

5

What is the genetic mutation that causes hypertrophic cardiomyopathy?

Mutation in sarcomeric genes resulting in mutated cardiac muscle proteins

6

Is the hypertrophy symmetrical or asymmetrical?

Asymmetrical - usually involving interventricular septum

7

Which is the most common heart chamber involved in hypertrophic cardiomyopathy?

Left ventricle

8

When does hypertrophic cardiomyopathy commonly present itself?

Post-puberty in early adulthood

9

What are some of the serious complications of hypertrophic cardiomyopathy?

Atrial fibrillation
Ventricular arrhythmias
Sudden death

10

What is the first line treatment for hypertrophic cardiomyopathy?

Beta-blockers

11

What is the second line treatment for hypertrophic cardiomyopathy?

Calcium channel antagonists i.e. verapamil

12

What is arrhythmogenic right ventricular cardiomyopathy (ARVC)?

Progressive loss of right ventricular myocytes with associated fibrosis, inflammation and adipose tissue replacement

13

What is the most important/common complication of ARVC?

Ventricular arrhythmias due to loss of functional RV myocardium by fibrofatty replacement

14

What genetic mutation leads to ARVC?

Genetic defect of genes encoding for desmosomal proteins
Inherited in autosomal dominant pattern

15

What are some of the symptoms of ARVC?

Syncope
Palpitations

16

How might ARVC present on an ECG?

T wave inversion in leads V1-V3
Right bundle branch block epsilon wave after QRS

17

What is the first line treatment for ARVC?

Sotalol

18

What other drugs may be used in the treatment of ARVC?

Amiodarone
Metoprolol

19

What other type of drug must be considered when dealing with ARVC? (Consider decreased ejection fraction in RV).

Anticoagulants i.e. warfarin

20

What is dilated cardiomyopathy?

Enlarged & weakened heart wall with no thickening of the myocardium

21

What is the most important complication of dilated cardiomyopathy?

Heart failure

22

What symptoms might dilated cardiomyopathy present with?

Breathlessness
Syncope
Cardiomegaly
Raised JVP
Low pulse pressure
Signs of mitral & tricuspid regurgitation
Tachycardia

23

What is the treatment for dilated cardiomyopathy?

ACEI
Diuretics
Digoxin
Anticoagulants

24

What is the most common atrial septal defect?

Patent foramen ovale

25

Which is the most common form of congenital heart defect?

Ventricular septal defect - accounts for 25% of defects

26

What are some of the complications of an atrial septal defect?

Cardiac failure in 3rd or 4th decade of life
Increased blood flow in pulmonary circulation

27

What murmur may be heard in atrial septal defect and why?

Diastolic rumbling murmur
Increased blood flow across tricuspid valve

28

What complications in the right heart develop if an atrial septal defect is left open?

Pulmonary hypertension
Right ventricular hypertrophy

29

Which murmur may be heart in ventricular septal defect?

Pansystolic murmur
May have associated thrill

30

What complications may arise from a ventricular septal defect?

Infective endocarditis
Cardiac failure