Cardiac Pathology

Question 1

What is the abnormality in hypertrophic cardiomyopathy?

Answer 1

Variable hypertrophy in distribution and extent (asymmetric) of the LV or both ventricles

Question 2

What is the mortality rate of hypertrophic cardiomyopathy in adults?

Answer 2

2-3% (higher in young pts)

Question 3

What is the aetiology of hypertrophic cardiomyopathy?

Answer 3

Genetic (AD) mutations in various genes encoding sarcomeric proteins (e.g. cardiac myosin, troponins)

Question 4

Describe the morphology observed in hypertrophic cardiomyopathy

Answer 4

Variable hypertrophy of LV +/- RV Myocyte hypertrophy and disarray Interstitial fibrosis Thickened intima of small intramyocardial arteries

Question 5

What are some of the possible clinical presentations of hypertrophic cardiomyopathy?

Answer 5

Asymptomatic Impaired ventricular compliance (leads to diastolic failure) Impaired LV outflow Ischaemia from increased muscle mass and abnormal intramyocardial arteries +/- increased wall stress related to outflow obstruction (leads to arrhythmias, angina and HF) Angina can also be caused by abnormal myofibres

Question 6

When does hypertrophic cardiomyopathy usually present?

Answer 6

4th decade

Question 7

What is the most common type of cardiomyopathy?

Answer 7

Idiopathic dilated cardiomyopathy

Question 8

What is the abnormality in idiopathic dilated cardiomyopathy?

Answer 8

Dilatation and impaired contraction of the LV or both ventricles, often also atria

Question 9

Describe the morphology observed in idiopathic dilated cardiomyopathy

Answer 9

Eccentric hypertrophy of LV +/- RV Myocyte hypertrophy Interstitial and endocardial fibrosis

Question 10

When does idiopathic dilated cardiomyopathy usually present?

Answer 10

3rd or 4th decade

Question 11

What are some of the possible clinical presentations of hypertrophic cardiomyopathy?

Answer 11

Deteriorating biventricular failure Arrhythmias Sudden cardiac death from arrhythmias Thromboembolic complications Steady course +/- improvement

Question 12

What is the suspected aetiology in idiopathic dilated cardiomyopathy?

Answer 12

20-30% genetic (various patterns of inheritance) mutations in various genes encoding e.g. cardiac cytoskeletal and myofibrillar proteins

?viral

?toxic

?AI

Question 13

List 6 causes of dilated cardiomyopathy

Answer 13

Peripartum period

Haemochromatosis

Alcohol

Drugs (e.g. anthracyclines)

Ischaemic

Neuromuscular disease (e.g. DMD)

Question 14

List the 5 types of cardiac myopathy

Answer 14

Dilated

Hypertrophic

Restrictive

Arrhythmogenic

Unclassified