Restrictive and Infiltrative Cardiomyopathy

Question 1

Define what restrictive and infiltrative cardiomyopathy is?

Answer 1

Ventricles are too rigid to expand so therefore relaxing and filling phase is abnormal

Question 2

What is the aetiology?

Answer 2

Non-infiltrative:

• Familial
• Forms of HCM
• Scleroderma
• Diabetic
• Pseudoxanthoma elasticum

Storage Diseases:

• Haemachromatosis
• Fabry disease

Infiltrative:

• Amyloid
• Sarcoid

Endomyocardial:

• Fibrosis
• Radiation
• Carcinoid
• Drug effect

Question 3

What are the clinical symptoms?

Answer 3

Fatigue (due to decreased muscle perfusion) 
Dyspnea (tissue hypoxia)
Tachypnea
Peripheral oedema 
Angina 
Palpitations
Syncope

Question 4

What are the clinical signs?

Answer 4

Elevated JVP 
Ascites
Hepatomegaly (hepatic congestion)
Peripheral edema 
(All due to increase ventricular P=RHF)
4th heart sound

Question 5

What are the investigations?

Answer 5

ECG:
-conduction defects+LBBB

N terminal pro brain natriuetic peptide

Basic bloods:

• FBC
• U+E
• Sarcoid + haemachromatosis

Auto antibodies for sclerotic CT disease

Echo

CMRI: show filling characteristics

Fabry: Decrease plasma alpha galactosidase A activity

Question 6

What are the treatments?

Answer 6

B-Blockers (pronanolol)
Anticoagulants(heparin, warfarin)
Limited diuretic use (low filling P cause problems)
SCD risk assessment with ICD or CRT-DP implant 
Cardiac transplant 
Amiodarone 
Low Na intake 
Cardiac transplant

Question 7

What is the prognosis like?

Answer 7

Unless reversible then poor prognosis