Cardiomyopathy, Myocarditis, and diseases of the pericardium Flashcards by Rodwan Halimi

What is cardiomyopathy?

Heart disease resulting from an abnormality in the myocardium.

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How is a cardiomyopathy diagnosed?

Traditionally it is a diagnosis of exclusion:

{No major disease or abnormality or coronary arteries

No systemic hypertension or history of hypertension

No disease or abnormality of heart valves

No shunt inside or outside of the heart}

Genetic testing

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How is cardiomyopathy classified?

Aetiological:

Primary - heart only

Secondary - Multiple system disorder

Clinico-pathological:

Dilated (DCMP)
Hypertrophic (HCMP)
Restrictive (RCMP)
Arrthmogenic (ARVC)

Extrinsic vs Intrinsic

Extrinsic: Ischaemic HD, Hypertensive HD, alcohol related
Intrinsic: Hypertrophic CM, Dilated CM

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What are the types of primary Cardiomyopathy?

Idiopathic (DCMP, HCMP, RCMP)
Familial (DCMP, HCMP)
Eosinophilic endomyocardial disease (RCMP)
Endomyocardial fibrosis (RCMP)
Arrhythmogenic right ventricular CMP (DCMP)

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What causes secondary CMP?

Infective (DCMP)
Metabolic (DCMP)
Familial storage disease
Deficiency (DCMP, RCMP)
Connective tissue disorders (DCMP)
Infiltrations and granulomas (RCMP, DCMP)
Neuromuscular (DCMP)
Sensitivity and toxic reaction (DCMP)
Peripartum heart disease (DCMP)

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How is cardiomyopathy diagnosed?

Endomyocardial biopsy

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What is dilated in DCMP?

All chambers are hypertrophied and dilated with bowing of the IV septum to the right side

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What percentage of cardiomyopathies are DCMP?

90%

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What causes DCMP?

Previous myocarditis

Idiopathic

Toxins

Connective tissue disorders

Muscular dystrophies

Peripartum

Can morphologically resemble end stage heart failure from other causes

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How is DCMP genetic causes inherited?

Genetically heterogenous:

Autosomal dominant

Autosomal recessive

Mtchondrial inheritence

X-linked transmission also documented

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What are the signs of DCMP?

Shortness of breath

Easy fatigability

Poor exertion capacity

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Can DCMP be reversed?

Yes with cessation of alcohol abuse, cocaine use, etc

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How much larger is the heart than normal in DCMP?

Up to 3x normal

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What is the heart structure like with DCMP?

Dilated and flabby heart

Mural thrombi

Patchy fibrous scarring of myocardium and endocardium

Histological abnormalities are non-specific

Hypertrophic myocyes

Endocardial and interstitial fibrosis of variable degree

Subendocardial scars

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What age group is DCMP seen in?

20 - 50 years old

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How common is hypertrophic cardiomyopathy (HCMP)?

1 in 500 of general population

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How does hypertrophic cardiomyopathy occur?

Heart does not fill properly due ti increased thickness of ventricular wall

Single gene mutations

Mutations in any one of the genes that encode sarcomeric proteins

Mostly autosomal dominant

Sporadic

Mutations are found most commonly in beta-myosin heavy chain

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What happens to heart of someone with HCMP?

LV capacity is reduced in size

Marked hypertrophy of heart muscle without dilation of ventricle

Disproportionate thickening of ventricular septum (asymmetric septal hypertrophy)

Ventricular cavity has a banana-like configuration due to bulging of septum into the lumen

May have endocardial thickening in outflow tract

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What happens to septum in hypertrophic cardiomyopathy?

it grows into the outflow tract.

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What are the clinical features of HCPM?

Variable clinical course slowly progressive

Dyspnea

Angina pectoris

Fatigue

Syncope (big warning)

Double or triple apical precordial impulse, harsh systolic murmur, fourth heart sound

Atrial fibrillation with thrombosis and embolism, infective endocarditis on mitral valve, non-responsive heart failure, ventricular arrhythmias and sudden death

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What is restrictive cardiomyopathy?

Abnormal diastolic function with excessively rigid left ventricular wall which impedes ventricular filling during diastole

What causes restrictive cardiomyopathy?

Diseases that affect myocardium such as amyloid disease of the heart, radiation fibrosis, sarcoidosis, metastatic tumours or deposition of metabolites

What are differential diagnosis to the symptoms of restrictive cardiomyopathies?

Constrictive pericarditis

HCMP

What pathologies are similar to RCMP? What is a common complication in all these conditions?

Endocardial fibroelastosis of infancy

Endomyocardial fibrosis

Loeffler’s endomyocarditis

Mural thrombi are a complication in all these conditions

What do ventricles look like in RCMP?

Normal in size or slightly enlarged. Cavities are not dilated and the myocardium is firm and non-compliant

What do the atria look like in RCMP?

Biatrial dilation

What does RCMP look like on microscope?

Patchy or diffuse interstitial fibrosis which can vary from minimal to extensive

What is myocarditis?

Inflammation of myocardium which can result in cardiac failure or sudden death

Who can myocarditis affect?

Can affect well fit people

What causes myocarditis?

Infections Immune mediated reactions (post viral and post strep) Others

What causes myocarditis infections most commonly? List in order of frequency the most common causes afterwards.

Viruses (coxsachie, ECHO, influenza, HIV) Bacteria (diphtheria toxin) Protozoa Rickettsial Fungal

What are the phases of myocarditis?

Active phase - heart is normal or dilated Advanced - Ventricular myocardium flabby and mottled. Mural thrombi may be present.

What happens to the heart histologically during myocarditis?

Interstitial inflammation + myocyte necrosis (Dallas criteria) Diffuse, predominantly lymphocytic infiltrate Usually patchy so biopsy may be false negative

What is giant cell myocarditis prognosis?

Poor prognosis

What is characteristic of hypersensitivity myocarditis?

Abundant eosinophils

What are some other causes of myocardial disease?

Cardiotoxic drugs (chemotherapy drugs, lithium, clozapine Catecholamines Amyloidosis Isolated atrial amyloidosis Iron overload

What causes pericardial disease?

Inflammatory exudates such as serous, sero-fibrinous, fibrinous, haemorrhagic, suppuratice, or caseous exudates

What do pericardial conditions sound like?

They can be heard as a "pleural rub"

What do serous or fluid effusion into the pericardium look like on chest X-ray?

"Globular" appearance

Do slowly accumulating effusions into the pericardium interfere with heart function?

Generally if effusion is less than 500ml it does not interfere greatly with heart function

Does rapid accumulation of fluid in pericardium cause problems?

Yes, it leads to cardiac tamponade -> Restrictive HF -> can be rapidly fatal

How is pericarditis clinically classified in regards to time?

Acute (<6 weeks) [fibrinous, effusive] Subacute (6 weeks to 6 months) [Effusive-constrictive, constrictive] Chronic (>6 months) [Constrictive, effusive, adhesive]

How is pericarditis clinically classified in regards to aetiology?

Infectious Non-infectious Hypersenstivity/autoimmune

What causes infectious pericarditis?

Viral Pyogenic Tuberculous Fungal Others

What are the non-infectious causes of pericarditis?

Uraemia Neoplasia Myxedema Trauma Aortic dissection

What causes hypersensitive or autoimmune pericarditis?

Rheumatic fever Collagen Vascular disease Drug-induced Post-cardiac injury

What is adhesive pericarditis?

A healed or chronic pericarditis Can be diffuse fine, or dense adhesions but does not interfere with cardiac function

What happens during adhesive mediastinopericarditis?

Heart work against traction by tethered pericardium and hypertrophies. Systolic retraction of ribs and diaphragm Cardiac hypertrophy and dilation

What happens in constrictive pericarditis?

Dense fibrosis with or without calcification Filling is reduced resulting in low output failure without hypertrophy or dilation Pericardiectomy is recommended to prevent restriction

What makes up the pericardium?

Mesothelium