1. Cardiomyopathy, Pericarditis & Cardiac Tumors
Ali Al Khader, M.D.
Faculty of Medicine
Al-Balqa’ Applied University

2. Objectives Cardiomyopathies Pericarditis Cardiac tumors
Dilated cardiomyopathy
Cardiomyopathies
Pericarditis
Cardiac tumors
Hypertrophic cardiomyopathy
Restrictive cardiomyopathy

3. Cardiomyopathies, overview
The most common (90% of the cases)
3 major pathological/functional patterns
The least common

4. Functional/Pathological patterns of cardiomyopathies
Robbins basic pathology 9th edition

5. More common than cardiomyopathies
primary
More common than cardiomyopathies
These are intrinsic abnormalities in the heart muscle
= cardiomyopathies
Secondary (as part of systemic disorder)
Robbins basic pathology 9th edition

6. Dilated cardiomyopathy (DCM)
Some causes:
-Genetic …25-50% of the cases
…over 40 genes may be mutated
…AD is the most common pattern of inheritance…mostly
cytoskeletal proteins
…X-linked…mostly dystrophin (a membrane protein that
couples ECM to cytoskeleton)
…Arrhythmogenic Right Ventricular Cardiomyopathy is also included
…AD
…can cause sudden death
…severe vent. Wall thinning & infiltration by fat, in addition to fibrosis
…the left can also be involved (but to a lesser extent)
*congenital conduction abnormalities also can be a feature of
inherited forms of DCM…some genes are needed for both
contractile myocytes & conduction fibers

7. Dilated cardiomyopathy (DCM) causes, cont’d
-Infective myocarditis:
…Coxsackie B is the most common
-Alcohol…Ethanol and its metabolite (acetaldehyde) are toxic to cardiac
myocytes
…Beriberi heart disease may also occur
-Peripartum cardiomyopathy…multifactorial
…about 50% of the patients recover normal function
-Iron overload…the most common presentation in the heart is: DCM

8. DCM, morphology in heart size (2-3 times of normal weight)
Flappy with dilation of all champers
Thickness…thinner, normal or thicker
Mural thrombi are often present
By definition: no valvular or ischemic lesion caused this dilation
Microscopically the changes are non-specific…except in iron overload
-Most myocytes are hypertrophic
-Many are attenuated
-Variable interstitial fibrosis

9. DCM, clinical notes Age: 20-50 years…but can occur at any age
Slowly progressive congestive heart failure
…ejection fraction can become less than 25% (Normal: 50-65%)
Secondary mitral regurgitation
Secondary arrhythmias
Mural thrombi & resultant emboli
50% die within 2 years
Trt.: Cardiac transplantation is the definitive treatment

10. Hypertrophic cardiomyopathy (HCM)
Defective diastolic function
No dilation
Ventricular outflow obstruction in about 1/3 of the cases…see slide #13
Hypercontractile
Systolic function is usually preserved
This is an intrinsic disorder (cardiomyopathy)…different from hypertrophy due to HTN or aortic stenosis (AS)…although similar functional consequences (diastolic dysfunction)

11. HCM, cont’d Mostly: AD…missense mutations in contractile proteins
The most commonly affected: sarcomeric proteins…esp. beta myosin
heavy chain
Some genes are also affected in DCM (e.g., beta myosin)
…but in DCM: contractility
Morphology: the septum more than free wall… = asymmetric septal
hypertrophy
…but in 10%:
concentric hypertrophy

12. HCM, morphology…cont’d
-Myocyte hypertrophy
-Disarray & branching
-Fibrosis
Robbins basic pathology 9th edition
Visit for references

13. HCM, clinical notes
Typically postpubertal growth spurt…but can occur at any age
Impaired diastolic filling stroke volume
In about 1/3 of the cases: outflow is also affected:
Visit for references

14. HCM, clinical notes…cont’d
Exertional dyspnea and systolic ejection murmur
Intramural arteries are compromised (even without atherosclerosis)
…cardiac ischemia…angina
Arrhythmias and mural thrombi
Infective endocarditis of mitral valve (because it became abnormal)
Congestive heart failure
Sudden death…especially in young athletes

15. Restrictive cardiomyopathy
The ventricular wall is stiff (no compliance)…diastolic dysfunction
(impaired filling)
Systolic (contractile) function is not affected
Of the causes: amyloidosis, sarcoidosis, radiation-induced fibrosis
The walls are of normal thickness or slightly thickened
Microscopically: variable interstitial fibrosis
Endomyocardial biopsy can reveal a specific cause

16. Restrictive cardiomyopathy, cont’d
**Amyloidosis:
Extracellular deposition of proteins (insoluble β-pleated sheets)
Can be part of systemic amyloidosis or isolated
..especially: senile cardiac
amyloidosis
…normal (or mutant) forms
of transthyretin…a liver-
synthesized circulating
protein that transports
thyroxine and retinol
In 4% of African Americans:
a specific mutation in this protein…increased incidence of isolated cardiac amyloidosis

17. Restrictive cardiomyopathy, cont’d
**Endomyocardial fibrosis:
Children and young adults in Africa and other tropical areas
Dense diffuse fibrosis of the ventricular endocardium and subendocardium…often: tricuspid and mitral valves
May be related to nutritional deficiencies or helminthic infections
The most common form of restrictive cardiomyopathy worldwide
**Loeffler endomyocarditis:
Endomyocardial fibrosis
Formation of large mural thrombi is typical
Eosinophilia in blood and tissue
Major basic protein from eosinophils causes endomyocardial tissue necrosis with subsequent fibrosis
Some patients: underlying hypereosinophilic myeloproliferative disorder…overactive PDGFR tyrosine kinase…can be controlled by tyrosine kinase inhibitors

18. Myocarditis In USA: The most common are: viruses…coxsackie B
…also coxsackie A, CMV, HIV, influenza…etc.
Chagas disease (caused by???) is the most common infective myocarditis in South America…10% die in acute attack…others: chronic CHF and arrhythmias years
Toxoplasma gondii (the most common vector is??)…esp. in immunocompromised
Trichinosis…the most common helminthic cause
Lyme disease…myocarditis occurs in 5% of the cases
Noninfectious causes:
-SLE, polymyositis…etc.
-Drug hypersensitivity reactions… = hypersensitivity myocarditis…usually mild
Wide spectrum of symptoms
…can progress to DCM

19. Pericarditis Pericardial effusion:
Can be isolated (primary…esp. viral (with myocarditis)) but in most instances: secondary (heart surgery, acute MI, pneumonia…etc.) or systemic disorder
The most common systemic disorder is: uremia
Can be seen in SLE, metastatic malignancies …etc.
Can be: -self-limited
-massive…can cause cardiac tamponade
-becomes chronic fibrosing…constrictive
pericarditis
Pericardial effusion:
…serous (any cause of generalized edema)
…serosanguinous
…chylous
*if rapid (even if small amount) may cause tamponade…if slow (even if large amount) can be tolerated

20. Cardiac tumors
Most common malignancy is: metastasis (in descending order: lung, lymphoma, breast, leukemia, melanoma…etc.)
Most primary tumors are benign
The most common primary tumor in adults: myxoma…most common site is: left atrium…in fossa ovalis (atrial septum)
The most common primary tumor in children and infants: rhabdomyoma
…high frequency in: tuberous sclerosis (TSC1 or TSC2 loss of function…tumor suppressor genes)
The most common primary malignant tumor: angiosarcoma
Others: fibromas, lipomas, papillary fibroelastomas…etc.

21. Thank You