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Introduction
1. Anatomy
2. Microbiology
3. Physiology
4. Pathology
4.1 General pathology
4.2 Central and peripheral nervous system
4.3 Cardiovascular system
4.3.1 Endocarditis, myocarditis and pericarditis
4.3.2 Cardiomyopathies
4.3.3 Hypertrophy of the heart
4.3.4 Atherosclerosis and arteriosclerosis
4.3.5 Ischemic heart disease (IHD)
4.3.6 Diagnosis of AMI/ ACS
4.3.7 Heart failure
4.3.8 Valvular heart disease
4.3.9 Arrhythmias
4.3.10 Vascular disorders
4.3.11 Common types of emboli
4.3.12 Vasculitis
4.3.13 Diseases of the veins
4.3.14 Additional information
4.4 Respiratory system
4.5 Hematology and oncology
4.6 Gastrointestinal pathology
4.7 Renal, endocrine and reproductive system
4.8 Musculoskeletal system
5. Pharmacology
6. Immunology
7. Biochemistry
8. Cell and molecular biology
9. Biostatistics and epidemiology
10. Genetics
11. Behavioral science
Wrapping up
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4.3.1 Endocarditis, myocarditis and pericarditis
Achievable USMLE/1
4. Pathology
4.3. Cardiovascular system

Endocarditis, myocarditis and pericarditis

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I) Infective endocarditis: Endocarditis is inflammation of the endocardium (the innermost lining of the heart). It may be infective or non-infective. Virulent microbes like S. aureus cause acute endocarditis, which can destroy previously healthy valves and presents abruptly. Less virulent bacteria like S. viridans cause subacute endocarditis, which runs a longer course and typically infects previously damaged valves.

Bacteria reach the endocardium during episodes of bacteremia. This can happen with:

  • Day-to-day activities like brushing teeth
  • Infections such as pneumonia and periodontitis
  • Invasive procedures such as GIT, GUT, and respiratory tract scopies; cardiac procedures; biopsies; and surgeries
  • Intravenous drug abuse

People at increased risk include those with congenital heart disorders (VSD, PDA, bicuspid aortic valve, Marfan’s syndrome, mitral valve prolapse, aortic stenosis), prosthetic valves, previous rheumatic heart disease, and immunocompromised states.

Turbulent blood flow damages valves and promotes formation of platelet-fibrin thrombi. These thrombi act as a nidus that traps bacteria circulating in the bloodstream. Adhesion molecules such as integrins Mac 1 (CD11b/18), ICAM 1-3, VCAM 1, and E selection are activated, allowing bacteria to adhere to the endocardium. This triggers both local and systemic inflammatory responses.

On gross examination, vegetations are seen, most commonly on the mitral valve, followed by the aortic valve. Right-sided valves (especially the tricuspid valve) are more commonly involved in intravenous drug abuse. Vegetations in acute bacterial endocarditis (ABE) are bulkier than those in subacute bacterial endocarditis (SABE). In ABE, ulceration, valve perforation, and perivalvular abscess may occur.

Microscopically, vegetations show:

  • An eosinophilic outer layer of fibrin and platelets
  • A middle basophilic zone containing bacterial colonies
  • An inner zone showing inflammation or repair

Clinical features of infective endocarditis

  • Fever, chills, malaise, pyrexia of unknown origin
  • Systemic embolism involving the brain (stroke, infectious or mycotic aneurysm, meningitis, brain abscess, spinal epidural abscess, encephalopathy, seizures); spleen or kidney infarcts
  • Pulmonary abscess from right sided endocarditis
  • Petechiae on skin and conjunctiva from infective emboli
  • Osler’s nodes: painful, tender nodules on finger tips from vasculitis and microembolization
  • Janeway lesions: painless, maculopapular lesions on palms and soles from microembolization, microabscess formation in the dermis with small vessel thrombosis
  • Roth’s spots: Retinal hemorrhages with central white spots. Due to micro-embolization and platelet clot formation. Seen in SABE
  • Splinter hemorrhages in nail beds
  • Splenomegaly
  • Immune complex mediated glomerulonephritis
  • New onset cardiac murmur

Complications include valvular stenosis or insufficiency, valve or chordae tendinae rupture, perivalvular or myocardial abscess formation, pericarditis, and heart failure.

II) Libman-Sacks or atypical verrucous endocarditis: This is seen in non-infectious inflammatory conditions such as SLE, TTP, and collagen diseases. It most commonly affects the mitral and tricuspid valves. It shows multiple, small, “mulberry-like”, sterile vegetations on both surfaces of the valves, chordae tendineae, and adjoining endocardium.

Microscopically, the vegetations show:

  • An outer exudative zone of fibrin, nuclear debris, and haematoxylin-stained bodies
  • A middle zone of proliferating capillaries and fibroblasts
  • An inner zone of neovascularization showing distinct, thin-walled junctional vessels

III) Marantic or non-bacterial thrombotic endocarditis: This is a non-infectious endocarditis seen in hypercoagulable states such as terminal cancers (especially mucinous adenocarcinomas), pancreatic and colon cancers, sepsis, etc. It commonly involves the mitral valve. Vegetations are small and friable, occur along the line of closure, and do not cause tissue destruction.

Embolization can occur from vegetations in marantic endocarditis, causing infarcts in the brain, spleen, lungs, and kidneys. Secondary infection of these vegetations can occur, leading to infective endocarditis.

IV) Myocarditis: Myocarditis is inflammation of the heart muscle. It presents with chest pain, shortness of breath, fatigue, myalgia, arrhythmias, and (in severe cases) heart failure. Sudden cardiac death can occur.

Grossly, all four chambers of the heart are dilated. Microscopically, edema, inflammatory infiltrate, and necrosis are seen. CK-MB and troponins are elevated. ECG shows nonspecific ST-T changes or arrhythmias. Endomyocardial biopsy is the gold standard for diagnosis of myocarditis.

Myocardial injury in myocarditis is caused due to various factors as follows:

Causes of myocarditis

  • Viral infections like influenza, measles, mumps, hepatitis, infectious mononucleosis, Coxsackie virus etc.
  • Bacterial myocarditis e.g. S.aureus, S.pyogenes, Borrelia burgdorferi, Rheumatic fever, Rickettsia
  • Bacterial toxins e.g. Diphtheria toxin
  • Parasites e.g. Trypanosoma cruzi, Toxoplasma gondii, Trichinella spiralis
  • Fungi e.g. Candida, Aspergillus, systemic fungal infections
  • Connective tissue disorders e.g. Rheumatoid arthritis, SLE, scleroderma, dermatomyositis, sarcoidosis
  • Drugs e.g. doxorubicin, daunorubicin, catecholamines
  • Fiedler’s idiopathic or Giant cell myocarditis
  • Trauma, radiation, cardiac surgery, CO, arsenic, phosphorus, uremia, hypokalemia

V) Pericarditis: Pericarditis is inflammation of the pericardium. It may be acute or chronic. Acute pericarditis may be serous, fibrinous, purulent, or hemorrhagic. Almost all causes of myocarditis can also cause pericarditis.

  • Serous pericarditis: A thin, watery fluid accumulates in the pericardial sac. It is seen in viral infections such as Coxsackie virus (enterovirus), parvovirus B19, adenovirus, EBV, HHV 6, CMV; and in rheumatic fever, RA, SLE, etc.
  • Fibrinous pericarditis: Seen in uraemia, AMI, rheumatic fever, trauma, and bacterial infections. It is characterized by fibrinous exudate; the cardiac surface looks shaggy with a “bread and butter” appearance. It may heal by forming fibrous adhesions.
  • Purulent pericarditis: Seen in infections with pyogenic bacteria like S. aureus, Streptococci, etc. The exudate is pus and may later lead to chronic constrictive or adhesive pericarditis.
  • Hemorrhagic pericarditis: Characterized by blood in the pericardial sac. Causes include malignancies, tuberculosis, and bleeding disorders.

Types of chronic pericarditis

  • Tuberculous pericarditis: Turbid, bloody exudate, tubercles seen on pericardium, tubercle granulomas seen on microscopy, heals by fibrosis and calcification

  • Chronic constrictive pericarditis: Thickened, fibrotic, scarred, calcified pericardium, sequelae of tuberculous, purulent or hemorrhagic pericarditis, previous history of cardiac surgery, normal or smaller sized heart, diastolic dysfunction, right heart failure

  • Chronic adhesive pericarditis: Follows any fibrinous, suppurative or hemorrhagic pericarditis; fibrous adhesions formed, cardiac hypertrophy and dilation

Patients with pericarditis typically present with sharp, pleuritic chest pain that improves on sitting up and leaning forward, and fever. A pericardial friction rub may be heard, and ECG may show widespread, concave ST segment elevation. The friction rub is best auscultated at the left sternal border with the patient leaning forward and holding their breath.

Pericardial effusion may be visualized on echocardiography. Large pericardial effusions may produce a “water bottle shaped” cardiac silhouette on chest X ray.

VI) Pericardial effusion: Pericardial effusion is accumulation of fluid in the pericardial cavity. The most common type is serous, seen in congestive heart failure, hepatic failure, renal failure, etc. Other types include sero-sanguinous (trauma), chylous (lymphatic obstruction or damage), and cholesterol effusion (myxoedema).

Blood may accumulate in the pericardium following rupture of the heart after AMI, dissecting aortic aneurysm, trauma, or bleeding disorders. Massive accumulation of blood can lead to cardiac tamponade.

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