Rheumatic heart disease

CVS 4 Hisham Alkhalidi

Rheumatic fever

• • • • Definition Aetiology Pathological changes (Aschoff body) Cardiac and non-cardiac manifestations of rheumatic fever with special emphasis on valvular manifestations (mitral and aortic valves involvement)

Rheumatic fever

• • • • Acute Immunologically mediated Multisystem inflammatory disease Occurs few weeks after an episode of:

group A β-hemolytic streptococcal pharyngitis

Rheumatic fever

• Usually affects children 5-15 years, but first attacks can occur in adults • In economically depressed urban areas or developing countries, RF and RHD remain important public health problems

Rheumatic fever

• Acute rheumatic mitral valvulitis superimposed on chronic rheumatic heart disease.

Chronic RHD

• • Mitral stenosis with diffuse fibrous thickening and distortion of the valve leaflets, commissural fusion (arrows) Thickening and shortening of the chordae tendineae

Chronic RHD

• • • Organization of the acute inflammation and subsequent scarring Aschoff bodies are replaced by fibrous scar, so diagnostic forms of these lesions are rarely seen in chronic RHD The major functional consequence of RHD is:

Valvular stenosis and regurgitation

MITRAL VALVE IS THE COMMONEST VALVE AFFECTED

• • • MAT 70 % M 25 % M+A

Diagnosis of acute RHD

• • serologic evidence of a previous streptococcal infection + two or more of the following Jones

criteria:

(1) carditis, (2) migratory polyarthritis of the large joints, (3) subcutaneous nodules, (4) erythema marginatum of the skin, and (5) Sydenham chorea, a neurologic disorder with involuntary purposeless, rapid movements One of the Jones criteria manifestations and two minor manifestations (nonspecific signs and symptoms that include fever, arthralgia, or elevated blood levels of acute-phase reactants)

Chronic rheumatic heart disease

• • More likely to occur when the first attack: – – In early childhood Sever – Recurrence The long-term prognosis is highly variable • Surgical repair or replacement of diseased valves has greatly improved the outlook for patients with RHD

Chronic RHD

• • • • • • • • The signs and symptoms of valvular disease depend on which valve(s) are involved Mitral stenosis is the most common manifestation Cardiac murmurs Cardiac hypertrophy and dilation CHF Arrhythmias (atrial fibrillation in the setting of mitral stenosis) Thromboembolic complications Increased risk of subsequent infective endocarditis.

Endocarditis and pericarditis

CVS 5 Hisham Alkhalidi

Lectures 5

• Pathology of endocarditis and pericarditis: definitions, classification and causes with emphasis on subacute bacterial endocarditis, marantic endocarditis and chronic constrictive pericarditis

Infective endocarditis (IE)

• A serious infection, characterized by: – Microbial invasion of heart valves or mural endocardium – Often with destruction of the underlying cardiac tissues – Results in bulky, friable vegetations composed of necrotic debris, thrombus, and organisms

IE, Types

• •

Acute endocarditis, usually:

– Suggests a destructive infection – Involvement of a highly virulent organism (staph. Aureus) – – Attacking a previously normal valve Death within days to weeks in more than 50% of patients despite antibiotics and surgery Subacute endocarditis: – Low virulence organisms (strept. Viridans) – Colonizing a previously abnormal heart, especially when there are deformed valves – The disease typically appears insidiously – Follows a protracted course of weeks to months – Most patients recover after appropriate antibiotic therapy

IE

Clinical presentation and complications • Acute: – Fever, rigor, malaise – Large vegetation -> emboli: • Infarction • • Metastatic infection affects distant organs like spleen, brain or heart • Kidney: Ag-Ab complex -> GN-> nephrotic syndrome or Renal failure – – Congestive heart failure due to valve disease Can lead to ring abscess and perforation of the aorta and myocardium – Death up to 60%

IE

Clinical presentation and complications • Subacute: – Insidious – Splenomegaly – Non specific fever, weight loss – Small vegetations, so less embolic – Low mortality

IE

• Diagnosis is largely made on the basis of positive blood cultures, echocardiographic findings, and other clinical and laboratory findings

IE

Pathogenesis • • Bacteremia is a pre-requisite – Other organs infection – IV drug abuse: • Usually Staph aureus, right heart side (Tricuspid) – Dental or surgical procedure – Trivial injury, skin, gut, urinary bladder Contributory conditions are immunosupression and neutropenia

IE

Favorable conditions of infections • • • • • • • Congenital defects Chronic RHD MV Prolapse Deg. Calcific stenosis Bicusped aorta Prosthetic valve (5% in 5 years) Indwelling catheters

Diagnsosis

• • Blood C/S is a major step Duke’s criteria

Nonbacterial Thrombotic Endocarditis NBTE (Marantic endocarditis)

– Attributed to: • • Disseminated intravascular coagulation Hypercoagulability – Association with malignancy (specially adenocarinoma)

Nonbacterial Thrombotic Endocarditis NBTE (Marantic endocarditis)

• • • • Gross: loose adherent groups of small nodules on the lines of valve closure (similar to those of acute rheumatic fever), valve leaflets are normal Micro: SMALL, sterile, fibrin and platelets aggregates, no inflammation or fibrosis.

Clinically asymptomatic, if large -> may embolize, may become infected Typically mitral valve

Endocarditis associated with SLE

• • • • Unknown etiology Both sides of the valve Deformity to the valve by healing of fibrinoid necrosis and mucoid degeneration Sterile

Pericarditis

• • • Primary vs secondery Acute vs chronic Uremia is the most common systemic disorder associated with pericarditis

Pericarditis

• • • • • Serous Fibrinous, serofibrinous Purulent Hemorrhagic Caseous

Pericarditis

• Serous: – Exudate – Non bacterial causes: • Rheumatic fever • • SLE Tumor • Uremia • Primary viral

Pericarditis

• • Fibrinous, serofibrinous • Same causes of serous • Most commonly due to MI Purulent – Bacteria (staph, strept, pneuomcocus) – Fungi – Parasite – Can lead to mediastionpericaridtis or congestive pericarditis

Pericarditis

• • Hemorrhagic: – Blood + fibrin or pus – Surgery – TB – Tumor Caseous: – TB – Fungi – Fibrocalcific constrictive pericarditis

Healing

• • Resolution Fibrosis: – Epicardial plaque – Thin – Thick massive adhesion

• • Adhesive mediastinopericarditis: – heart contracts against the surrounding structures Constrictive pericarditis: – 1 cm thick dense fibrous obliteration – Limit diastolic expansion and cardiac output

Chronic Pericarditis: Morphology

• • Ranges from delicates adhesions to dense fibrotic scars that obliterate the pericardial space.

In extreme cases the heart can’t expand during diastole : constrictive pericarditis

Clinical picture

• • • • Atypical chest pain (worse on reclining) High pitch friction rub.

Significant exudate  cardiac tamponade  faint distant heart sounds, distended neck veins, declining cardiac output and shock Chronic constrictive pericarditis  distension and low cardiac output.

venous