3.2b CVS Pathology 2 Flashcards
What is the adaptive response in systemic HHD?
Concentric hypertrophy
- Stems from the increased demands placed on the heart by HPN
- Refers to the effects on the heart as a result of increased pressure
Hypertensive heart disease (HHD)
Pulmonary HHD is characterized by (2)
- RV hypertrophy (chronic) or
2. RV dilatation (acute)
- Adapt over whose to PRESSURE overload
2. Can lead to: myocardial dysfunction, cardiac dilatation, CHF, sudden death
systemic HHD
Diagnostic criteria of systemic HHD (3)
- LVH (concentric thickening >2cm towards inner chamber)
- History or pathologic evidence of HPN
- Absence of other CVS lesions
Its gross features include:
- Concentric hypertrophy
- Thickened LV wall
- Thickened IV septum
- Thick and robust papillary muscles
- Initially without ventricular dilatation
- Increased heat weight (>500g)
- Stiffness and impairment of diastolic filling
Systemic HHD
Its microscopic features include:
- Increased transverse diameter of myocytes
- Enlarged myocytes and nuclei (box-like)
- Diffused interstitial fibrosis
Systemic HHD
- Pressure overload in RV due to increased pulmonary resistance usually caused by chronic lung disease that can lead to pulmonary vasculature fibrosis (ie. COPD)
- Characterized by: RV hypertrophy, RV dilatation, (potentially) failure due to increased pulmonary resistance
Pulmonary HHD
Types of pulmonary HHD (2)
- Acute cor pulmonale (RV dilatation)
2. Chronic cor pulmonale (RV hypertrophy)
Predisposing factors of pulmonary HHD (4)
- Diseases of pulmonary PARENCHYMA
- Diseases of pulmonary VESSELS
- Disorders affecting chest movement
- Disorders inducing pulmonary arterial constriction
The ff are examples of which predisposing factor of pulmonary HHD?
- COPD
- Disuse pulmonary interstitial fibrosis
- Pneumoconiosis
- Cystic fibrosis
- Bronchiectasias
Diseases of pulmonary parenchyma
The ff are examples of which predisposing factor of pulmonary HHD?
- Recurrent pulmonary thromboembolism
- Primary pulmonary HPN
- Extensive pulmonary arteritis
- Drug-, toxin-, radiation-induced vascular obstruction
- Extensive pulmonary tumor
- Microembolism
Diseases of pulmonary vessels
The ff are examples of which predisposing factor of pulmonary HHD?
- Kyphoscoliosis
- Marked obesity
- Neuromuscular disease
Disorders affecting chest movement
The ff are examples of which predisposing factor of pulmonary HHD?
- Hypoxemia
- Metabolic acidosis
- Chronic altitude sickness
- Obstruction to major airways
- Idiopathic alveolar hypoventilation
Disorders inducing pulmonary arterial constriction
(Pulmonary HHD) RV hypertrophy and dilatation can lead to (3)
- Compression of LV
- Tricuspid thickening
- Regurgitation
On cross section of acute cor pulmonale, what is the shape of RV?
Ovoid (normal is crescent)
What are the microscopic feature of pulmonary HHD? (2)
- Normal fat in the wall disappears
2. Circumferentially arranged myocytes (normal: haphazardly arranged)
- Heart muscle disease (problem in heart muscle)
2. INTRINSIC heart disease resulting from a primary abnormality in the myocardium
Cardiomyopathy (CMP)
CMPs usually produce (2)
- Abnormalities is cardiac wall thickness, chamber size
2. Mechanical and/or electrical dysfunction
Clinical manifestations of CMP are either primary of secondary CMPs. Describe each.
- Primary: confined to the heart
2. Secondary: part of a generalized systematic disorder
Types of CMP (3)
- Dilated CMP
- Hypertrophic CMP
- Restrictive CMP
Type of CMP:
- Progressive hypertrophy and global dilatation
- Contractile and systolic dysfunction
- HYPOcontracting heart
- Can cause mitral regurgitation, abnormal cardiac rhythms
Dilated CMP
S/Sx of dilated CMP (such as shortness of breath, easy fatigability, poor exertional capacity) are manifestations of?
Progressive CHF
The ff are a causes of which CMP?
- Idiopathic
- Previous viral or complicated myocarditis (Coxsackie virus, enteroviruses)
- Alcohol abuse, other toxins (cobalt, CO, Doxorubicin)
- Pregnancy (peri- and postpartum CMP)
- Genetic (autosomal dominant, defects in cytoskeleton)
- Malnutrition (B12 and B1 deficiency)
- Adriamycin therapy (dose related)
- Cobalt added to beer
Dilated CMP
This CMP is a disease of exclusion and it’s negative criteria for its diagnosis include the ff: NO
- Major coronary artery disease or anomaly
- Valvular disease or anomaly
- Systemic HPN
- Shunts within or outside the heart
Dilated CMP
Define dilated CMP
Progressive hypertrophy
Global dilatation
The ff are gross features of which CMP?
- Increased weight
- Global enlargement
- Generalized chamber dilatation (flabby)
- Abnormal release of blood flow (due to mural thrombi)
- Annular ring dilatation –> valvular dilatation –> regurgitation
Dilated CMP
The ff are microscopic features of which CMP?
- Hypertrophied muscle cells
- Enlarged nuclei
- Interstitial endocardium fibrosis
- Subendocardial scars
Dilated CMP
It is aka
- Idiopathic Hypertrophic Subaortic Stenosis (IHSS)
- Hypertrophic Obstructive CMP
- Asymmetric Septal Hypertophy
Hypertrophic CMP
What is the most important finding in hypertrophic CMP?
Myocardial hypertrophy
In this type of CMP:
In the atrioventricular septum, the ventricular lumen is reduced –> abnormal diastolic filling, LV outflow obstruction
Hypertrophic CMP
Hypertrophic CMP is characterized by? (2)
- Heart is muscular and HYPERcontracting
* remember that in dilated CMP the heart is HYPOcontracting - Increased/normal LV ejection
Pathogenesis of Hypertrophic CMP (1)
MUTATION in at least one gene encoding proteins that are part of the sarcomere
*most cases are familial, autosomal dominant
What are the clinical features of hypertrophic CMP? (4)
- Impaired diastolic filling
- Exertional dyspnea
- Harsh systolic murmur
- Anginal pain
The ff are major clinical problems seen in which CMP?
- Atrial fibrillation with mural thrombus
- Infective endocarditis of mitral valve
- Intractable cardiac failure
- Ventricular arrhythmias
- Sudden death
Hypertrophic CMP
Its gross features include the ff:
- Massive myocardial hypertrophy WITHOUT DILATATION
- Asymmetric septal hypertrophy (septum:wall ratio is >1.3)
- BANANA-like ventricular cavity
- Endocardium thickening
- Mural plaque formation in the LV outflow tract
- Anterior mitral valve thickening
Hypertrophic CMP
Its microscopic features include the ff:
- Extensive myocyte hypertrophy
- HAPHAZARD myocardial fiber disarray
- Interstitial fibrosis
Hypertrophic CMP
- Decreased cardiac compliance
- Impaired ventricular filling
- Decreased LV ejection fraction
Restrictive CMP (RCM)
Its causes include the ff:
- Idiopathic
- Amyloidosis, Radiation, fibrosis, sarcoidosis
- Immune or metabolic
- Metastatic tumors
- Deposition of metabolites (d/t inborn errors of metabolism)
Restrictive CMP
Other types of RCM (3)
- Endomyocardial fibrosis
- Loeffler endomyocarditis
- Endocardial fibroelastosis
The ff describes which type of RCM?
- Diffuse fibrosis of ventricular subendocardium
- Markedly diminishes volume and compliance of affect chambers
- Restrictive functional effect
Endomyocardial fibrosis
The ff describes which type of RCM?
- Eosinophilia
- Endocardial fibrosis
- Thrombosis
Loeffler endomyocarditis
The ff describes which type of RCM?
- Focal or diffuse fibroelastic thickening
- Heart is infiltrated by fibroelastic tissue
Endocardial fibroelasatosis
- An inherited disease of cardiac muscle
- Causes RV failure, ventricular tachycardia or fibrillation —> sudden death
- Autosomal-dominant inheritance
- Variable penetrance
- (+) defective cell adhesion proteins in desmosomes (link adjacent myocytes)
Arrythmogenic RV CMP/Dysplasia
What disorder is characterized by
- Arrythmogenic RV CMP
- Hyperkeratosis of plantar and palmar skin surfaces associated with mutations in the gene encoding plakglobin
Naxos Syndrome
The ff are gross characteristics of?
- RV wall: severely thinner due to loss of myocytes
- Extensive fatty infiltration and fibrosis
Arrhythmognic RV CMP/Dysplasia
This includes inflammatory diseases of the myocardium that results in injury to cardiac myocytes but is NOT reflective of the injurious pattern related to IHD
Myocarditis
What are the major causes of myocarditis? (4)
- Infections
- Immune-mediate reactions
- Unknown
- Cardiotoxic drugs
The ff are clinical features of?
- Can mimic AMI
- Broad clinical manifestations
- Entirely asymptomatic
- Precipitous onset of heart failure or arrhythmias, occasionally with sudden death
- May develop late CMP as a late complication
Myocarditis
The ff symptoms are seen in?
- Fatigue
- Dyspnea
- Palpitations
- Precordial discomfort
- Fever
Myocarditis
The ff are gross features of?
- Normal or dilated
- Lesions are diffuse (if viral) or patchy (parasitic)
- Hypertrophy (depends on disease duration)
- Ventricular myocardium is flabby and mottled by focal foci/minute hemorrhagic lesions
- Beefy red (d/t associated vascular congestion)
Myocarditis
The ff are microscopic features of?
- Interstitial inflammatory infiltrate associated with focal myocyte necrosis
- Edema, vascular congestion
- Focal necrosis, degeneration of myocytes adjacent to inflammatory cells
- Viral inclusions, parasites may be present
- A diffuse mononuclear, lymphocyte infiltrate is most common
Myocarditis
Type of myocarditis:
- Multinucleated giant cells
- Lymphocytes, eosinophils, necrosis
- Differential: sarcoid, hypersensitivity
- Aggressive clinical course
Giant Cell Myocarditis (Fiedler’s Myocarditis)
Type of myocarditis:
- Interstitial infiltrate: macrophages, eosinophils
- No/little necrosis
- No granuloma
- Multinucleated giant cells may be present
- Associated with adverse effects of methyldopa
Hypersensitivity myocarditis
Types of myocarditis (2)
- Giant cell myocarditis (Fiedler’s Myocarditis)
2. Hypersensitivity myocarditis
- Anomalies of the heart or great vessels present from birth
- Arises from faulty embryogenesis (1st trimester: 3-8th week when major cv structure develop)
CHD
Etiology of Coarctation of Aorta
Trisomy
Etiology of ASD, VSD, PDA
Down’s Trisomy 21
Etiology of CHD (5)
- Genetic
- Infection (viruses)
- Chemical substances
- Multifactorial
- Idiopathic
In Holt-Oram Syndrome (CHD):
- Gene affected?
- Congenital cardiac disease (3)
- TBX5
2. ASD, VSD, conduction defects
In Di’George Syndrome (CHD):
- Gene affected?
- Congenital cardiac disease (1)
- TBX1
2. Cardiac outflow tract defects
In Alagille Syndrome (CHD):
- Gene affected?
- Congenital cardiac disease (2)
- JAG1, NOTCH2
2. Pulmonary artery stenosis
In Marfan Syndrome (CHD):
- Gene affected?
- Congenital cardiac disease (2)
- Fibrillin
2. Aortic aneurysm, valve abnormalities
Th ff are clinical effects of?
- Failure to thrive
- Cyanosis at birth (R-L shunt)
- Cardiac failure
- Pulmonary HPN
- Infective endocarditis
CHD
What are the classifications of CHD? (3)
- Left to right shunt anomaly (acyanotic)
- Right to left shunt anomaly (cyanotic)
- Obstructive anomaly
Which defects are a categorized under Left to Right shunt anomaly? (4)
- ASD
- VSD
- AVSD
- PDA
Which defects are a categorized under Right to Left shunt anomaly? (5)
- Tetralogy of Fallot
- Transposition of great vessels
- Truncus arteriosus
- Tricuspid atresia
- Total anomalous pulmonary venous return
Which defects are a categorized under obstructive anomaly? (3)
- Coarctation of Aorta
- Pulmonary stenosis or atresia
- Aortic stenosis or atresia
Type of LR shunt:
- Opening in the atrial septum –> communication of blood between LA and RA
- Acyanotic at birth
- Most common is a patent foramen ovale
- Its late effect is pulmonary HPN
- The most common CHD that is asymptomatic
ASD
It is the most common CHD that is ASYMPTOMATIC
ASD
Types of ASD (3)
- Septum Secundum ASD
- Septum Primum ASD
- Sinus Venosus Defect
Type of ASD:
- 90% of ASD
- Defect in fossa ovale
- May be solitary or with another anomaly
Septum Secundum ASD
Type of ASD:
- 5% ASD
- Defect near AV valve
- Associated with a clefted anterior mitral valve (partial ASD)
Septum Primum ASD
Type of ASD:
- 5% of ASD
- Located at the top, near entrance of SVC
- Associated with anomalous pulmonary venous return to RA
- Associated with TAPVR
Sinus Venosus defect
The ff are effects of which LR shunt anomaly?
- Right atrial enlargement and hypertrophy
- Right ventricular enlargement and hypertrophy
- Increased pulmonary blood flow –> pulmonary HPN
- Murmur due to increased blood flow to pulmonary valve
ASD
- Incomplete closure of ventricular septum –> free communication between RV and LV
- Most common CHD
- 30% isolated anomaly
- Most are associated with other congenital cardiac defects such as Tetralogy of Fallot
- ACYANOTIC at birth
- Can have associated mitral valve abnormality
VSD
Types of VSD (3)
- Membranous VSD
- Infundibular VSD
- Muscular VSD
Type of VSD:
- 90% of VSD
- Located in upper portion of interventricular septum
Membranous VSD
Type of VSD:
1. Located below pulmonary valve
Infundinular VSD
Type of VSD:
- Found in muscular wall of interventricular septum
- Spontaneous closure in 50% of cases
Muscular VSD
Type of CHD:
- Abnormal development of AV canal
- Failure of fusion of superior and inferior endocardial cushions
- Incomplete closure of atrioventricular septum
- Inadequate formation of tricuspid and mitral valves
Atrioventricular Septal Defect (AVSD)
More than 1/3 of those with complete AVSD have what other disorder/syndrome?
Down’s Syndrome
Types of AVSD (2)
- Partial AVSD
2. Complete AVSD
Type of AVSD:
1. Combination of Primum ASD + a cleft anterior mitral valve
Partial AVSD
Type of AVSD:
1.Total absence of atrial and ventricular septum –> free communication of all four chambers
Complete AVSD
Type of CHD:
- Persistence of communication between pulmonary artery and aorta via ductus arteriosus
- 90% isolated anomaly
- 10% associated with VSD, coarctation, pulmonary or aortic stenosis
- Classic sign: machinery murmur
- Late effect: pulmonary HPN
PDA
What is the classic sign of PDA?
Machinery murmur
What is given to preserve ductal patency (for infants with various forms of CHD with obstruct pulmonary or systemic blood flow such as on aortic valve atresia)?
Prostaglandin E
What are the (4) components of a Tetralogy of Fallot?
- VSD
- Subpulmonic stenosis
- Aorta overriding VSD
- Right ventricular hypertrophy
What is the morphological feature of tetralogy of Fallot?
Boot-shaped heart
due to marked RV hypertrophy, particularly the apical region
In tetralogy of Fallot, the degree of cyanosis is dependent on the degree of pulmonary stenosis.
- Severe pulmonary stenosis is what type of shunt and leads to what?
- Mild pulmonary stenosis is what type of shunt and leads to what?
- Right to left shunt –> classic tetralogy of Fallot
2. Left to right shunt –> no cyanosis –> pink tetralogy of Fallot
In transposition of great vessels, there is ventriculo-arterial discordance. Define ventriculo-arterial discordance.
- AoRta arises from the Right ventricle (it’s normally a the LV)
- PuLmonary artery arises from the Left ventricle (normally at RV)
In this type of CHD, there is switching of the aorta and pulmonary artery
Transposition of great vessels
What is the result of transposition of great vessels?
Separation of systemic and pulmonary circulations
incompatible with life
What is the embryo logic deft in complete transposition?
Abnormal formation of the truncal and aortopulmonary septa
Type of CHD:
- Developmental failure of separation of the embryologic truncus arteriosus into the aorta and pulmonary artery
- Result: single great artery that receives blood from both ventricles, gives rise to the systemic, pulmonary, and coronary circulations
- Associated lesion: VSD
- Incompatible with life unless combined with another defect (PDA, ASD, VSD)
Truncus Arteriosus
Type of CHD:
- Complete occlusion of tricuspid valve (d/t unequal division of atrioventricular canal)
- Incompatible with life unless combined with another defect
- Associated lesions: large mitral valve underdeveloped RV, ASD, VSD, PDA
- Early cyanosis
- High mortality rate
Tricuspid atresia
Type or CHD:
- Pulmonary veins drain into left innominate vein or coronary sinus (instead of LA)
- Needs a patent foramen ovale or VSD for blood to go back to left side of heart
Total anomalous pulmonary venous return (TAPVR)
Th ff are morphological features of?
- Right atrial and ventricular dilatation
- Pulmonary trunk dilatation
- Hypoplastic LA
- LV is normal in size
Total anomalous pulmonary venous return
Type of Obstructive anomaly:
1. (+) obstruction at a segment of the aorta
Coarctation of Aorta
Types of coarctation of aorta (2)
- Infantile coarctation
(constriction Before ductus arteriosus = Baby) - Adult coarctation
(constriction After ductus arteriosus = Adult)
Type of coarctation of aorta:
1. Tubular hypoplasia of aortic arch proximal to PDA
Infantile Coarctation
Type of coarctation of aorta:
1. Ridge-like coarctation of aorta at level of ductus arteriosus
Adult coarctation
Type of Obstructive Anomaly:
- Obstruction at pulmonary valve
- With or without cyanosis
- S/Sx dependent on size of pulmonary stenosisl
- May be an isolated event or part of a complex anomaly
Pulmonary Stenosis or Atresia
The ff are associated lesions of which obstructive anomaly?
- RV hypertrophy
- Pulmonary artery hypoplasia
- RV hypoplasia in pulmonary valve atresia
- ASD
Pulmonary Stenosis or atresia
Type of obstructive anomaly:
- Narrowing/obstruction of aorta
- Adaptive response: LVH
Aortic stenosis or atresia
What are the types of aortic stenosis or atresia? (3)
- Valvular aortic stenosis
- Subaortic stenosis
- Supravalvular stenosis
Adaptive response of aortic stenosis
LVH
Type of aortic stenosis:
1. Cusps may be hypoplastic, dysplastic, abnormal in number
Valvular aortic stenosis
Type of aortic stenosis:
- Thickened ring or collar of dense endocardial fibrous tissue below the level of the cusps
- Associated with prominent systolic murmur
Subaortic stenosis
Type of aortic stenosis:
1. Represent an inherited form of aortic Dysplasia: ascending aortic wall is thickened –> luminal constriction
Supravalvular aortic stenosis
Types of valvular disease (6)
- Stenosis
- Insufficiency
- Functional regurgitation
- Singular valvular disease
- Mixed stenosis/regurgitation
- Combined multiple valvular disease
Type of Valvular Disease:
1. Failure of a valve to OPEN completing thus impending blood flow
Stenosis
Type of Valvular Disease:
1. Failure of a valve to CLOSE completely thus allowing regurgitation
Insufficiency
Type of Valvular Disease:
1. Incompetence of a valve resulting from an abnormality on one of its support structures
Functional regurgitation
Type of Valvular Disease:
1. Mitral valve is commonly affected
Singular valvular disease
Type of Valvular Disease:
1. Occurs when valvular stenosis and regurgitation coexist
(Example. RHD)
Mixed stenosis/regurgitation
Causes of valvular disease (2)
- Congenital valvular heart disease
2. Acquired valvular heart disease
Examples of congenital valvular heart disease (3)
- Congenital bicuspid valve
- Congenital calcific aortic stenosis
- Congenital mitral valve prolapse
Examples of acquired valvular heart disease (2)
- Rheumatic valvulitis
2. Infective endocarditis
Factors that affect clinical impact of valvular disease (4)
- Valve impairment m
- Degree of impairment
- Rate/duration of rate of involvement
- Rate and quality of compensatory mechanism
Major valvular heart diseases (7)
- Calcific aortic valve stenosis
- Congenital bicuspid aortic valve calcification
- Mitral annular calcification
- Infective endocarditis
- Non-bacterial thrombotic endocarditis
- Endocarditis in systemic lupus erythematosus (SLE)
- Carcinoid heart disease
- The most common of all valvular abnormalities
- Acquired, age related
- “Wear and tear”
Calcified aortic valve stenosis
Morphologic hallmark of calcified aortic valve stenosi
Heaped-up calcified masses within aortic cusps
(No commissural fusion
(3) Clinical manifestations of calcified aortic valve stenosis
- LVH
(Increased LV pressure 200mmHg) - Angina Pectoris
(Diminished microcirculations of hypertrophied myocardium) - Chronic Heart Failure, Syncope
(Impaired systolic and diastolic myocardial functions)
- The most frequent congenital cardiovascular malformation in humans
- Cusps are usually of unequal size (larger one has a midline raphe due to incomplete commissural separation during development)
- Associated with: aortic dissection, aortic coarctation, aneurysm
Congenital Bicuspid Aortic Valve Calcification
This is frequently the major site for calcific deposits (Congenital Bicuspid Aortic Valve Calcification)
Raphe
- Degenerative calcific deposits that developed in the peripheral annulus of the mitral valve
- Common in elderly women (60 years old), individuals with mitral valve prolapse, with elevated left ventricular pressure
- Doesn’t affect valvular function
Mitral Annular Calcification
What is the gross appearance of Mitral Annular Calcification
Irregular, stony hard, ulcerated nodules behind the mitral leaflets
What are the etiological agents in Infective Endocarditis? (5)
- Streptococcus viridans
- Staphylococcus aureus
- HACEK Group
- Rickettsia
- Chlamydia
The ff are predisposing disease to which Valvular Disease?
- Rheumatic valvulitis
- Degenerative atherosclerotic/syphilitic valve disease
- Congenital defects
- Prosthetic heart valves
Infective Endocarditis
Types of Infective Endocarditis (2)
- Acute IE
2. Subacute IE
The ff describe which type of valvular disease?
- Occurs in previously normal valve
- Bulky vegetation that may produce erosions/valvular ruptures
- Produces Necrotizing, ulcerative, destructive lesions
- Severe clinical s/sx, high morbidity
- Difficult to cure, requires surgery
Acute IE
The ff describe which type of valvular disease?
- Occurs in previously diseased valve
- Histology: granulation tissue indicative of healing (at base)
- Caused by less virulent organisms, insidious clinical onset
Subacute IE
- A serious infection characterized by colonization/invasion of heart valves or mural endocardium by a microbe
- Frequently involves AV or MV
- Tricuspid valve involved in drug mainliners
Infective Endocarditis
Hallmark of IE
Friable, bulky, potentially destructive vegetation (contain fibrin, inflammatory cells, bacteria)
The ff are clinical features of:
- Fever
- Nonspecific signs: weight lodge, fatigue, flu-like symptoms, anorexia
- Murmurs
Infective endocarditis
What is the most consistent sign in IE? (Clinical feature)
Fever
What are the embolic complications seen in IE? (3)
- Janeway Lesions
- Osler Nodes
- Roth Spots
Embolic Complication (IE):
- Small erythematous or hemorrhagic, macular, non-tender lesions
- Palms, soles
- The consequence of septic embolic events
Janeway lesions
Embolic Complication (IE):
- Small tender subcutaneous nodules
- Pulp of digits, fingers
Osler Nodes
Embolic Complication (IE): 1. Oval retinal hemorrhages with pale centers
Roth spots
Type of vascular disease:
- Sterile, nondestructive, noninvasive vegetations
- Does not elicit inflammation
- Associated with debilitated patients, carcinoma, sepsis, cachexia
- May be part of Trousseau Syndrome of migratory thrombophlebitis
Non-bacterial Thrombotic Endocarditis (NBTE)
Pathogenesis of NBTE (3)
- Hypercoagulable states
- Malignancy
- Istrumentation
The ff are gross features of which valvular disease?
- Small nodules along the lines of closure
- Bulky and friable, may be single or multiple
- Leaflets are often normal
- Atrial and mitral valve
NBTE
The ff are gross features of which valvular disease?
- Bland thrombi loosely attached to underlying valve
- No inflammation
- No organism identified/present
NBTE
Which valvular disease is associated with SLE?
Libman-Sacks Endocarditis
Which valves are affected by Libman-Sacks Endocarditis? (2)
- MV
2. TV
Type of valvular disease:
- Sterile, small vegetations on the undersurface of cords, mural (sterile vegetations beneath valves)
- Fibrinoid necrosis of valves
Libman-Sacks Endocarditis
The are histologic features of which valvular disease?
1. Finely granular, fibrinous eosinophilic material containing “hematoxylin bodies”
Libman-Sacks Endocarditis
What constitutes Carcinoid Syndrome? (5)
- Episodic skin flushing
- Cramps
- Nausea
- Vomiting
- Diarrhea
Right sided endocarditis
Carcinoid endocarditis/heart disease
Which valves are affected by carcinoid endocarditis? (2)
- Pulmonary valve
2. Tricuspid valve
Lesion seen in carcinoid endocarditis (1)
Endocardial thickening/fibrosis
In this valvular disease, there is elaboration of serotonin, histamine, bradykinin, prostaglandin, gastroactive amines
Carcinoid endocarditis
An acute immunologically mediated, multiway stem inflammatory disease following group A streptococci pharyngitis
Rheumatic Fever
Rheumatic fever is an effect of what?
An antibody against streptococcus
It is not a direct effect of strep
What are the clinical features or rheumatic fever (major criteria of jones)? (5)
- Carditis
- Arthritis
- Subcutaneous nodules
- Erythema
- Sydenham’s Chorea (neurologic disorder, epileptic-like involuntary rapid, muscular, purposeless movement)
What are the clinical features or rheumatic fever (minor criteria of jones)? (5)
- Fever
- Arthralgia
- C-reactive protein
- ESR (erythrocyte sedimentation rate)
- Anti-streptolysin O
In RF, what will you see if pericardium is affected? (Gross)
Fibrinous pericarditis or bread and butter appearance
In RF, what will you see if myocardium is affected? (Histo)
Aschoff body in INTERSTITIUM (not muscle cells)
In RF, what will you see if endocardium is affected? (Gross)
Verrucal vegetations (on lines of closure)
Plump activated macrophages
Anitschkow cells aka caterpillar cells (zig zag pattern)
Necrotic vegetations on lines of valve closure that resemble rosary beads
Hint: RF
Verrucae
Irregular thickenings, usually in LA caused by subendocardial lesions
Hint: RF
MacCallum bodies
It is a complication of recurrent bouts of RF
Rheumatic heart disease (RHD)
The ff are Morphologic features of which valvular disease?
- Multivalvular (MV –> AV –> TV)
- Commissural fusion of cusps –> stenosis
- Fish mouth deformity / buttonhole stenosis
- Shortening/thickening of chordae
- Fibrosis
RHD
What are the four major forms of vegetative endocarditis?
- RHD
- IE
- NBTE
- LSE
What is the key histologic change in mitral valve prolapse?
Myxomatous degeneration of mitral valve
Notes
- Mitral valve is enlarged, hooded, or floppy –> tendency to prolapse into LA during systole
- Developmental anomaly of connective tissue
- Most are idiopathic
- Fibrillin 1 mutations (Marfan’s Syndrome)
The ff are gross features of which valvular disease?
- Interchordal hooding or ballooning of MV into left atrium
- MV leaflets are floppy, enlarged, redundant, thickend, rubbery, fibrotic, calcified
- Associated tendinous cords may be elongated, thinned, ruptured
- Annular dilatation is common
MVP
The ff are clinical features if which valvular diseas?
- Majority are asymptomatic
- Mid-systolic click
- Late systolic or holosystolic murmur from mitral regurgitation
- Symptoms: chest pain, dyspnea, fatigue, depression
MVP
Complications of MVP (4)
- IE
- Mitral insufficiency
- Thrombo-embolic complications
- Arrhythmias
Disadvantage of a bioprosthetic valve (2)
- Mechanical detioration
2. Calcification
Disadvantage of a man-made prosthetic valve (1)
- Thrombi formation
Types of pericardial disease (6)
- Serous pericarditis
- Fibrinous
- Hemorrhagic
- Purulent
- Caseous
- Healed
Type of pericardial disease:
- Causes: RHD, SLE, uremia, viral
- Scanty inflammation
- (+/-) pericardial serous effusion
- Rare sequelae: organization fibrosis
- Histology: mild inflammatory infiltrate in epipericardial fat (mostly lymphocytes)
Serous pericarditis
Type of pericardial disease:
- Causes: RHD, post-radiation, post-infarction, AMI, Dressler Syndrome
- The most common type of pericarditis
- Shaggy, rough pericardium (bread and butter)
- Sequelae: resolution, lysis, organization
- Most striking characteristic: loud pericardial rub
Fibrinous pericarditis
It is a secondary form of pericarditis that occurs in th stating of injury to the heart or pericardium. It consists of a triad of features:
- Fever
- Pleuritic pain
- Pericardial effusion
Dressler Syndrome
What is the triad of features of Dressler Syndrome?
- Fever
- Pleuritic pain
- Pericardial effusion
Type of pericardial disease:
- Causes: TB, tumor metastasis, bacteria
- Mixture of a huge purulent and fibrinous effusion
- Exudate: blood + fibrinous or suppurative effusion
Hemorrhagic pericarditis
Pathways of spread of Purulent Endocarditis (3)
- Hematogenous seeding
- Lymphatic extension
- Iatrogenic
Type of pericardial disease:
- Rare type
- Tuberculous in origin
- A frequent antecedent of disabling, fibrocalcific, chronic constrictive pericarditis
Caseous Pericarditis
Type of pericardial disease:
- Organization produces “soldier’s plaque” (plaque-like fibrous thickenings)
- Rarely causes impairment of cardiac function
Healed pericarditis
(2) types of healed pericarditis
- Adhesive mediastinopericarditis
2. Constrictive pericarditis
Type of healed pericarditis:
1. Obliteration of pericardial sac with adhesion to parietal pericardium
Adhesive mediastinopericarditis
Type of healed pericarditis:
- Obliterated sac with encasement by dense fibrous tissue
- Diastolic expansion
Constrictive pericarditis
Types of cardiac neoplasms (2)
- Myxoma
2. Rhabdomyoma
Type of cardiac neoplasm:
- Most common primary cardiac tumor in adults
- Associated with clonal abnormalities of chromosomes 12 and 17
- 90% located in LA
- Complication: ball-valve obstruction and embolization
Myxoma
Favored site of origin of myxoma
Fossa ovalis in atrial septum
The ff are gross features of which cardiac neoplasm?
- Sessile or pedunculated masses (globular)
- Soft, translucent, papillary, or villus lesions
- Gelatinous appearance
Myxoma
Type of cardiac neoplasm:
- Most frequent primary cardiac tumor in children
- Location: ventricular chamber
- Causes valvular or outflow tract obstruction
Rhabdomyoma
The ff are gross features of which cardiac neoplasm?
- Gray-white, fleshy, myocardial masses
- Location: either on left or right side of heart, extending in ventricular Chambers
Rhabdomyoma
The ff are microscopic features of which cardiac neoplasm?
- Large, rounded, or polygonal cells
- Cytoplasmic glycogen separated by strands of cytoplasm running from plasma membrane to nucleus
Rhabdomyoma