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