52 Questions
What is the most common risk factor for aortic dissection?
Hypertension
Which type of aneurysm is characterized by weakened arterial wall secondary to infection?
Mycotic aneurysm
Which condition is associated with aortic regurgitation as a clinical presentation?
Marfan's syndrome
What is the hallmark feature of a true aneurysm?
Surrounded by complete vessel wall
What is the main pathogenesis leading to the formation of syphilitic aneurysms?
Endarteritis obliterans of vasa vasorum
Which condition is characterized by fibrinous thickenings and adhesions in the pericardium?
Chronic pericarditis
What is the main cause of abdominal aortic aneurysms?
Inflammatory diseases
What histological feature is typically seen in aortic aneurysms due to cystic medial degeneration?
Mucoid deposition
Which factor is implicated in the formation of amyloid in abnormal fibrillar form?
Micelle formation
Which of the following is NOT a risk factor for abdominal aortic aneurysms?
Female gender
What process leads to local hypoxia, medial neovascularization, and inflammation in aortic aneurysms?
Mural thrombus formation
What is a common cause of intrarenal aneurysms?
Kawasaki syndrome
'Rupture associated with straining or orgasm' is a characteristic of which type of aneurysm?
Syphilitic aneurysm
Which complication can occur due to distal embolization in patients with aneurysms?
Blue toe syndrome
'Unequal upper limb pulses' as a clinical presentation is primarily seen in which condition?
Marfan's syndrome
'Attenuation of aortic media' is a characteristic feature of which condition?
Abdominal aortic aneurysm
What is the common cause of left heart failure?
Connective tissue diseases
Which of the following is a characteristic of right ventricular failure morphology?
Cardiac cirrhosis
What is the main cause of dilated cardiomyopathy?
Familial factors
Which condition is known for mutations in genes encoding sacromeric proteins?
Hypertrophic cardiomyopathy
Which feature is typically seen in the microscopic morphology of dilated cardiomyopathy?
Interstitial fibrosis
What is the hallmark pathogenesis of hypertrophic cardiomyopathy?
Mutations in sacromeric proteins
What is the most common motif found in amyloidosis?
Beta sheet structure
Which disease is NOT associated with amyloid like fibrils in the brain and heart?
Alzheimer's disease
What type of amyloidosis is associated with plasma cell dyscrasias such as myeloma?
Systemic AL amyloidosis
Which amyloidosis type is NOT correctly matched with its associated protein?
ACal - Calcitonin protein
Which disease is NOT a form of transmissible spongiform encephalopathy?
Huntington's disease
Which technique is NOT useful for definitive tissue diagnosis of amyloidosis?
Fluorescence in situ hybridization (FISH)
What characteristic finding can be observed on echocardiogram in patients with amyloidosis?
Sparkling appearance
Which treatment option is NOT commonly used for systemic AL amyloidosis?
Cardiac catheterization
What is the primary cause of restrictive cardiomyopathy?
Decrease in ventricular compliance
Which of the following is a common symptom of left heart failure?
Elevated pulmonary capillary wedge pressure
What is a characteristic microscopic finding in myocarditis?
Focal myocyte necrosis
Which of the following is typically observed in pericarditis morphology?
Lymphocytic infiltrate
What is a common cause of acute pericarditis?
Viral infections
What distinguishes the gross morphology of restrictive cardiomyopathy from myocarditis?
Normal ventricles with firm myocardium
Which symptom is most likely associated with right heart failure?
Weight gain
Which of the following diseases is associated with the abnormal accumulation of beta-amyloid proteins in the brain and heart?
Huntington's disease
In the context of amyloidosis, which type is characterized by the deposition of transthyretin protein in various tissues?
ATTR amyloidosis
Which treatment option is commonly used to target prion diseases by inhibiting post-translational modifications of the prion protein?
Pharmacological chaperone therapy
Which of the following is a key feature of the beta sheet structure predominant in amyloid fibrils?
Antiparallel arrangement
Which of the following conditions is NOT typically treated using immunosuppressive therapy for systemic involvement of amyloidosis?
Systemic Aβ2M amyloidosis
Which type of structure is most commonly associated with amyloidosis?
Beta sheet structure
Which disease is characterized by amyloid-like fibrils in the brain and heart, and is heritable with trinucleotide repeats?
Huntington's disease
Which of the following is NOT a characteristic of prions?
Associated with trinucleotide repeats
Which type of amyloidosis is associated with myeloma and plasma cell dyscrasias?
Systemic AL
What is a possible treatment option for hereditary amyloidosis?
Liver transplant
What is the most common motif found in amyloidosis?
Beta-sheet structure
Which disease is NOT associated with amyloid like fibrils in the brain and heart?
Parkinson's disease
What treatment option is NOT commonly used for systemic AL amyloidosis?
Surgical resection
Which of the following is typically observed in pericarditis morphology?
Fibrinous thickenings
What is the hallmark pathogenesis of hypertrophic cardiomyopathy?
Mutations in genes encoding sacromeric proteins
Study Notes
Aneurysms
- Aneurysms are localized, abnormal, permanent dilations of a blood vessel.
- True aneurysms are surrounded by a complete vessel wall, with all layers affected.
- False aneurysms (pseudoaneurysms) are extravascular hematomas that communicate with the intravascular space.
- Types of aneurysms include:
- Saccular (berry) aneurysms
- Fusiform aneurysms (e.g. abdominal aortic aneurysm)
- Mycotic aneurysms (weakened arterial wall due to infection)
Aortic Dissection
- Most (>80%) aortic dissections develop without a pre-existing thoracic aortic aneurysm.
- Risk factors include hypertension, old age, atherosclerosis, Marfan's, Turner's, bicuspid aortic valve, Takayasu's arteritis, familial, and pregnancy.
- Histology: cystic medial degeneration, decreased vascular smooth muscle, mucoid deposition, elastin deficiency, and fragmentation.
Amyloidosis
- Amyloid is a protein in an abnormal fibrillar form, resistant to degradation.
- Amyloid formation relies on the polymerization of fibrils from an unstable intermediate state.
- Types of amyloid include:
- Systemic AL (Ig LC, lambda)
- Systemic AA (SAA, reactive systemic, chronic inflammatory)
- Abeta2M (Beta2-microglobulin, dialysis associated, chronic renal failure)
- Localized Abeta2 (APP, senile cerebral, Alzheimer's)
- ACal (Calcitonin, Endocrine, Medullary thyroid carcinoma)
- ATTR (Transthyretin, Familial amyloidotic neuropathies or systemic senile amyloidosis)
Cardiomyopathy, Myocarditis, and Pericarditis
- Causes of heart failure:
- Left ventricular failure: volume overload, pressure overload, loss of muscles, and restricted filling
- Right heart failure: left heart failure, lung disorders
- Morphology of left ventricular failure: gross depends on disease processes, LVH, and dilation; microscopy shows myocyte hypertrophy and interstitial fibrosis
- Morphology of right ventricular failure: heart varies with cause, typically RVH and dilation, possibly atrial dilation; liver shows nutmeg liver (chronic passive congestion), centrilobular necrosis, and cardiac cirrhosis; subcutaneous tissue shows pedal and sacral edema
Heart Failure
- Dilated cardiomyopathy/congestive heart failure: progressive cardiac dilation and contractile dysfunction, usually with concomitant hypertrophy
- Gross morphology: enlarged "flabby" heart due to dilation of all chambers
- Microscopic morphology: non-specific, may see hypertrophic myocytes
- Pathogenesis: secondary to ischemic heart disease, familial, post-myocarditis, toxins, hemochromatosis, sarcoidosis, and peripartum
Test your knowledge on the morphology of pericarditis and aneurysms by answering questions on different types of pericarditis, such as serous, fibrinous, purulent, and chronic/healed. Additionally, assess your understanding of true and false aneurysms, including their characteristics and causes.
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