path cvs - formatted Flashcards
- 67.APRIL02 A 7-year-old girl presents with a systemic illness after a documented group A streptococcal pharyngitis. Acute rheumatic fever is suspected. Which of the following combination of manifestations IS NOT diagnostic of this condition using the Jones criteria:
- Carditis and Sydenham chorea
- Migratory polyarthritis of large joints and subcutaneous nodules
- Migratory polyarthritis of large joints and Sydenham chorea
- Erythema marginatum skin lesions and carditis
- Subcutaneous nodules and fever
- Subcutaneous nodules and fever - F - this only constitutes 1 major and 1 minor criteria
• Criteria apply only to an initial attack
• “High probability” of ARF if evidence of recent Gp A Strep inf (throat cx, streptozyme, or elevated or rising strep Ab titers) and (2 major or 1 major + 2 minor)
Major Minor
Carditis Arthralgia
Polyarthritis Fever
Chorea Elevated ESR
Erythema marginatum Elevated CRP
Subcutaneous nodules Prolonged PR interval
*LW: major criteria "JONES" J - Joint involvement O - O looks like a heart, so carditis. N - Nodules, subcutaneous E - Erythema marginatum S - Syndeham corea.
• Rheumatic fever is characterized by a constellation of findings that includes as major manifestations
(1) migratory polyarthritis of the large joints
(2) carditis
(3) subcutaneous nodules
(4) erythema marginatum of the skin
(5) Sydenham chorea– a neurologic disorder with involuntary purposeless, rapid movements
- 65.APRIL02 An obstetric ultrasound examination reveals a fetus with an abnormal pulmonary outflow tract. The pulmonary artery appears to arise from the left ventricle but the AV connections are normal. The aorta appears to arise from the right ventricle. Which of the following statements about this condition is most correct?
- Two thirds of cases are associated with a patent foramen ovale or patent ductus arteriosus
- A VSD is a rare association (<1%)
- Untreated cases often present with pulmonary hypertension in their 2-3 rd decades
- Berry aneurysms of the circle of Willis are a common association
- Right ventricular hypertrophy only develops when there is associated pulmonic stenosis
- Two thirds of cases are associated with a patent foramen ovale or patent ductus arteriosus (Those with only a patent foramen ovale or PDA (about 65%), however, have unstable shunts that tend to close and therefore require immediate intervention to create a shunt (such as balloon atrial septostomy) within the first few days of life)
- 65.APRIL02 An obstetric ultrasound examination reveals a fetus with an abnormal pulmonary outflow tract. The pulmonary artery appears to arise from the left ventricle but the AV connections are normal. The aorta appears to arise from the right ventricle. Which of the following statements about this condition is most correct?
- Two thirds of cases are associated with a patent foramen ovale or patent ductus arteriosus (Those with only a patent foramen ovale or PDA (about 65%), however, have unstable shunts that tend to close and therefore require immediate intervention to create a shunt (such as balloon atrial septostomy) within the first few days of life)
- A VSD is a rare association (<1%) (Patients with transposition and a VSD (about 35%) have a stable shunt)
- Untreated cases often present with pulmonary hypertension in their 2-3 rd decades (Without surgery, most patients die within the first months of life)
- Berry aneurysms of the circle of Willis are a common association
- Right ventricular hypertrophy only develops when there is associated pulmonic stenosis (Right ventricular hypertrophy becomes prominent as this chamber functions as the systemic ventricle)
• Transposition implies ventriculoarterial discordance, such that the aorta arises from the right ventricle, and the pulmonary artery emanates from the left ventricle.
• The AV connections are normal (concordant), with right atrium joining right ventricle and left atrium emptying into left ventricle
• The essential embryologic defect in complete transposition is abnormal formation of the truncal and aortopulmonary septa.
• The aorta arises from the right ventricle and lies anterior and to the right of the pulmonary artery;
o in contrast, in the normal heart, the aorta is posterior and to the right.
• The result is separation of the systemic and pulmonary circulations, a condition incompatible with postnatal life, unless a shunt exists for adequate mixing of blood.
o Patients with transposition and a VSD (about 35%) have a stable shunt.
o Those with only a patent foramen ovale or PDA (about 65%), however, have unstable shunts that tend to close and therefore require immediate intervention to create a shunt (such as balloon atrial septostomy) within the first few days of life.
o Right ventricular hypertrophy becomes prominent as this chamber functions as the systemic ventricle.
o Concurrently the left ventricle becomes thin-walled (atrophic) as it supports the low-resistance pulmonary circulation.
• The outlook for infants with transposition of the great vessels depends on the degree of mixing of the blood, the magnitude of the tissue hypoxia, and the ability of the right ventricle to maintain the systemic circulation.
• Without surgery, most patients die within the first months of life.
• Currently, most patients undergo a reparative operation (usually entailing transection and switching of the great arteries as well as the coronary arteries) during the first several weeks of life.
- 100.APRIL02 A 60-year-old man with a 2-year history of myeloma develops cardiac failure associated with a restrictive cardiomyopathy. The most likely cause is:
- Drug associated cardiomyopathy secondary to chemotherapy
- Amyloidosis with deposition of AA type amyloid 2
- Viral cardiomyopathy associated with immune suppression
- Amyloidosis with deposition of AL type protein
- Simple ischaemic cariomyopathy
- Amyloidosis with deposition of AL type protein
Restrictive cardiomyopathy is characterised by decreased ventricular compliance, resulting in impaired filling during diastole. It can be idiopathic or associated with distinct diseases that affect the myocardium, principally radiation fibrosis, amyloidosis, sarcoidosis, metastatic tumor, or products of inborn errors of metabolism.
• AL (immunocyte-associated / 1o) - immunoglobulin light chains (lambda)
o cannot distinguish from AA but often involves heart, kidney, GIT, peripheral Ns, skin, tongue,
o also odd sites – eyes, respiratory tract
o Associated diseases: MM & other monoclonal B-cell proliferations
Heart
• More common in AL – amyloidosis, and in (senile systemic amyloidosis?)
• Usually normal but may be firm & enlarged
• Myocardial & subendocardial deposits → conduction abnormalities, restrictive CMO, CCF
Involvement of the cardiovascular system by amyloidosis occurs in four general forms:
- The most common presentation of cardiac amyloidosis is that of Restrictive CM. Right-sided findings dominate the clinical presentation; peripheral edema is a prominent finding, whereas paroxysmal nocturnal dyspnea and orthopnea are absent. Amyloid infiltration of the myocardium results in increased stiffness of the myocardium, producing the characteristic diastolic dip and plateau (square root sign) in the ventricular pressure pulse that may simulate constrictive pericarditis. In contrast to the accelerated early left ventricular diastolic filling found in constrictive pericarditis, cardiac amyloidosis is marked by an impaired rate of early diastolic filling.
- A second common presentation is congestive heart failure due to systolic dysfunction. Hemodynamic evidence of restriction of ventricular filling may not be prominent in these patients. In some patients amyloid deposition in the atria may be responsible for loss of atrial transport function despite the maintenance of electrical “sinus” rhythm, with the production of congestive heart failure. The course of this form of the disease is often one of relentless progression, usually poorly responsive to treatment. Angina pectoris occurs on occasion despite angiographically normal coronary arteries.
- Orthostatic hypotension occurs in about 10 percent of cases. Although most likely due to amyloid infiltration of the autonomic nervous system or of blood vessels, amyloid deposition in the heart and adrenals may contribute to the pathogenesis of this variant. Hypovolemia as a result of the nephrotic syndrome secondary to renal amyloidosis may aggravate the postural hypotension.
- An abnormality of cardiac impulse formation and conduction is the fourth and least common mode of presentation and may result in arrhythmias and conduction disturbances. Sudden death, presumably arrhythmic in origin, is relatively common and may be preceded by episodes of syncope
- 71.APRIL02 A 54-year-old man with headache has a normal CT head with intravenous contrast. He leaves your hospital after 15 minutes but returns 1 hour later as an unwitnessed collapse, dead on arrival. Autopsy of the heart reveals a mottled slightly yellow area in the anterior wall of the left ventricle. Histology shows necrotic myocytes with an intense acute inflammatory infiltrate devoid of granulation tissue. This is mostly likely to represent;
- Contrast associated myocarditis
- A hyper-acute myocardial infarct (1 – 2 hours old)
- A myocardial infarct less than 24 hours old
- A myocardial infarct of approximately 48 hours old
- A myocardial infarct of approximately 7 days old
- A myocardial infarct of approximately 48 hours old
12-24 hr Dark mottling
Ongoing coagulation necrosis; pyknosis of nuclei; myocyte hypereosinophilia; marginal contraction band necrosis; beginning neutrophilic infiltrate
1-3 days Mottling with yellow-tan infarct center
Coagulation necrosis, with loss of nuclei and striations; interstitial infiltrate of neutrophils
3-7 days Hyperemic border; central yellow-tan softening
Beginning disintegration of dead myofibers, with dying neutrophils; early phagocytosis of dead cells by macrophages at infarct border
- 69.APRIL02 A 27-year-old male presents with hypertension in the upper extremities and , weak pulses in the lower limbs. A chest radiograph shows an abnormal aortic arch contour and inferior rib notching. Which of the following IS A LEAST LIKELY association;
- Bicuspid aortic valve
- Berry aneurysms of the Circle of Willis
- Cor pulmonale
- Mitral regurgitation
- Atrial septal defect
- Cor pulmonale
Coarctation Associations¬
• bicuspid aortic valve in 50 % congenital aortic stenosis
• ASD
• VSD
• mitral regurgitation
• Berry aneurysms of the circle of Willis
- 60.APRIL02 A patient has a cardiac scan two days after confirmed myocardial infarct. This shows that an area of viable myocardium in an area that is non-contractile on echocardiography. This is best explained by:
- Hibernating myocardium
- Stunned myocardium post infarct
- Luxury perfusion post infarct
- Hyperdynamic myocardium adjacent to the infarct
- Troponin cross reactivity
- Stunned myocardium post infarct ACUTE
- Hibernating myocardium is viable myocardium with depressed contractile dysfunction resulting from chronic ischemia (months to years) insufficient to cause tissue necrosis. Contractility recovers quickly once coronary flow is restored.
- Stunned myocardium exhibits sustained (hours to days) reduced regional contractility after coronary reperfusion from an acute ischemic insult. However, it will respond to inotropic stimulation, and this behavior of the myocardium can be exploited to differentiate between stunned and infarcted myocardium.
- Low-dose dobutamine stress echocardiography (0–20 µg/kg per minute) has been used to test for viability in the form of transient recovery of function.
- Assessment for reversible dysfunction is important for prognosis and to identify high-risk patients, who may benefit most from aggressive revascularization therapy.
- 61.APRIL02 A 42-year-old man is referred for a nuclear heart scan and cardiac enzyme assay. He had 4 hours of “severe reflux” one week earlier. The enzyme diagnosis of a week old myocardial infarct best be supported by finding elevations of which of the following;
- CK MB (Creatinine kinease MB)
- LDH (Lactate dehydrogenase)
- LDH and AST (lactate dehydrogenase and aspartamine transaminase)
- AST and CK (aspartamine transaminase and creatinine kinease)
- LDH and TnT (Tn) (Lactate dehydrogenase and Troponin T)
- LDH and TnT (Tn) (Lactate dehydrogenase and Troponin T)
.21 Man with right sided heart failure LEAST LIKELY finding is ?
- Leibmann Sachs endocarditis (SLE)
- Arrythmogenic right ventricular cardiomyopathy
- Asbestosis
- Ischaemia involving right ventricle
- Ischaemia involving right ventricle and posterior wall of ventricle
- Leibmann Sachs endocarditis (SLE) (no heart failure)
- 11.02.32 What is most important factor in myocardial infarction?
- Size
- Site
- Transmural versus subendocardial
- Involvement of right ventricle wall
- Transmural versus subendocardial - higher risk of complications
- 11.03.17 Patient with AMI, delevops chest pain, 5 days later LEAST LIKELY cause is ?
- Second AMI
- Dresslers syndrome
- Bronchopneumonia
- PE
- Myocardial rupture
- Dresslers syndrome (onset 2-3 weeks)
- 11.03.17 Patient with AMI, develops chest pain, 5 days later LEAST LIKELY cause is? Rob p365
- Second AMI
- Dresslers syndrome (onset 2-3 weeks)
- Bronchopneumonia
- PE (thromboembolism occurs with 2 weeks in upto 40%)
- Myocardial rupture (occurs anytime in first 2 weeks)
April 2004 – myocardial rupture – peak time of occurrence
- 7.02.20 Man dies from AMI macroscopic pathology shows yellow area in myocardium with granulation tissue at the margin. How old is the infarct?
- 3 days
- Less than 2 weeks
- 2-8 weeks
- 8-10 weeks
- Months
- Less than 2 weeks (10days)
7-10 days Maximally yellow-tan and soft, with depressed red-tan margins
Well-developed phagocytosis of dead cells; early formation of fibrovascular granulation tissue at margins
- 11.02.49 Sudden cardiac death is associated with the following except:
- Mitral valve prolapse
- Mitral stenosis
- Aortic stenosis
- HOCM
- Pulmonary Hypertension
- Mitral stenosis
SCS: looks like PAH patients get ventricular arrhythmias leading to SCD. Or compresion of LCA by big MPA.
- 11.03.23 In hypertensive heart disease which is LEAST TRUE ?
- Circumferential enlargement of left ventricle
- Mild ventricular dilatation classically accompanies cardiac enlargement
- Mild diastolic filling impairment
- Increase in muscle mass more than cardiac size
- Mild ventricular dilatation classically accompanies cardiac enlargement (concentric hypertrophy of the left ventricle without dilatation and no other possible causitive lesion)
- 11.03.23 In hypertensive heart disease which is LEAST TRUE ? Rob p372
- Circumfrential enlargement of left ventricle (by definition)
- Mild ventricular dilatation classically accompanies cardiac enlargement (concentric hypertrophy of the left ventricle without dilatation and no other possible causitive lesion)
- Mild diastolic filling impairment (increased thickness eventually impairs dystolic function)
- Increase in muscle mass more than cardiac size (increase in weight disproportionate to the increase in overall size)
- 11.03.06 In rheumatic fever, valve damage is via ?
- Cross reaction of antigens to Group B Staph and endocardium
- Cross reaction to bacterial glycoproteins in capsule
- Entry is by breach in skin and mucous membranes
- Initially cysts begin at microscopic level
- Direct toxic effect on valve
- Cross reaction to bacterial glycoproteins in capsule - T - M proteins of certain streptococcal strains induce host antibodies that cross react with tissue glycoproteins in the heart, joints, and other tissues in genetically susceptible individuals (3% of infected patients).
- 11.03.06 In rheumatic fever, valve damage is via ? Rob p111, 375-378 (GC)
- Cross reaction of antigens to Group B Staph and endocardium - F - group A (beta-haemolytic) strep.
- Cross reaction to bacterial glycoproteins in capsule - T - M proteins of certain streptococcal strains induce host antibodies that cross react with tissue glycoproteins in the heart, joints, and other tissues in genetically susceptible individuals (3% of infected patients).
- Entry is by breach in skin and mucous membranes - F - acute RF is a hypersensitivity reaction. Onset of symptoms occurs 2-3 wks after original infection, and strep is absent in lesions (the original infection is usually pharyngitis, rarely skin/other infection). Valve (endocardial) involvement in acute RF is the result of fibrinoid necrosis along closure line forming vegetations (verrucae); it is the chronic stage (organisation and fibrosis) that results in valvular damage - leaflet thickening, commisural fusion, ‘fish mouth’ stenoses etc.
- Initially cysts begin at microscopic level - F - histologic hallmark is the Aschoff body = central zone of degenerating hypereosinophilic extracellular matrix, with lymphocyte/plasma cell infiltration, plump macrophages (Anitschow cells).
- Direct toxic effect on valve - F - see option 3 above.
- 11.02.70 8yr old girl having anaesthetic for MRI to investigate choreiform movements. Murmur heard by anaesthetist most likely due to ?
- Previous rheumatic fever
- Previous rheumatic fever
- 11.03.25 Autopsy on 40 year old male shows abnormal aortic valve with 2 leaflets one of which is large with median raphe, which is TRUE ?
- Bicuspid Aortic valve occurs in 0.1% of poplulation
- Bicuspid Aortic valve occurs in 1% of poplulation
- Patient has rheumatic heart disease
- Patient has calcific stenosis
- Patient has Marfans
- Bicuspid Aortic valve occurs in 1% of population - T - 1-2% of the population has bicuspid aortic valve as an isolated abnormality. The two cusps are of unequal size with the larger cusp having a midline raphe. (Robbins)
- 11.03.25 Autopsy on 40 year old male shows abnormal aortic valve with 2 leaflets one of which is large with median raphe, which is TRUE ? Rob p379 Big Rob p568 (JS)
- Bicuspid Aortic valve occurs in 0.1% of poplulation - F
- Bicuspid Aortic valve occurs in 1% of population - T - 1-2% of the population has bicuspid aortic valve as an isolated abnormality. The two cusps are of unequal size with the larger cusp having a midline raphe. (Robbins)
- Patient has rheumatic heart disease - F - bicuspid valves are predisposed to infective endocarditis. They are also associated with aortic coarctation, aneurysm and dissection.
- Patient has calcific stenosis - F - predisposed to progressive degenerative calcification
- Patient has Marfans - F - Marfans is associated with MVP
- 11.03.24 Complications of mitral valve prolapse, which is FALSE ?
- Aortic regurgitation secondary to annular dilation
- Infective endocarditis
- Stroke
- Sudden death
- Mitral insuffiency
- Aortic regurgitation secondary to annular dilation - F - this is see in Marfans with loss of medial support results in progressive dilatation of the aortic ring and root of the aorta, giving rise to severe aortic incompetance
- 11.03.24 Complications of mitral valve prolapse, which is FALSE ? Rob p379 (JS)
- Aortic regurgitation secondary to annular dilation - F - this is see in Marfans with loss of medial support results in progressive dilatation of the aortic ring and root of the aorta, giving rise to severe aortic incompetance
- Infective endocarditis - T - manyfold more frequent than in general population. Overall only 3% of patients with MVP develop complications.
- Stroke - T - emboli of leaflet or atrial wall thrombi can cause strokes or systemic infarcts.
- Sudden death - T - uncommon but due to ventricular or atrial arrhythmias
- Mitral insuffiency - T - due to leaflet deformity, dilation of the annulus or cordal lengthening or sudden onset due to cordal rupture.
- 11.03.19 Patient with large exophytic mass on cardiac valve referred for CT:
- If has splenic lesions is most likely Candida
- If has non dilated cardiomyopathy is most likely carcinoid
- If has pericardial effusion is likely Staph with abscess
- If has thromboemboli is unlikely to be marantic endocarditis
- If has pulmonary infiltrates patient may have Wegners
- If has pericardial effusion is likely Staph with abscess - T
* LW: probably most correct: Valvular vegetations can erode into underlying myocardium to produce ring abscess. Can be associated with pericardial effusion.
[Pretty limited info to go on in question stem, likely incomplete recall]
- 11.03.19 Patient with large exophytic mass on cardiac valve referred for CT: Rob p381 (–)
- If has splenic lesions is most likely Candida (Streptococci and staphylococci were the causative organisms in 85%):
* LW: could be true, as large valvular lesions are usually fungal. - If has non dilated cardiomyopathy is most likely carcinoid: *LW False, right sided valvular disease.
- If has pericardial effusion is likely Staph with abscess - T -
- If has thromboemboli is unlikely to be marantic endocarditis: *LW - False: Marantic endocarditis commonly associated with thromboemboli. (frequently occurs concomitantly with venous thromboses or pulmonary embolism, suggesting a common origin in a hypercoagulable state with systemic activation of blood coagulation such as disseminated intravascular coagulation)
- If has pulmonary infiltrates patient may have Wegners: False - (most likely IV drug user - S. aureus, Candida parapsilosis and C. albicans)
- 11.03.22 Patient with restrictive cardiomyopathy, LEAST LIKELY finding on Chest CT is ?
- Basal bullous change
- Radiotherapy changes
- Bilateral hilar lymphadenopathy
- Multiple pulmonary nodules
- Bilateral bronchiectasis
- Basal bullous change - F - This is not typical of any of the causes of restrictive cardiomyopathy. Restrictive cardiomyopathy is characterised by decreased ventricular compliance, resulting in impaired filling during diastole. It can be idiopathic or associated with distinct diseases that affect the myocardium, principally radiation fibrosis, amyloidosis, sarcoidosis, metastatic tumor, or products of inborn errors of metabolism.
- 11.03.22 Patient with restrictive cardiomyopathy, LEAST LIKELY finding on Chest CT is ? Rob p387 (JS)
- Basal bullous change - F - This is not typical of any of the causes of restrictive cardiomyopathy. Restrictive cardiomyopathy is characterised by decreased ventricular compliance, resulting in impaired filling during diastole. It can be idiopathic or associated with distinct diseases that affect the myocardium, principally radiation fibrosis, amyloidosis, sarcoidosis, metastatic tumor, or products of inborn errors of metabolism.
- Radiotherapy changes - T - radiation fibrosis
- Bilateral hilar lymphadenopathy - T- sarcoidosis
- Multiple pulmonary nodules - T - metastatic tumour/sarcoid.
- Bilateral bronchiectasis - T - amyloidosis secondary to chronic lung disease/ sarcoid- traction bronchiectasis from fibrosis.
20. Sep03.22 4 y.o. in ICU with cardiomegaly. Discharged, then one month later – left atrial enlargement. Cause? Kawasaki Takayasu Rheumatic fever Subacute bacterial Endocarditis
- Sep03.22 4 y.o. in ICU with cardiomegaly. Discharged, then one month later – left atrial enlargement. Cause?
Rheumatic fever
- mitral valve involved by itself 70% of the time, with mitral and aortic valves involved 25% of the time
- chronic mitral stenosis leads to left atrial hypertrophy
SCS Added options.
- Sep03.24 Atrial myxoma – atypical feature.
- 3cm papillary soft tissue mass sessile
- 8 cm globular mass pedunculated
- atrial septum
- can prolapse into ventricle
- more common in the right atrium
- more common in the right atrium - F - left to right ratio is 4:1 with 90% located in the atria
- Sep03.24 Atrial myxoma – atypical feature. (JS)
- 3cm papillary soft tissue mass sessile - T - sessile or pedunculated masses that vary from hard globular masses mottled with haemorrhage to soft, translucent, papillary or villous lesions having a gelatinous appearance
- 8 cm globular mass pedunculated - T - size range is 1 to 10cm and they are usually solitary
- atrial septum - T - fossa ovalis in the atrial septum is a common site
- can prolapse into ventricle - T - pedunculated form mobile enough to move into or through the AV valves during systole causing intermittent and often position dependent obstruction or “wrecking ball” effect
- more common in the right atrium - F - left to right ratio is 4:1 with 90% located in the atria
- Sep03.35 Mitral ring calcification
- Soft
- hard, MVR & conduction defects
- normal variant
- hard, MVR & conduction defects
- USUALLY ASYMPTOMATIC
- THIS COULD BE BAD RECALL, CAREFUL IF SEE IT IN EXAM
- Degenerative calcific deposits can develop in the ring ( annulus) of the mitral valve, visualized on gross inspection as irregular, stony hard, and occasionally ulcerated nodules (2 to 5 mm in thickness) that lie behind the leaflets
- The process generally does not affect valvular function.
- In unusual cases, however, it may lead to regurgitation by interfering with systolic contraction of the mitral valve ring, to stenosis by impairing opening of the mitral leaflets, or to arrhythmias and occasionally sudden death by the calcium deposits penetrating sufficiently deeply to impinge on the AV conduction system.
- Because ulcerated calcific nodules may provide a site for thrombi that can embolize, some patients with mitral annular calcification have an increased risk of stroke.
- The calcific nodules can also be the nidus for infective endocarditis.
- Heavy calcific deposits are sometimes visualized on echocardiography or seen as a distinctive, ringlike opacity on chest radiographs.
- Mitral annular calcification is most common in women over 60 years of age and individuals with myxomatous mitral valve (see later) or elevated left ventricular pressure (as in systemic hypertension, aortic stenosis, or hypertrophic cardiomyopathy).
- Sep03.68 Pericardial effusion on ultrasound. Which is the least likely cause?
- Cocksackie virus
- Sarcoid
- Uraemia
- Rheumatoid arthritis
- Sarcoid
- 11.03.26 In secundum ASD which is MOST TRUE ?
- Accounts for 60-70% of ASD’S
- Occurs in artioventricular valve
- Usually isolated anomaly
- Associated with left ventricular enlargement
- Present in neonatal period
- Usually isolated anomaly (Most are isolated (not associated with other anomalies))
- 11.03.26 In secundum ASD which is MOST TRUE ? Rob p388
- Accounts for 60-70% of ASD’S (90% of all ASDs)
- Occurs in artioventricular valve (fenestrated oval fossa near the mid-septum - valve or limbus of the fossa ovalis)
- Usually isolated anomaly (Most are isolated (not associated with other anomalies))
- Associated with left ventricular enlargement (When associated with another defect, such as tetralogy of Fallot, the other defect is usually hemodynamically dominant.)
- Present in neonatal period (most do not become evident till age 30)
- The secundum ASD, accounting for approximately 90% of all ASDs, comprises a defect located at and results from a deficient or fenestrated oval fossa near the mid-septum.
- Most are isolated (not associated with other anomalies).
- When associated with another defect, such as tetralogy of Fallot, the other defect is usually hemodynamically dominant.
- The atrial aperture may be of any size and may be single, multiple, or fenestrated
- 7.02.30 Carcinoid of the heart ?
- 7.02.30 Carcinoid of the heart ? Big Rob p577
• Cardiac involvement
o Pulmonic and tricuspid valve thickening and stenosis
o Endocardial fibrosis, principally in the right ventricle
o (Bronchial carcinoids affect the left side)
- Carcinoid tumors of the ileum are the most likely to metastasize, with involvement of the regional lymph nodes and liver.
- Usually only carcinoid tumors that invade the liver result in carcinoid heart disease.
- The cardiac lesions may be related to large circulating quantities of serotonin, bradykinin, or other substances secreted by the tumor, which usually are inactivated by the liver, lungs, and brain.
- Hepatic metastases apparently allow large quantities of tumor products to reach the heart.
- The preferential right-sided involvement presumably is related to inactivation of the offending humoral substance(s) by the lungs.
- In 5 to 10 percent of cases, significant left-sided valvular disease develops, related in most to passage of blood directly from the right to the left side of the heart through a patent foramen ovale, or less commonly by tumor involvement of the lungs
- Physical examination usually reveals a systolic murmur along the left sternal border, produced by tricuspid regurgitation; in some cases, there may be a concomitant murmur of pulmonic stenosis and/or regurgitation
- The chest roentgenogram is normal in half of the patients, but it may reveal enlargement of the heart and pleural effusions or nodules; the pulmonary artery trunk is typically of normal size, without evidence of poststenotic dilatation as occurs in congenital pulmonic stenosis
- The hemodynamic findings most commonly encountered are those of tricuspid regurgitation and occasionally pulmonic stenosis.
- Sep03.80 Patient suffering from transient myocardial depression after AMI for one week:
- stunned myocardium
- cardiogenic shock
- stunned myocardium - T - abnormalities in cellular biochemistry may persist for several days after ischaemia and lead to a noncontractile state. Such stunning can produce a state of transient reversible cardiac failure that may require pump assistance to support the pt until cardiac fxn returns.
- Sep03.80 Patient suffering from transient myocardial depression after AMI for one week: (GC)
- stunned myocardium - T - abnormalities in cellular biochemistry may persist for several days after ischaemia and lead to a noncontractile state. Such stunning can produce a state of transient reversible cardiac failure that may require pump assistance to support the pt until cardiac fxn returns.
- cardiogenic shock - F - occurs in 10-15% of patients after an AMI, generally with a large infarct (>40% of LV); 70% mortality rate.
- PATH2004 Which of the following would be an UNEXPECTED finding following coronary angioplasty?
- Luminal expansion
- Plaque rupture
- Thickened intact intima
- Medial dissection
- Proliferative restenosis in 30-50% of patients at 6 months
- Thickened intact intima - F - The split encompasses the intima and media
- PATH2004 Which of the following would be an UNEXPECTED finding following coronary angioplasty? (JS)
- Luminal expansion - T - The key elements of luminal expansion in angioplasty are plaque rupture, medial dissection and stretching of the media of the dissected segment.
- Plaque rupture - T
- Thickened intact intima - F - The split encompasses the intima and media
- Medial dissection - T
- Proliferative restenosis in 30-50% of patients at 6 months - T - The long-term success of angioplasty is limited by the development of proliferative restenosis that occurs in approximately 30 to 50% of patients within the first 4 to 6 months after angioplasty
- PATH2004 AMI, develops chest pain, 5 days later LEAST LIKELY cause is ?
- PATH2004 AMI, develops chest pain, 5 days later LEAST LIKELY cause is ?
• myocardial rupture (2.5%) o occurs in 10% of patients dying in hospital from acute infarct o occurs any time first 2 weeks o most common between 4 and 7 days o develops over several days
• septal rupture
• acute pericarditis at 2 to 3 days (nearly 100% of transmural infarcts)
o acute pericarditis in 15% within 2 to 4 days
• papillary muscle rupture (1%)
o day 3 -> acute left ventricular failure -> high mortality rate
• Not Ventricular aneurysm
o This is a late complication that most commonly results from a large transmural anteroseptal infarct
• Not Dressler syndrome
o Onset 2-3 weeks
- PATH2004 Regarding acute rheumatic fever, which of the following is the LEAST CORRECT:
- It follows pharyngeal infection with group A streptococcus
- Serofibrinous pericarditis is common
- Acute arthritis typically affects large joints like the knees
- Aschoff bodies are an uncommon histological feature of subcutaneous nodules
- Rheumatic pneumonitis is a recognised rare complication
- Aschoff bodies are an uncommon histological feature of subcutaneous nodules - F - Aschoff bodies are foci of fibrinoid degeneration surrounded by lymphocytes, plasma cells and macrophages and can be found in any of the three layers of the heart
- PATH2004 Regarding acute rheumatic fever, which of the following is the LEAST CORRECT: (JS)
- It follows pharyngeal infection with group A streptococcus - T - acute immunologically mediated multisystem inflammatory disease that occurs a few weeks after an episode of group A streptococcal pharyngitis. Major manifestations are migratory polyarthritis, carditis, subcutaneous nodules, erythema marginatum, Sydenham chorea.
- Serofibrinous pericarditis is common - T - causes a pancarditis with a serofibrinous pericardial exudate (bread and butter pericarditis), a myocarditis and endocardial involvement
- Acute arthritis typically affects large joints like the knees - T - migratory polyarthritis involves the large joints
- Aschoff bodies are an uncommon histological feature of subcutaneous nodules - F - Aschoff bodies are foci of fibrinoid degeneration surrounded by lymphocytes, plasma cells and macrophages and can be found in any of the three layers of the heart
- Rheumatic pneumonitis is a recognised rare complication T - diffuse pulmonary consolidation and tachypnoea, unresponsive to antibiotic or steriod therapy in a patient with active Rheumatic fever
- rheumatic fever occurs in 3% of patients after infection with group A streptococcus
- pericardium - fibrinous or serofibrinous exudate (bread and butter pericarditis) which generally resolves without sequelae
- migratory large joint arthritis
- Aschoff bodies. They constitute foci of fibrinoid degeneration surrounded by lymphocytes (primarily T cells), occasional plasma cells, and plump macrophages called Anitschkow cells (pathognomonic for rheumatic fever). These distinctive cells have abundant amphophilic cytoplasm and central round-to-ovoid nuclei in which the chromatin is disposed in a central, slender, wavy ribbon (hence the designation caterpillar cells).
- In cases of acute rheumatic fever with severe carditis, areas of patchy pneumonitis are sometimes seen. Many observers believe that these pulmonary infiltrates represent a specific rheumatic pneumonia
- PATH2004 Regarding the macroscopic appearance of acute MI, which of the following is the most correct:
- The left circumflex coronary shows severe stenosing atherosclerosis in 40-50% of cases
- Isolated infarct of the right ventricle occurs in 1-3% of cases
- The median time to rupture is 2-3 days
- Pericarditis usually develops at 24hrs post event
- Infarcts <48hrs old are usually inapparent on gross examination
- Isolated infarct of the right ventricle occurs in 1-3% of cases
- PATH2004 Regarding the macroscopic appearance of acute MI, which of the following is the most correct:
- The left circumflex coronary shows severe stenosing atherosclerosis in 40-50% of cases
- Isolated infarct of the right ventricle occurs in 1-3% of cases - True (word for word from Robbins>
- The median time to rupture is 2-3 days
- Pericarditis usually develops at 24hrs post event
- Infarcts <48hrs old are usually inapparent on gross examination
Location
• 95% involve a portion of the left ventricle
• LAD (45%)
o anterior wall of left ventricle near apex, anterior 2/3’s of interventricular septum
• RCA (35%)
o inferior/posterior wall of left ventricle, posterior 1/3 of IV septum, posterior right ventricular free wall in some cases
• Left circumflex (15%)
o lateral wall of the left ventricle
• Isolated infarction of the right ventricle, however, occurs in only 1 to 3%
Myocardial Rupture (2.5%)
• occurs in 10% of patients dying in hospital from acute infarct
• occurs any time first 2 weeks, most common between 4 and 7 days
Pericarditis
• 2 to 3 days (nearly 100% of transmural infarcts) acute pericarditis in 15% within 2 to 4 days
Dresslers syndrome (autoimmune pericarditis) (2%) onset 2-3 weeks Gross morphology • Dark mottling may present by 12 hrs, definitely by 24hrs
- PATH2004 Regarding infective endocarditis, which of the following is the LEAST CORRECT:
- Right sided heart valves in 50% of narcotic related cases
- Diffuse glomerulonephritis seen in 50% of untreated cases
- Positive blood cultures are seen in 85-90% of cases
- Strep pneumonia is the leading cause of acute endocarditis
- With mechanical prostheses infections are usually located on the margin of the sewing ring
- Strep pneumonia is the leading cause of acute endocarditis – F – (note that path notes say more than ½ cases due to various streptococci – however most commonly gingival disease) – since 1980s IE due to S aureus (primary pathogen) primary risk factor is intravascular devices (eMed).
- PATH2004 Regarding infective endocarditis, which of the following is the LEAST CORRECT: (TW)
- Right sided heart valves in 50% of narcotic related cases – T - in 75% of IVDA IE, no underlying valvular abnormalities are noted, and 50% of these infections involve the tricuspid valve (eMed).
- Diffuse glomerulonephritis in 50% of untreated cases – T ~30% UpToDate
- Positive blood cultures in 85-90% of cases – T – path notes / UpToDate (but varies depending on series and geography)/ eMed
- Strep pneumonia is the leading cause of acute endocarditis – F – (note that path notes say more than ½ cases due to various streptococci – however most commonly gingival disease) – since 1980s IE due to S aureus (primary pathogen) primary risk factor is intravascular devices (eMed).
- With mechanical prostheses infections are usually located on the margin of the sewing ring – T – endocarditis is located at the prosthesis-tissue intervace, causing a ring intervace (Robbins).
- Endocarditis of native but previously damaged or otherwise abnormal valves is caused most commonly (50 to 60% of cases) by alpha-hemolytic (viridans) streptococci
- Staphylococcus aureus organisms commonly found on the skin can attack either healthy or deformed valves; they are responsible for 10 to 20% of cases overall and are the major offender in intravenous drug abusers.
- The roster of the remaining bacteria includes enterococci and the so-called HACEK group ( Haemophilus, Actinobacillus, Cardiobacterium, Eikenella, and Kingella), all commensals in the oral cavity
- Prosthetic valve endocarditis is caused most commonly by coagulase-negative staphylococci (e.g., Staphylococcus epidermidis).
- Other agents causing endocarditis include gram-negative bacilli and fungi. In about 10% of all cases of endocarditis, no organism can be isolated from the blood ( culture-negative endocarditis) because of prior antibiotic therapy, other difficulties in isolation of the offending agent, or deeply embedded organisms within the enlarging vegetation are not released into the blood.
- Bowel Ischaemia:
a. Can be related to superior mesenteric vein thrombosis
b. Is associated with paroxysmal nocturnal haemoglobinuria
c. Vasospasm indicates underlying atherosclerosis and stenosis
d. Occlusion is typically 10cm distal to the SMA origin
e. Plain film changes are only seen after bowel infarction
a. Can be related to superior mesenteric vein thrombosis - T - venous occlusion <10%. SMV > IMV > PV.
b. Is associated with paroxysmal nocturnal haemoglobinuria - T - PNH is a disorder characterised by a defect in the GPI anchor due to an abnormality in th PIG-A gene. Result in abnormalities in hemopoietic function, including hemolytic anemia, a hypercoagulabel state, and diminished hematopoiesis.
- Bowel Ischaemia: (TW)
a. Can be related to superior mesenteric vein thrombosis - T - venous occlusion <10%. SMV > IMV > PV.
b. Is associated with paroxysmal nocturnal haemoglobinuria - T - PNH is a disorder characterised by a defect in the GPI anchor due to an abnormality in th PIG-A gene. Result in abnormalities in hemopoietic function, including hemolytic anemia, a hypercoagulabel state, and diminished hematopoiesis.
c. Vasospasm indicates underlying atherosclerosis and stenosis - F
d. Occlusion is typically 10cm distal to the SMA origin - F - SMA thrombosis (20-40%) at origin + site of atherosclerotic narrowing. Embolus (40-50%) just distal to middle colic artery.
e. Plain film changes are only seen after bowel infarction - F - ischaemia, oedematous bowel, thumb printing. Could also have bowel obstruction as cause of ischaemia (pre-infarction).
- Aortic Stent Grafts:
a. Sac size greater than 8 cm is a contra indication
b. The neck of the aneurysm is defined as the distance from the inferior most renal artery to the start of the aneurysm
c. Immediate endoleak always needs further intervention
d. Retro leak from lumbar arteries needs further intervention
e. Luminal diameter is best measured via angiography with a graded catheter
b. The neck of the aneurysm is defined as the distance from the inferior most renal artery to the start of the aneurysm - T - the proximal neck is defined as the segment of aorta between the origin of the lowest renal artery and superior aspect of the aneurysm. Most endographs require a length of greater than 15mm to ensure adequate proximal seal.
- Aortic Stent Grafts: (TW)
a. Sac size greater than 8cm is a contraindication - F - for example the Zenith AAA grafts range from 82mm to 149mm in length.
b. The neck of the aneurysm is defined as the distance from the inferior most renal artery to the start of the aneurysm - T - the proximal neck is defined as the segment of aorta between the origin of the lowest renal artery and superior aspect of the aneurysm. Most endographs require a length of greater than 15mm to ensure adequate proximal seal.
c. Immediate endoleak always needs further intervention - F - depends on type. type I endoleak (ie incompetent seal at either proximal or distal attachment site) must be repaired as soon as possible. Type III endoleaks represtn flow into sac from separation between components of a modular system, or tears in the endograft - these are repaired with an additional endograft. Type IV leaks are due to egress of blood through the pores of the fabric - these heal spontaneously. Type II enodleak (most common) - management is controversial. Some argue that as spontaneous resolution occurs 30-100% of cases, a “wait and see” approach is preferable. UTD recommendations are occluding type II endoleaks that have not spontaneously thrombosed in one month (as systemic pressures have been noted in aneuyrsm sac in the presence of type II endoleaks
d. Retro leak from lumbar arteries needs further intervention - ?T/F - controversial, see ans c.
e. Luminal diameter is best measured via angiography with a graded catheter - ?F
- Regarding Cervico Cephalic Vessel Dissection: which is false?
a. Causes 5% to 20% of CVAs in young to middle aged patients
b. Has a rim of T1 hyper intensity surrounding a void in the vessels
c. Imaging identifies vasculopathy in the majority
d. The location of vertebral artery dissections is most often proximal (between C2 and C6)
e. The location of an ICA dissection is most often just immediately distal to the bulb
d. The location of vertebral artery dissections is most often proximal (between C2 and C6) - F - at the level of C1/2 in 65% [Dahenrt]. ie. predominantly located in the pars transversaria (V2, 35%) or the atlas loop (V3, 34%) [RG]. Bilateral vertebal artery dissections in 5%.
c. Imaging identifies vasculopathy in the majority: *LW: vasculopathy in terms of atherosclerosis is not a risk factor for dissection, so hence not commonly found, would favour this to also be false.
* *LJS - agree
- Regarding Cervico Cephalic Vessel Dissection: (GC)
a. Causes 5% to 20% of CVAs in young to middle aged patients - T - 5-20% in those aged 40-60yrs [Dahnert]; up to 1/4 of strokes,with a peak prevalence in the 5th decade [RG].
b. Has a rim of T1 hyperintensity surrounding a void in the vessels - T - subacute hematoma (7days-2mths) on T1 + fat sat appears as a bright crescent-shaped area around an eccentric flow void. Note that in the early and chronic stage, the haematoma is usually isointense to surrounding structures on T1.
d. The location of vertebral artery dissections is most often proximal (between C2 and C6) - F - at the level of C1/2 in 65% [Dahenrt]. ie. predominantly located in the pars transversaria (V2, 35%) or the atlas loop (V3, 34%) [RG]. Bilateral vertebal artery dissections in 5%.
e. The location of an ICA dissection is most often just immediately distal to the bulb - T - extracranial ICA dissection affects the cervical part of the artery distal to the carotid bulb and tends not to extend beyond its entry into the petrous portion. [Craniocervical a. dissection, RG 2008]
- Fixed inferior defects in a thallium scan are caused by: which one is false
a. Diaphragmatic attenuation b. Mastectomy c. Right coronary artery infarct d. Pacemaker leads e. Left bundle branch block
b. Mastectomy - F - implants could cause attentuation artifact.
- Fixed inferior defects in a thallium scan are caused by: (GC)
a. Diaphragmatic attenuation - T - variation in tracer intensity by 15-20% btn regions on planar images may be normal (due to soft tissue attentuation artifacts from subdiaphragmatic abdominal contents or breast tissue).
b. Mastectomy - F - implants could cause attentuation artifact.
c. Right coronary artery infarct - T - RCA territory is 5-8 o’clock on the short axis (oblique coronal).
d. Pacemaker leads - T - attentuation artifact.
e. Left bundle branch block - ?T
- With regard to cerebral aneurysms: (multiple true)
a. In children are associated with trauma and infection
b. Multiple aneurysms are strongly associated with male gender
c. A truly azygos anterior cerebral artery is associated with lobar holoprosencephaly and saccular aneurysms
d. Are multiple in 15% to 20%
e. Occur in the posterior circulation in 40%
there are 3 trues : a, c, d
- With regard to cerebral aneurysms: (TW)
a. In children are associated with trauma and infection - T - Aneuyrsms in children may be congenital or idiopathic (77%), inflammatory or mycotic (11%), or traumatic (11%).
b. Multiple aneurysms are strongly associated with male gender - F - Female > Male (Neuroradiology 2004). Female preponderance has been reported in up to 70% of multiple aneurysms and a 2.4:1 ratio of females > males was reported in the Cooperative Study of Intracranial Aneurysms and SAH.
c. A truly azygos anterior cerebral artery is associated with lobar holoprosencephaly and saccular aneurysms - T - azygos ACA is a rare variation of the COW in which the A2 segments of both ACAs are represented by a singl common vessel from which arise all the major vessels supplying most of both anterior cerebral hemispheres, as well as the corpus callosum (ie a true azygos). Associated with holoprosencephaly. High indicence of berry aneurysms. Abnormal medial and intimal elements in the vessel wall of this developmental variant may account for the increased prevalence of associated saccular aneurysms.
d. Are multiple in 15% to 20% - T
e. Occur in the posterior circulation in 40% - F - 90-95% of aneurysms arise from COW
- 90% from anterior circulation, 1
0% from posterior circulation.
ACom 30-35%,
PCom 30-35%,
MCA 20-30%, B
asilar apex 5-10%, other posterior 1-3%.
Variations of the distal ACA: trus azygos artery, from which all major branches are given off to both cerebral hemispheres; a bihemispheric ACA, where both right and left ACAs are present, but one is rudimentary and most of the major branches to both hemispheres arise from the other ACA; a triple ACA, with the accessory ACA arising from the anterior communcating artery.
- Cerebral angiography: which is false?
a. The supraclinoid internal carotid artery is best seen in a caudal (Waters) projection
b. The origin of the opthalmic artery is intradural in the majority of people
c. Internal carotid injections are of 8mls at 2mls per second
d. Cavernous haemangiomata are angiographically occult
e. The enhancement of meningiomas persist late into the venous phase
a. The supraclinoid internal carotid artery is best seen in a caudal (Waters) projection - ?F -
Cerebral angiography: (TW)
a. The supraclinoid internal carotid artery is best seen in a caudal (Waters) projection - ?F - ICA/ophthalmic: transorbital oblique (15-30 degree rotation, and 7 degrees cephalad); Caldwell’s oblique (15-30 degrees rotation, and 7 degrees caudad). Waters is ~30 degrees caudad?
b. The origin of the opthalmic artery is intradural in the majority of people - T - The origin of the ophthalmic artery is intradural in approx 90% of anatomic dissections (Diagnostic cerebral angiography, AG Osborn).
c. Internal carotid injections are of 8mls at 2mls per second - T - CCA 4-5ml/s for 2s (ie total 8-10mls)
d. Cavernous haemangiomata are angiographically occult - T - cavernous malformation: DSA is usually normal “angiographically occult vascular malformation”
e. The enhancement of meningiomas persist late into the venous phase - T - mother-in-law - “comes early, stay late”
- alternate memory aid to remember big nando: the pre-mature ejaculator who wants to cuddle afterwards.
- Which of the following statements with regards to fatty plaques?(streaks?) is LEAST CORRECT?
- Moreover, they frequently affect individuals in geographic locales and populations in which atherosclerotic plaque is uncommon.
- ?% of kids that have them
- ?site
- bld vess and heart pg 516 -517 robbins.
A is right if it is streak
can occur in all children even poor skinny ones
involve 4-5% of aorta by 10yo
Regarding polyarteritis nodosa. Which of the following is/are LEAST CORRECT:
a. The kidneys are most commonly involved in autopsy
a. Aneurysms are seen at angiography in 50%
b. 70% are seropositive for Hepatitis B surface antigen
c. Churg Strauss syndrome frequently involves pulmonary and splenic vessels
d. Serum anti-neutrophil antibody titres correlate with disease activity
2 and 4 false
- Regarding polyarteritis nodosa. Which of the following is/are false: (GC)
- The kidneys are most commonly involved in autopsy - T - vessels of the kidneys, heart, liver, and GIT are involved in descending order.
- Aneurysms are seen at angiography in 50% - T - multiple small intrarenal microaneurysms typically at branchpoints in 50-60% - hallmark feature. Lesions usually involve only part of the vessel circumference; the inflammatory process weakens the arterial wall and can lead to aneurysms or even rupture.
- 70% are seropositive for Hepatitis B surface antigen - F - 30% have hepatitis B antigenaemia, and HBsAg-HBsAb immune complexes can be demonstrated in the lesions.
- Churg Strauss syndrome frequently involves pulmonary and splenic vessels - T - aka allergic granulomatosis and angiitis; vascular lesions can resemble PAN and microscopic polyangiitis, but in the lung, heart, spleen, peripheral nerves and skin there are also intra- and extravascular granulomas. PAN spares the pulmonary circulation.
- Serum anti-neutrophil antibody titres correlate with disease activity - F - Adelaide path notes: p-ANCA is often present in serum and correlates with disease activity. *LW: I favour false per Robbins.
* *WJI disagree with rationale. PAN is ANCA negative. So still false.
- Which of the following would be an UNEXPECTED finding following coronary angioplasty?
a. Luminal expansion
b. Plaque rupture
c. Thickened intact intima
d. Medial dissection
e. Proliferative restenosis in 30-50% of patients at 6 months
- Thickened intact intima - F - The split encompasses the intima and media
- Which of the following would be an UNEXPECTED finding following coronary angioplasty? (JS)
- Luminal expansion - T - The key elements of luminal expansion in angioplasty are plaque rupture, medial dissection and stretching of the media of the dissected segment.
- Plaque rupture - T
- Thickened intact intima - F - The split encompasses the intima and media
- Medial dissection - T
- Proliferative restenosis in 30-50% of patients at 6 months - T - The long-term success of angioplasty is limited by the development of proliferative restenosis that occurs in approximately 30 to 50% of patients within the first 4 to 6 months after angioplasty
- Which of the following conditions IS TRUE with regards to hypertension?
- 5-10% of dissections have no obvious intimal tear - true
- Which of the following conditions IS TRUE with regards to hypertension?
- 5-10% of dissections have no obvious intimal tear - true
- Focal renal infarct, least likely:
a. Aortic atherosclerosis
b. Mitral valve regurgitation
c. Post AMI
d. PAN
e. Adult Haemolytic Uremic Syndrome
f. Candida endocarditis
- Mitral valve regurgitation - F - MR is not a cause of embolism in itself, but could cause embolic renal infarction if the regurg is due to infective endocarditis.
* *LW: Mitral valve regurgitation by itself would not cause renal infarction, while HUS will cause microthrombi within renal vasculature, thus is more likely than isolated MR. Hence preferred answer is MR - Focal renal infarct, least likely: (GC)
- Aortic atherosclerosis - T - thromboembolism
- Mitral valve regurgitation - F - MR is not a cause of embolism in itself, but could cause embolic renal infarction if the regurg is due to infective endocarditis.
- Post AMI - T - LV mural thrombus
- PAN - T - kidney involved in 70-90%. Focal panmural necrotising vasculitis – mucoid degeneration & fibrinoid necrosis begins in media – intimal proliferation & thrombosis.
- Adult Haemolytic Uremic Syndrome - ? T/F - similar to childhood (classic) HUS but seen in association with:
• Infection – verocytotoxic E.coli (O157:H7), shigella, viral
• Postpartum
• 2° HUS – scleroderma, SLE, malignant HTN, drugs etc.
Classic triad:
4. Thrombocytopaenia
5. Microangiopathic haemolytic anaemia
6. Acute renal failure
Path: capillary & endothelial injury – mechanical damage to RBCs – formation of hyaline thrombi (predominantly glomeruli, but may extend into arterioles & larger arteries if severe) – cortical necrosis (fleabitten).
Several MCQ teams have answered that HUS is False as it causes cortical necrosis and not a focal renal infarct. Dahnert says focal infarction DOES occur.
6. Candida endocarditis - T - embolism
Causes of renal infarction:
Most due to embolism
Major source of emboli = mural thrombi from LA & LV from AMI
Less common sources
• vegetative endocarditis
• thrombosis in aortic aneurysms and aortic atherosclerosis
Rare causes
• thrombosis in advanced atherosclerosis
• acute vasculitis of polyarteritis nodosa
[Robbins, Adelaide path notes, Dahnert pg 929]
. Least likely site for hypertensive bleed in the brain is:
a. hippocampus
b. cerebellum
c. basal ganglia
d. thalamus
e. putamen
f. brainstem
- hippocampus
. Cavernous Angioma – What is not typical
a. detectable at angiography
b. Bleeding tendency
c. No intervening brain
d. Pseudo capsule + surrounding hemosiderin laden macrophages
e. Associated venous angioma
- detectable at angiography F - angiographically occult lesions
- Cavernous Angioma – What is not typical (JS)
- detectable at angiography F - angiographically occult lesions
- Bleeding tendency - T - contain blood degredation products of varying ages, haemorrhage risk is 0.5-1% per year but occult bleeds are more common (Osborne)
- No intervening brain - T - dilated endothelial cell-lined spaces with no normal brain within the lesion
- Pseudo capsule + surrounding hemosiderin laden macrophages - T - A low signal haemosiderin ring is typical on MR
- Associated venous angioma - T - up to 1 third of DVAs are associated with cavernous angiomas (Osborne)
- Wegener’s granulomatosis is characterised by all of the following except
- Generalised vasculitis:
- GN
- Granulomatosis of the upper and lower respiratory tract
- ANA +ve
- ANCA +ve
- ANA +ve - F 18.
- Wegener’s granulomatosis is characterised by all of the following except (JS)
- generalised vasculitis - T - A necrotising vasculitis characterised by (1) acute necrotising granulomas of the upper and lower respiratory tracts, (2) focal necrotizing or granulomatous vasculitis affecting small to medium sized vessels and (3) renal disease in the form of focal or necrotising, often crescentic glomerulitis.
- GN - T
- Granulomatosis of the upper and lower respiratory tract - T
- ANA +ve - F
- ANCA +ve - T - c-ANCA is present in 90% of patients with active disease
- Regarding cardiomyopathies, which of the following is the most correct:
a. Autosomal recessive cases are seen in over 50% of cases of hypertrophic CMP
b. Heart always increased in weight in dilated CMP
c. Atria never involved in hypertrophic CMP
d. Endomyocardial fibrosis is mainly a disease of children and young adults in South America
e. Dilation of the heart in peripartum CMP is irreversible
- Heart always increased in weight in dilated CMP - T - characterised by a massively hypertrophied LV without dilatation. Classically, there is disproportionate thickening of the IV septum (asymmetrical septal hypertrophy), although 10% are symmetrical/concentric. LV cavity has a ‘banana-like’ configuration as septum bulges into lumen. Paradoxically provides a markedly reduced stroke volume due to overall small size of chamber and impaired diastolic filling, 25% also have a dynamic LVOT obstruction due to a thickened mitral valve anterior leaflet.
* RY - this discription is for hypertrophic CM, not dilated. Dilated “usually 2-3x heavier” Big Robbins 9th pg567 - Regarding cardiomyopathies, which of the following is the most correct: (GC)
- Autosomal recessive cases are seen in over 50% of cases of hypertrophic CMP - F - genetic basis in most cases; 50% familial with autosomal dominant transmission (variable expression). >100 mutations have been identified, the most common are beta-myosin heavy chain, myosin-binding protein C, and troponin T. These 3 genes account for 70-80% of HCM.
- Heart always increased in weight in dilated CMP - T - characterised by a massively hypertrophied LV without dilatation. Classically, there is disproportionate thickening of the IV septum (asymmetrical septal hypertrophy), although 10% are symmetrical/concentric. LV cavity has a ‘banana-like’ configuration as septum bulges into lumen. Paradoxically provides a markedly reduced stroke volume due to overall small size of chamber and impaired diastolic filling, 25% also have a dynamic LVOT obstruction due to a thickened mitral valve anterior leaflet.
- Atria never involved in hypertrophic CMP - F - left atrial dilatation due to high LV pressures - AF with mural thrombus formation are potential complications. But true if the question refers to LA “hypertrophy” - only LV is hypertrophic.
- Endomyocardial fibrosis is mainly a disease of children and young adults in South America - F - technically true but not “most correct”. Primarily a disease of children and young adults in Africa [Robbins]; but is also encountered in tropical and subtropical regions elsewhere in the world, including areas in India and South America that are within 15° of the equator [eMedicine]. EMF is the most common type of restrictive cardiomyopathy worldwide.
- Dilation of the heart in peripartum CMP is irreversible in the majority - F - half of these patients will spontaneously recover. Aetiology is multifactorial - preg-assocd HTN, volume overload, nutritional deficiency, metabolic derangement, immunologic response (eg. abnormal cytokine production).
- Aortic dissection: false
a. Associated with Marfan’s
b. Stanford classification system
c. Bicuspid valve
d. Intimal flap visible in 90%
e. DeBakey type 2 is the most common
- DeBakey type 2 is the most common - F - DeBakey Type 1 invovles ascending and descending aorta (50%), Type 2 involves ascending aorta only (10%), Type 3 involves descending aorta (40%)
*LW note small robbins diagram has Debakey images and classification wrong. correct in big robbins.
Remember using pneumonic “BAD”
B: Both ascending and descending aorta - Type 1
A: Ascending: type 2
D: Descending: type 3
- Aortic dissection, which is false (JS)
- Associated with Marfan’s T - Causes include hypertension, Marfans, Ehlers-Danlos, Aortic coarctation and bicuspid or unicuspid valve
- Stanford classification system - T - Stanford classification - Type A involves ascending aorta, Type B involves descending aorta
- Bicuspid valve T - Causes include hypertension, Marfans, Ehlers-Danlos, Aortic coarctation and bicuspid or unicuspid valve
- Intimal flap visible in 90% - T - Intimal flap is usually present with haematoma spreading between the middle and outer 1/3 of the media.
- DeBakey type 2 is the most common - F - DeBakey Type 1 invovles ascending and descending aorta (50%), Type 2 involves ascending aorta only (10%), Type 3 involves descending aorta (40%)
. Regarding acute rheumatic fever, which of the following is the LEAST CORRECT:
a. It follows pharyngeal infection with group A streptococcus
b. Serofibrinous pericarditis is common
c. Acute arthritis typically affects large joints like the knees
d. Aschoff bodies are an uncommon histological feature of subcutaneous nodules
e. Rheumatic pneumonitis is a recognised rare complication
- Aschoff bodies are an uncommon histological feature of subcutaneous nodules - F - Aschoff bodies are foci of fibrinoid degeneration surrounded by lymphocytes, plasma cells and macrophages and can be found in any of the three layers of the heart
- Regarding acute rheumatic fever, which of the following is the LEAST CORRECT: (JS)
- It follows pharyngeal infection with group A streptococcus - T - acute immunologically mediated multisystem inflammatory disease that occurs a few weeks after an episode of group A streptococcal pharyngitis. Major manifestations are migratory polyarthritis, carditis, subcutaneous nodules, erythema marginatum, Sydenham chorea.
- Serofibrinous pericarditis is common - T - causes a pancarditis with a serofibrinous pericardial exudate (bread and butter pericarditis), a myocarditis and endocardial involvement
- Acute arthritis typically affects large joints like the knees - T - migratory polyarthritis involves the large joints
- Aschoff bodies are an uncommon histological feature of subcutaneous nodules - F - Aschoff bodies are foci of fibrinoid degeneration surrounded by lymphocytes, plasma cells and macrophages and can be found in any of the three layers of the heart
- Rheumatic pneumonitis is a recognised rare complication T - diffuse pulmonary consolidation and tachypnoea, unresponsive to antibiotic or steriod therapy in a patient with active Rheumatic fever
