Cardio

Question 1

How does carotid USS differ from carotid CTA or MRA (or catheter angiography) in determining degree of carotid artery stenosis?

Answer 1

Carotid USS estimates the degree of stenosis based on elevations in blood flow velocity observed in the target vessel as opposed to the other modalities which undertake anatomic measurements of luminal narrowing (e.g. measure residual lumen diameter at the most stenotic portion of the vessel and compare this with the lumen diameter in the normal internal carotid artery distal to the stenosis)

Velocity criteria that correlate with angiography measurements have been developed that are categorical (rather than linear) ie 50-69% stenosis

Question 2

What is the gold standard for diagnosing internal carotid artery stenosis?

Answer 2

Catheter cerebral аոgiοgrарhу BUT it is invasive, time consuming, assoc with stroke risk

Question 3

What is the preferred diagnostic strategy in symptomatic (eg stroke/TIA) carotid artery disease?

Answer 3

Usually CT or MR head and neck angiogram (given usually performed in ED at time of stroke presentation)

Question 3

What is the preferred diagnostic strategy for asymptomatic carotid artery disease?

Answer 3

Carotid duplex USS

Question 3

If a carotid stenosis less than 50% is identified on initial imaging, what further evaluation needs to be done?

Answer 3

Usually none - effectively excludes haemodynamically significant CAD

Only exceptions:
- carotid stenosis that is unusually distal and beyond the field of insonation for CDUS

• short-segment stenosis in regions of heavily calcified plaque on СΤA in which adequate assessment of the residual lumen may be difficult

Question 4

What further evaluation is indicated if a symptomatic carotid artery stenosis 50-99% is detected?

Answer 4

Confirmation with an alternative modality is generally advisable, as two separate noninvasive diagnostic modalities concordant for high-grade stenosis increase accuracy;
If concordant - proceed to revasc
If discordant - consider catheter angiogram

However some experts are happy with just one positive test

Question 5

What further evaluation is indicated if an asymptomatic carotid artery stenosis 50-99% is detected?

Answer 5

Depends on if they are candidate for revascularisation

If not - serial carotid duplex USS

If they are - obtain alternative test + treat with statin and antiplatelet and aggressive risk factor modification

Question 6

If a patient is found to have complete carotid artery occlusion on CTA can you trust it?

Answer 6

No, whilst CDUS and МRA are very accurate ; have to be suspicious of CTA because with increasing speed of image acquisition, can have “pseudo-occlusion” whereby image acquisition occurs before contrast bolus can fully opacify the affected vessel

Therefore if complete occlusion found on CTA suggest alternative study eg CDUS or MRA to confirm

Question 7

What are the advantages of CT (head and neck) angiogram for the detection of carotid artery disease?

Answer 7

Quick (an important advantage in patients who are uncooperative or claustrophobic)
Can be useful in situations of difficult anatomy e.g. severe kinking

Question 8

What are the disadvantages of CT (head and neck angiogram) for the detection of carotid artery disease?

Answer 8

Significant contrast bolus (same or higher dose than catheter angiogram) which poses risks to kidneys
+
Radiation risk
+
Pseudo-occlusion risk

Question 9

What are the advantages and disadvantages of carotid duplex USS for evaluation of carotid artery disease?

Answer 9

Adv:
- noninvasive, safe, and inexpensive
- no claustrophobia
- no contrast
- can be easily repeated
- highly accurate

Disadv:
- operator dependent
- tends to overestimate the degree of stenosis
- less precise at identifying minimal stenoses (ie gets more accurate the higher the degree of stenosis)
- may be limited by features such as calcific carotid lesions, tortuous or kinked carotid arteries, and patient body habitus
- only a portion of the cervical internal carotid artery extending just past the carotid bifurcation can be evaluated (Fortunately, the vast majority of atherosclerotic stenoses occur within this region)

Question 10

Is CDUS a good modality for detecting carotid artery dissection and why?

Answer 10

No - CDUS can only evaluate a portion of the cervical internal carotid artery extending just past the carotid bifurcation

However, carotid dissections often occur in the more distal portion of the cervical carotid artery and thus will not be accurately identified by СDUS

Question 11

After carotid artery intervention (ie endarterectomy or stenting) what is the best imaging modality to evaluate for restenosis?

Answer 11

CDUS is commonly used to conduct serial monitoring of the carotids over time to ensure patency of the vessel and evaluate for restenosis because it is:
low cost,
easy to use
accurately monitors changes

But structural changes to the carotid wall after endarterectomy or stenting may alter compliance of the vessel wall which in turn may impact the relationship between flow velocity and degree of stenosis such that standard velocity criteria may be inaccurate - note modified criteria have been developed to address this

Note the accuracy of CTA and especially MRA may be negatively impacted by stent artifact

Question 12

What is the definition of symptomatic extracranial carotid atherosclerotic disease?

Answer 12

Neurologic symptoms (incl TIA, transient monocular blindness, stroke) that are sudden in onset and referable to the appropriate internal carotid artery distribution (ipsilateral to significant carotid atherosclerotic pathology)

Question 13

Αthеrοѕϲlerоѕis of the internal carotid artery at the bifurcation accounts for what percentage of ischaemic strokes?

Answer 13

10-12%

Question 14

What is the mechanism by which carotid artery disease may cause an ischaemic stroke?

Answer 14

Progressive atheromatous plaque at the carotid bifurcation results in luminal narrowing, often accompanied by ulceration

This may then lead to embolization, thrombosis, or reduced brain perfusion (more likely in the setting of bilateral disease)

Question 15

What is the treatment of symptomatic carotid artery disease?

Answer 15

Intensive medical management +/- carotid artery revascularisation

Question 16

What does intensive medical management of carotid artery disease entail?

Answer 16

1) Antithrombotic therapy
2) High-potency stаtiո therapy
+
3) Risk factor modification, including blood pressure control, glucose control, weight control, and lifestyle modification with smoking cessation, exercise, and recommended dietary modifications

Question 17

Which patients are most likely to benefit from carotid artery revascularisation?

Answer 17

Patients with recently symptomatic carotid stenosis of 70 to 99 percent who have a life expectancy of at least two years (ideally within 2 weeks of symptoms)

Patients with recently symptomatic carotid stenosis of 50 to 69 percent who have a life expectancy of at least three years, particularly if can be done within 2 weeks of symptoms (beyond 2 weeks - benefit is less certain overall, and females, in particular, may not benefit from revascularization beyond two weeks from symptom onset)

Question 18

What is the preferred modality of carotid artery revascularisation?

Answer 18

Carotid endarterectomy rather than transfemoral carotid artery stenting when there is a surgically accessible carotid artery lesion; no clinically significant cardiac, pulmonary, or other disease that would greatly increase the risk of anaesthesia and surgery; and no prior ipsilateral endarterectomy

Question 19

Which patients are unlikely to benefit from carotid artery revascularisation?

Answer 19

• Stenosis less than 50 percent.
• Severe comorbidity due to other surgical or medical illness.
• Strоkе associated with persistent, severe neurologic deficits and disability that precludes preservation of useful function.
• Near occlusion of the symptomatic ipsilateral internal carotid artery
• Total occlusion of carotid artery

Question 20

What are the benefits of carotid artery revascularisation?

Answer 20

To prevent one event at five years for ipsilateral carotid territory ischemic ѕtrоke and operative strοke or death, the number needed to treat (NNT) was 6

Note whilst CEA and CAS have similar long-term benefits, CAS has higher rates of periprocedural complications and 30d death (note was transfemoral ?if transradial approach mitigates these risks somewhat)

Question 21

In what circumstances may you consider carotid artery stenting over endarterectomy?

Answer 21

• A carotid lesion that is not suitable for surgical access
• Radiation-induced stenosis
• Carotid restenosis after endarterectomy
• Clinically significant cardiac, pulmonary, or other disease that greatly increases the risk of anaesthesia and surgery
• Unfavorable neck anatomy including contralateral vocal cord paralysis, open tracheostomy, or prior radical surgery

Question 22

Which factors influence the benefit and risk of Carotid endarterectomy?

Answer 22

Baseline stroke risk - the higher the greater the benefit from CEA

Location of event: Hemispheric ischaemia rather than retinal ischaemia

Age: Older age (>70yo)

Gender: Males > females

Presence of contralateral carotid artery disease - if present, favour surgical revasc over medical mx (despite increased periprocedural risk)

Combined perioperative morbidity and mortality that exceeds 6 percent

Timing of revasc - best between 2 days and 2 weeks after event (ie stroke)

Question 23

What is the definition of asymptomatic carotid atherosclerotic diease?

Answer 23

The presence of atherosclerotic narrowing of the extracranial internal carotid artery (significant = >50% stenosis) in individuals WITHOUT a history of ipsilateral carotid territory ischemic strokе or transient ischemic attack in the last six months

Question 24

What is the annual stroke risk in patients with asymptomatic carotid artery disease?

Answer 24

0.5 to 1.0 percent annually + Also a marker for MI and vascular death

Question 25

Does an isolated unilateral carotid stenosis cause vertigo, diplopia, lightheadedness or syncope?

Answer 25

No

Question 26

What is the preferred treatment approach for asymptomatic extracranial carotid artery disease?

Answer 26

Intensive medical management Revascularisation is controversial given periprocedural risks and low absolute risk reduction assoc with revasc ie 2.9% risk of periprocedural stroke or death vs 1% absolute risk reduction; NNT = 33 Controversial but some evidence of equivalence between intensive medical tx and CEA in asymptomatic px ie risk of stroke similar Others would argue still benefit for CEA in asymptomatic 70-99% SPACE-2 trial suggested nil significant differences in 30d death, cumulative stroke or ipsilateral stroke out to 5 years between intensive medical mx, CEA + medical mx, CAS + medical mx but limitations incl stopped early due to slow recruitment and low event rates

Question 27

What are the four key manifestations of acute pericarditis?

Answer 27

1) Chest pain 2) Pericardial friction rub 3) ECG changes 4) Pericardial effusion

Question 28

What are the typical features of chest pain in acute pericarditis?

Answer 28

Sudden onset Anterior chest Sharp and pleuritic + worse with coughing Better sitting up and leaning forward May radiate to trapezius ridge

Question 29

Chest pain may be minimal or absent in patients with pericarditis due to which aetiologies?

Answer 29

Uraemia Rheumatologic disorder eg SLE

Question 30

What are the key features of the pericardial friction rub?

Answer 30

Classically triphasic (but may have only one or two phases) Superficial scratchy or squeaking quality Often intermittent/waxes and wanes Best heard with diaphragm of stethoscope over LLSE with patient leaning forward and suspending breath (intensifies in this position)

Question 31

What are the four stages of ECG changes in acute pericarditis

Answer 31

Stage 1 - (hours to days) - widespread concave-up ST elevation with reciprocal ST depression in aVR and V1 with PR depression in leads (except elevation in aVR) - ie PR and ST segments change in different directions Stage 2 - (first week) - normalisation of ST and PR segments Stage 3 - (weeks to ?months) - diffuse T wave inversion after ST segments have become isoelectric Stage 4 - normalisation of ECG

Question 32

Why might some patients not experience classical ECG changes with acute pericarditis?

Answer 32

If the parietal pericardium is more involved than epicardial pericardium as parietal pericardium is electrially inert E.g. uraemic pericarditis

Question 33

What ECG features may distinguish acute pericarditis from STEMI in patients with chest pain and troponin elevation?

Answer 33

1) STE in acute pericarditis begins at J-point, rarely exceeds 5mm and retains its normal concave experience whereas STEMI typically convex (dome-shaped) and may be >5mm (also begins at J-point) 2) STEMI typically involves STE in anatomical groupings of leads that corresponds to vascular area of infarct (e.g. inferior leads) whereas acute pericarditis STE more generalised/most leads (as pericardium envelops the heart) 3) STEMI often associated with reciprocal ST segment changes whereas pericarditis is not (except for aVR/V1) 4) Concurrent STE and TWI do not generally occur simultaneously in pericarditis WHEREAS they commonly do in STEMI 5) Widespread PR depression (with PR elevation in aVR) is often seen in pericarditis but rarely in STEMI 6) Hyperacute T waves, new pathologic Q waves and QTc prolongation are all rare in pericarditis but common in acute MI

Question 34

How might acute pericarditis ECG be differentiated from a benign early repolarisation ECG?

Answer 34

1 - Acute pericarditis, STE in both limb and precordial leads whereas half the time BER patients have no STE in limb leads 2 - PR changes strongly favour pericarditis and are not seen in BER 3 - ratio of STE to T wave amplitude in V6 >0.24 favours pericarditis

Question 35

What are the two main forms of pericarditis in advanced chronic kidney disease and how might they be differentiated?

Answer 35

Uraemic pericarditis - pericarditis beginning withing 8 weeks of dialysis initiation Dialysis-associated pericarditis - pericarditis arising any time after 8 weeks of dialysis - complex pathogenesis - less response to increasing the frequency/intensity of dialysis - more likely to be associated with haemodynamic instability and serosanguinous pericardial effusion

Question 36

Does the absence of a pericardial effusion exclude pericarditis?

Answer 36

No

Question 37

What are the key initial tests to order for a patient with acute pericarditis?

Answer 37

ECG Bloods incl FBC, Troponin, ESR, CRP, HIV and HCV CXR Echo Consider: - BC if febrile - viral studies but yield is low/rarely alters management - ANA (esp young women / history suggests underlying SLE) - tuberculin skin test/interferon-gamma if suspect TB - cardiac MRI - nondiagnostic echo, constrictive pericarditis, complicated course, suspicion of specific aetiology - percardiocentesis - tamponade

Question 38

What is an echocardiogram likely to show in acute pericarditis?

Answer 38

Often normal May show associated pericardial effusion (which supports the diagnosis) - often small/without haemodynamic consequence

Question 39

What does the CXR typically show in patients with acute pericarditis?

Answer 39

Normal Rarely shows cardiomegaly - however acute pericarditis should be considered in the evaluation of a patient with new/otherwise unexplained cardiomegaly

Question 40

What is the significance of finding elevated cardiac biomarkers in patient with acute pericarditis?

Answer 40

Indicates myopericarditis

Question 41

What is the significance of raised inflammatory markers in patient with acute pericarditis?

Answer 41

Supports the diagnosis but is neither sensitive nor specific; also in hyperacute phase may remain normal Their role is often more in determining optimal duration and approach to tapering of therapy

Question 42

What are the typical findings of pericarditis on cardiac MRI and what other information can it provide?

Answer 42

Pericardial inflammation - bright and thickened pericardium on T2 weighted imaging + late gadolinium enhancement +/- non-calcified pericardial thickening May reveal concomitant myocarditis May evaluate for concomitant pleuropulmonary diseases and lymphadenopathies eg TB, lung ca

Question 43

Which patients should undergo pericardiocentesis?

Answer 43

Patients with symptomatic effusions and evidence of cardiac tamponade Large effusions refractory to medical therapy If pericardial effusion + specific aetiology suspected and diagnosis cannot be reached by other means (e.g. malignant or bacterial cause)

Question 44

What are the diagnostic criteria for acute pericarditis?

Answer 44

Need 2 of 4: 1) Typical chest pain (sharp, pleuritic, improved sitting up and leaning forward) 2) Pericardial friction rub 3) Characteristic ECG changes 4) New or worsening pericardial efusion

Question 45

What high-risk features indicate need for hospital admission in patients with acute pericarditis?

Answer 45

Fever Subacute course over days to weeks Cardiac tamponade Moderate-to-large effusion (end-diastolic free space of >20mm) Immunosuppression Warfarin/DOAC therapy Acute trauma Failure to improve on 7 days of appropriate anti-inflammatory therapy Elevated cardiac troponins

Question 46

What approach should be taken to establishing a definite aetiology of acute pericarditis?

Answer 46

A specific non-viral/idiopathic cause of acute pericarditis is found in ~17% Because of the relatively benign course associated with the common causes of реricаrditis, along with the relatively low yield of much of the diagnostic testing, it is not necessary to establish a definite etiology in all patients with acute реriϲаrditis. Initial efforts should focus on excluding a significant pericardial effusion or cardiac tamponade, and the identification of patients in whom a more comprehensive evaluation should be performed to exclude causes that require specific therapy (eg, malignancy, tսbеrϲսlοsis, or purulent реricаrditiѕ) In addition, among patients at high risk of coronary disease, МΙ must be ruled out by appropriate studies

Question 47

What are the non-viral/infective causes of pericarditis?

Answer 47

- Autoimmune eg SLE - Neoplastic eg lung cancer, breast cancer, and lymphomas and leukemias - Metabolic eg uraema - Traumatic - Post-AMI (Dressler syndrome) - Iatrogenic secondary to immune checkpoint inhibitor therapy and COVID-19 vaccination

Question 48

What is the classic triad of cardiac tamponade?

Answer 48

Soft heart sounds, elevated JVP, hypotension

Question 49

In most patients (ie patients without specific cause) with pericarditis, what is the mainstay of treatment?

Answer 49

1) Restriction of exercise (until resolution of symptoms AND normalisation of biomarkers OR if competitive athlete = 3mths OR if competitive and myopericarditis present = 6mths) 2) Drug therapy with: Colchicine (500mcg BD if wt >70kg for 3 months; once daily dose if wt<70kg) + NSAIDs (e.g. Aspirin 1g Q8H OR Ibuprofen 600mg Q8H for 1-2 weeks) Usually wean NSAIDs over 1-2 weeks then stop after symptoms have resolved and normalisation of inflammatory markers

Question 50

What is the role of oral steroids in acute pericarditis?

Answer 50

Can provide relief but are associated with usual complications of long-term corticosteroid therapy AND recurrence is common after stopping therapy THEREFORE should only be considered if NSAIDS are contraindicated or ineffective or for some immune-mediated causes

Question 51

Are there any alternative drug therapies for resistant or recurrent cases of pericarditis?

Answer 51

Yes - IL-1 inhibitors (anakinra) is showing promising but is not currently approved by TGA

Question 52

What does failure to respond to aspirin/NSAID therapy within 7 days (ie persistence of fever/pleuritic chest pain/new pericardial effusion/worsening general illness) indicate?

Answer 52

Suggests cause other than idiopathic/viral pericarditis is present OR aspirin resistance present OR constrictive pericarditis NSAIDs alone effective in reducing inflammation and pain relief in 70-80%; no evidence they alter natural history of acute pericarditis

Question 53

Which NSAIDs must be used in Dressler's syndrome?

Answer 53

Aspirin (+ Colchicine) Avoid others since anti-inflammatory therapy may impair scar formation

Question 54

What are the risks/issues with using NSAID therapy in acute pericarditis?

Answer 54

Theoretical risk that the antiplatelet activity may promote the development of a haemorrhagic pericardial effusion - however has not been convincingly established GI toxicity (eg gastritis, ulcers) - caution in px >65yo, hx of PUD, concurrent steroids/anticoagulant use Interaction with other medications especially warfarin (NSAIDs alter metabolism of VKA thus enhancing anticoagulant effect)

Question 55

Which patients with pericarditis benefit from colchicine and which don't/should avoid?

Answer 55

Benefit - idiopathic or viral pericarditis - autoimmune disease - post-cardiac injury syndromes/Dressler's Not benefit - bacterial pericarditis (may impair clearance of infectious agent) - malignancy-related - pericardial effusion

Question 56

What is the role/benefit of colchicine in patients with pericarditis?

Answer 56

Reduces symptoms Decreases the rate of recurrent pericarditis (HR 0.40)

Question 57

What are the side effects/risks of colchicine therapy?

Answer 57

Common = GI eg diarrhoea, nausea, vomiting Uncommon = bone marrow suppression, hepatotoxicity, myotoxicity - CKD seems to be major risk factor for these + drug-interactions/altered metablism

Question 58

If oral steroids are used for acute pericarditis, what is the optimal dose and guidelines around tapering?

Answer 58

0.2 to 0.5 mg/kg/day of prednisolone - usually begin tapering at 2-4 weeks after resolution of symptoms and CRP normalisation - more rapid tapering increases risk of treatment failure/recurrence

Question 59

Are there any adjunctive therapies that can be used in pericarditis?

Answer 59

For patients with elevated HR (>70bpm) - with nil evidence of tamponade but persistent symptoms, can trial beta-blockers if nil c/i

Question 60

How do you treat acute pericarditis in patients with advanced chronic kidney disease?

Answer 60

Uraemic pericarditis - commence dialysis )if not already) Dialysis-associated pericarditis - intensify dialysis regime + selective medical therapy with NSAIDs, colchicine, corticosteroids

Question 61

What are the risks of acute pericarditis?

Answer 61

Rare to get tamponade (usually only if malignancy/TB/purulent pericarditis) 1% risk of constrictive pericarditis 15-30% risk of recurrence/incessant disease if NOT treated with colchicine (approx halved by colchicine use); women at higher risk

Question 62

What is the key challenge in managing myopericarditis?

Answer 62

Excluding ACS How? coronary angiography (either invasive of CTCA) if intermediate or high risk for ACS (ie if: - hx of prior CAD - non-classical ECG findings - markedly elevated trop - high GRACE or TIMI score - unstable arrhythmias - persistent symptoms

Question 63

What is the difference between myopericarditis and perimyocarditis?

Answer 63

Often used interchangeably but technically two different disease processes with different approach to diagnosis and treatment Myopericarditis = confirmed acute pericarditis with elevated troponin but without LV systolic dysfunction Perimyocarditis = acute pericarditis + elevated troponin + LVEF <55% (approach same as myocarditis)

Question 64

What clinical features increase the likelihood of myopericarditis over isolated acute pericarditis?

Answer 64

Younger age Male sex Recent febrile illness ST elevation Ventricular arrhythmias Presence of pericardial effusion decreases likelihood of myopericarditis (ie raises chance of isolated acute pericarditis)

Question 65

What are the key tests to order in patients with myopericaditis?

Answer 65

Cardiac MRI with late gadolinium enhancement (within 2 weeks of symptom onset)

Question 66

How do you treat myopericarditis?

Answer 66

Exclude ACS/myocarditis/perimyocarditis Exercise restriction + NSAIDs + colchicine (as per pericarditis tx)

Question 67

How do you treat Vaccinia-associated myopericarditis (ie myopericarditis assoc with smallpox vaccination)?

Answer 67

Same as pericarditis Exercise restriction + NSAIDS/colchicine

Question 68

How do you treat myocarditis/perimyocarditis?

Answer 68

General therapy (for all) + specific therapy (targeting specific causes) General therapy - standard HF therapy eg ACEI, evidence-based beta-blocker, MRA, diuretics as required

Question 69

What are the treatment options for patients with myocarditis and haemodynamic instability or refractory HF despite Optimal Medical Therapy (OMT)?

Answer 69

Mechanical circulatory support e.g. ECMO / VAD Cardiac transplantation

Question 70

If arrhythmias develop in a patient with myocarditis, what therapy should be given?

Answer 70

None if asymptomatic APBs or VPBs or asymptomatic nonsustained arrhythmia (eg NSVT) If indicated - cardioversion OR amiodarone (mainstay) OR if no class IV HF cautious use of BB and CCB Temporary pacing if CHB or symptomatic bradycardia - usually transient so doesn't always require PPM Avoid class I and II antiarrhythmic drugs due to proarrhythmic and negative inotropic effects

Question 71

What should be avoided in the treatment of myocarditis/perimyocarditis?

Answer 71

NSAIDS - not effective and may increase mortality + exac HF Heavy alcohol consumption - may enhance the severity of myocarditis Exercise - 3-6 month abstinence from competitive sports

Question 72

What are the two most common types of cardiac amyloidosis?

Answer 72

Transthyretin amyloidosis (ATTR Amyloidosis) Light chain amyloidosis (AL amyloidosis) These account for 95% of cases and are named after the precursor protein of the amyloid deposit eg transthyretin and immunoglobulin light chains respectively

Question 73

What is the pathogenesis/pathophysiology and 2 main subtypes of ATTR amyloidosis?

Answer 73

ATTR amyloidosis results from the misfolding and deposition of tranthyretin in cardiac tissues Transthyretin (formerly called pre-albumin) is a protein produced by the liver that normally functions to transport thyroid hormone and retinol (vitamin A) There are 2 types: Wild-type ATTR (wtARRT) - previously called senile systemic amyloidosis caused by misfolding of wild-type/normal transthyretin Hereditary amyloidosis (hATTR) - caused by gene mutations in the transthyretin gene

Question 74

What is the typical mode of inheritance of hereditary amyloidosis?

Answer 74

Autosomal dominant inheritance with variable penetrance

Question 75

In what population is wtATTR underdiagnosed?

Answer 75

Elderly patients with HFpEF Patients with severe aortic stenosis

Question 76

What are the clinical features of light chain cardiac amyloidosis?

Answer 76

Age >40 Multisystem involvement - liver, kidneys, spleen, autonomic and peripheral nervous system, lungs, heart

Question 77

What is the main determinant of prognosis in AL amyloidosis?

Answer 77

Cardiac involvement/amyloidosis

Question 78

How does cardiac amyloidosis typically present?

Answer 78

SOB Peripheral oedema Elevated JVP Hepatic congestion Ascites (ie symptoms consistent with restrictive cardiomyopathy and predominantly right ventricular failure

Question 79

Do patients with cardiac amyloidosis commonly present with ischaemic heart disease?

Answer 79

No - angina is uncommon but microvascular dysfunction is common

Question 80

Why do patients with cardiac amyloidosis commonly present with syncope or presyncope?

Answer 80

Due to bradyarrhythmias or advanced AV block OR postural/exertional hypotension secondary to excessive diuresis or autonomic neuropathy Critical to recall that patients with ATTR amyloidosis often develop progressive conduction system disease and require PPM insertion This is far less common in AL amyloidosis

Question 81

Are patients with amyloid cardiomyopathy at increased risk for cardiac thromboembolism?

Answer 81

Yes - amyloid deposits in atrial and ventricular walls causes atrial dysfunction incl electromechanical dissociation during sinus rhythm associated with risk of atrial thrombus formation

Question 82

What are the typical features of extracardiac involvement in AL amyloidosis?

Answer 82

Nonspecific symptoms eg fatigue, poor appetite, early satiety, weight loss Kidney disease - asymptomatic proteinuria -> nephrotic syndrome Peripheral nerve involvement - peripheral neuropathy incl carpal tunnel syndrome GI involvement - incl hepatomegaly, GI bleeding Tongue involvement - macroglossia (nearly pathognomonic) Bleeding issues - incl periorbital purpura (nearly pathognomonic)

Question 83

Other than restrictive cardiomyopathy, what other cardiac condition is strongly associated with cardiac amyloidosis?

Answer 83

Severe aortic stenosis, particularly low-flow AS

Question 84

What extra-cardiac manifestations may be seen in ATTR amyloidosis?

Answer 84

Autonomic neuropathy Peripheral neuropathy - carpal tunnel syndrome Spinal stenosis Biceps tendon rupture

Question 85

What is the hallmark of cardiac amyloidosis on ECG?

Answer 85

Discordance between QRS voltage (often reduced) and LV wall thickness (e.g. on echocardiogram) - low sensitivity - more frequent in those with AL amyloidosis

Question 86

What other ECG features may be seen in amyloidosis?

Answer 86

LV hypertrophy LBBB Pseudo-infarct patterns AV block (in older patient with LVH should prompt consideration of cardiac amyloidosis) AF

Question 87

When should you suspect cardiac amyloidosis?

Answer 87

Unexplained LV hypertrophy # HF and unexplained LV hypertrophy # Patients with cardiac symptoms and unexplained LVH # Patients with Aortic stenosis + low-flow, low-gradient or impaired longitudinal strain # Patients with HF and other typical features eg history of bilateral carpal tunnel syndrome # Patients with condition highly assoc with cardiac amyloidosis eg systemic AL amyloidosis

Question 88

What finding on echocardiogram is more suggestive for cardiac amyloidosis?

Answer 88

Reduction in global longitudinal stain with relative apical sparing of longitudinal strain One of the earliest markers is reduced GLS; pattern of relative apical sparing can help differentiate cardiac amyloidosis from other hypertrophic phenocopies Sens 93%, spec 82%

Question 89

What is the typical approach to evaluation in patient with suspected cardiac amyloidosis?

Answer 89

1) Echocardiogram - often non-specific but may be suggestive 2) Cardiac MRI If cardiac MRI suggestive: 3) Test for evidence of monoclonal protein (serum free light chain assay + SPEP + UPEP) If monoclonal protein detected -> often go on to have bone marrow biopsy If monoclonal protein not detected -> cardiac scintigraphy with 99mtechnetium to identify presence/extent of cardiac uptake

Question 90

Is the presence of a monoclonal protein specific for AL amyloidosis in patient with suspected cardiac amyloidosis?

Answer 90

No - whilst suggestive, can also be seen in ATTR with MGUS

Question 91

Is bone scintigraphy helpful in patients with systemic AL amyloidosis and CMR suggestive of cardiac amyloidosis?

Answer 91

No it is not and cardiac MRI usually sufficient

Question 92

Can cardiac MRI distinguish between AL amyloidosis and ATTR amyloidosis?

Answer 92

No

Question 93

What is the characteristic appearance of cardiac amyloidosis on CMR with late gadolinium enhancement?

Answer 93

Initially diffuse subendocardial LGE Later transmural myocardial LGE pattern Sens 85%, spec 92%

Question 94

What is a key limitation for cardiac MRI in the evaluation of cardiac amyloidosis?

Answer 94

Gadolinium-based contrast agents relatively contraindicated in patients with severe renal dysfunction which is fairly common in AL amyloidosis

Question 95

If you are uncertain whether a wide complex tachycardia is SVT with aberrancy or VT - what are you best to treat it as?

Answer 95

VT Inadvertently treating VΤ as though it were SVΤ could precipitate hemodynamic collapse and/or cardiac arrest, while "mistreating" SVΤ as though it were VΤ will rarely cause a clinically significant adverse effect

Question 96

What ECG features suggest VT as the cause of wide complex tachycardia?

Answer 96

Regular rhythm (although polymorphic VT can be irregular) Extreme right axis deviation or any axis shift >40deg from baseline (OR QRS to left of -30deg in px with RBBB-like WCT or to right +90deg in LBBB-like WCT) AV dissociation (atrial activity independent of ventricular activity) Fusion beats (QRS complex has a morphology intermediate between that of a sinus beat and a purely ventricular complex) Capture beats (QRS complexes during a WCT that are identical to the sinus QRS complex) 'Warm up phenomenon' - a period of some irregularity and acceleration Wide QRS >160msec Concordance (QRS complexes in all precordial leads are monomorphic and of same polarity ie lack biphasic QRS)

Question 97

What is the Brugada criteria for VT?

Answer 97

1 - inspect V1-V6 for RS complex (biphasic QRS) - if nil RS = concordance present = VT 2) If RS complex present measure interval between onset of R wave and nadir of S wave - if longest RS interval is >100ms and R wave is wider than S wave = VT 3 - if longest RS interval is <100ms, assess for AV dissociation - if present = VT 4 - if nil AV dissociation, assess QRS morphology in V1 or V2 and V6 - if

Question 98

What are the causes of VT?

Answer 98

Coronary heart disease Dilated cardiomyopathy Infiltrative cardiomyopathy Chagas heart disease Complex congenital heart disease Cardiac sarcoidosis Arrhythmogenic right ventricular cardiomyopathy Left ventricular noncompaction Drugs - flecainide Idiopathic eg RVOT VT or LVOT VT Congenital long QTc Inherited long QTc eg drugs

Question 99

What is the role of cardiac MRI in the evaluation of patients with sustained monomorphic VT?

Answer 99

Can identify certain diagnoses eg arrythmogenic RV cardiomyopathy, cardiac sacroidosis, other infiltrative cardiomyopathies Can guide ablation (ie finding areas of delayed gadolinium enhancement)

Question 100

What is the most definitive means for establishing the diagnosis of sustained monomorphic VT?

Answer 100

Electrophysiology studies

Question 101

How do you treat patients with sustained monomorphic VT?

Answer 101

Unstable and pulseless - advanced life support eg prompt defibrillation Unstable but responsive/has a pulse - urgent cardioversion Stable - pharmacological or electrical cardioversion - pharm = IV amidarone, IV lidocaine or IV procainamide, IV sotalol Ongoing/other measures: - beta-blockers to reduce sympathetic facilitation esp in px with frequent recurrences of VT within short time period - correct hypokalaemia and hypomagnesaemia (although electrolyte abnormalities alone should not be accepted as cause of VT) - cease pro-arrhythmic drugs - interrogate ICD (if present) - urgent coronary revascularisation if active myocardial ischaemic felt to be contributing factor

Question 102

What are the pillars of chronic therapy for sustained monomorphic VT?

Answer 102

ICD insertion Beta-blockers Amiodarone Sotalol Catheter-based radiofrequency ablation