- Mitral regurgitation - systolic with ejection quality at LLSB - INCREASES with valsalva (decreased preload) - INCREASES with nitrates (decreased afterload

Cardiology - Cardiomyopathy Flashcards by Jen P

Is Takotsubo Cardiomyopathy more common in females or males?

Females

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Pathogenesis of Takotsubo Cardiomyopathy

Transient LV ballooning

- thought to be related to catecholamine excess

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Hallmark feature of a Takotsubo Cardiomyopathy…which part of the heart is affected?

There is wall motion abnormalities BEYOND a single territory

Brought on by acute emotional stressors

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ECG features of Takotsubo Cardiomyopathy?

ST elevation in precordial leads
QT prolongation
Widespread T wave inversion

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Management of Takotsubo Cardiomyopathy?

Acute: ACE inhibitor and BB
Supportive treatment
Don’t give GTN

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Natural progression of Takotsubo Cardiomyopathy

95% will have resolution of symptoms and LV recovery within 7 days

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What is the leading cause of death in young athletes <35 years old?

HOCM

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HOCM:

Inheritance pattern?

Autosomal dominant

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HOCM:

Which genes are mutated usually?

Usually genes encoding BETA MYOSIN HEAVY CHAIN PROTEIN

Usually sarcomere genes:

MYBPC3: cardiac myosin binding protein C
MYH7: beta myosin heavy chain

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What happens the FUNCTION of the heart in HOCM?

DIASTOLIC dysfunction and poor myocardial COMPLIANCE

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What are the cardinal structural features of HOCM

Septal hypertrophy
and
Anterior displacement of the mitral valve

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What kind of outflow problem happens in HOCM?

Dynamic LVOT in 66%

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What WORSENS the outflow problem in HOCM?

Reduced preload (ie dehydration)
Reduced afterload (ie vasodilators)
Drugs that enhance outflow: digoxin and inotropes

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Pulse in HOCM?

Rapid and bifid carotid upstroke (=pulsus bisferiens) if there is LVOT obstruction

And

‘Jerky’ pulse

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JVP in HOCM

high ‘a’ wave if significant infundibular hypertrophy

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Apex in HOCM

Sustained, localised and bifid or trifid

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Extra heart sounds in HOCM?

S4 (especially if young)

Paradoxical splitting of S2 if significant LVOT obstruction

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Murmur in HOCM?

Mitral regurgitation
systolic with ejection quality at LLSB
INCREASES with valsalva (decreased preload)
INCREASES with nitrates (decreased afterload

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Other diseases associated with HOCM?

Freidreich’s ataxia

WPW

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Complications of HOCM?

AF in 20 - 25%

Sudden cardiac death

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Risk factors for Sudden Cardiac Death in HOCM?

Family history of premature cardiac death
Recurrent syncope
Nonsustained VT (in adults)
Severe LVH (septum >2.5 - 3cm)
Severe obstruction
Abnormal exercise BP response
Level of myocardial fibrosis on MRI (contraversial)
Genotype: Arg719Trp

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What is the MOST SENSITIVE test to diagnose HOCM?

ECG

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What ECG findings in HOCM?

LVH in 80 - 90%
Progressive T wave inversion
Deep Q waves
+/- AF

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What echo findings in HOCM?

Systolic anterior motion of anterior mitral valve leaflet
and
Asymmetric hypertrophy

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How do you quantify LVOT obstruction in HOCM? What makes it severe?

Severe LVOT obstruction if pressure gradient is: >=30mmHg at rest >=50mmHg at exertion/provocation

Why is MRI useful in HOCM?

Better for eccentric and apical atrophy

Primary prevention measures in HOCM?

Betablockers ICD if indicated Anticoagulation if AF

What indications for ICD insertion in HOCM?

- FHx of sudden death - interventricular septum >30mm - Unexplained syncope - NSVT >3 beats on holter - Blunted increase (<20mmHg) in BP on exercise

Drugs to avoid in HOCM?

Nitrates ACE inhibitors Inotropes Digoxin

How to reduce the LVOT obstruction in HOCM?

Septal surgical myectomy: - GOLD STANDARD with symptom relief in >90% - low peri-op mortality Alcohol ablation (better if high risk BUT then needs PPM)

Who needs genetic screening if their relative has HOCM?

``` In ALL first degree relatives (examination, ECG and echo): Age 0 - 10yrs: every 2-3 years Age 11-20yrs: every 1 year Age 21-30yrs: every 2-3 years Age >31yrs: every 3-5 years ```

Poor PROGNOSTIC factors for HOCM?

- Syncope - FHx of sudden death - YOUNG age at presentation - NSVT - Abnormal BP response to exercise - increased septal wall thickness

Causes of RESTRICTIVE cardiomyopathy?

``` Amyloidosis Post-Radiotherapy Scleroderma Fabry's Loeffler's endocarditis Haemochromatosis Familial restrictive cardiomyopathy ```

In RESTRICTIVE cardiomyopathy, how does the apex compare on examination to DILATED?

Apex is LESS DISPLACED than in dilated CM

In RESTRICTIVE cardiomyopathy, how does the apex compare on examination to DILATED?

Apex is LESS DYNAMIC than in dilated CM

First line management in RESTRICTIVE cardiomyopathy?

Negative chronotropic agents (CCBs and BBs) to lengthen diastolic filling time and improve myocardial relaxation ACE inhibitors MAY improve diastolic dysfunction

In RESTRICTIVE cardiomyopathy when might digoxin need to be avoided?

In AMYLOID cause of restrictive cardiomyopathy don't use digoxin as it binds to amyloid fibrils and patient then becomes digoxin-toxic

Differentiating Constrictive versus Restrictive: | Apex beat?

Diminished in constrictive

Differentiating Constrictive versus Restrictive: | Diastolic elevated BP?

Seen in BOTH

Differentiating Constrictive versus Restrictive: | Kaussmaul's sign?

Seen in BOTH

Differentiating Constrictive versus Restrictive: | High BNP levels?

Seen in RESTRICTIVE

Differentiating Constrictive versus Restrictive: | CT with pericardial thickening and calcification?

Seen in CONSTRICTIVE

Differentiating Constrictive versus Restrictive: | Echo with equalisation of R and L ventricular diastolic pressures?

Seen in CONSTRICTIVE

Differentiating Constrictive versus Restrictive: | Peripheral oedema?

Seen in BOTH

Differentiating Constrictive versus Restrictive: | Symptoms of HF OUT OF PROPORTION to systolic dysfunction?

Seen in BOTH

Differentiating Constrictive versus Restrictive: | Elevated JVP? Which JVP abnormality might you see?

Seen in BOTH | ** prominant 'y' descent due to accentuation of early filling

Differentiating Constrictive versus Restrictive: | Presence of pulmonary hypertension?

Seen in RESTRICTIVE more commonly (as more severe diastolic abnormality)

Differentiating Constrictive versus Restrictive: | Early diastolic filling sounds?

In CONSTRICTIVE: 'knock' | In RESTRICTIVE: 'S3'

Dilated Cardiomyopathy: | What infective diseases can cause it?

VIRAL: coxsackie B, HIV, adenovirus and Hep C BACTERIAL: diphtheria PARASITE: Chagas and Toxoplasma Q fever

Dilated Cardiomyopathy: | Which rheum conditions are associated?

Poly and Dermatomyositis Sarcoid Collagen vascular disease Giant cell myocarditis and eosinophilic myocarditis

Dilated Cardiomyopathy: | Which toxins/drugs may cause it?

``` Alcohol Catecholamines Chemo: anthracyclines and Trastuzumab Interferon Hydroxychloroquine Lead Mercury ```

Dilated Cardiomyopathy: | Which neuromuscular diseases are associated?

Duchennes Beckers muscular dystrophy Mitochondrial myopathy

Dilated Cardiomyopathy: | Which endocrine conditions are associated?

HypERthyroidism Diabetes Pheochromocytoma

Dilated Cardiomyopathy: | Which nutritional deficiencies are causes?

``` Thiamine deficiency Selenium deficiency Niacin deficiency Carnitine deficiency (= cofactor in long chain fatty acid metabolism) ```

Dilated Cardiomyopathy: | Which electrolyte abnormalities can cause it?

Hypocalcaemia Hypomagnesaemia Hypophosphataemia

Dilated Cardiomyopathy: | Which genetic conditions can cause it?

- Gene coding TTN (titin) is most common - LMNA - SCN5A - PRKAG2 (also assoc WPW and AV block) - LAMP (Danon's disease)

What is Danon's Disease?

X-linked mutationin LAMP (lysosome associated membrane protein) - skeletal myopathy - mental retardation - LFT derangement - EXTREME LVH!!

Other than dilated cardiomyopathy what is the gene PRKAG2 associated with?

WPW | AV block

When does Peri-Partum Cardiomyopathy present?

Develops in the last month of pregnancy up until 5 months post partum

Pathological process implicated in Peri-Partum Cardiomyopathy?

Prolactin Processing with 16kDA prolactin fragment causing endothelial and myocardial damage

Risk factors for Peri-Partum Cardiomyopathy

- Age >30 years - African - Multi-foetus pregnancy - Greater parity - Multiple gestations - Maternal COCAINE abuse - Hx of pre-eclampsia / eclampsia / postpartum hypertension - Longterm (>4 weeks) tocolytics with beta-agonists (ie TERBUTALINE)

What to avoid in peri-partum cardiomyopathy?

ACE inhibitors, ARBs | Aldosterone antagonists

What is a woman at high risk of if she has peri-partum cardiomyopathy?

HIGH risk of thrombus

When to avoid pregnancy with hx of peri-partum cardiomyopathy?

Avoid 2nd pregnancy if: - EF persistently <50% - EF <25% at diagnosis

How does BROMOCRIPTINE work in peri-partum cardiomyopathy?

Bromocriptine causes prolactin blockade activating dopamine D2 receptors and actives post-synaptic dopamine receptors in TUBEROFUNDIBULAR pathway (--> inhibits prolactin secretion) and in NIGROSTRIATAL pathway (--> enhances motor control)

Cardiology - Cardiomyopathy Flashcards

(65 cards)