£ (52-27) (0.54-0.85)

Cardiunegaly 3.3 0.33

© (2.4-47) (023-0.48)

ECO Figure 40.Congestive heart failure

AFib 3.8 0.79

(12-8.8) (0.65-0.96)

Any abnormal 2.2 0.64

finding (1.6 3.1) (0.47 0.88)

Carotid Pulse * Cl = confidence interval

a wave

JVP Wavelorm Dichotomies of HF

• Forward vs.backward

• Left-sided vs.right-sided

• Systolic vs.diastolic dysfunction

• Low output vs.high output

Cardiac

S Sr Sounds

x descent

ECG P T

QRS Use EF to GradeIV

Dysfunction

• GradeI(EF >60%) (Normal)

• GradeII(EF - 40-59%)

• GradeIII(EF - 21-39%)

• GradeIV (EF <20%)

Features ol Abnormal JVP Wave Formation

Atrial fibrillation:absent a wave

3rd degree heartblock:cannon a waves

Tricuspid regurgitation:cv wave,elevated JVP

Cardiac tamponade:x descent only,absent y descent

Constrictive pericarditis:prominent y descent,Kussmaul's sign

(paradoxical increaseinJVP with inspiration)

2

S r "i

L J .5

(3

c

y descent

-t +

Figure 41. JVP waveform

C ll Cardiology and Cardiac Surgery Toronto Notes 2023

Table 15. Signs and Symptoms of Left vs. Right HF

Left Failure Right Failure

See Landmark CardiacTnatsfor more information on

DAPA-HF which detailstheefticacy of SGLT2 inh tktion

m patientswith HFrEFand without 120M.

Left failure symptoms if decreased RV output

leads to LV underfilling

low CO (Forward) Fatigue

Syncope

Systemic hypotension

Cool extremities

Slow capillary refill

Peripheral cyanosis

Pulsus alternans

IR

S3 (right-sided)

See Landmark Cardiac Inalsfor more information on

PARADIOM-HF which detailsthe survival outcomesof

HFrff patientstreated with an ACE I or a n angiotensinntprilysln inhibitor.

MR

S3

Venous Congestion (Backward) Dyspnea, orthopnea. PND

Cough

Crackles

Peripheral edema

Elevated JVP with abdominojugulat reflux, and

tKussmaul'

ssign

Hepatomegaly

Pulsatile liver

It Validated Clinical and BiochemicalScore

lor the Diagnosis ol Acute Flearl Failure:

I he ProBHP Investigation ol Dyspnea in the

Emergency Department (PRIDE) Acute Heart

failure Score

Am Heat J 2006;151:48-S4

Pathophysiology

• most common causes are ischemic heart disease, risk factors for CAD, LVH (HTN), valvular heart

disease, and tachyarrhythmia

• myocardial insult causes pump dysfunction/impaired filling leading to myocardial remodeling and

the following maladaptive changes:

• pressure overload (e.g. AS or HTN) leads to compensatory hypertrophy (i.e. concentric

remodeling) and eventually interstitial fibrosis

• volume overload (e.g. aortic insufficiency) leads to dilatation (i.e. eccentric remodeling)

• remodeling results in decreased forward CO resulting in activation of the SNS and RAAS

• SNS causes tachycardia

• RAAS causes Na f and water retention to increase preload and afterload

• net result is increased cardiac demand leading to eventual decompensation

Predictor Possible Score

Age »75 yr

Orthopnea present 2

Lack of cough

Current loop

diuretic use (before

presentation)

Rales nn lung eiant t

lack of lever

Elevated NT-proBNP 4

( >4S0 pgfmL if <50 yr,

»900 pgfmlif »50 yr|

Interstitial edema 2

on UR

1

1

1

Heart Failure with Reduced Ejection Fraction (HFrEF: LVEF <40%)

• impaired myocardial contractile function -> decreased LVEF and SV -> decreased CO

• volume overload is the typical phenotype

• findings: apex beat displaced, S3, cardiothoracic ratio >0.5, decreased LVEF, LV dilatation

• causes

ischemic (e.g. extensiveCAD, previous Ml)

non-ischemic

HTN

DM

EtOH (and other toxins)

myocarditis

DCM (multiple causessee Dilated Cardiomyopathy,C47 )

tachycardia-induced

2

Total /14

likelihood of HF

low *

0-5

Intelmediate *

6 -8

High »

914

Heart Failure with Mid-Range Ejection Fraction (HF-mrEF: LVEF 41-49%)

• includes patients who are recovering from HErEE, declining from HI pEE, and transitioning to HEpEE

• characterization of HEmEF ongoing; guideline management does not currently exist

BNP is secreted by Vs due to LV stretch

and wall tension.Cardiomyocytes

secrete BNP precursor that is cleaved

into proBNP.After secretion into Vs,

proBNP is cleaved into the active

C-terminal portion and the inactive

NT-proBNP. The above scoring algorithm

developed by Baggish et al.Is commonly

used. A score of <6 has a negative

predictive value of 98%,while scores>6

had a sensitivity of 96% and specificity

of 84% (P<0.001) for the diagnosis of

acute HF

Heart Failure with Preserved Ejection Fraction (HFpEF: LVEF >50%)

• previously known as “diastolic HE"

• concentric remodelling with a “stiff" LV is the typical phenotype

• 50% of patients with HE have preserved EE;confers similar prognosis to HErEE; more common in the

elderly and females

• reduced LV compliance causes increased LV filling pressures, increased LA pressure/volume, and

pulmonary congestion

• findings: HTN. apex beat sustained, S-l, normal-sized heart on CXR, LVH on ECCi/echo, normal EE

• causes

transient: ischemia (e.g. CAD, Ml)

permanent:severe hypertrophy (HTN,AS, HCM), RCM (e.g. amyloid), Ml

NYHA Functional Classification of HF

• Class I: ordinary physical activity

does not cause symptoms of HF

• Class II: comfortable at rest; ordinary

physical activity results in symptoms

. Class III:marked limitation of

ordinary activity:less than ordinary

physical activity results in symptoms

• Class IV:inability to carry out any

physical activity without discomfort:

symptoms may be present at rest

High-Output Heart Failure

• caused by demand for increased CO

• often exacerbates existing HE or decompensates a patient with other cardiac pathology

• DDx:anemia, thiamine deficiency (beriberi), hyperthyroidism, arteriovenous (A-V) fistula or left to

right (L-R) shunting, Paget’s disease, renal disease, hepatic disease

r t

L J

Precipitants of Symptomatic Exacerbations

• consider natural progression of disease vs. new precipitant

• always search for reversible cause +

G12 Cardiology and Cardiac Surgery Toronto Notes 2023

• DDx can also be organized as follows:

new cardiac insult/disease: Ml, arrhythmia, valvular disease, cardiotoxic chemotherapy

new demand on CV system:HTN, anemia, thyrotoxicosis, infection

medication non-compliance

• dietary indiscretion (e.g.salt intake)

obstructive sleep apnea

Five Most Common Causes of CHF

. CAD (60-70%)

. HTN

. Idiopathic (often DCM)

• Valvular (e.g. AS. AR. and MR)

• EtOH (DCM)

Investigations

• identify and assess precipitating factors and treatable causes of CHI-

'

• blood work:CBC,electrolytes (including calcium and magnesium), blood urea nitrogen (BUN),Cr,

fasting blood glucose, hemoglobin Ale, lipid profile,LFTs,serum TSH ± ferritin, BNP (>100 pg/mL),

NT-ProBNP (>300 pg/ml), uric acid

• urinalysis

• ECU:look for chamber enlargement, arrhythmia, ischemia/infarction

• CXR: cardiomegaly, pleural effusion, redistribution, Kerley B lines, bronchiolar-alveolar culling

• echo:systolic function (LVEP),diastolic function (E/A ratio,E/e’),cardiac dimensions, wall motion

abnormalities, RV systolic pressure (from TR jet), valvular disease, pericardial effusion

• radionuclide angiography: LVEP

• myocardial perfusion scintigraphy (thallium or sestamibi SPE(.T)

<§>

Precipitants of HF

HEART FAILED

HTN (common)

Endocarditis/environment (e.g. heat

wave)

Anemia

Rheumatic heart disease and other

valvular disease

Thyrotoxicosis

Failure to take medications (very

common)

Arrhythmia (common)

Infection/Ischemia/Infarction (common)

Lung problems (PE. pneumonia. COPD)

Endocrine (pheochromocytoma.

hypcraldosteronism)

Dietary indiscretions(common)

Additional Diagnostic Investigations

• cardiac catheterization

• cardiopulmonary'exercise testing

. other tests (CMR, MPI, MUGA,CT scan)

Acute Treatment of Pulmonary Edema

• treat acute precipitating factors(e.g.ischemia,arrhythmias)

L Lasix* (furosemide) 40-500 mg IV

M morphine 2-4 mg IV: decreases anxiety and preload (venodilation)

N nitroglycerin: topical/IV/SL - use with caution in preload-dependent patients (e.g. right HP or

RV infarction) as it may precipitate CV collapse

O oxygen:in hypoxemic patients

• P positive airway pressure (continuous positive airway pressure (CPAP)/bilevel positive airway

pressure (BiPAP)):decreases preload and need for ventilation when appropriate

P position:sit patient up with legs hanging down unless patient is hypotensive

• in ICU setting or failure of LMNOPP: other interventions may be necessary

nitroprusside IV

hydralazine PO

sympathomimetics

dopamine

- low dose:selective renal vasodilation (high potency D1 agonist)

- medium dose: inotropic support (medium potency pi agonist)

- high dose: increases SVR (low potency pi agonist), which is undesirable

* dobutamine

- pi-selective agonist causing inotropy, tachycardia, hypotension (low dose) or HTN (high

dose);most seriousside effect is arrhythmia, especially AEib

phosphodiesterase inhibitors (milrinone)

- inotropic effect and vascularsmooth muscle relaxation (decreased SVR),similar to

dobutamine

• consider pulmonary artery catheter to monitor PCWP if patient is unstable or a cardiac etiology is

uncertain (PCWP >18 indicateslikely cardiac etiology)

• mechanical ventilation as needed

• rarely used, but potentially life-saving measures:

IABP - reduces afterload via systolic unloading and improves coronary perfusion via diastolic

augmentation

LVAD/RVAD

cardiac transplant

The most common cause of right HF is

left HF

Measuring NT-proBNP

BNP is secreted by Vs due to IV stretch and

wall tension

Cardiomyocytes secrete BNP precursor

that is cleaved into proBNP

After secretion into Vs.proBNP is cleaved

into the active C-terminal portion and the

inactive NT-proBNP portion

NT- proBNPIevels

Wirt)

HF very likely

*

pe

<50 >450

50-75 >900

>75 »1800

IMlaUons:Age.body hjtrtuv WMIlunctton.PI

Features

*

of HF on CXR

HERB-B

Hea rt enlargement (cardiothoracic

ratio >0.50)

Pleural Effusion

Re-distribution (alveolar edema)

Kerley B lines

Bronchiolar-alveolar cuffing

Long-Term Management

• overwhelming majority of evidence-based management applies to H FrEF

• currently no proven pharmacologic therapiesshown to reduce mortality in Hl-pEE;control risk factors

for HTpEE (e.g.HTN)

• prevent fluid overload with appropriate diuretic strategies

Conservative Measures

• symptomatic measures: oxygen in hospital, bedrest, elevate the head of bed

• lifestyle measures:diet, exercise, DM control,smoking cessation, decrease EtOH consumption, patient

education,sodium, and fluid restriction

• multidisciplinary'H E clinics:for management of individuals at higher risk,or with recent

hospitalization

n

L J

Patients on p-blocker therapy who

have acute decompensated HF should

continue (5-blockers where possible

(provided they are not in cardiogenic

shock or in severe pulmonary edema)

+

013Cardiology and Cardiac Surgery Toronto Notes 2023

Non-Pharmacological Management

• from 2021 CCS guidelines

• restrictsalt intake to 2-3 g/d

• monitor daily weight for patients with HI-

'

,fluid retention, or congestion that is difficult to control with

diuretics or renal dysfunction

• restrict daily fluid intake to approximately 2 L/d for patients with fluid retention or congestion that is

difficult to control with diuretics

• cardiac rehabilitation: participation in a structured exercise program for N YHA class 1-111 after

clinicalstatus assessment to improve quality of life (HF-ACT10N trial)

Pharmacological Therapy

. ACEI/ARB: HAAS blockade

ACE1:slows progression of LV dysfunction and improvessurvival

all symptomatic patientsfunctional class 11-1V

all asymptomatic patients with LVEF <40%

post-MI

• angiotensin II receptor blockers

second-line to ACEI (if ACEI not tolerated), or as adjunct to ACEI if (3-blockers not tolerated

combination of p-blockers with ACEI is not routinely recommended and should be used with

caution asit may precipitate hyperkalemia,renal failure, and the need for dialysis(CHARM,

ONTARGET)

CCS/CHFS Heart Failure Guidelines Update:

Defining a Hew Pharmacologic Standard of Care

for Heart FailurewKh Reduced ejection Fraction

Can JCardiolM2UJ:531-40

Management of HFtlF: ll isiKommendedthal,

in the absence of contraindications. KFrfF patients

be treated witbcombinabon therapy intinding

1drug from each of the following categories:

ARM (orACEL'UB). 3- blocker, mineratocorticoid

receptor antagonist (MRA) and SGLT2 inhibitor.II

is recommended that patients admittrtwith acute

decom pensated HFiEF should be switched to an ARM.

Horn an ACEHARB when stabilized.It isrecommended

that 3-blockers be initiated assoon as passible after

HF diagnosis, not waiting until hospitaldischarge

to Initiate treatment in stabilized patients.MRA

treatment isrecommended lor patientswith acuteMI

and LYEF < 40'Vand KF symptoms or DM.to reduce

CV mortality and hospitalization for CVeients.SGLI2

inhibitorsshould be used in patients with HFrEF.with

or without concomitant I2DM, to improresymptoms

and reduce hospdatiiations.

• antiarrhythmic drugs:for use in CHE with arrhythmia

can use amiodarone, (3-blocker, or digoxin

• anticoagulants: DOACs or vitamin K antagonist (warfarin) for prevention of thromboembolic events

prophylactic indications:

Afib

LV thrombus

Prior thromboembolic event

See Landmark Cardiac Inalsfor more information on

DAPA- HF which detaiisthe efficacy of SGU2 inhiiition

in patients with HFrEF and withoutI2DM.

See Landmark CardiacTrialsfor more information on

PARAOIGM- HF which details the surmaloutcomesoi

If rtf patients heated with an ACEI or an angiotensinneprllysln inhibitor.

• ARN1:combination angiotensin receptor-neprilysin inhibitors - slows down progression of LV

dysfunction and improvessurvival

RAAS inhibitor prevents volume overload and neprilysin inhibitor enhances effects of BNP

first line therapy or ifswitching from an ACEI or ARB among patients with residual N YHA 11-1V

symptoms and LVEE <40%

• (3- blockers:slow progression and improve survival

(3-adrenergic blocking agents blocks effects of epinephrine to reduce rate and force of myocardial

contraction

indicated for class 1-111 with LVEE <40% and stable class IV patients

carvedilol improvessurvival in class IV HE (COMET)

caution:should be used cautiously; titrate slowly because may initially worsen CHE

• diuretics: management of fluid overload and symptom control (e.g. dyspnea and PND)

furosemide (40-500 mg QD) for potent diuresis

metolazone once weekly may be used with furosemide to increase diuresis if patient becomes

refractory to furosemide

furosemide, metolazone, and thiazides oppose the hyperkalemia that can be induced by

P-blockers, ACEI, ARBs, and aldosterone antagonists

• digoxin and cardiac glycosides:increase myocardial contractility but decrease rate

improves symptoms and decreases hospitalizations; no effect on mortality

indications: patient in sinus rhythm and symptomatic on ACEI or CHE and Al-

'

ib

caution: patients on digitalis glycosides may worsen if these are withdrawn

• hydralazine plusisosorbide dinitrate:combination antihypertensive and vasodilator

consider forsymptom control and mortality benefit in Black patients with symptomatic HFrEF

despite guideline-directed medical therapy (GDMT)

also consider for Hl-rEE patients with drug intolerance to ACEIs, ARBs,or ARN1

• ivabradine:selective inhibition of the If current

recommended forCV death and hospitalization prevention in patients with HFrEF and

symptomatic despite:

treatment with appropriate doses of GDMT, resting HR >70 bpm, and in sinus rhythm

weaker level of evidence than either ARN1 or SGL’1‘2 inhibitor

r n

LJ

• mineralocorticoid receptor (aldosterone) antagonists:spironolactone or eplerenone +

mortality benefit in symptomatic HE and severely depressed EE

• for symptomatic HE in patients already on ACEI, (3-blocker, and loop diuretic

caution: potential for life threatening hyperkalemia

monitor K 'after initiation and avoid if Cr >220 pmol/L or K +

>5.2 mmol/L

C44 Cardiology and Cardiac Surgery Toronto Notes 2023

• SGLT2 inhibitor: empaglillozin, canagliflozin, dapaglitlozin

« recommended for treatment of patients with stable HErEE,irrespective of T2 DM

recommended in mild to moderate HErEE with concomitant T2DM to improve symptoms and

reduce mortality

Chronic Treatment of CHF

. ACEI*

• pblockers'

• i Mineralocortlcold receptor

antagonists*

• Diuretic

. ARNI

• t Inotrope

. Antiarrhythmic

• iAnticoagulant

'Mortality benefit

HFrEF Management

1. ARNI (or if on ACE1/ARB substitute to ARNI)

2. p-blockers

3. MRA

4. SGLT2 inhibitor

HFpEF Management

1. ARB

2. Systolic/Diastolic Hypertension Management according to CHER Guidelines (2017)

3. MRA (if serum K* <5.0 mmol/L and eGl R >30 ml/min)

Ivabradine and Outcomesin Chronic Heart failure

(SHIFT):A Randomized Placebo-Controlled Stud y

Lanced 2010:376:11-17

Study: Randomised, double-blind, placebocontrolled. parallel-group trial.

Population Patients with symptomatic Hfand IVEF

of 35% or loner,in sinus rh ythm with HR greater than

or equal to JO bpm, had been admitted to hospital

lot HF within presions year, on stabe background

treatment including 3-blocker iftoierated.

Intervention: hapiadme titrated toa minimum of 7.5

mg SID vs. placebo.

Outcome Primary endpoint was composite of CV

death or hospital admission for worsening Hf .

Results:133|24%|patients in the nrabradine

group and 93/|29%|of those tak ig placebo had a

primary endpoint crent (HR 0.82, 95% Cl 0.75-0.90.

P< 0.000l|. f ewetserious adverse events occulted

m the ivabtadL-egioup|3398 eierts) than in

the placebo group (3847; P >

0.025).15015%) of

ivabradine patients had symptomatic bradycardia

vs. 32|1%|oflhe pacebo group|P*0.0001|.Median

follow up was 22.9 mo(interquartile range 18-281 .

Conclusions Resullssuppnrtfenrporlanceol HR

reduction with rvabradine fur improiement ol clinics

outcomes in HF and confirm the important role of

HR in the pathophysiology of this disorder. Hole:

limitation of thisstudy was that only 28% ol patients

were an target 3 blocker doses.Ivabradine currently

recommended in these patients when HR is not

controlled on man mum tolerated J-blocker dnseor

there isa contraindi ration to J-hlocker use.

Surgical Management

• revascularization is the most frequently performed operation in HE patients with the aim to restore

blood flow to hibernating myocardium (<10% operative mortality in some patient groups)

• mitral valve surgery for the treatment of M R secondary to ischemic LV dilation

• LV remodeling (Batista procedure - partial left ventriculectomy; Dor procedure - left ventricular

restoration) improves ventricular function by reducing ventricular radial dimensions and thus

decreasing wall tension via Laplace's law

• VADs (see Cardiac transplantation, C50)

• heart transplantation (seeCardiac transplantation, C50)

IPCN Cell membrane current ivabradine administration -

Slowed rate ol I

diastolic depolarization I

•

0 mV

= reduced HR

/ ,-channel at SA node —

Higher New York Heart Association Classes

and Increased Mortality and Hospitalization in

Patients with Heart Failure and Preserved left

Ventricular function

Am HeartJ 2006:151:444 4S0

Purpose: Toestablish the association between

NY HA dassand outcomeswith HF and preserved

systolic function.

Methods Retrospective loilow -DPStudy (median

38.5 mo) of 988 patients with Hf with tf >45%.

Estimated risks ol various outcomes using Con

propod ora! hazard models.

Results:Adjusted HR forall-cause mortality for

NYHA classll. III. IV patentswas1.54.2.56, and

8.48.respectively. Adjusted HR for a cause

hospitalization Ipi HVHA class II.II. IV patents was

1.23.1.71.and 3.4, respectively.

Conclusions:Higher NYHA classes were associated

with poorer outcomes in patients with Hf an d

preserved systolic function.

Proportiohsof NYHA I. II. III. and IV patents who d ed

ol allcausesdutingllic study were 14.3%,

Figure 42. Ivabradine mechanism of action

Procedural Interventions

• resynchronization therapy:symptomatic improvement with CRT-P or CRT-D

consider if QRS >130 msec with I.BBB morphology, LVEE <35%, and persistent symptoms despite

QMT

greatest benefit likely with marked LV enlargement, MR, QRS >150 msec

CRT'

-R is indicated for patients eligible for resynchronization therapy but not 1CD;if the patient is

also eligible for an 1CD the decision for CRT-D is individualized in accordance with overall goals

of care

• 1CD: mortality benefit i n i"prevention of SCD

consider if:prior Ml,OMT, LVEE <30%, clinically stable

consider if:prior Ml, non-sustained VI, LVEE 30-40%, EPS inducible VT

• LVA D/ RVA D (see Ventricular Assist Devices, C52 )

• cardiac transplantation (see Cardiac Transplantation, (.5(7)

• valve repair if patient is surgical candidate and hassignificant valve disease contributing to CHI (see

Valvular Heart Disease, C54)

L J

NYHA Propovtion

of All-Cause

Hospitalization

Proportion

of All-Cause

Modality

I 60.7% 14.3% +

65.2% 21.3%

72.7% 35.9%

IV 75.0% 58.3%

015Cardiology and Cardiac Surgery Toronto Notes 2023

RATIONALE FOR HF TREATMENT:

Reduce afterload t augment contractility -improve CO + relieve pulmonary congestion

A =diuretic or venodilator

(nitrates)

A'

=aggressive diuresis or

venodilation

I=inotopes (contractility)

D = ACEI (vasodilation)

B=inotrope ACEI

NORMAL

m

_

I I

UJ (0

sc vs

HF +

TREATMENT

=

1

S S

cc ~

uI

LEFT VENTRICULAR END-DIASTOUC PRESSURE

(preload) © Young M.Kim 2012

Figure 43.Effect of HF treatment on the Frank-Starling curve

Sleep-Disordered Breathing

• patients with CHI can have sleep disturbances; 40% of patients have central sleep apnea with CheyneStokes breathing and 11% of patients have obstructive sleep apnea

• associated with a worse prognosis and greater LV dysfunction

• nasal CPAP may be effective to treat symptoms of sleep apnea with secondary benefits to cardiac

function

Cardio-oncology

• cardiotoxicity of chemotherapeutic agents is a leading cause of long-term morbidity and mortality

among cancer survivors

• dose-dependent LV systolic dysfunction with anthracvclines and potentially reversible decline in

LVLT with trastuzumab

• evaluate CV risk factors and optimize treatment of pre-existing CV disease before, during, and after

receiving cardiotoxic cancer therapy

• follow patient using same imaging modality and methods (e.g. echo with contrast, echocardiographic

global longitudinal strain (GLS), 3 dimensional echo, or multiple-gated acquisition (MUGA) scan) to

assess LV function before, during, and upon completion of chemotherapy

• recommended that clinical HF or an asymptomatic decline in LVEF (>10% decrease in LVEF from

baseline or LVEF <53%) during or after treatment is managed according to CCS guidelines

Myocardial Disease

Definition of Cardiomyopathy

• intrinsic or primary myocardial disease not secondary'to congenital, hypertensive, coronary, valvular,

or pericardial disease

• results in both morphologic and functional abnormalities

• functional classification:dilated, hypertrophic, or restrictive

• LV dysfunction secondary to Ml,often termed “ischemic cardiomyopathy',” is not a true

cardiomyopathy (i.e. primary myocardial disorder) since the primary pathology is obstructive CAD

Cardiomyopathy

HARD

Hypertrophic cardiomyopathy (HCM)

Arrhythmogenic right ventricular

cardiomyopathy

Restrictive cardiomyopathy (RCM)

Table 16 Dilated cardiomyopathy (DCM)

. Comparison of Cardiomyopathies, Secondary Causes, and Consequent HF Phenotypes

Heart Failure Reduced Ejection Fraction

(HFrEF)

Heart Failure Preserved Ejection Fraction (HFpEF)

Dilated Cardiomyopathy Secondary Causes

( DCM )

Idiopathic, infectious

(e.g. myocarditis),

EtOH.familial,

collagen vascular

disease

Hypertrophic

Cardiomyopathy ( HCM)

Restrictive

Cardiomyopathy (RCM )

Secondary Causes

CAD. Ml. DM. valvular (e.g.

AR. MR)

Genetic disorder affecting Amyloidosis, sarcoidosis. HIM. DM. valvular (e.g.

AS). post- MI. transiently by

ischemia

cardiac sarcomeres(most scleroderma,

common cause of SCD in hemochromatosis.

Fabry's, Pompe'

s Disease,

loclflei's

r i

L J

young athletes)

+

GI6Cardiology and Cardiac Surgery Toronto Notes 2023

Myocarditis

Definition

• inflammatory process involving the myocardium ranging from acute to chronic

• important cause of DCM

• spectrum of severity ranging from non-specific symptomssuch as fatigue to cardiogenic shock

Etiology

• idiopathic

• infectious

viral (most common overall cause):coxsackie A and B,parvovirus B19, adenoviruses, influenza,

coxsackie B, echovirus, poliovirus, HIV, mumps,coronavirus disease 2019 (COV1D-19)

• bacterial: S.aureus.Streptococcus, perfringens,C.diphtheritic. Mycoplasma, Rickettsia

• fungi

spirochetal (Lyme disease Borrelia burgdorferi)

• Chagas disease (Trypanosoma cruzi), Toxoplasma gondii

• toxic:catecholamines, chemotherapy, cocaine

• hypersensitivity/eosinophilic:drugs (e.g. antibiotics, diuretics,lithium, clozapine), insect/snake bites

• systemic diseases: collagen vascular diseases(e.g.SLE,rheumatoid arthritis),sarcoidosis,

autoimmune

• other:giant cell myocarditis, acute rheumatic fever

Signs and Symptoms

• constitutional symptoms

• acute CHE: dyspnea, tachycardia, elevated ) VH

• cardiogenic shock

• chest pain:due to pericarditis or cardiac ischemia

• arrhythmias

• systemic or pulmonary emboli

• presyncope/syncope/sudden death

Investigations

• ECG: non-specific ST-T changes and/or conduction defects (used for initialscreening and risk

stratification)

• echocardiography:lack of cardiac dilation and increased thickness ofseptum (fulminant

myocarditis),spherical ventricle that remodels to elliptical aftersome months(acute myocarditis)

• blood work

• increased creatine kinase (CK), cardiac troponins (cTnl and cTnT), NT-proBNP (if LV dysfunction

occurs), LDH, and AST'

with acute myocardial necrosis ± increased WBC, C-reactive protein

(CRP),erythrocyte sedimentation rate (ESR), antinuclear antibody test (ANA), rheumatoid factor,

complement levels

• blood culture, viral titres, and cold agglutininsfor Mycoplasma

• CXR:enlarged cardiac silhouette

• TEE:systolic dysfunction (dilated, hypokinetic chambers,segmental wall motion abnormalities) and/

or diastolic dysfunction

• CMR:functional and morphological abnormalities as well as tissue pathology (gadolinium

enhancement)

• endomyocardial biopsy:only done in certain clinical scenarios (e.g. on inotropic and/or mechanical

circulatory support)

• coronary angiography: to exclude ischemic heart disease

Management

• supportive care

• mechanical circulatory support and inotropic support if cardiogenic shock

• restrict physical activity during early recovery

• treat CHE per current HE guidelines

guideline-directed medical therapy

• advanced therapiessuch as ventricular assist and transplantation

• treat arrhythmias

• anticoagulation

• treat underlying cause if possible

Prognosis

• often unrecognized and may be self-limited

• myocarditistreatment trialshowed 5 yr mortality between 25-50%

• giant cell myocarditis, although rare, can present with fulminant CHE and be rapidly fatal, with 5 yr

mortality >80%

• sudden death in young adults

• may progress to DCM

r

cJ

+

C47Cardiology and CardiacSurgery Toronto Notes 2023

Dilated Cardiomyopathy

Definition

• unexplained dilation and impaired systolic function of one or both ventricles

• if present,comorbid CAD is unable to fully account for extent of dysfunction observed Major Risks Factors tor DCM

FMHx.EtOH,cocaine.

Etiology

• familial/genetic ~60%

. EtOH -20-30%

• myocarditis

• infectious:viral (coxsackie B,HIV,COV1D-19), Chagas disease,Lyme disease,Rickettsial diseases,

acute rheumatic fever, toxoplasmosis

• collagen vascular disease: SLE, polyarteritis nodosa,dermatomyositis, progressive systemic sclerosis

• idiopathic (presumed viral or idiopathic)

• uncontrolled tachycardia (e.g. persistent, rapid Al-

'

ib)

• neuromuscular disease:Duchenne muscular dystrophy,myotonic dystrophy,Eriedreich’

s ataxia

• metabolic:uremia, nutritional deficiency (thiamine,selenium, carnitine)

• endocrine:hyper/hypothyroidism, DM, pheochromocytoma

• peripartum

• toxic:cocaine, heroin,organic solvents

- drugs: chemotherapies(doxorubicin,cyclophosphamide), anti-retrovirals, chloroquine, clozapine,

TCA

• radiation

Abnormal Labs in DCM

. High BNP

. HighCr

• High LFTs

• Low bicarbonate

• LowNa+

Signs and Symptoms

• may present as:

systolic HE

systemic or pulmonary emboli

• arrhythmias

sudden death (major cause of mortality due to fatal arrhythmia)

Investigations

• blood work:CBC, electrolytes, Cr, bicarbonate, BNP, CK, troponin, LET*

,TSH, total iron binding

capacity (TIBC)

• ECG: variableST-T wave abnormalities, poor R wave progression, conduction defects (e.g.BBB),

arrhythmias(e.g. non-sustained VT)

• CXR: global cardiomegaly (i.e. globular heart),signs of CHE, pleural

• echo:systolic dysfunction (chamber enlargement, global hy pokinesis, depressed LVEE, MR and TR,

mural thrombi)

cardiac MR1:myocardial fibrosis

• endomyocardial biopsy:not routine, used to rule out a treatable cause

• coronary angiography: in select patientsto exclude ischemic heart disease

Management

• treat underlying disease:e.g. abstinence from EtOH

treat CHE as per current guidelines (see Heart Failure, C40)

includes medical management and devices (1CD and CRT)

• advanced therapies considered for medication-refractory disease

e.g. LVAD, transplant, and volume reduction surgery

• thromboembolism prophylaxis: anticoagulation with warfarin

indicated for: AEib, history of thromboembolism or documented thrombus

• treat symptomatic or serious arrhythmias

• immunize against influenza and S. pneumoniae

• indication to screen first-degree relatives when unclear etiology

effusion

Prognosis

• depends on etiology, often parallels prognosis of systolic HE

• belter with reversible underlying cause; worst with infiltrative diseases, HIV,drug-induced

• early reverse remodelling with optimal HE management (i.e. medications and devices) improves

prognosis

• myocardial fibrosis increases SCD risk

• cause of death usually CHE (due to pump failure) or sudden death secondary to ventricular

arrhythmias

• systemic emboli are significantsource of morbidity

• 20% mortality in first yr, 10% per year thereafter

+

C48Cardiology and Cardiac Surgery Toronto Notes 2023

Hypertrophic Cardiomyopathy

•see 2020 American Heart Association (AHA)/American College of Cardiology (ACC) Guideline for the

Diagnosis and Treatment of Patients With Hypertrophic Cardiomyopathy for details

Definition

•unexplained left ventricular hypertrophy (LVH)

•LVH can occur in any distribution

asymmetric septal hypertrophy is most common

•systolic anterior motion of mitral valve and hyperdynamic LV are common but non-diagnostic

Etiology

•cause is felt to be a genetic defect involving one of the cardiac sarcomeric proteins

>400 mutations associated with autosomal dominant inheritance, incomplete penetrance,

variable age of onset

• 70% of pathogenic variants occur within 2 genes: beta myosin heavy chain 7 (MV H7) and

myosin-binding protein C3 (MYBPC3)

•prevalence of 1 in 500 to 1 in 1000 in general population

equally prevalent in men and women although women are diagnosed less often

•generally presents in early adulthood

Pathophysiology

•histopathologic featuresinclude myocyte disarray, myocyte hypertrophy,dysplastic

interstitial fibrosis

•dynamic obstruction of LVOT (LVOTO) due to both septal hypertrophy and systolic anterior motion

(SAM)of mitral leaflets

•diastolic dysfunction due to LVH, ischemia, and interstitial fibrosis

•myocardial ischemia due to supply-demand mismatch

•autonomic dysfunction inappropriate vasodilation during exercise and abnormal HR recovery

arterioles and

Prior event

(SCD. VF.

sustained VT)

Yes An ICD is

» recommended

HI Hemodynamic Classification

•HOCM (hypertrophic obstructive cardiomyopathy):defined as peak LVOT gradient of at least 30

mmHg either at rest or with provocation

peak LVOT gradient of at least 50 mmHg at rest or provoked isthe typical threshold for

considering invasive septal reduction in patients with insufficient response to medical

management

•non-obstructive HCM (one-third): no LVOT obstruction

TNO

At least one of

the following:

• FHSCD*

• Massive LVH*

• Unexplained

Syncope*

• Apical

aneurysm

• EF 550%

An ICO is

reasonable

Yes

(2a)

Signs and Symptoms

•clinical manifestations:asymptomatic (common, therefore screening is important), SOBOE,angina,

presyncope/syncope (due to LV outflow obstruction or arrhythmia),CHI'

, arrhythmias,SCD

•pulses:rapid upstroke, “spike and dome"

pattern in carotid pulse (in HCM with outflow tract

obstruction)

•precordial palpation:PM1 localized,sustained, double impulse,‘

triple ripple’(triple apical impulse in

HOCM), LV lift

•precordial auscultation: normal or paradoxically split S2, SI, harsh systolic diamond-shaped murmur

at LLSB or apex, enhanced by squat to standing or Valsalva (murmursecondary to LVOTO as

compared to AS); often with pansystolic murmur due to MR

Investigations

•3-generation family history

first

-degree relatives receive directed cascade genetic testing and routineTTE and ECG screening

first-degree relatives are screened every 1-3 yr as children and every 3-5 yr as adults provided they

are asymptomatic and initial assessment is negative

•TTE for initial diagnosis, monitoring every 1-2 yr and evaluating clinical concerns

for patients not meeting LVOTO criteria (LVOT'

gradient of at least 50mmHg) at rest, a

provocative maneuver and/or exercise stress test is performed to assess for dynamic LVOTO

development

•TEE for preoperative planning ofseptal reduction, assessment of MR etiology,SAM and LVOTO

•cardiac MR1 to clarify inconclusive echocardiogram results or determine method ofseptal reduction

•ECG/holter monitor for initial workup, regular follow-up, and assessment of SCD risk

• LVH, high voltages across precordium, prominent Q waves (lead I, aVL, V5, V6), tall R wave in V1,

V wave abnormalities

•cardiac catheterization (only when patient being considered for invasive therapy)

•genetic studiesto clarify* uncertain diagnoses and facilitate screening of family members

I No ( Children ]

Yes f NSVT’

I )

No ( Adults! )

Extensive

LGE on CMR An ICD may be

considered

12b)

No

An ICD is not

indicated

(3:Harm)

* ICD decisions in pediatric patientswith HCM

arc based on >1of thesemajor risk factors:

familyhistory of HCM SCD,NSVT on

ambulatorymonitor,massive LVH,and

unexplained syncope

tInpatients>l6yrof age,5-yr risk estimates

canbe consideredtofullyinform patients

during shared decision-making discussions

tItwould seem most appropriate toplace

greater weightonfrequent longer,and faster

runs of NSVT

CMR - cardiovascular magnetic resonance;

EF= ejection fraction;HCM= hypertrophic

cardiomyopathy.ICD = implantable

cardioverter-defibrillator;LGE = late gadolinium

enhancement;LVH= left ventricular

yNSVT = nonsustained

'

hypertrophy

tachycardia;SCO = sudden cardiac death

rcntncular

Figure 44. ICDimplantation in HCM +

019Cardiology and Cardiac Surgery Toronto Notes 2023

Management

• avoid factors which increase obstruction (e.g. volume depletion)

avoidance of high-intensitv competitive sports unless exceptional circumstances

mild-to-moderate-intensity exercise issafe

• treatment of HOCM

• medical agents: p-blockers, verapamil or diltiazem (started only in monitored settings),

disopyramide, phenylephrine (in setting of cardiogenic shock)

avoid digoxin and vasodilators (e.g. nitrates,dihydropyridine calcium channel blockers, and

ACEl/ARB) as they are inotropic and afterload reducing, respectively

• patients with HOCM and drug-refractory symptoms require septal reduction therapy at experienced

centres

surgical myectomy

• alcohol septal ablation - percutaneous intervention that ablates the hypertrophic septum with

100% ethanol via the septal artery

dual chamber pacing (rarely done)

• treatment of non -obstructive HCM

symptomatic: p-blockers or non-dihydropyridine calcium channel blockers and diuretics if

refractory symptoms

• comorbid atrial fibrillation:direct oral anticoagulant or warfarin regardless of CHA2DS2-VASc score

• consequent systolic dysfunction: consider candidacy for transplant

• treatment of patients at high-risk of sudden death: ICD (see Figure 44,ICI)implantation in HCM,C48)

history ofsurvived cardiac arrest/sustained VT

1

'

MHx of premature sudden death

other factors associated with increased risk of SCD

syncope (presumed to be arrhythmic in origin)

LVET <50%

LV apical aneurysm

non-sustained VT on ambulatory monitoring

marked LVH (maximum wall thickness >30 mm)

Prognosis

• life expectancy may or may not be reduced

the majority of those with HCM do not experience severe symptoms or require aggressive

treatments

• potential complications:ATib,stroke,CHF(diastolic and systolic), VT,SCD (1% risk/yr; most common

cause of SCD in young athletes)

Mavacanten far treatment af Symptamallt

Obstructive Hypertrophic Cardiamyopattiy

(EXPLORER HCM ): A Randomized, Double Blind ,

Placebo-Cantrelled, Phase 3Trial

The Lancet 2020Sep12;39S|10253):759 69.

Purpose Assess the safety and efficacy af

mavacamten, a cardiac myosin inhibitor,in

symptomatic HOCM.

Methods: Patients with HOCM (llf0I»50mmHj, NTHA

fill)from (8clinical centersin t3 countries were

ra nd enured to ntavacamten or placebo for 30 wk.

the pemaryendpointwasa '

I.SmL/trq/min increase

In p«k 0r consumption andal least orreHYHt class

reduction,or >3.0mUkf'

mm Increase in peak Oz

consumption with no NTHA class reduction.

Results:4S|37%) of 123 patientson mavacamten is.

22 (12%) of 128 on placebo met the primary endpoint.

Patients on maiacamten had greater reductions In

post-eiercise LVOT gradient and greater increase

in peak Oz consumption.34% more patients in the

mavacamten group improved by at least1NYHA class.

Safety and tolerability werecomparable to placebo.

Conclnsion:Mavacamten improved exercise

capacity.LVOT obstruction, NYHA functional class,

and health statusin patients with HOCM.

Restrictive Cardiomyopathy

Definition

• impaired ventricular filling with preserved systolic function in a non-dilalcd, non-hypertrophied

ventricle secondary to factors that decrease myocardial compliance (fibrosis and/or infiltration)

• biatrial enlargement is often present despite normal z\V valve functioning

Etiology

• most commonly:amyloidosis,sarcoidosis, and hemochromatosis

• infiltrative: amyloidosis,sarcoidosis

• non-infiltrative:scleroderma, idiopathic myocardial fibrosis, diabetic cardiomyopathy

• storage diseases: hemochromatosis, Fabry’s disease,Gaucher'

s disease, glycogen storage diseases

• endomyocardial

endomyocardial fibrosis (late presentation), Loefiler's endocarditis, or eosinophilic

endomyocardial disease

radiation heart disease

carcinoid syndrome (may have associated tricuspid valve or pulmonary valve dysfunction)

RCM vs. Constrictive Pericarditis

Present similarly but constrictive

pericarditis is treatable with surgery

Clinical Manifestations

• CHI'

(usually with preserved LV systolic function), arrhythmias

• elevated )VP with prominent x and y descents, Kussmaul'

s sign

. S3,S4, MR, TR

• thromboembolic events

RCM Constrictive

Pericarditis

. . •

iltry

• no putsusparadoxus

• systolic murmurs

. LVH

. y r i .:

1

in somecases

• pulsus paradoxus Investigations

• EGG:low voltage, non-specific,diffuse ST-T wave changes ± non-ischemic Q waves

• CXR:mild cardiomegalv due to biatrial enlargement

• echo: LAE, R AE;specific Doppler findings with no significant respiratory variation

• cardiac MRI: assessment of myocardial fibrosis, determination of etiology and exclusion of

constrictive pericarditis

• cardiac catheterization:increased end-diastolic ventricular pressures

• endomyocardial biopsy: to determine etiology (especially for infiltrative RCM)

may be present

• . : :-

'

'

:d

'

J

_ . -Jf i-y: :

(ntiacardiac . no LVH

• pericardial

calcification

and per.catdral

pathology)

. urjo- and

endocardial

later gadolinium thickening

enhancement (LGE) • pericardial late

gadolmiom

enhancement (LGE)

. reduced BNP

+

. elevated BNP

CSO Cardiology and Cardiac Surgery Toronto Notes 2023

Management

• exclude constrictive pericarditis

• control HR, anticoagulate if AFib

• treat underlying disease:(e.g. cardiac amyloidosis, cardiac sarcoidosis, hemochromatosis)

• supportive care and treatment for CHF, arrhythmias, and prevention of SCI) when indicated

• judicious use of diuretics(excess volume reduction reducesfilling pressures versus pathologic

requirements triggering hypoperfusion)

• cardiac transplant: might be considered for CHF refractory to medical therapy

Key Investigations

• Echo:may show respiratory

variation in Wood (low in constrictive

pericarditis

. CT:may show very thickened

pericardium and calcification in

constrictive pericarditis

. MRI:best modality to directly

visualize pericardium and

myocardium

Prognosis

• depends on etiology

Left Ventricular Noncompaction Cardiomyopathy

Definition

• failure of LV compaction leading to endomyocardial trabeculationsthat increase in number and

prominence

• characterized by abnormal trabeculations in the LV, most frequently at the apex

Etiology

• genetics are incompletely understood

• mutations have been mainly observed in genes coding sarcomeric, cytoskeletal and mitochondrial

proteins

• can occur in healthy individuals (e.g. athletes and pregnancy) as well as concomitantly with

genital heart diseases and other cardiomyopathies(i.e. HCM, RCM, DCM,ARVC)

be reversible

con

• can

Clinical Manifestations

• if occurring in absence of concomitant cardiomyopathy and congenital heart disease, LV noncompaction can be benign

• symptoms range from SOBOL to rest symptoms

• many patients are asymptomatic

• ventricular arrhythmias or complete AV block (presents assyncope and sudden death)

• thromboembolic events

• more likely when systolic dysfunction and LV dilatation are present

Investigations

• directed by primary pathology when LV non-compaction is comorbid with congenital disease or other

cardiomyopathies

• TTL and cardiac MRI

• most common diagnostic method is the ratio of the thickness of the non-compacted layer to that

of the compacted layer (greater than 2:1 at the end of diastole)

• role of routine genetic screening remainsin question

typically performed in the setting of LV non-compaction with comorbid cardiomyopathy

Management

• at-risk first-degree relatives are recommended to undergo screening

• therapy islargely driven by concomitant myocardial dysfunction, arrhythmias, and congenital heart

disease

• ICD is an option if patients have syncope or documented VT

• antiplatelets or systemic anticoagulation should be considered in adults, especially when the LV or

atria are dilated

Prognosis

• dependent on LV function and presence of comorbid conditions(e.g. congenital heart disease and

cardiomyopathy)

Cardiac Transplantation

• treatment for end-stage heart failure

•median survival is 12 yr

•matching is according to blood type, body size and weight (should be within 25%), HLA tissue

matching, and geographical considerations(to minimize ischemic time)

Indications for Surgery

•severe cardiac disability despite maximal medical therapy (e.g.recurrent hospitalizationsfor CHF,

NYHA 111 or IV, peak metabolic oxygen consumption <14 mL/kg/min in absence of p-blocker) with a

life expectancy of 12-18 mo

•symptomatic cardiac ischemia refractory to conventional treatment (e.g. unstable angina not

amenable to CABG or PCI with LVEF <20-25%;recurrent,symptomatic ventricular arrhythmias)

+

C51Cardiology and CardiacSurgery Toronto Notes 2023

•high-risk HFSS

HFSS is an algorithm that incorporates the patients HR,serum sodium, ischemic

cardiomyopathy, LVEF, peak myocardial oxygen consumption, MAP, interventricular conduction

delay

• patients with medium-risk (HFSS 7.2-8.1, 73% l-yr survival) and high-risk (HFSS <7.2, 43% 1-yr

survival) benefit from cardiac transplant

•cardiogenic shock requiring IV inotropic agents or mechanical circulatory support to sustain organ

perfusion

•exclusion of all surgical alternatives to cardiac transplantation

Eflttls ol Donor Pro-treatment with Oopamine

on Survival oiler Heart Transplantation:tCohort

Study ol Heart Transplant Recipients Nested in a

Randomized Controlled Hulticentre Trial

JAta Coll Cardiol 201058:1768-1777

Treatment ol brain-dead donorswith dopamine of

4 iigfkgta will not harm raider allografts but

appearsto improve the limital tourse of the heart

allograft retipieat.

Absolute Contraindications

•active alcohol use disorder orsubstance use disorder

•actively smoking

•coagulopathy

•incurable malignancy

•irreversible major organ disease

•irreversible pulmonary HTN (i.e. >5 Wood units, transpulmonary gradient <18 mmHg,or systolic

pulmonary artery pressure >60 mmHG)

•majorsystemic illness

•mental illness or other cognitive factorslikely to affect ability to adhere to post-transplant regimens

•repeated non-adherence to medications

•severe COED (i.e. I EVI <1L)

•severe symptomatic cerebrovascular disease

Relative Contraindications

•active systemic infection

•acute PUD

•age >70 yr

•DM with end-organ disease

•lack of family/social support

•obesity (>35 kg/m2)

•significantsymptomatic carotid disease or PVD

Long-Term Use of a LeftVentrlrular Assist Device

for End-Stage Heart Failnre

KJH 2001:345:1435-1443

Intleased survival of 23% vs.i% with WAD vs.

medical management of Hf after 2 yr.

Heartmate VAD has a biologic surface and.therefore,

does not require long-term anticoagulation but

confers a higher risk of infection.

Canadian Cardiovascular Society Focused

Position Statement Update on Assessment

<f the Cardiac Patient lor Fitness to Orhre:

Fitnessfollowing left Ventricular Assist Device

Implantation

Can J Cardiol 2U12;2fct37-U0

Patients with a continuonsflow LVAD (NYHAclassl-lll)

whoare stable 2 mo post-LVAD implantation qualify

foe private driving My)and

*'*

disqualified from

commercial driving.

Prerequisites

•psychosocialstability

•medically compliant and motivated

Canadian Cardiovascular Society!Canadian

Cardiac Transplant network Position Statement

ordfeart Transplantation:Patient Eligibility,

Selection, and Post-Transplantation Care

CanJCardiol 2020;36:33S 56

Selection Criteria: Cardiac transplantation is

recommended for consideration in HF patients <70 yr

old.For all patients being considered,an assessment

of frailty with a validated tool is recommended.

Caution isrecommended for patients with BUI >3S.

WAD rmplantalron Is recommended lot eligible

patents with pulmonary hypertension on right heart

catheterization.Finally,cerdiactransplantation is

not recommended for patientswho show repeated

aooadheience to medications, alcohol orilicit drug

use. menial Illness, and/or cognitive concerns that

will tender patients mlAely toadliere to posttransplantation regimens.

Complications

•rejection

declining incidence with improved post-transplant immunosuppression regimens:<13%

experience an episode that needs to be treated and <5% have serious hemodynamic compromise

• gold standard to detect rejection: endomyocardial biopsy

• risk of acute rejection is greatest during the first 3 mo after transplant

• hyperacute rejection (minutes to hours after transplant) due to ABO mismatch, acute rejection

(days to months after transplant),or chronic rejection (years after transplant)

•infection

leading cause of morbidity and mortality after cardiac transplantation

risk peaks early during the first few months after transplantation and then declines to a low

persistent rate

•allograft vasculopathy

approximately 50% develop graft vasculopathy within 10 yr of transplantation

most common cause of late death following transplantation

•malignancy

developsin 15% of cardiac transplant recipients due to immunosuppressive medication

second most common cause of late death following transplantation

• cutaneous neoplasms most common,followed by non-Hodgkin lymphoma and lung cancer

•medication side effects

immunosuppressives(e.g. prednisone, cyclosporine nephrotoxicity, tacrolimus) may have

nephrotoxic effects

•cardiac denervation

• as the donor heart is completely denervated, it does not receive parasympathetic vagal

stimulation or intrinsic postganglionic sympathetic stimulation so it will not respond to

anticholinergicslike atropine

•RV dysfunction

RV dysfunction with TR, particularly in patients with preoperative pulmonary HTN, due to

myocardial dysfunction caused by long ischemic time and/or reperfusion injury

requires aggressive management for treatment using agents that dilate the pulmonary

vasculature or, rarely, RVAD support

r -i

L J

+

C52 Cardiology and Cardiac Surgery Toronto Notes 2023

Ventricular Assist Devices

Advanced Heart Failure Ireatedwith ContinuousFlow Left Ventricular Assist Device

HEJM 2009:361:2241-51

Purpose: Assessquality of life in patients with

advanced HF treated with implanted pulsatile-flow

LVAD or new continuous-flow devices.

Methods: Patients with advanced medicallylefractory HF were randomutd|«i a 2:1ratio)

to implantation ol a continuous-flow LVAD or a

pjlsat lie-flow LVAD.Quality-oMife tests and 6-rain

walk test data were collected at haselme.1 month,

3mo.6 mo.thto every 6 mo untilstudy completion.

The primary endpoint wasa composite of 2-yrr survival

free of disabling stroke (Rankin score >3),or device

cooperation for replacement.

Results:86% of patients with the continuous-flow

device and 76% of patientswith the pulsatile-flow

device were discharged from the hospitalwith the

device in- place,the primary endpoint was achieved in

46% of patients implanted with the continuous-flow

device, compared to11% with the pulsatile-flow

device (hataid ratio 0.38;35% Cl 0.27 to 0.54;

P<0.001).fhe Kaplan- Meiec esbmatesof survival

revealed significantly better outcomesfor patients

with the cpntiooous

-Howderices compared with the

pulsatile-flow device (RR 0.54;95% Cl 0.34 to0.86:

M.008).

Conclusions:Implantation of a continuous-flow

device, compared toa pulsate- flow deice improved

strohe-lree survival and gualty ol life in patients with

advanced medically-refractory HF.

• work to unload the ventricle while maintaining output; also results in decreased myocardial oxygen

consumption permitting recovery of the myocardium that is not irreversibly injured

• can support the left (LVAD), right (RVAD), or both ventricles (BiVAD); typical circuit is comprised of

a pump, an outflow graft, and a driveline to connect to an external power source and controller (see

Figure -75)

• indications;

• bridge to transplantation, bridge to decision (for transplant), or long term permanent therapy

(“destination therapy")

postoperative mechanical support when unable to separate from CPB despite inotropic and IABP

support

IABP is a catheter-based device inserted into the femoral artery and advanced to the

descending aorta that decreases afterload, thus myocardial O: demand and increases blood

flow to coronary arteries

inflation of the balloon occurs during diastole to increase ascending aorta and coronary

artery perfusion pressure;deflation occurs at systole to reduce intra-aortic pressure thus

reducing afterload

cardiogenic shock

Extracorporeal Membrane Oxygenation

• circuit includes:centrifugal pump, membrane oxygenator, venous and arterial cannulas(see figure

46)

• venoatrial (VA) HCMO is treatment for cardiogenic shock due to broad availability, technical

simplicity, and rapid deployment

• outcomesfor HCMO poor with 35% survival

• indications: postcardiotomy shock, allograft failure, fulminant myocarditis, decompensated HP

• extracorporeal life support through HCMO use is an effective method of resuscitation in moribund

patients 11

>

OCO rPressure Heat exchanger y

Monitor I Aorta

-

Battery

Warm water -

? Accesssite pap1. L

Percutaneous

driveline

Membrane

Oxygenator II

External controller

Remrusion

cannula

Oxygen

Blender HeartMato III

• Femoral

OCO pressure

Monitor

vein Outflow

J Access site Drainage

cannula

Femoral

artery

Figure 46 Intrapericardial pump . ECMO

Cardiac Tumours HeartMate II

Incidence

• cardiac tumours are more commonly derived from metastases than primary tumours

• primary cardiac tumours have an estimated incidence of 1-30 in 100000 people per year

metastatic involvement of the heart is much more common and is present in 10-20% of autopsies

of patients who die from cancer

Outflow

graft

Inflow r T

p <nr Jls LJ

Diagnosis Pump

• TTH,TEH,MRI, PET and/or CTscan can be used to detect cardiac tumours

• transvenous and open biopsy offer definitive diagnosis when required

• once delected,CT or PET scansscreen for distant metastasis while cardiac MRI helps determine

suitability forsurgery

• coronary angiography determines presence of concomitant CAD and neoplastic involvement of

coronary vasculature

Figure 45. LVAD

+

C53Cardiology and Cardiac Surgery Toronto Notes 2023

Physiological Consequences of Cardiac Tumours

• systemic or pulmonic embolization

• symptoms of HI'

due to obstruction of circulation

• regurgitation due to interference with heart valves

• myocardial invasion causing impaired left ventricular function, arrhythmias, heart block, or

pericardial effusion

• constitutional or systemic symptoms

Subtypes of Cardiac Tumours by Location

• right atrial tumours

• may obstruct blood flow and present with symptomssimilar to those of TS and right HI-

'

• fragmentsfrom right atrial tumours may cause pulmonary emboli

• tumours affecting the AV node can cause heart block

myxomas are one of the most common

• right ventricular tumours

• can induce right-sided HF by interfering with filling or outflow from the KV

• may obstruct blood flow or beget I K and, as a result,simulate mitral valve disease and produce

HF orsecondary pulmonary HTN

• left atria] tumours

• may release tumour fragments or thrombi into the systemic circulation

myxomas are one of the most common

• left ventricular tumours

• intramural left ventricular tumours may induce arrhythmias or conduction defects

• intracavitary tumours can present with systemic embolization or outflow obstruction

may ultimately result in left ventricular failure

• valvular tumours

• papillary fibroelastomas are most common, equal incidence at AV and semilunar valves

asymptomatic untilsentinel eventssuch as distal embolization and coronary ostial obstruction

resection and repair of the valvular tissue is preferred over valve replacement

• pericardial tumours

• includes lipomas and metastatic tumours

• external compression of the heart as a result of both mass effect and propensity to generate

pericardial effusions

Subtypes of Cardiac Tumours by Histopathology

• benign tumours

• roughly 75% of cardiac tumours are benign

• myxomas make up the majority of benign cardiac tumours and they most commonly arise in the

LA

in patients over age 16, the three most common primary tumours are myxomas(50%), lipomatous

tumours (21%), and papillary fibroelastomas (16%)

in patients under age 16, the four most common tumours are rhabdomyomas (55%), teratomas

(16%), fibromas (10%),and myxomas(10%)

myxomasshould be surgically resected to minimize the risk of cardiovascular complications,

including embolization

• primary malignant cardiac tumours

sarcomas are the most common form of primary malignant cardiac tumours(75%)

these tumours progress rapidly and can infiltrate the myocardium, obstruct circulation, and

release metastatic cells

prognosis dictated by anatomic location as opposed to histopathology

right-sided tumours are more invasive and metastasize earlier than left-sided ones

• although the recommended treatment strategy is surgical resection when possible, these tumours

are likely to recur

a combination of chemotherapy and surgical resection for primary cardiac sarcomas prolongs

survival as compared with eithersurgery or chemotherapy alone

• metastatic involvement of the heart

metastatic cancer cells may reach the heart through hematogenousspread, direct invasion,or

tumour growth through the venae cavae into the RA

incidence is highest in external layers of heart and reduced toward luminal layers, reflecting

seeding through the coronary arteries and direct extension of adjacent thoracic tumours

when a cancer patient develops cardiovascularsymptoms,cardiac or pericardial metastases

should be suspected

• although most metastases are asymptomatic, the most common symptom is pericardial effusion

with or without tamponade

+

C.51Cardiology and Cardiac Surgery Toronto Notes 2023

Valvular Heart Disease

• see the 2020 American College of Cardiology (ACC)/American Heart Association (AHA) Guideline for

the Management of Patients with Valvular Heart disease and the 2017 AHA/ACC focused Update of

the 2014 AHA/ACC Guidelines for the Management of Patients with Valvular Heart disease for details

Infective Endocarditis

• see Infectious Diseases.11)15

• American Heart Association (AHA ) 2007 guidelines recommend Hi prophylaxis

only for patients with:

prosthetic valve material

past history of IE

cyanotic CHD

cardiac transplant recipients who develop valvulopathy

« only for the following procedures:

dental

respiratory tract

procedures on infected skin/skin structures/MSK structures

not Gl/GU proceduresspecifically

Rheumatic Fever

• see Paediatrics. P65

Prognosis

• acute complications:myocarditis(DCM/CHP), conduction abnormalities (sinus tachycardia, AFib),

valvulitis(acute MR), acute pericarditis(not constrictive pericarditis)

• chronic complications: rheumatic valvular heart disease fibrous thickening, adhesion, calcification of

valve leaflets resulting in stenosis/regurgitation, increased risk of IE ± thromboembolism

• onset ofsymptoms usually after 10-20 yr latency from acute carditis of rheumatic fever

• mitral valve most commonly affected

Twenty-Year Outcome After Mitral Repair Versus

Replacement lor Severe Degenerative Mitral

R egurgitation: Analysis of a large.Prospective,

Multicenter. International Registry

Circulation 2011;135:410-22

Purpose: Analyze very-long term outcomesafter MY

repair and replacement lor degenerative MR with a

Rail leaflet.

Methods: Employing the Mitral Regurgitation

International Database,outcomes after MV repair

and replacement were anatyred by propensity score

matching and by inverse piobability of treatment

weighting.

Results: Operative mortality was lower after MV

repair than replacement in the propensity-matched

population (0.2% vs.4.4%; P- 0.001|and 20-y r

survival was better after MV repair than rep'acement

<i the same popu lahon (41% vs. 24%; P <0.001|. MV

repair wasalso associated with a red.ced rate ot

vatrular complications.

Conclusions: MV repair was associated with lower

operative mortality,better 20-yr survival and lower

complication rates than MV replacement, in patients

with degenerative mitral regurgitation with a flail

leaflet.

Valve Repair and Valve Replacement

• indication for valve surgery depends on the severity of the pathology:typically recommended when

medical management has failed to adequately clear the infection or improve symptoms

• surgical valve repair:surgical valvuloplasty (commissurotomy, annuloplasty), chordae tendineae

repair, tissue patch

• surgical valve replacement: typically for aortic or mitral valves only; mitral valve repair is favoured in

younger individuals(and patients with MVP with severe MR)

• surgical decision between mechanical vs. bioprosthetic prosthesisfor patients 50-70 v/o remains

uncertain as valve techniques evolve

Choice of Valve Prosthesis

Table 17. Mechanical Valve vs. Bioprosthetic Valve vs. Pulmonary Autograft-Ross Procedure

Mechanical Valve Bioprosthetic Valve Pulmonary Autograft in Aortic

Position (Ross Procedure')

Good durability Limited long term durability (mitiafaorlic) Only aortic valve replacement that restores

life expectancy to the age- and sex-matched

general population

Closest flow profile lo native aortic roots

low risk of thromboembolism

less preferred in small aorlic root sites

Increased risk ol thromboembolism (1-3%/

yr):requires long-term anticoagulation with

coumadin

Good flow in small aorlic root sires

Decreased risk olthromboembolism: longterm anticoagulation notneeded for aortic

valves

Target INR - aortic valves: 2 0 3.0 (mean 2.5); Some recommendation lor limited

anticoagulation for mitral valves

Decreased risk of hemorrhage

>65 yr tor both aorlic and mitral valves

No anticoagulation required, enables higher

activity and straightforward pregnancies

I Lw risk of hemorrhage

Classically in children and young adults <50 yr

mitral valves: 2.S-3.5 (mean 3.0) r

LJ