Unit 3

what is cardiomyopathy

disorders in which the hear muscle is structurally and functionally abnormal caused by cardiovascular dx such as HTN, ischemic heart disease, or valvular dx can be genetic, acquired or mixed two types

primary: the dx process is confined to the heart

secondary: condition in which cardiac involvement occurs as part of systemic condition

and out of these two types they can dilated, restrictive, and hypertrophic

restrictive cardiomyopathy

a rare form of cardiomyopathy

heart muscle is stiff (decrease compliance) not thicken causing impaired ventricle filing but systolic function is preserved early on marked by biatrial enlargment from chronically elevated filling pressure

what is the EF for restrictive cardiomyopathy

normal or mild reduce

causes of restrictive cardiomyopathy

SHAPE

S- sarcodosis

H-hemochromatosis

A- amyloidosis

P post radiation fibrosis

E- endomycardial fibrosis

ventricles can not relax that cause diastolic failure and blood backs up and causes venous congestion and decrease cardiac output

how does amyloidosis cause RCM

deposition of amyliod causes stiff non compliance

how does sarcoidosis cause RCM

granulomatous infiltrates of the myocardium

Loeffler endocarditis

eosinophilic infiltrates of the endocardium/myocardium

how does Hemochromatosis case RCM

Iron deposition in the myocardium

how does endomyocardial fibrosis cause RCM

– Idiopathic fibrotic replacement of the endocardium

how does carcinoid cause RCM

•Fibrotic involvement of the heart

what is amyloidosis

Infiltrative disorder with deposition of proteins (amyloid fibrils) in the extracellular tissues (heart, kidney, liver, nerves, bone marrow, fat, skin•

ATTR is now the most common cause of cardiac amyloidosis and is increasingly recognized in older adults with HFpEF and unexplained LV wall thickening.

sx of RCM

progressive HF

fatigue

SOB

JVP

acites

hepatomegaly

decrease exercise tolerance

peripheral edema

s3/s4 gallop, stroke, angina, palpitation, syncope

could have weight loss, macroglossia (ENLARGED tongue) periorbital purpura, peripheral neuropathy

dx RCM what is gold standard

endomyocardial biopsy only when echo and MRI are inconclusive

echo findings of rcm

•Biatrial enlargement

•Normal or mildly increased ventricular wall thickness

•Normal or small ventricular cavities

•Preserved or mildly reduced LVEF

•Severe diastolic dysfunction

•Elevated filling pressures

what imaging can identify the disease

cardiac MRI Identifies infiltrative disease (especially amyloidosis or sarcoidosis)

RCM tx

•Treat the underlying cause

•Gentle loop diuretics for congestion (avoid over diuresis—patients depend on adequate filling pressures)- with caution fine line of decreasing sx and worsening CO

•Maintain sinus rhythm whenever possible

•Anticoagulate if atrial fibrillation (all cardiac amyloidosis patients with AF require anticoagulation)

•Etiology-specific therapy

•Amyloidosis: Disease-specific therapy

•Sarcoidosis/Loeffler's: Corticosteroids

•Refer all patients to cardiology for evaluation and management

hypertrophic cardiomyopathy

HCOM genetic disorder of the heart muscle and asymmetric thickening of the LV wall

think when there is LVH with no other cause

increase risk of sudden cardiac death

usually presents in early adulthood

patho of HCOM

•HCM is a clinically caused by various mutations associated with hypertrophy of the LV.

oLV outflow obstruction - 2/3 of cases 

oDiastolic dysfunction

oMyocardial ischemia

oMitral regurgitation

•The LV volume is average or reduced in HCM, and diastolic dysfunction is usually present. Thick, stiff, less complaint ventricle.

•Asymmetric interventricular septum growth in most

•Also predisposed to arrhythmias. 

•Most common cause of  SCD in athletes

sx of HOCOM

•Frequently asymptomatic. Cardiac arrest can be the first presentation

•Exertional Chest Pain

•Syncope

•Dyspnea

•Palpitations

•Arrhythmias

•Sudden death à In children with exertional cardiology complaints- must consider HCM

sustained PMI and s4 gallop, harsh crecendo decrescendo systolic murmur at left sternal border

may have bifid carotid carotid pulse feeling like it has two peaks

HCOM dx

•ECG

•Left ventricular hypertrophy (LVH), deep, narrow ("dagger-like") Q waves may be present

•Atrial fibrillation or ventricular arrhythmias may occur

•Echocardiogram (first-line test)

•LV wall thickness ≥15

•Asymmetric septal hypertrophy (most common)

•Systolic anterior motion of the mitral valve

•Dynamic LV outflow tract obstruction

•Diastolic dysfunction

•Cardiac MRI

•Defines myocardial hypertrophy and fibrosis

•Used when echo is inconclusive or for risk stratification

•Additional Evaluation

•Ambulatory ECG (Holter) to detect arrhythmias

•Exercise stress testing to assess LVOT obstruction, blood pressure response, and exercise tolerance

HOCOM tx

•Beta blockers - metoprolol, atenolol, propranolol (first-line)

•Verapamil or diltiazem if beta-blocker is not tolerated

•Avoid digoxin (may worsen LVOT obstruction)

•Maintain sinus rhythm and anticoagulate atrial fibrillation

•Regular exercise is encouraged

•Shared decision-making for vigorous or competitive sports

•ICD for patients at high risk of sudden cardiac death

•Septal reduction therapy (surgical myectomy or alcohol septal ablation) for persistent severe symptoms

•Refer to cardiology/HCM center and screen first-degree relatives

HCOM surgerical options

•Septal Reduction Therapy

•Persistent severe symptoms despite optimal medical therapy

•Surgical septal myectomy -gold standard

•Removes a portion of the hypertrophied interventricular septum

•Alcohol septal ablation

•Injects alcohol into a septal branch of the left anterior descending (LAD) artery to create a controlled infarction and reduce LVOT obstruction

•Heart transplantation

•Rare; end-stage HCM with refractory heart failure

When to Refer

•Refer all patients with HCM to cardiology

what is the MC form of cardiomayopathy

dilated cardiomyopathy - left ventricular or biventricular dilation

when does DCM typically occur age wise

20-60 but can happen any age

DCM impairs what

systolic function, will present with reduced HFrEF less than or equal to 40%

what race is DCM more common in

black pt and males

how does DCM present

with heart failure sx

DCM means there is an increase risk of

atrial and ventricular arrhythmias including sudden cardiac death

what is the MC cause of DCM

idiopathic

what is the MC cause of acquired DCM

ischemic heart disease like post MI, remodeling, chronic CAD

what is the mc cause of infectious DCM

viral myocarditis

what are the other causes of DCM

• toxic like drugs and alc

• stress induced

• endocrine like DM or thyroid

• tachycardia induced cardiomyopathy

• infiltrative/ inflammatory: amyloid and sarcoidosis

• peripartum

• nutritional deficiency: thiamine wet beriberi

hive, iron overload, end stage kidney disease

what is the patho for DCM

myocyte injury causes cell death eccentric remodeling of the heart that caused chamber dilation and thinning, causes a problem with systolic and therefore there will be less contractability and less EF meaning less CO that causes increase in neurohormonal activation of RAAS and SNS that causes progressive HF, functional MR and ventricular arrhythmias - OVERALL: weak dilated heart muscle

sx of DCM

• progressive SOB

• fatigue and weakness

• orthopnea and or paroxysmal nocturnal dyspnea (SOB that wakes them up) (thick more fluid return when you lay down so the heart has even more work to do w/o gravity

• peripheral edema in late

PE lungs for DCM

pulmonary rales

PE heart findings for DCM

• sinus tachy

• S3 gallop (rapid to slow filling seen in overload)

• functional holosystolic murmur of mitral and or tricuspid regurg (holosystolic cause s3 during filling and then regurg during diastolic)

• elevated JVP

• peripheral edema

• atrial or ventricular arrhythmias

initial eval for DCM

• BNP, CBC, CMP, TSH

• 12 lead EKG

  • chest x ray

what is used to confirm DCM diagnosis

eccho- can show LV dilation, reduce LVEF, MR/TR and global hypokinesis (means there entire heart is pumping less)

additional testing for DCM

• coronary angiography: to eval ischemic cardiomyopathy (also gold standard for coronary artery disease

• right heart cath: hemodynamic assessment

• cardiac MRI: myocarditis, infiltrative cardiomyopathy, and myocardial scar/ fibrosis

tx for DCM

• beta blocker: carvedilol, metoprolol, bisoprolol

• MRA: spironolactone or eplerenone

• ARNI: sacubitril/ valsartan

• SGLT2: dapagliflozin or empagliflozin

= guideline-directed medical th - GDMT the four pillars initiate them all and titrate to max tolerate dose this th decrease mortality

takotsubo syndrome

stress cardiomyopathy/ broken heart syndrome. Is defined as left ventricular transient systolic dysfunction. wall abnormalities extend beyond a single coronary artery territory

what can have an increase EF

HCM

atypical ballooning

stress cardiomyopathy

proposed patho for stress cardiomyopathy

• catecholamine surge

• coronary microvascular dysfunction

• coronary vasospasm

most cardiomyopathy occurs in

postmenopausal women

sx of stress cardiomyopathy

chest pain, st changes, elevated troponin

tx of stress cardiomyopathy

tx as ACS until obstructive coronary dx is excluded- most pt will recover LV function days to weeks later but can used temporary GDMT as LV dysfunction persist

New onset of HF, chest pain, or arrhythmia following a recent viral illness

myocarditis

what to do after stress cardiomyopathy

repeat echo to document recovery

dagger Q wave

HCM

tx for HCM

beta blocker is septal reduction is severe

RCM think the cause is

amyloidosis until proven otherwise

what is mycocarditis

rare inflammatory dx of cardiac muscle caused by both infectious and noninfectious conditions. can be acute, chronic, subacute. can lead to acquired cardiomyopathy with HF

infectious myocarditis etiology

• viral is MC from (enterovirus, parvovirus, HPV6, adenovirus, influenzas)

• chas disease (common in latin america ), borrelia burgdorferi- lyme

most viral follow flu like sx and is common after covid

autoimmune causes of myocarditis

• SLE

• RA

• IBD

drug induced causes of myocarditis

cocaine, lithium, chloroquine, hypersensitivity reactions

cancer th myocarditis

immune checkpoint inhibitors, chemo, radiation

systemic inflammatory dx that can cause myocarditis

• sarcoidosis

• giant cell myocarditis

• granulomatosis with polyangiitis

• celica dx

myocarditis sx

• SOB,

• chest pain (often pleuritic)

• fever

• palpitations, syncope, arrhythmias

PE for myocarditis

sinus tachycardia, conductional abnormalities or arrhythmias, heart failure

what is the preferred imaging for myocarditis

cardiac MRI

what is the gold standard for myocarditis

endomyocardial biopsy but only do when it wil change tx cause it is invasive

myocarditis tx

• no activity until inflammation has resolved and will be signed off by CARDS

• supportive care and tx underlaying cause

• aviod NSAIDS unless tx isolated pericarditis or normal LVEF

• Heart failure: the pillars as tolerated

• targeted therapy: like abx for infection

fulminant mycocarditis tx

mechanical circulatory support

when can athletes return with myocarditis

aviod strenuous activity for 3-6 months, resume activity only after cardiac evaluation confirms recovery including normal ventricular function, resolution of myocardial injury and inflammation and no significant arrhythmias

impella

is a percutaneous ventricular assist device is a temporary mechanical circulatory support for severe left ventricular failure. a catheter mounted pump that is inserted across the aortic valve and pumps the blood from the left ventricle into the ascending aorta

what are the benefits of impella

increase CO, unloads the left ventricle and improve ends organ perfusion

indications for impella

cardiogenic shock, high risk PCI, bridge therapy to recovery, durable LVAD, heart transplant

what does impella not do

provide o2 or right ventricular support

ECMO

stands for extracorporeal membrane oxygenation it pumps and oxygenated pt blood outside the body allowing the heart and lungs to rest there are two type 1. connected to both a vein and artery and 2. connected to one or more veins

primary cardiac tumors

are rare most are metastatic

primary cardiac tumors most common cause

benin that includes myxoma (MC), papillary fibroelastoma, lipoma, paraganglioma

malignant cardiac tumors

sarcomas

myxomas where most often found

benin and most often found in the left atrium discovered accidently

pregnancy does what to the heart

is like a stress test increasing the risk of HF, arrhythmias, and decompensation in women with underlaying heart conditions

tumor plop

myxomas can also cause a murmur that mimics MS

myxomas sx

depende on tumor size and location: obstruction can be SOB, syncope, HF - embolization: stroke or systemic emboli -constitutional sx: fatigue, fever, weight loss

tx of myxomas

cardiac MRI will give info for size, location, and attachment. tx is surgical excision to prevent obstruction or embolization

can myoxoma reoccur

rare but yes likely in familial like carney complex

what has the highest chance of metastatic to the heart

melanoma

metastatic tumors can cause

pericardial effusion, tamponade, arrhythmias, HF, systemic embolization

tx for metastic tumors

usually palliative and directed at the underlaying malignancy

In HF there is

• increase preload

• increase afterload

• decrease contractability

BP can look normal because the body is compensating for bad CO

HF is defined as

as a state in which the ventricle at normal filling pressure cannot maintain an adequate CO to meet the metabolic needs of peripheral tissue or can do so with only with an elevated filling pressure