1. Craig Ernst MHS, PA-C Lock Haven University
Cardiomyopathy
Craig Ernst MHS, PA-C
Lock Haven University

2. Cardiomyopathy
General term indicating disease of cardiac muscle resulting in abnormal function
Divided into three types:
Dilated cardiomyopathy-ventricular dilation
Hypertrophic cardiomyopathy-myocardial hypertrophy
Restrictive cardiomyopathy-impaired ventricular filling
Can have characteristics of more than one

3. Dilated Cardiomyopathy (DCM)
Characterized by dilation and impaired systolic function of left &/or right ventricle
Most common DCM is ischemic cardiomyopathy
Idiopathic (ICM) next most common
Familial autosomal dominant in 20% of cases.
Role of coxsackie/adenovirus and immune mediated etiology unknown.

4. DCM
Many cases of systemic heart muscle disease present with features of DCM including:
Ischemic/rheumatic CVD
Generalized disease- hemochromatosis, sarcoid
Connective tissue disease-SLE, systemic sclerosis
Neuromuscular disease-Friederich’s ataxia etc
Glycogen storage disease
Primary heart muscle disease- amyloidosis
Alcohol excess
Cytotoxic drugs-doxorubicin, cyclophosphamide
Pregnancy

7. Starling Curve

8. Starling Curve
Contractility
Volume

9. DCM Clinical features: R/L heart failure Arrhythmia Emboli
Cardiomegaly
Tachycardia
JVD
3rd/4th heart sounds
basiler crackles
displaced PMI

10. DCM Evaluation CXR: cardiomegaly
EKG: diffuse non specific ST-T wave changes, LBBB common, tachycardia, conduction abnormalities, arrhythmias
Echo: poor chamber contraction and dilated chambers
If CAD suspected, cardiac catheterization
Endomyocardial biopsy for research only.

12. DCM Treatment Sudden death – Rx failure & arrhythmias
Ace Inhibitors a must in failure management
Non-specific Beta blockade:
Carvedilol, ??, ??
Anticoagulation for A.fib/mural thrombus.
CRT-D (Bi-V AICD)
Transplant
Sudden death –
Due to V. Tach. or V. Fib
Failure: ACEI, sodium/fluid-restriction, diuretics, carvedilol(Coreg)
Arrythmias: electrophysiologist consult. DC proarrythmic drugs.

14. Hypertrophic Cardiomyopathy (HCM)
Complex heart disease due to the asymmetric left ventricular hypertrophy, left ventricular stiffness, mitral valve changes and cellular changes (myocardial disarray)
60 % inherited % sporadic
HTN, Aging, Unknown
Most autosomal dominant w/ variable penetrance

15. Other Terms Hypertrophic Obstructive Cardiomyopathy (HOCM)
Idiopathic hypertrophic subaortic stenosis (IHSS)
Asymmetrical septal hypertrophy (ASH)
Systolic anterior motion (SAM) of mitral apparatus

16. Pathophysiology HOCM is a subvalvular obstruction!
Distinct from valvular Aortic Stenosis (pressure gradient across valve)
Gradient/obstruction increases with lower LV volume
HOCM pts here do better when full and slow
Standing after squatting/Valvsalva
lower venous return & increase outflow obstruction and intensity of murmur

17. MV leaflet
HCM without obstruction
HCM with obstruction HOCM)

18. HOCM: Clinical Features:
Chest pain
Dyspnea
Syncope/Pre-syncope (typically with exertion)
Palpitations
Sudden Death (arrhythmia)
Typically occurs in asymptomatic young adults or adolescents (10-35 y/o)
Family history of sudden death, sustained ventricular tachycardia, & B/P response to exercise are recognized risk factors
Diastolic dysfunction with impaired filling
Outflow tract obstruction occurs in 1/3 of cases

19. Physical Exam
Systolic murmur; with little to no radiation to neck vessels (increased by maneuvers that decrease preload such as Valvsalva or squatting)
Decreases ventricular filling
May hear systolic murmur of MR
Weak late carotid pulse (late obstruction)
Diagnosed by echocardiogram.

20. Evaluation EKG-LVH with ST-T wave changes CXR-normal
ECHO indicated if PE suggests IHSS
Pedigree analysis (ECHO to screen 1st relatives)
Genetic analysis
XST/Holter

21. Treatment of HOCM Relief of symptoms, prevention of endocarditis, arrhythmias and sudden death
B-Blockers or verapamil
better filling, slow, bigger heart-less obstruction
Amiodarone or procainamide for A Fib
need atrial kick to adequately fill LV
Avoid afterload reducing agents and vasodilators (no ACEI/A2RB, NITRO)-refractory hypotension
These agents increase outflow obstruction
actually do better with increased SVR
Slow controlled emptying from increased SVR
SBE prophylaxis

22. Treatment of HOCM Implantable defibrillators
may be indicated if at risk for SCD
Dual-chamber pacemakers: reverse of resynchronization therapy for LVEF
Surgical: myotomy & myomectomy
Non surgical ablation of the septum (alcohol ablation through cath)

23. Sudden Cardiac Deaths 2-3 % per year Sudden & unexpected
Sudden death may be the initial (only) presentation
NPR link
Risk for SCD:
Extreme LVH
Family history of SCD
History of Vtach or syncope
Failure of BP to rise with exercise
Treatments that lower gradient do not prevent SCD

24. Restrictive Cardiomyopathy (RCM)
May cause systolic & diastolic dysfunction
All increase LV stiffness
Characteristic ventricle filling pressures
Over time filling dramatically ceases

25. Restrictive Cardiomyopathy
Some cardiomyopathies do not present with dilation or hypertrophy but rather restricted ventricular filling (as with pericarditis)
Amyloidosis
Sarcoidosis
Hemochromoatosis
Endomyocardial fibrosis
Atrial dilation, atrial fibrillation and clot formation common in restrictive

27. Restrictive Cardiomyopathy
Clinical Features
Dyspnea
Fatigue
Embolic phenomena
Elevated venous pressures
JVD
Hepatomegaly
Edema
Ascites

28. Restrictive Cardiomyopathy
CXR=cardiac enlargement
EKG-low voltage and ST-T wave abnormalities (Exaggerated Septal Q’s – Think MI)
Echo-symmetrical myocardial thickening
Endomyocardial biopsy may be useful.
Is it restrictive pericarditis?

29. Pre-op effusion
Post op effusion

30. Restrictive Cardiomyopathy
Treatment
No specific treatment
Treat underlying cause… results?
Those with amyloidosis may recur after transplant

31. Amyloidosis
Systemic disorder, but if DHF occurs usually from multiple myeloma
Fibrillar protein deposited throughout the myocardium leading to rubbery consistency and concentric hypertrophy
RV & LV hypertrophy
Absence of high voltage QRS on EKG despite LVH on ECHO
Appearance on Echo

33. Amyloidosis
Fat pad aspirate or tissue biopsies for systemic amyloidosis
Endomyocardial biopsy if questionable etiology
Poor prognosis

34. Hemochromatosis
Hereditary disorder characterized by excess dietary iron absorption and deposition in tissues with resulting end-organ damage.
Affects liver first and most frequently
Pancreatic involvement results in DM
Cardiac deposits leads to dilated cardiomyopathy
Skin deposits leads to bronze discoloration that results from increased melanin production. Hyperpigmentation
Remember: liver, pancreas, heart
Dx: AST, ALT, serum iron, TIBC, ferritin

35. http://www. mdconsult. com/das/pdxmd/body/107058015-3/757942852