An exhaustive list of conditions that can present with cardiomyopathy.

1. Cardiomyopathies
Frank W. Meissner, MD, FACP, FACC, FCCP
LtCol, USAF, MC, FS

2. Overview
●Dilated
●Restrictive
○Myocardial
■NONINFILTRATIVE (E.G. SCLERODERMA)
■INFILTRATIVE (E.G. AMYLOID, SARCOID)
■STORAGE (E.G., HEMOCHROMATOSIS)
○Endomyocardial
●Hypertrophic

3. Case #1
●35 y/o Black Male
●Recent Respiratory Illness
●(+) Cold Aggluttins
●Presented with dyspnea

4. Infectious Myocarditis- Essentials of
Dx
● Oten predated by resp infection
● Presentation
○ Chest pain (pleuritic or nonspecific)
○ CHF
○ Arrhythmias
● ECG
○ sinus tachycardia
○ nonspecific repolarization changes
○ intraventricular conduction abnormalities

5. Microbiology
●Coxsackieviruses most common
○Coxsackie A&B
●Rickettsial myocarditis
○Scrub typhus,
○Rocky Mountain spotted fever
○Q fever
●Diphtheritic myocarditis 2° toxin
○Conduction abnormalities + CHF
●Chagas Disease
○CHF + RBBB in Central or S. America

6. Bacterial Cardiomyopathy I
●Diphtheria
●Tuberculosis
●Typhoid Fever
●Psittacosis
●Brucellosis
●Chylamdia trachomatis
●Actinomycosis

7. Bacterial Cardiomyopathy II
●Tetanus
●Tularemia
●Meliodosis
●Legionaires Disease
●Mycoplasma

8. Bacterial Cardiomyopathy III
●Syphilis
●Leptospirosis
●Relapsing Fever
●Lyme Disease

9. Rickettsial Cardiomyopathy
●Typhus
●RMSF
●Q Fever

10. Fungal Cardiomyopathy
●Blastomycosis
●Candidiasis
●Aspergillosis
●Histoplasmosis
●Sporotrichosis
●Coccidoidomycosis
●Cryptococcosis
●Mucormycosis

11. Protozoal Cardiomyopathy
●Chagas Disease
●African Sleeping Sickness
●Toxoplasmosis
●Malariae
●Leishmaniasis
●Balantidiasis
●Sarcosporidosis
●Amebiasis

12. Helminthic Cardiomyopathy I
●Trichinosis
●Echinococcosis
●Schistosomiasis
●Ascariasis
●Heterophydiasis

13. Helminthic Cardiomyopathy II
●Filarisis
●Paragonimiasis
●Strongylodiasis
●Cysticercosis
●Visceral larva Migrans

14. HIV - A completely Seperate
Lecture!!!!!!

15. Diagnostic Studies
●Echocardiography convenient evaluation
●Ga-67 scintigraphy (+) acute / subacute
myocarditis
●Paired serum viral titers & serologic tests

16. Endomyocardial Biopsy
●Round cell inflammatory reaction
●Patchy necrosis
●"Active" inflammatory stage for mo’s

17. Treatment
●Specific therapy if infecting agent ID’ed
●Controlled steriod trials not positive
●Routine myocardial Bx uncertain utility
●NO Empiric immunosuppressive therapy
●Treat CHF & arrhythmias

18. Prognosis
●Many resolve spontaneously
●Cardiac function can progressively
deteriorate
●May evolve into dilated cardiomyopathy
●Molecular biology techniques suggest
idiopathic dilated cardiomyopathy may
represent end stage of viral myocarditis

19. Case # 2
●18 y/o hispanic female
●No cardiac complaints
●C/o ‘skin rash’

20. A Preventable
Cardiomyopathy

21. Lyme Dz- Stg 1 early localized
●Erythema migrans
●1 week post tick bite (range, 3-30 days)
●Flat or slightly raised red lesion at bite
●Commonly groin, thigh, or axilla
●Lesion expands several d’s, with central clearing
●20% do not have typical skin lesions or lesions
unnoticed
●Without Rx, erythema migrans resolve 3-4 wks

22. Lyme Stg 2- early
disseminated
●Days to weeks
●50% 2ndary lesions similar appearance primary
usually smaller
●Headache & stiff neck + migratory pains in joints,
muscles, & tendons
●Persistent fatigue & malaise common
●Neurologic & musculoskeletal symptoms intermittent -
hours to day
●Cardiac (4-10% of patients) - myocarditis, with
arrhythmias and heart block.

23. Case #3
●40 y/o Korean Female
●Stg IV Breast Cancer
●Adriamycin Therapy total dose
●Echocardiogram

24. Drug-induced & Toxic Myocarditis
●Doxorubicin & other chemotherapy cytotoxic agents
●Emetine (an antiparasitic agent for amebiasis)
●Catecholamines (especially with pheochromocytoma)
●Phenothiazines, lithium, chloroquine, disopyramide,
antimony-containing compounds, and arsenicals
●Hypersensitivity reactions sulfonamides, penicillins,
and aminosalicylic acid
●Radiation acute inflammatory reaction & chronic
fibrosis,
○Usually with pericarditis

25. Hypersensitivity Myocarditis
●Chest discomfort, dyspnea, palpatations
●Elevated JVP, Gallop Rythmn
●Sinus Tach, ST segment changes, BBB,
AV Block, Vtach
●Incr CK-MB, Cardiomegaly
●Eosinophils, atypical lymphs, giant cells
in BX

26. Hypersensitivity Myocarditis
●Amphotericin B
●Ampicillin
●Chloramphenicol
●PCN
●TCN
●Streptomycin

27. Hypersensitivity Myocarditis
●Sulfadiazine
●Sulfisoxazole
●Phenindione
●Phentoin
●Carbamazepine
●INH
●Para-aminosalicylic acid

28. Hypersensitivity Myocarditis
●Indomethacin
●Oxyphenbutazone
●Phenylbutazone
●Acetazolamide
●Chlorthalidone
●Hydrochlorothiazide

29. Hypersensitivity Myocarditis
●Spironolactone
●Amitriptyline
●Methyldopa
●Sulfonaureas
●Tetanus Toxoid

30. Case #4
●27 y/o Caucasian Male
●Episoidic severe headache, palpitations, tachycardia,
profuse sweating, abdominal pain
●Incr nervousness & irritability, incr appetite, & loss of
weight.
●Postural tachycardia (change of more than 20
beats/min) & postural hypotension.
●Pulmonary edema on CXR

31. Cocaine cardiotoxicity
●Marked Increase in incidence
●Coronary artery spasm, myocardial infarction,
arrhythmias, & myocarditis
●Mediated by inhibitory effect on norepinephrine
reuptake by sympathetic nerves
●Beta-blockers have been used therapeutically
●Coronary spasm, Ca++ channel blockers

32. Protein-Calorie Malnultrition
●Vacuolar degeneration of myofibrils
●Esp in conducting tissue
●Sudden death common, possibly from
arrythmia
●During recovery findings of CHF often seen
●Malnourished children sensitive to digoxin,
use diuretic only

33. Beriberi Heart Disease
●Thiamine deficiency 2° ingesting highly milled
rice as staple food
●Also occurs in chronic alcoholism
●Peripheral vasodilatation - high output state
●Reduced renal blood flow with retention of
Na+ & water
●Increased blood volume & biventricular failure

34. Beriberi - diagnostic criteria
●Hx/O Thiamine Deficiency
●Exclusion of other causes of heart disease
●High output failure
●Evidence of peripheral neuritis or pellagra
●Rapid response to therapeutic trial of
Thiamine

35. Beriberi- treatment
●Thiamine hydrochloride 100 mg IV QD
X 5 days
●Oral thiamine 50 mg qd X 1-2 weeks
●Bed rest
●± diuretics

36. Alcoholic Cardiomyopathy
●More common in men 30-55
●Heavy consumption of alcohol > 10yrs
●Often without liver disease or social
dysfunction
●PAF often initial presentation
●RX- IMMEDIATE & COMPLETE
ABSTINENCE

37. Cobalt Cardiomyopathy
●Fulminating CHF mid-60’s
●1st recognized in Canada
●Cobalt Sulfate added as foam stablizer in
beer

38. Septic Shock
●Acute mod to severe biventricular failure
●Preserved Atrial dimensions
●NO MR or TR
●Tumor Necrosis Factor directly
Cardiotoxic
●Reversible

39. Case #5
●44 y/o caucasian male pilot
●Sustained asymptomatic Vtach @ pk
exercise
●RV enlargement + focal Apical RV
dyskinesia
●Abnormal MRI

40. Arrhythmogenic RV Dysplasia
●Thinning & fatty infiltration of RV myocardium
(+-) fibrosis
●Ventricular arrythmias & sudden death
●Typical presentation young males with
symptoms during exercise
●VTach usually of LBB type
●Consider in family history of sudden death

41. DILATED CARDIOMYOPATHY- Essentials of
Diagnosis
●Symptoms & signs of heart failure.
●ECG
○Low OR Increased QRS voltage
○Nonspecific repolarization abnormalities
○Intraventricular conduction abnormalities
●X-ray => cardiomegaly
●Echo => LVdilation, thinning, & global
dysfunction

42. Symptoms & Signs
●Syms may develop gradually
●Asymptomatic cardiomegaly
●EKG abnormalities or ventricular ectopy
●Initial presentation can be severe biventricular
failure
●Cardiomegaly
●S3 gallop rhythm
●Murmur of functional mitral regurgitation

43. Ischemic Cardiomyopathy
●Most COMMON form of dilated
Cardiomyopathy
●Segmental Wall Motion Abnormalities
●‘Stunned’ vs ‘Hibernating’ Myocardium
●GROWTH MARKET in Cardiomyopathy

44. Case # 6
●32 y/o Caucasian female
●Progressive dyspnea
●Exertional Syncope
●Abnormal EKG - LVH with strain
●ECHO

45. Case #7
●40 y/o F-16 Fighter Pilot
●Hx/o Abnml EKG with deep inverted
Twaves precordial leads
●Asymptomatic
●Abnml Thallium Treadmill test with
COMPLETE RI of APEX
●Echocardiogram & Cardiac MRI

46. HYPERTROPHIC CARDIOMYOPATHY
●Dyspnea, chest pain, syncope
●Bisferiens carotid pulse, sustained (triple
apical impulse), Loud S4, systolic EM
(incr by upright posture/valsalva)
●ECG left ventricular hypertrophy
●Echo asymetric hypertrophy normal or
incr contractility & signs of dynamic
obstruction

47. Mechanics
●Inappropriate--ie, unrelated to pressure or
volume overload--myocardial hypertrophy
●Tends to impinge upon left ventricular cavity
●IVS disproportionately involved (asymmetric
septal hypertrophy)
●In some cases hypertrophy localized to apex
●LV outflow tract narrowed during systole

48. Pathophysiology
●Dynamic obstruction
●Worsened by increased myocardial contractility
○Sympathetic stimulation
○digoxin
○postextrasystolic beat
●Worsened by factors that decrease left ventricular
filling
○Valsalva's maneuver
○peripheral vasodilators

49. Genetics/Mechanism
●Hypertrophic cardiomyopathy inherited
autosomal dominant trait with variable
penetrance
●Caused by mutations of the gene coding for
myosin heavy chains
●These patients present in early adulthood
●Elderly with long hx/o HTN
●Some cases sporadic

50. Treatment
●Beta-blockers
●Dyspnea, angina, & arrhythmias respond 50%
●Calcium channel blockers, esp verapamil
●Myomectomy
●Dual-chamber pacing may prevent progression
of hypertrophy & obstruction
●Amiodarone &, possibly, sotalol may be
valuable

51. Prognosis
●Natural history highly variable
●Many asymptomatic many years or for life.
●Sudden death, especially during exercise, may
be initial event
●Most frequently associated with sudden death
in athletes
●Gradually progressive symptom
●May transition into dilated cardiomyopathy

52. Case #8
●84 y/o Hispanic male
●6 mo hx/o dramatic decrease in
functional status
●Marked bilat effussions
●Echocardiogram

53. RESTRICTIVE CARDIOMYOPATHY
● Impaired diastolic filling with preserved contractile function
● Relatively uncommon
○ Amyloidosis
○ Radiation
○ Myocardial fibrosis after open heart surgery
● In Africa, endomyocardial fibrosis, common
○ Severe fibrosis of the endocardium
○ Often with eosinophilia (Loffler's syndrome)
● Other causes
○ Infiltrative cardiomyopathies (eg, sarcoidosis,
hemochromatosis, carcinoid syndrome)

54. Amyloidosis
●Frequent cause of restrictive cardiomyopathy
●More frequently dilated cardiomyopathy with
congestive heart failure
●Almost invariably conduction disturbances
●Rectal, abdominal fat, or gingival biopsies or
myocardial biopsy

55. Primary Diagnostic Problem
●Restrictive cardiomyopathy vs constrictive pericarditis
●Status of left ventricular function - helpful
○Usually normal with pericarditis + thickened
pericardium
○Slightly depressed with restrictive cardiomyopathy
●Myocardial biopsies usually negative with pericarditis
●In some cases, only surgical exploration can make
diagnosis
○ Connective tissue diseases (eg, scleroderma)

56. Unfortunately
●Little useful therapy available for
causative conditions
●OR restrictive cardiomyopathy
●Diuretics can help
●Excessive diuresis can produce
worsening symptoms

57. Case # 9
●35 y/o active duty navigator
●Transfered for AICD placement
●FH (+) sudden death
●Ferritin Level = 2500

58. Hemochromatosis
●Autosomal recessive disease with linkage to
HLA-A3 & HLA-B14 or HLA-A3 and HLA-B7
●Hemosiderin in liver, pancreas, heart,
adrenals, testes, pituitary, & kidneys
●Hepatic, pancreatic, & cardiac insufficiency
●Diabetes + Hypogonadism =>
Hemochromatosis
●Rarely recognized before fifth decade

59. Case #10
●44 y/o black male
●Atrial Fibrillation with bradycardia rate
=45 bpm
●(+) Gallium scan (parotid uptake)

60. Sarcoidosis
●Pulmonary fibrosis => Cor Pulmonale
●Autopsy studies Cardiac 20-30% cases
●Clinical manifestations < 5% pxts
●Conduction system disturbances
●Restrictive + Dilated features

61. Case # 11
●74 y/o caucasian male with progressive
LE edema
●Increasing dyspnea
●Persistent Eosinophlia = 40%
●Echo obliteration of apex

62. Loffler Endocarditis
●Typically male 4th or 5th decade of life
●Biventricular cardiac involvement is the rule
●Must differentiate Cuhurg-Strauss syndrome
(asthma, nasal-polyposis, necrotizing
vasculitis)
●Death due to CHF although embolism can
occur
●Corticosteroids are Rx
●Routine CHF Rx

63. Tropical Endomyocardial Fibrosis-
Epidemiology
●Uganda, Kenya, Zambia (E. Africa);
Nigeria, Ghana, Ivory Coast (W.
Africa)
●Brazil, Columbia, Venezuela,
Mexico (S. & Cntrl America)
●Kerala & Haryana (India)

64. Tropical Endomyocardial Fibrosis-
Epidemiology
●In Uganda - as common a cause of
cardiac failure as RHD
●Uganda - seen in 25% of cardiac
necropsies
●More common in poorer socioeconomic
conditions
●In endemic areas 50% occur in persons
< 15 yrs of age

65. Tropical Endomyocardial Fibrosis-
Etiology
●Tropical environment
●Familial occurrence
●Circulating autoimmune heart antibodies
●Deposition of immune complexes in
heart
●Elevated malaria antibody titers
●Tropical spleenomegaly

66. Tropical Endomyocardial Fibrosis-
Pathology
●Fibrosis of mural endocardium
●Thrombus deposition followed by fibrotic
organization
●Early in disease embolization may occur
●Usual extends to the mitral and tricuspid
valve apparatus

67. Tropical Endomyocardial Fibrosis-
Pathology
●Valvular regurgitation can often occur
●Restriction of cardiac filling/cardiac output
●R-ventricle infundibulum hypertrophied and
dilated
●Severe R-sided failure symptoms can be
seen (ascites/hepatomegaly)
●L-ventricular involvement results in MR &
PAH

68. Tropical Endomyocardial Fibrosis- Clinical
Findings
●May manifest in first several months in
life
●Usually recognized in advanced stages
●Symptoms advance rapidly
●Process is usually biventricular
●High venous pressure causes
exopthalmos, periorbital facial edema,

69. Tropical Endomyocardial
Fibrosis- Clinical Findings
●Ascites always, peripheral edema rare
●Pericarditis present approx 40% cases -
aggravates restrictive cardiomyopathy
●Peripheral cyanosis & clubbing common
2° low cardiac output
●Cachexia, protein-losing enteropathy,
cardiac cirrhosis with hepatic failure -
terminal events

70. Tropical Endomyocardial Fibrosis- Clinical Findings
●Hyperdynamic RV outflow - L upper
parasternum
●Murmur of TR + R-sided S3
●Early peaking systolic MR murmur
●Late opening snap (MV) + L-sided S3 often
heard
●Early peaking systolic MR murmur + opening
snap unique to LV endomyocardial fibrosis

71. Case # 12
●65 y/o caucasian female
●Severe TR by Physical Exam & Echo
●Known ? tumor metastatic to liver

72. Carcinoid Heart Disease
●Liver mets needed for Carcinoid Heart
Disease
●Path findings: fibrious plaques
downstream of TV & PV
●TR + or - PS
●Occ pxts develop High Output failure
●Surgical Rx for severe disease

73. CARDIAC FAILURE- Essentials of
Diagnosis
● Left ventricular failure
○ Cough & fatigue
○ Exertional dyspnea, orthopnea, PND
○ Cardiac enlargement
○ Rales & gallop rhythms
● Right ventricular failure
○ Elevated venous pressure
○ Hepatomegaly
○ Dependent edema
● Both: Combination of above
● Dx => noninvasive or hemodynamic

74. Systolic Function
●Contractile state of the myocardium
●Preload of the ventricle
○End-diastolic volume
○Fiber length of ventricles
○Prior to onset of the contraction)
○Afterload (impedance to left ventricular
ejection)
●Heart rate

75. Heart Failure 2° Abnormal Systolic Fxn
●Primary derangement decr myocardial contractility
○Loss of functional muscle (MI , etc)
○Diffuse myocardial disease
●Pump failure 2° excessive preload (Valvular
Regurgitation)
●OR excessive Afterload (Aortic stenosis or HTN)
●Pump function compromised 2° Brady/Tachycardia

76. High Output Failure
●Pump function supranormal but inadequate
○Incr metabolic demands
○Incr requirements blood flow
●Causes of high output failure
○Thyrotoxicosis
○Bberiberi
○Severe anemia
○Arteriovenous shunting
○Paget's disease of bone

77. Diastolic Dysfunction
●Filling of the left or right ventricle is impaired
●Chamber is noncompliant ("stiff")
○Excessive hypertrophy
○Abnormal myocardium
●Contractility preserved
●Diastolic pressures elevated
●Occassionally cardiac output reduced

78. Pathophysiology
●Adaptations occur Heart & Vascular
●Decr SV either ventricle 2° decr
contractility or excessive afterload
○End-diastolic volume & pressure Incr
○=> end-diastolic myocardial fiber length
○=> greater systolic shortening
●If chronic, ventricular dilation will occur

79. Ventricular Dilatation
●May restore resting cardiac output
●Chronic incr diastolic mmHg => atria &
pulmonary & systemic circulations
●Incr capillary pressure => transudation
fluid
○Pulmonary or systemic edema

80. Reduced C.O.
● Decr kidney arterial pressure/perfusion
● Activates neural & humoral systems
○ Incr activity of sympathetic nervous system
○ Stimulates myocardial contractility, heart rate, and venous tone
○ Rise in effective central blood volume, incr preload
● Tachycardia & increased contractility=> ischemia
● Incr preload worsens pulmonary congestion
● Incr peripheral vascular resistance
○ Maintains perfusion to vital organs
○ When excessive reduces renal & other tissue blood flow
○ Incr LV afterload => further decr C.O.

81. Neurohumoral Activation
●Decr RBF & GFR
●=> Na+ & fluid retention
●Renin-angiotensin-aldosterone system activated
●=> incr PVR & LVafterload
●Further Na+ & fluid retention
●Increased arginine vasopressin
○Vasoconstrictor & inhibitor of H20 excretion
●Atrial natriuretic peptide incr 2° Incr atrial press
○Resistance to natriuretic & vasodilating effects

82. Hemodynamic Alterations
●Myocardial failure due to two
hemodynamic derangements
●Clinical presentation determined by
severity of each
●1° reduction in C.O. or cardiac reserve
●2° incr ventricular diastolic pressures
○Result of compensatory processes

83. Right-sided vs Left-sided
● Left heart failure
○ Low cardiac output & incr pulmonary venous pressure
○ Dyspnea predominant sym
● Right heart failure
○ Fluid retention predominates
○ Edema, hepatic congestion, & occassionally ascites
● Most exhibit R- & L-sided failure
● LV dysfunction is most common cause RV failure
● Occ severe LV dysfunction simulates isolated right heart failure
● May be clinically indistinguishable from cor pulmonale

84. Diastolic Failure
● Diastolic dysfunction => symptoms systolic dysfxn
● Difficult to distinguish clinically
● Diastolic pressures incr with normal or low diastolic volumes
● Pressures transmitted to pulmonary & systemic venous systems
○ Dyspnea & edema
● Most frequent cause LVH
○ Hypertension
○ Hypertrophic or restrictive cardiomyopathy
● Diabetes
● Pericardial disease
● Ischemia
● Diuretics useful other systolic therapies inappropriate