This document discusses cardiomyopathy and myocarditis. It defines cardiomyopathy as disorders of the heart muscle that are not caused by other diseases. Cardiomyopathy is classified into dilated, restrictive, and hypertrophic types based on echocardiography. Myocarditis is inflammation of the heart muscle that is often caused by viral infections. The document provides details on the presentation, evaluation, causes, and treatment of various types of cardiomyopathy and myocarditis.
2. Cardiomyopathy and Myocarditis
▪ Disease of heart muscle.
▪ Accounts for 5-10% of HF.
▪ Defined as disorders characterized by morphologically and
functionally abnormal myocardium in the absence of any other
disease that is sufficient by it self to cause observed phenotype.
▪ Many attributable to genetic causes.
▪ Classified based on echocardiographic features in to three
▪ Dilated
▪ Restrictive
▪ Hyperthrophic
3.
4. General presentation
▪ Early symptoms are exertional intolerance with breathlessness or
fatigue.
▪ Peripheral edema may be absent despite sever fluid retention.
▪ May also present atypical chest pain with palpitation or syncope.
▪ Embolism from intracardiac thrombus.
▪ Palpitation or syncope related to rhythm disorder.
▪ Acute cardiogenic shock in fulminant myocarditis.
5. ▪ Initial evaluation begins with detailed history and examination.
▪ Clues to cardiac, extracardiac and genetic causes of heart disease.
▪ Echocardiography is initial imaging modality.
6. Genetic causes of cardiomyopathy
▪ Well recognized in hypertrophic cardiomyopathy.
▪ 30% of dilated cardiomyopathy.
▪ Should be suspected in :
▪ Known cardiomyopathy and heart failure.
▪ Family member who had sudden death.
▪ Atrial fibrillation and pace maker implantation by middle age.
▪ Inherited autosomal dominant pattern commonly.
▪ Mutation in sarcomeric genes encode the thick and thin
myofilament proteins.
7. Dilated cardiomyopathy-DCM
▪ An enlarged left ventricle with reduced systolic function as
measured by ejection fraction.
▪ Systolic failure more prominent than diastolic dysfunction.
▪ Dynamic remodeling of interstitial scaffolding affects diastolic
function and amount of ventricular dilation.
▪ MR commonly develops.
▪ Many cases that present acutely have progressed silently through
these stages over months to years.
▪ About 1/3 demonstrate spontaneous recovery.
8. ▪ Chronic DCM may improve to near normal ejection fraction during
recommended therapy by neurohormonal modulation,
▪ Cardiac resynchronization therapy of LBB.
▪ Diuretics as needed to maintain fluid balace.
9. Myocarditis
▪ Inflammation of the heart muscle.
▪ Most often attributable to infective agents.
▪ With out obvious infection
▪ Sarcoidosis, giant cell myocarditis, SLE, polymyositis.
▪ Cant be assumed from presentation of decreased systolic function
in the setting of acute systemic infection.
▪ Fulminant myocarditis can result from viral infection, check point
inhibitors, giant cell myocarditis.
▪ Often complicated by arrythmia's.
▪ Early recognition is crucial.
10. Infective myocarditis
▪ Can injure myocardium through:
▪ Direct invasion.
▪ Disruption of normal cellular process.
▪ Production of cardiotoxic substance.
▪ Stimulation of chronic inflammation.
▪ Most commonly viruses and protozoan T.cruzi.
▪ Viral infection and replication can cause myocardial injury and
lysis.
11. Clinical presentation
▪ Acute viral myocarditis often presents with symptoms and signs of
heart failure.
▪ May present with chest pain and ECG changes suggestive of
pericarditis or acute MI.
▪ Occasionally atrial and ventricular tachyarrhythmias.
▪ Typical patient with presumed viral myocarditis is
▪ Young to middle aged adult.
▪ Progress to dyspnea.
▪ Few days to weeks after viral prodrome that was accompanied by fever
and myalgia
12. ▪ Subacute presentation may occur with in few weeks or month of
viral infection.
▪ Some presents with fulminant myocarditis.
▪ Rapid progression from sever febrile respiratory syndrome to
cardiogenic shock.
▪ Leads to MOF
▪ Renal failure
▪ Hepatic failure
▪ Coagulopathy
▪ Early Recognition is potentially life saving.
13. Ejection fraction often returns to normal.
50% can survive with aggressive therapy with catecholamine and
Mechanical ventilation.
Chronic viral myocarditis
Rare as cause of DCM
Many cause attributed to previous viral infection.
14. Laboratory evaluation
▪ Initial evaluation
▪ ECG
▪ Echo
▪ Serum troponin and CPK.
▪ Endocardial biopsy indicated when
▪ New HF with conduction block or ventricular tachyarrythmias
▪ Dallas criteria for myocarditis
▪ Lymphocytic infiltrate
▪ Myocyte necrosis
▪ Negative in 80-90% of patient with clinical myocarditis.
15. ▪ Suggest possible etiologies of non infectious inflammatory cause
like sarcoidosis or giant cell myocarditis.
▪ Viral titers b/n acute and convalescent blood sample.
▪ Respiratory virus panels can detect
▪ Adenovirus
▪ Influenza
▪ Corona virus
16. ▪ Possible subclinical myocarditis
▪ Typical viral syndrome occurs with out cardiac symptoms.
▪ Elevated biomarkers of cardiac injury.
▪ ECG suggestive and/or
▪ Reduced EF
▪ Probable acute myocarditis
▪ Above criteria met
▪ Cardiac symptoms which result from myocarditis, SOB, Chest pain.
▪ Elevated troponin, CK-MB
▪ Abnormal cardiac wall motion
▪ Some times called peri myocarditis or myopericarditis.
17. ▪ Definitive myocarditis
▪ Histologic or immunohistology evidence of inflammation.
▪ Doesn’t require any other laboratory or clinical criteria.
18. ▪ Specific viruses
▪ Enteroviruses
▪ Coxsackie virus
▪ Echovirus
▪ Poliovirus
▪ Influenza virus
▪ DNA viruses
▪ Adeno virus, vaccina and herpes virus (varicella zoster virus,
cytomegalovirus, EBV, HHV-6)
▪ B19 and HHV-6 can be found in normal heart.
19. ▪ HIV associated incidence of DCM is 1-2%.
▪ HAART reduced incidence.
▪ CMP in HIV may be from other viruses such as CMV and HCV.
▪ ART can cause CMP both directly and through hypersensitivity.
▪ Complicated by pericardial effusion and PHTN..
▪ HCV in German and Asian.
▪ May improve with interferon therapy.
▪ Interferon may depress cardiac function.
▪ HBV is uncommon.
▪ Can be seen associated with PAN.
20. ▪ Additional viruses
▪ Mumps
▪ RSV
▪ Arbovirus-dengue fever and yellow fever.
▪ Arena viruses –less likely
▪ For any serious infection the SIRS can causes non specific depression of
cardiac function.
▪ This is responsive for SARS-CoV-2 associated cardiac dysfunction
▪ Vasoconstrictive and prothrombotic endothilopathy of SARS-CoV-2 may
contribute to MI and/or stroke.
▪ Dominant injury to lung where ARDS develops.
▪ When HF develops it is onset of respiratory failure.
21. Therapy for viral myocarditis
▪ Non specific therapy.
▪ During acute infection
▪ Ant inflammatory or immunosuppression is avoided.
▪ Neither antiviral nor anti inflammatory therapy is recommended.
22. Other infectious causes
▪ Parasitic myocarditis
▪ Chagas disease most common infectious cause of DCM.
▪ T.cruzi transmitted by bite of reduviid bug.
▪ Transmission can also occur
▪ Blood transfusion.
▪ Organ donation
▪ Mother to fetus.
▪ Orally occasionally.
23. Pathogenesis
▪ The parasite it self can cause myolysis and primary neuronal
damage.
▪ Autonomic dysfunction and microvascular damage that may
contribute to cardiac or GI disease.
▪ Acute phase parasitemia unrecognized.
▪ In 5% cases non specific symptom, occasionally myocarditis and
meningoencephalitis.
▪ Silent stage progress 10-30 years.
▪ Manifest chronically with cardiac and gastrointestinal symptoms.
24. ▪ Manifest chronically with cardiac and gastrointestinal symptoms.
▪ Conduction system abnormalities.
▪ SA node and AV node dysfunction.
▪ RBB.
▪ Atrial fibrillation, ventricular tachyarrhythmia.
▪ Small ventricular aneurysm.
25. Treatment of T.cruzi CMP
▪ Treatment of HF.
▪ Pacemaker-defibrillators and
▪ Anticoagulation.
▪ Most common antiparasitic therapies are benznidazole and
nifurimox.
▪ Didn’t prevent progression disease in advanced CMP.
26. African Trypanosomiasis
▪ Results from tsetse fly bite.
▪ T.brucei and T.rhodesience
▪ Can progress rapidly through perivascular infiltration to cause
myocarditis and HF with frequent arrythmias.
▪ Diagnosis made by identification of trypanosomes in the blood,
lymph nodes or other infected tissue.
27. Toxoplasmosis
▪ Contracted through ingestion of undercooked infected beef or
pork.
▪ Transmission
▪ Feline feces
▪ Organ transplantation.
▪ Transfusion
▪ Maternal-fetal
▪ Immunocompromised hosts are more likely to experience
reactivation.
▪ May present with encephalitis or chorioretinits.
28. ▪ In heart it can cause
▪ Myocarditis
▪ Pericardial effusion
▪ Constrictive effusion and
▪ HF.
▪ Diagnosis in immunocompetent is made
▪ When IgM positive and IgG become positive latter.
▪ May be suspected in immunocompromised patient with
myocarditis and positive IgG titer.
▪ Treatment is Pyrimethamine and Sulfadiazine or clindamycin.
29. Trichinellosis
▪ Larva ingested with undercooked meat.
▪ Larva cause myalgias, weakness and fever.
▪ Periorbital and facial edema and conjunctival and retinal
hemorrhages.
▪ Diagnosis made by specific serum antibody.
▪ Supported by presence of eosinophilia.
▪ Treatment
▪ Albendazole, mebendazole
▪ Glucocorticoid for sever inflammation.
30. Bacterial infections
▪ Can involve heart occasionaly.
▪ Through direct invasion and abscess formation.
▪ Do so rarely.
▪ Diphtheria specifically affects the heart in ½ cases.
▪ Cardiac involvement is most common cause of death with
diphtheria.
▪ May particularly affect conduction system.
▪ Specific anti toxin should be administered as soon as possible.
31. ▪ Clostridial toxin can cause myocardial damage and gas bubbles
can be detected in the myocardium and pericardium.
▪ Streptococcal infection with ß-hemolytic streptococcus associated
with ARF.
▪ Can also lead to myocarditis.
32. Other systemic infection that can involve heart
muscle
▪ Brucellosis
▪ Legionella
▪ Meningococcus
▪ Mycoplasma
▪ Psittacosis and
▪ Salmonellosis
▪ Tuberculosis can involve directly or through TB pericarditis.
33. ▪ Tick borne infection-spirochetal myocarditis and borrelia
burgdoferi.
▪ Presents with arthritis and conduction system disease that resolves 1-
2 weeks after antibiotic.
▪ Treated with doxycycline.
34. Non infective myocarditis
▪ Can occur in the absence of infectious cause.
▪ Most common in sarcoidosis and Giant cell myocarditis
35. Sarcoidosis
▪ Higher prevalence in African-American.
▪ Patient with pulmonary sarcoidosis at high risk for cardiac
involvement.
▪ Patient may present with
▪ Rapid onset HF and ventricular tachyarrhythmias.
▪ Conduction block.
▪ Chest pain syndrome.
▪ Minor cardiac finding.
36. Sarcoidosis
▪ Ocular involvement
▪ Infiltrative skin rash
▪ No specific febrile illness.
▪ May present months to years.
▪ High suspicion when Ventricular tachycardia or conduction block
dominates HF with out CAD.
▪ Ventricles may appear restrictive or dilated.
▪ Small ventricular aneurysm.
▪ Chest CT reveals pulmonary lymphadenopathy.
▪ Pathologic confirmation to rule out TB or histoplasmosis.
37. Sarcoidosis
▪ Biopsy of enlarged mediastinal lymphadenopathy.
▪ Immunosuppressive treatment with high dose glucocorticoids
▪ More effective suppressing arrythmias than improving depressed EF.
▪ If lesion persists , immunosuppression.
▪ Pace maker and implantable defibrillator to prevent life
threatening heart block and ventricular tachycardia respectively.
38. Giant cell myocarditis
▪ Less common than sarcoidosis.
▪ Account for 10-20% of biopsy proven cases of myocardial disease.
▪ Presents with rapidly progressive
▪ Heart failure and tachyarrhythmias.
▪ Patient older than viral myocarditis.
▪ Associated thymomas, thyroiditis, pernicious anemia.
▪ Glucocorticoid alone rarely effective.
▪ In combination with other immunosuppression.
▪ Therapies similar to sever transplant rejection.
39. Peripartum cardiomyopathy
▪ Develops during last trimester or with 1st 6 month after pregnancy.
▪ Affects 1:2000-1:4000.
▪ Risk factors are
▪ Increased maternal age
▪ Increased parity.
▪ Twin pregnancy.
▪ Malnutrition.
▪ Tocolytic for premature labor.
▪ Preeclampsia.
▪ no evidence of preexisting cardiac disorder.
▪ Heart failure presenting earlier in pregnancy is termed pregnancy
associated cardiomyopathy-PACM.
40. Toxic cardiomyopathy
▪ Alcohol is the most common toxin in DCM.
▪ >10% of HF.
▪ Worsening of HF with structural heart disease.
▪ Men>women.
▪ Left ventricular dysfunction in 1/3 of asymptomatic.
▪ 80-100g of pure ethanol/d for 5-10 years.
▪ Frequent binge drinking.
▪ Toxicity is to both alcohol and metabolite acetaldehyde.
41. ▪ Many patient fully functional with out apparent stigmata of
alcoholism.
▪ Atrial fibrillation occurs both in early disease –Holiday heart and
advanced stages.
▪ Medical therapy –neurohormonal antagonist and diuretic therapy
as needed.
▪ Withdrawal.
▪ Marked improvement even with advanced stages 3-6 month after
abstinence.
▪ Grim prognosis if alcohol consumption continues.
42. Chemotherapy agents
▪ Most common drugs implicated in toxic CMP.
▪ Anthracyclines E.g doxorubicin.
▪ Risk increases with
▪ Older age
▪ Preexisting cardiac disease.
▪ Higher doses.
▪ Combination therapies.
▪ Left chest irradiation.
▪ Therapy for reduced ejection fraction with ß-blockers, inhibition of
RAAS.
43. ▪ Improve to normal with careful management.
▪ Prevention of second hit insults such as Atrial fibrillation and
Hypertension.
▪ Highest cardiotoxicity when administered with trastuzumab.
▪ Cyclophosphamide and ifosfamide
▪ Occurs acutely with very high doses.
▪ Other therapeutic drugs like, TNF-α antagonist, carbamazepine,
clozapine, lithium, ART.
▪ Chloroquine and hydroxychloroquine reduces EF by either
restrictive or dilated phenotype-often in association of conduction
block.
45. Metabolic causes of CMP
▪ Endocrine –hyper/hypothyroidism doesn’t cause HF in normal
heart.
▪ Exacerbate HF.
▪ Test for thyroid function is routine in evaluation of CMP.
▪ Hyperthyroidism should always be considered in new onset
Ventricular tachycardia, Atrial fibrillation and Atrial fibrillation with
difficult to control fast response.
▪ The most common reason is amiodarone which is high iodine
content.
▪ Hypothyroidism should be treated with very slow escalation.
46. ▪ Pheochromocytoma
▪ Rare.
▪ Should be considered when a patient has HF and very labile BP
and HR.
▪ Some times episodic palpitation.
▪ Postural hypotension.
▪ α-adrenergic receptors antagonist and surgical extirpation.
47. Nutritional deficiencies
▪ Beri-beri heart due to thiamine deficiency.
▪ Poor nutrition.
▪ Patient driving their calorie from alcohol.
▪ Initially high output failure latter low out-put failure.
▪ Thiamine replenishing lead to prompt recovery.
48. Hemochromatosis
▪ Metabolic storage disease.
▪ Included among causes of restrictive CMP, but clinical presentation
is that of dilated.
▪ If diagnosed early, can be managed by repeated phelebotomy.
49. Familial DCM
▪ Familial involvement has increased >30%.
▪ Mutation in TTN encoding giant sarcomeric protein.
▪ Men develop CMP a decade than female.
▪ Muscular dystrophies extracardiac manifestation common.
▪ Implantable defibrillators to prevent sudden death.
50. Tokotsubo CMP
▪ Apical ballooning syndrome.
▪ Acute stress CMP.
▪ Older women after sudden intense emotional and physical stress.
▪ Global ventricular dilation with basal contraction.
▪ Presentation include
▪ Pulmonary edema
▪ Hypotension
▪ Chest pain
▪ ECG changes mimicking acute infarction.
51. ▪ Resolves days to weeks.
▪ No proven therapies.
▪ In hospital mortality and complication similar to MI.
53. Overlapping types of CMP
▪ Sarcoidosis and hemochromatosis can present as dilated or
restrictive.
▪ Early stages of amyloidosis are often mistaken for hypertrophic
CMP.
▪ Common in inherited metabolic disorder.
54. Disorders of metabolic pathway
▪ Cause myocardial disease due to infiltration of abnormal products
or cells.
▪ Due to their accumulation with in cells.
▪ Fabry's disease
▪ Mucopolysacridosis
55. Restrictive CMP
▪ Dominated by abnormal diastolic function.
▪ Mildly decreased contractility.
▪ Ejection fraction 30-50%.
▪ Both atria enlarged sometimes massively.
▪ Modest left ventricular dilatation.
▪ Subtle exercise intolerance is the 1st symptom.
▪ Often present right sided symptoms such as edema, abdominal
discomfort and ascites.
56. ▪ Cardiac impulse is less displaced than DCM, less dynamic in
hypertrophic CMP.
▪ Atrial fibrillation is common.
▪ Positive kussmaul’s sign.
▪ 4th heart sound common.
▪ Most are due to infiltration of abnormal substance b/n myocytes,
storage of abnormal metabolic products with in myocytes or
fibrotic injury.
▪ Ddx include constrictive pericardial disease.
57. Causes of restrictive cardiomyopathy
▪ Infiltrative disease
▪ Amyloidosis is most common.
▪ Half in age> 90.
▪ Men>women.
▪ Endomyocardial biopsy is virtually 100% reliable for diagnosis.
▪ All most all amyloid that affects the heart is caused by assembly
of either immunoglobulin light chain from clonal plasma cells- AL
or primary amyloid or
▪ Transthyretin ATTR which is made in liver.
▪ Can be inherited mutant protein-ATTRm
▪ Normal protein –ATTRwt-wild type
58. ▪ History of carpal tunnel syndrome is common in ATTRm and wt.
▪ ATTRwt is also associated with spinal stenosis.
▪ Atrial enlargement is prominent.
▪ Diastolic dysfunction more sever than that of other causes of
hypertrophy.
▪ Nephrotic syndrome is common in AL amyloid.
▪ Amyloidosis should be suspected when ventricular myocardium
appears thick on imaging with low voltage ECG.
59. Fibrotic restrictive CMP
▪ Progressive fibrosis can cause restrictive myocardial disease with
out ventricular dilatation.
▪ Thoracic radiation can produce early/late restrictive CMP.
▪ May present with possible constrictive pericarditis.
▪ Right hear failure dominates clinical presentation.
▪ Conduction system disease and Atrial fibrillation are common.
60. Treatment
▪ AL amyloidosis can be treated with bortezomib.
▪ Sometimes be treated with cardiac transplantation followed by
stem cell transplantation.
▪ Tarnsthyretic amyloidosis can be treated by tafamidus and
diflusinal for associated neuropathy.
▪ Patisiran –small interfering RNA.
▪ Inotersen – anti sense mRNA.
▪ Both for treatment of polyneuropathy associated with TTR amyloid.
▪ Those therapies have not yet approved for cardiac indication.
61. Endomyocardial disease
▪ Loffler’s endocarditis.
▪ Common in men than women.
▪ Hyper eosinophilia >1500 for at least 6 months.
▪ Can cause acute phase eosinophilic myocarditis.
▪ Occasionally explained by parasitic or allergic disease.
▪ May be due to myeloproliferative variant.
▪ May present with HF, embolic events and atrial arrythmias.
▪ Pericardial effusion frequently accompany endocardial fibrosis, but not
common in Loffler’s endocarditis.
▪ For endomyocardial fibrosis no gender difference.
▪ High prevalence in African-American.
62. ▪ In tropical countries ¼ of HF may be due to endomyocardial
fibrosis.
▪ Glucocorticoid for hyper eosinophilia when present.
▪ Anticoagulation recommended.
▪ Atrial fibrillation difficult to suppress.
▪ Serotonin secreted by carcinoid tumor can produce fibrous plaques
in the endocardium and right sided cardiac valves.
▪ Stenotic or regurgitant valve lesion.
▪ Systemic symptoms flushing and diarrhea.
63. Hypertrophic CMP
▪ Ventricular hypertrophy that develops in the absence of causative
hemodynamic factors such as HTN, aortic valve disease or
systemic infiltrative stage disease.
▪ Prevalence 1:500
▪ Leading cause of sudden death in young.
▪ Important causes of HF.
▪ Sarcomere mutation is present in 50%.
▪ 80% mutation in either MYH7 or MYBPC3.
▪ Age dependent and incomplete penetrance.
64. ▪ Fibrosis and microvascular disease may present.
▪ The interventricular septum is the typical location if maximal
hypertrophy.
▪ Left ventricular outflow tract obstruction the most common focus
of diagnosis and intervention.
▪ Obstruction present in 30% at rest.
65. ▪ Provoked by exercise.
▪ Low preload- dehydration and low afterload such as arteriolar
vasodilation may lead to near syncope.
▪ Systolic ejection murmur is harsh and late peaking can be
enhanced by Valsalva maneuver and standing from squatting
position.
67. Treatment
▪ Treatment symptoms and prevention of sudden death and stroke.
▪ Left ventricular out flow tract obstruction can be controlled by
medically.
▪ β-blocker and verapamil are 1st line agents to reduce severityof
obstruction by slowing HR.
▪ Exertional dyspnea and chest pain can some times be controlled
by addition of disopyramide.
70. ▪ Fluid retention require diuretic therapy for venous congestion.
▪ Sever medically refractory symptom develop in 5%.
▪ Surgical myectomy or alcohol septal ablation may be effective.
▪ Mitral valve repair or replacement unnecessary.
▪ Neither procedure has been shown to improve outcomes other
than symptoms.
▪ Complete heart block complication of procedure require pacing.
▪ Patients have an increased risk of sudden death from ventricular
tachyarrhythmias.
71. ▪ Sudden death is not reduced by medical or procedure intervention.
▪ Atrial fibrillation is common.
▪ Rapid ventricular response is poorly tolerated and may worsen out
flow tract obstruction.
▪ β-blocker and L-type calcium channel blocker.
▪ Cardiac glycoside should be avoided.
▪ Anticoagulation recommended to prevent cardioembolic event on
atrial fibrillation.
72. Prognosis
▪ Mortality remains high.
▪ Sudden death risk <1%/year.
▪ 1:20 patient will progress to overt systolic dysfunction with
reduced ejection fraction <50%.
▪ Burned out or end stage hypertropic cardiomyopathy.
▪ Those patients are at high risk of death unless they undergo
transplantation.