Specific Myocardial Disease

1
Specific Myocardial Disease
2
Alcoholic Cardiomyopathy

• Reported to account for 1/3 of clinical DCM and
  45 at autopsy
• arrhythmia and chest pain not uncommon
• risk for SVT is 2.6x for pts drinking gt6 /day
• prognosis poor for pt who continues to drink
• 64 alcoholic followed for 4 years9 mortality
  for 1/3 who remained abstinent50 mortality for
  the active alcoholics

3
Peripartum Cardiomyopathy

• A form of dilated cardiomyopathy
• symptoms occur during the last trimester,
  diagnosed in the peripartum period
• 1/10,000 in the U.S., 1/100 in parts of Africa
• greatest in twin preganancies, multiparas, gt30
  years of age, African-American
• 50-60 show near complete recovery in 6 months
• subsequent pregnancies discouraged

4
Acquired Immunodeficiency Syndrome

• occurs in ¼ to ½ of patients (echo, biopsy,
  autopsy)
• Kaposi, myocarditis, pericarditis, endocarditis,
  DCM
• clinically apparent in only 10
• most common symptom is CHF
• dyspnea may be attributed to pulmonary infection
• LV dilatation and dysfunction
• pathological findings with myocarditis are common
• HIV, viral, fungal, bacterial, parasitic, AZT

5
Lyme Disease

• Infection with the spirochete Borrelia
  bergdorferi
• characterized by erythema chronica migrans,
  myalgias, arthralgias, headache, fever,
  lymphadenopathy, fatigue
• may progress to Lyme carditis with complete heart
  block unusual to cause ventricular dysfunction
• Treatment
• tetracycline during the early phase
• corticosteroids for Lyme carditis following
  tetracycline

6
Sarcoidosis

• a systemic granulomatous disease of undetermined
  etiology involving the lymph nodes, lung, liver,
  spleen, eyes, phalangeal bones, and parotid
  glands
• Cardiac manifestations
• 1/5 of patient have myocardial sarcoid at autopsy
• only 5 may have cardiac related symptoms
• sarcoid granuloma preferentially affect the basal
  septum and LV papillary muscles
• conduction abnormalities, mitral regurgitation,
  wall motion abnormalities are frequently seen
  ventricular tachyarrhythmias are frequent and may
  cause sudden death

7
Chagas DiseaseAmerican trypanosomiasis

• The most common cause of CHF in the world
• infection with Trypanosoma cruzi is endemic in
  rural Central and South America
• characterized by CHF, heart block, arrhythmia,
  megacolon, and megaesophagus
• Treatment
• standard heart failure therapy
• pacemaker for CHB amiodarone for vent arrhythmia
• nifurtimox for prophylaxis and first time
  treatment

8
Carcinoid Heart Disease

• tumor arise from argentiffin cells in the GI
  tract
• usually located in the ileum
• infrequently in the bronchus
• rarely in the jejunum, rectum, Meckels
  diverticulum, ovary
• secret large amount of serotonin
• lt10 associated with carcinoid syndrome

9
Carcinoid Heart Disease

• heart disease occurs in 50 of pts with carcinoid
  syndrome
• myofibromatous thickening of the tricuspiod and
  pulmonic valves
• severe tricupid regurgitation
• major causes of death
• hepatic failure
• right heart failure
• bowel obstruction and perforation
• treatment -
• 70-80 5-year survival with interferon and
  somatostatin analogues
• surgical management of right heart valves

10
Toxoplasmosis

• Following cardiac transplantation with possible
  transmission by the donor heart
• Diagnosis
• rise in antibody titer to T. gondii
• identification of toxoplasma cysts in areas of
  focal myocyte necrosis, edema, and inflammation
• Treatment
• pyrimethamine and sulfadiazaine
• curable when treated early

11
Cytomegalovirus

• Usually self-limited and asymptomatic
• May cause cardiac dysfunction in transplant pts
• Early CMV infection correlates with development
  of allograft coronary artery disease
• Treatment
• intravenous ganciclovir

12
Eosinophilic Myocarditis

• myocardial interstitial Infiltration by
  eosinophils
• characterized by
• endocardial thickening
• restrictive myocardial disease
• cardiac dysfunction
• eosinophilic myocarditis may occur without
  peripheral eosinophilia
• Treatment
• corticosteroids improved LV function in
  uncontrolled trials

13
Giant-Cell Myocarditis

• A particularly aggressive form of myocarditis
• associated with autoimmune disease
• myasthenia gravis
• autoimmune hemolytic anemia
• polymyositis
• characterized by brady- , tachy- arrhythmias, and
  left ventricular dysfunction
• Treatment
• Immunosuppressive therapy ? benefit

14
Pheochromocytoma

• Found in adrenal medulla and sympathetic ganglia
• catecholamine induces myocardial damage
• characterized by hypertension, brady-, tachy-
  arrhythmias, orthostatic hypotension, ischemia,
  congestive heart failure with LV dilatation
• Treatment
• medical therapy to reduce circulating
  catecholamines
• tumor removal

15
Hyperoxaluria

• Characterized by ca oxylate deposition in body
  tissues
• Primary - autosomal recessive (alanine glyoxylate
  aminotransferase)
• Secondary - chronic renal failure, ingestion of
  oxylates or oxylate precursors (ethylene glycol,
  xylitol, methoxyflurane), enteric hyperoxaluria
  (small bowel disease with pyridoxine deficiency)
• Cardiac abnormalities
• heart block
• arrhythmias
• congestive heart failure
• In primary oxalosis, cardiac dysfunction may
  reverse after combined kidney-liver transplant

16
Gout

• Uric acid crystal can be found in
• walls of blood vessels
• myocardial interstitium
• pericardium
• conducting system
• mitral annulus
• mitral, aortic, and tricuspid leaflets

17
Hemochromatosis

• Characterized by iron overload
• Genetic - autosomal recessive (severe only in
  homozygotes)
• Acquired - sideroblastic anemia, thalassemia,
  transfusion-related, iron ingestion
• Clinical presentation
• cirrhosis, diabetes, hyperpigmentation, cardiac
  dysfunction
• congestive heart failure is the leading cause of
  deathtransition from restricted disease to
  dilated cardiomyopathy
• ECG with low voltage and non-specific changes,
  and SVT
• Treatment
• phlebotomy, chelation therapy with deferoxamine
• cardiac function may normalize

18
Amyloid Heart Disease

• Amyloid AL (primary and myeloma-associated)
• Amyloid AA (secondary and familial medit fever)
• Amyloid AF (familial)
• Amyloid SSA (senile cardiac and senile systemic)
• Amyloid IAA (isolated atrial)
• Amyloid CAA (cerebral amyloid angiopathy)

19
Amyloid AL Myeloma-associated and Primary
Systemic Amyloidosis

• immunoglobulin light chain (kappa or lamda)
• monoclonal protein spike in the serum or urine
• 1/3 to 1/2 have cardiac involvement
• gt 1/4 have symptomatic heart failuremedian
  survival lt 1 year, 5 year survival lt 10

20
Amyloid AA Secondary Amyloidosis and Familial
Mediterranean Fever

• heart is not involved
• Chronic inflammatory disorders
• rheumatoid arthritis, jeuvenile RA, ankylosing
  spondylitis, Crohns diesease, cystic fibrosis,
  heroin use
• Familial Mediterranean Fever
• autosomal recessive disease of Sephardic Jews,
  Armenians, and other Mediterranean people

21
Amyloid AFFamilial Amyloidosis

• Autosomal dominant disorderdeposition of
  transthyretin (a variant of prealbumin)
• Clinical manifestation
• progressive neuropathy
• cardiomyopathy
• renal involvement
• heart disease is variable in different families

22
Amyloid SSA Senile Cardiac Amyloid and Senile
Systemic Amyloid

• deposition of transthyretin (a variant of
  prealbumin), similar to amyloid AF, but in older
  individuals
• Clinical variants
• affecting only the atria
• affecting only the aorta
• affecting the lung, kidneys, and myocardium
• uncommon cause of cardiac mortalitybetter
  prognosis than the immunoglobulin amyloids

23
Laboratory Evaluation in Amyloidosis

• Echocardiography
• most effective noninvasive screening
• concentric LV hypertrophy(diffuse
  hyperrefractile, granular sparkling)
• Endomyocardial biopsy
• Technetium 99m-pyrophosphate
• may identify early amyloid heart disease

24
Diabetic Cardiomyopathy

• Does it exist?