july 5 2007 anne marie kathryn p ingente md
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July 5, 2007 Anne Marie Kathryn P. Ingente MD. Medical grandrounds. LEARNING OBJECTIVES. To present a case of CHF secondary to restrictive cardiomyopathy secondary to cardiac amyloidosis To discuss the diagnosis and management of cardiac amyloidosis. IDENTIFYING DATA.

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july 5 2007 anne marie kathryn p ingente md
July 5, 2007

Anne Marie Kathryn P. Ingente MD

Medical grandrounds

learning objectives
LEARNING OBJECTIVES
  • To present a case of CHF secondary to restrictive cardiomyopathy secondary to cardiac amyloidosis
  • To discuss the diagnosis and management of cardiac amyloidosis
identifying data
IDENTIFYING DATA
  • 65-year-old Filipino male, married, resident from US
  • (+) HPN (since 1991)
  • (+) DM 2 (since 1991)
chief complaint
CHIEF COMPLAINT
  • Difficulty of breathing
history
HISTORY

January 2006: (+) easy fatigability

December 2006: (+) easy fatigability

(+) 2-pillow orthopnea

(+) bipedal edema

(+) occasional cough w/ whitish phlegm

(-) fever; (-) chest pain;

(-) palpitations

admitted at Stanford University Medical Center

(Palo Alto Medical Foundation)

2d echo palo alto medical clinic dec 28 2006
2D-ECHO(Palo Alto Medical Clinic; Dec 28, 2006)

Concentric LVH. Small left ventricular cavity. Mild-moderate LV systolic dysfunction (EF 40-50%).

Normal RV size. RV hypertrophy. Moderate RV systolic dysfunction.

Right and left atrial sizes are within normal limits.

Mild thickening of the aortic and pulmonic valves.

Large right pleural effusion. Ascites and small pericardial effusion noted.

history1
HISTORY

February 2007: (+) easy fatigability

(+) shortness of breath

(+) bipedal edema

(+) occasional cough, with scanty whitish phlegm

admitted at Stanford

responded to diuretics,

salt and fluid restriction

history2
HISTORY
  • MAY 1, 2007; Stanford University Medical Center
    • Right heart catheterization with right ventricular biopsy
    • RV biopsy was remarkable for CARDIAC AMYLOIDOSIS.
  • Immunofixation Electrophoresis of Serum:
    • Elevated free lambda light chains
history3
HISTORY
  • MAY 16, 2007; Stanford University Medical Center

Bone marrow biopsy with flow cytometric immunophenotyping was done.

history4
HISTORY
  • BONE MARROW BIOPSY:

- Moderate monoclonal plasmacytosis (10-20%) consistent with a plasma cell dyscrasia

  • FLOW CYTOMETRIC IMMUNOPHENOTYPING:

- Lambda light chain-restricted plasma cells

history5
HISTORY
  • May 31, 2007; Makati Medical Center

- sought consult for continuation of treatment

- easy fatigability, shortness of breath, bipedal edema, orthopnea

review of systems
REVIEW OF SYSTEMS
  • Skin: (+) periorbital bruising, (-) urticaria, (-) rash
  • Bones, joints, muscles: (-) pain, (-) muscle weakness
  • Hematopoietic: (-) bleeding; (-) delayed clotting
  • HEENT: (-) headache, (-) blurring of vision, (-) tinnitus,

(-) hearing loss, (+) dysphagia, (+) hoarseness

review of systems1
REVIEW OF SYSTEMS
  • ABDOMEN: (-) pain, (-) bloatedness, (+)constipation (-) diarrhea
  • GENITOURINARY: (-) hesitancy, intermittency, frequency (-) hematuria (-) dysuria
  • EXTREMITIES: (+) pricking sensation on the R tibia, (+) numbness on tips of toes and fingers
past medical history
PAST MEDICAL HISTORY
  • (+) S/P Appendectomy – 1960s
  • (+) S/P Surgery for Carpal Tunnel Syndrome – 1991
  • (+) Gout – 1980 (Allopurinol 100mg OD)
  • (+) Dyslipidemia – 1990s (Simvastatin 20mg OD)
  • No asthma, no allergies, no history of TB
  • No prior MI or CVA
past medical history1
PAST MEDICAL HISTORY
  • Maintenance meds:
    • Glipizide 5mg OD
    • Insulin
    • Simvastatin 20mg OD
    • Allopurinol 100mg OD
    • Hydrocholorothiazide 25mg OD
    • Bumetanide 1mg/tab 2 tabs BID (4mg/day)
    • KCl 10 mEq tab 1 tab with each tablet of Bumetanide, up to 4 tabs daily
family medical history
FAMILY MEDICAL HISTORY
  • (+) HPN – mother
  • (+) DM – mother
  • (+) heart disease – father
  • (-) asthma
  • (-) cancer
personal social history
PERSONAL & SOCIAL HISTORY
  • Non-smoker
  • Occasionally drinks
  • Retired architect
physical examination
PHYSICAL EXAMINATION
  • BP 110/70 HR 104 reg RR 22 afebrile
  • Conscious, coherent, conversant
  • Pink palp conjunctivae, anictericsclerae,

(+) periorbital discoloration

  • Trachea midline, thyroid not palpable, no CLAD, JVP 12 cm H20, no carotid bruit
physical examination1
PHYSICAL EXAMINATION
  • Lungs: symmetric chest expansion, no retractions, dullness to percussion on the R mid basal lung field, decreased breath sounds on the R mid to base, fine crackles on the left base
  • Heart: adynamic precordium, outer border 2 fingers outside the LMCL, tachycardic, regular rhythm,distinct heart sounds, no murmurs
physical examination2
PHYSICAL EXAMINATION
  • Protuberant abdomen with bulging flanks, normoactive bowel sounds, liver and spleen palpable, liver edge felt at 5 cm below the right costal margin, (+) dullness at Traube’s space, (+) shifting dullness.
  • (+) Grade 3 bipedal pitting edema, dorsalispedis strong and equal, pink nail beds
salient features
SALIENT FEATURES
  • 65-yr-old Filipino male
  • Diagnosed with cardiac amyloidosis
  • Came for continuation of treatment
  • Persistent shortness of breath, easy fatigability, bipedal edema, orthopnea
  • Periorbital edema
  • Dullness on percussion on the R mid to basal lung field, decreased breathsounds on R mid to base, fine crackles L base
slide22

Outer border 2 fingers outside the LMCL, tachycardic, regular rhythm, no murmurs

  • Protuberant abdomen with bulging flanks, NABS, liver and spleen palpable, liver edge felt at 5cm below the R costal margin (+) dullness at Traube’s space, (+) shifting dullness
  • Grade 3 pitting bipedal edema
admitting diagnosis
ADMITTING DIAGNOSIS
  • Congestive Heart Failure secondary to Restrictive Cardiomyopathy secondary to Cardiac Amyloidosis
  • Hypertensive atherosclerotic disease
  • Diabetes Mellitus
  • Gout
chest usg
Chest USG
  • Result showed massive amount of anechoic free fluid in the right hemithorax with a volume of at least 1100cc.
pleural fluid
PLEURAL FLUID
  • Protein 2.7 gm%
  • Glucose 204 mg%
  • LDH 57 U/L
  • RBC 584
  • WBC 3
  • Segmenters 3
  • 20cc yellow, hazy; specimen with clot
  • No microorganisms seen; WBC 4-6/OIF
  • No growth in 5 days
2d echo
2D- ECHO
  • concentric LVH with global hypokinesia. Ejection fraction of 39% by simpson and 45% by teicholz.
  • Dilated left atrium without evidence of thrombus. Normal right atrial and right ventriuclar dimensions.
  • Normal main pulmonary artery, aortic root and proximal ascending aortic dimensions.
slide30

Calcified right coronary, non coronary and left coronary cusps of the aortic valve with normal valve mobility. Pericardial effusion mild to moderate.

  • Normal tricuspid valve and pulmonic valve. Color flow and Doppler study showed mitral regurgitation, mild.
  • Aortic regurgitation, trivial. Tricuspid regurgitation mild. Pulmonic regurgitation, mild. Mild pulmonary hypertension. Restricted filling pattern of mitral valve leaflet velocity flow.
final diagnosis
FINAL DIAGNOSIS
  • Congestive Heart Failure secondary to Restrictive cardiomyopathy secondary to cardiac amyloidosis
  • Hypertensive atherosclerotic cardiovascular disease
  • Pleural effusion secondary to CHF
slide34

Chronic renal insufficiency secondary to cardiac decompensation

  • Diabetes Mellitus
  • Gout
discussion
DISCUSSION

Heart Failure

Right-sided

Left-sided

Cor pulmonale

Constrictive pericarditis

Tamponade

RV infarction

Restrictive cardiomyopathy

Aortic regurgitation

Post MI

restrictive cardiomyopathy
RESTRICTIVE CARDIOMYOPATHY
  • Defined as heart- muscle disease
  • results in impaired ventricular filling
  • with normal or decreased diastolic volume of either or both ventricles
slide37

Usually results from increased stiffness of the myocardium

  • Causes pressure within the ventricles to rise precipitously with only small increase in volume
slide38

Affects either or both ventricles

  • May cause symptoms and signs of R or L ventricular failure
  • Often R sided findings predominate
slide39

Considered in a patient presenting with heart failure but no evidence of cardiomegaly or systolic dysfunction

restrictive hemodynamics
RESTRICTIVE HEMODYNAMICS

AMYLOID DEPOSITION

INC STIFFNESS OF MYOCARDIUM

INCREASED FILLING PRESSURE

REDUCED FILLING VOLUME

LOW CARDIAC OUTPUT

CONGESTION

restrictive hemodynamics1
RESTRICTIVE HEMODYNAMICS

INCREASED FILLING PRESSURE

REDUCED FILLING VOLUME

LOW CARDIAC OUTPUT

CONGESTION

Bipedal edema, ascites, enlarged liver

Easy fatigability, weakness, azotemia

slide43

Amyloid deposition can disturb the tissue architecture and lead to organ dysfunction

  • J Clin Pathol 2005; 58: 125-133
what is amyloid
WHAT IS AMYLOID?
  • Nonbranching fibrillar structure, an indefinite length and a 9.5 nm width
  • Organized into a pure beta pleated sheet configuration making it highly insoluble
slide47

Formation is not clearly understood

  • But is thought that development of amyloid is a result of cleavage of the light chains of the immunoglobulins followed by aggregation of these light chains into beta pleated sheet
what is amyloidosis
WHAT IS AMYLOIDOSIS
  • Refers to the deposition of amyloid protein in organs and tissues
  • Protein fragments of normal antibody molecules produced by plasma cells in the bone marrow
slide49

Amyloid is deposited in various organs and tissues including tongue, intestines, skeletal and smooth muscles, nerves, skin, ligaments, heart, liver, spleen and kidneys

slide52

AMYLOID DEPOSITS STAIN AS RED WITH CONGO RED STAIN

AND SHOW APPLE-GREEN BIREFRINGENCE UNDER POLARIZED LIGHT

incidence
INCIDENCE
  • 8 cases per million per year
  • Occurs in both sexes
  • 2:1 (males:females)
  • Peak occurrence at 60-67 y.o
      • Mayo Reference services publications Sept 2002
syndromes
SYNDROMES
  • Infiltrative cardiomyopathy with restrictive hemodynamics
  • Nephrotic range proteinuria w/ or without renal insufficiency
  • Indiopathic peripheral neuropathy
slide56

Unexplained hepatomegaly

  • Unexplained splenomegaly
  • Carpal tunnel syndrome
  • Macroglossia
  • Gastrointestinal symptoms
cardiac amyloidosis
CARDIAC AMYLOIDOSIS

MYOCARDIUM

AMYLOID

PERIVASCULAR

(SMALL INTRAMURAL VESSELS)

CARDIAC CONDUCTION SYSTEM

VALVES

pe in patients with cardiac amyloidosis
PE in Patients with Cardiac Amyloidosis
  • Elevation of jugular venous pressure
  • Hypotension may be caused by a low cardiac output
  • Orthostatic hypotension
  • R sided 3rd heart sound is occasionally heard
  • Murmur of tricuspid or mitral regurgitation is occasionally heard
ecg findings
ECG FINDINGS
  • Low voltage in the limb leads occurring in approximately 50%
  • Conduction abnormalities
  • Atrial fibrillation
  • Pseudoinfarct patterns
echocardiography
ECHOCARDIOGRAPHY
  • noninvasive test of choice
  • Left ventricular wall thickening with evidence of diastolic dysfunction is the earliest echocardiographic abnormality
slide62

In more advanced disease, wall thickening progresses resulting in cardiomyopathy with a nondialted or small LV cavity

  • Biatrial enlargement occurs, and the R ventricle may dilate
  • Mitral and aortic valves may become thickened
slide64

Amyloid infiltration of the heart results in increased echogenicity

  • Described as “granular, sparkling” appearance of the myocardium and it resulted in unusually high quality myocardial visualization
  • “sparkling pattern is not sensitive because only a minority 26% has it
slide65
Long axis view from a 2-D echocardiogram showing concentric left ventricular hypertrophy, thickened mitral and aortic valve leaflets and left atrial dilatation. Courtesy of Thomas Binder, MD. University of Vienna.

Cardiac amyloidosis

Long axis view from a 2-D

slide66
Short axis view from a 2-D echocardiogram shows concentric left ventricular hypertrophy and thickened mitral valve leaflets. Courtesy of Thomas Binder, MD. University of Vienna.

Cardiac amyloidosis

Long axis view from a 2-D

slide67
Four chamber view from a 2-D echocardiogram shows concentric hypertrophy of the right and left ventricular myocardium which has a "sparkling" appearance. The mitral and tricuspid valves are thickened and the right and left atria are dilated. Courtesy of Thomas Binder, MD. University of Vienna

Cardiac amyloidosis

Long axis view from a 2-D

voltage to mass ratio
VOLTAGE TO MASS RATIO
  • Left ventricular thickening due to amyloid infiltration may be d misdiagnosed on echo as LEVH.
  • However, unlike true LVH, left ventricular thickening in cardiac amyloidosis is associated with a decrease in ECG voltage
diagnosis
DIAGNOSIS
  • Presence of cardiac amyloidosis should be ruled out in any patients with unexplained heart failure
tissue biopsy
TISSUE BIOPSY
  • Demonstrating amyloid deposits on endomyocardial biopsy
  • Amyloid deposits on histologic examination of a biopsy from other tissues (abdominal fat pad, rectum or kidney)
monoclonal paraprotein
Monoclonal paraprotein
  • Serum or urine monoclonal paraprotein
nuclear imaging
NUCLEAR IMAGING
  • Increased cardiac uptkae of radiolabeled Tc in patient with amyloid heart disease
  • Not sensitive
cardiovascular magnetic resonance
CARDIOVASCULAR MAGNETIC RESONANCE
  • Global and subendocardial late enhancement of the myocardium
  • Sensitivity of this test was not assessed and the predictive value of this test remains undetermined
bnp and n terminal pro bnp
BNP and N-terminal pro-BNP
  • Increased in heart failure
  • Seen in patients with AL amyloidosis before the onset of clinical heart failure and are a marker of cardiac involvment.
  • Sensitivity 93%
  • Specificity of 90%
treatment
TREATMENT
  • Usually ineffective and generally consists of supportive measures
treatment options
TREATMENT OPTIONS
  • Melphalan + stem cell transplantation
  • Melphalan + dexamethasone
  • High-dose dexamethasone
  • Thalidomide + dexamethasone
  • SWISS MED WKLY 2006; 136: 715-720
supportive treatment
SUPPORTIVE TREATMENT
  • Salt restriction
  • Judicious diuretic use
  • Control of neuropathic pain
  • Transplantation of organs
prognosis of al amyloidosis
Prognosis of AL amyloidosis
  • Median survival 6-9 months in those with heart failure
  • 1.1 years in those with any sign of cardiac involvement
slide79

Thank You

Good Morning

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