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Medical grandrounds

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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Medical grandrounds

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  1. July 5, 2007 Anne Marie Kathryn P. Ingente MD Medical grandrounds

  2. 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

  3. IDENTIFYING DATA • 65-year-old Filipino male, married, resident from US • (+) HPN (since 1991) • (+) DM 2 (since 1991)

  4. CHIEF COMPLAINT • Difficulty of breathing

  5. 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)

  6. 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.

  7. 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

  8. 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

  9. HISTORY • MAY 16, 2007; Stanford University Medical Center Bone marrow biopsy with flow cytometric immunophenotyping was done.

  10. HISTORY • BONE MARROW BIOPSY: - Moderate monoclonal plasmacytosis (10-20%) consistent with a plasma cell dyscrasia • FLOW CYTOMETRIC IMMUNOPHENOTYPING: - Lambda light chain-restricted plasma cells

  11. HISTORY • May 31, 2007; Makati Medical Center - sought consult for continuation of treatment - easy fatigability, shortness of breath, bipedal edema, orthopnea

  12. 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

  13. 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

  14. 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

  15. 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

  16. FAMILY MEDICAL HISTORY • (+) HPN – mother • (+) DM – mother • (+) heart disease – father • (-) asthma • (-) cancer

  17. PERSONAL & SOCIAL HISTORY • Non-smoker • Occasionally drinks • Retired architect

  18. 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

  19. 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

  20. 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

  21. 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

  22. 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

  23. ADMITTING DIAGNOSIS • Congestive Heart Failure secondary to Restrictive Cardiomyopathy secondary to Cardiac Amyloidosis • Hypertensive atherosclerotic disease • Diabetes Mellitus • Gout

  24. PROBLEM #1 SHORTNESS OF BREATH

  25. Chest USG • Result showed massive amount of anechoic free fluid in the right hemithorax with a volume of at least 1100cc.

  26. 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

  27. 4 DAYS POST PIGTAIL INSERTION

  28. PROBLEM # 2 CARDIAC AMYLOIDOSIS

  29. 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.

  30. 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.

  31. PROBLEM # 3 INCREASED CREATININE

  32. PROBLEM #4 RECURRENT PLEURAL EFFUSION

  33. FINAL DIAGNOSIS • Congestive Heart Failure secondary to Restrictive cardiomyopathy secondary to cardiac amyloidosis • Hypertensive atherosclerotic cardiovascular disease • Pleural effusion secondary to CHF

  34. Chronic renal insufficiency secondary to cardiac decompensation • Diabetes Mellitus • Gout

  35. DISCUSSION Heart Failure Right-sided Left-sided Cor pulmonale Constrictive pericarditis Tamponade RV infarction Restrictive cardiomyopathy Aortic regurgitation Post MI

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

  37. Usually results from increased stiffness of the myocardium • Causes pressure within the ventricles to rise precipitously with only small increase in volume

  38. Affects either or both ventricles • May cause symptoms and signs of R or L ventricular failure • Often R sided findings predominate

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

  40. RESTRICTIVE HEMODYNAMICS AMYLOID DEPOSITION INC STIFFNESS OF MYOCARDIUM INCREASED FILLING PRESSURE REDUCED FILLING VOLUME LOW CARDIAC OUTPUT CONGESTION

  41. RESTRICTIVE HEMODYNAMICS INCREASED FILLING PRESSURE REDUCED FILLING VOLUME LOW CARDIAC OUTPUT CONGESTION Bipedal edema, ascites, enlarged liver Easy fatigability, weakness, azotemia

  42. WHAT DOES AMYLOID DO TO THE HEART?

  43. Amyloid deposition can disturb the tissue architecture and lead to organ dysfunction • J Clin Pathol 2005; 58: 125-133

  44. WHAT HAPPENED TO OUR PATIENT?

  45. 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

  46. 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

  47. 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

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

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