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Morbidity and Mortality A# 50082309-1

Morbidity and Mortality A# 50082309-1. Elizabeth Kellogg Wolfe, M.D. November 21, 2001. What day is it today?. Monday Tuesday Wednesday Thursday Friday. History of the Present Illness 10/01. CC: Left lower extremity pain and dyspnea

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Morbidity and Mortality A# 50082309-1

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  1. Morbidity and MortalityA# 50082309-1 Elizabeth Kellogg Wolfe, M.D. November 21, 2001

  2. What day is it today? • Monday • Tuesday • Wednesday • Thursday • Friday

  3. History of the Present Illness10/01 • CC: Left lower extremity pain and dyspnea • HPI: 80 year old man with a complicated PMHx including pulmonary hypertension, atrial fibrillation, and hypertension who presents to the DHMC ED after awakening with acute worsening of his chronic SOB and left LE pain.

  4. Dyspnea Progressive exertional dyspnea for months New orthopnea 3# weight gain No chest pain/angina No cough or hemoptysis Given Furosemide 80 mg IV by paramedics Left LE pain Pain for decades Ambulatory until night prior to admission 10/10 ten pain in left ankle/tibia Shaking chills, no fever or sweats No change in LE swelling 80 yo man with acute on chronic SOB & LLE painHPI Continued

  5. 80 yo man with acute on chronic SOB & LLE painPast Medical History • Progressive exertional dyspnea • Dx with pulmonary hypertension, by echo 1997 • PFT’s 4/4/01: FVC 2.94 L (79% predicted), FEV1 1.98 L (84% predicted), FEV1/FVC 68% • Overnight oximetry normal 4/01 • Normal VQ scan 6/01 • Estimated PASP 79mmHg by Echo 8/29/01 • Home oxygen dependent since 8/2001

  6. 80 yo man with acute on chronic SOB & LLE painPMHx Continued • Possible Cardiomyopathy • ECHOCARDIOGRAM 8/29/01 • Borderline severe concentric LV hypertrophy • Possible restrictive cardiomyopathy • Calculated LVEF 65% • ASCVD • Sestamibi ETT 1/4/2000: inferoseptal scar with periinfarct ischemia • Chronic Atrial Fibrillation • Rate controlled, anticoagulated • Hypertension, well controlled

  7. 80 yo man with acute on chronic SOB & LLE painPMHx Continued • Chronic Renal Insufficiency • Baseline Cr =1.7 • Felt secondary to HTN • Hyperlipidemia • LLE fracture and shrapnel injury 1940’s • H/O LLE osteomyelitis • LLE varicose vein stripping1950’s and 1970’s

  8. Medications • Home oxygen at 2.5 L • Vitamin E 400 IU po qd • Warfarin 2.5 mg po qd • Toprol XL 100 mg po qd • Spironolactone 25 mg po qd • Atorvastatin 40 mg po qd • Furosemide 60 mg po bid Allergies: PCN caused diarrhea; Sulfa caused rash

  9. Social History • History of pipe smoking, quit at age 60 • EtOH: 6-8 oz of wine daily • Retired hotelier, now counsels for small businesses • Widowed in 1999 • Resident at Harvest Hill

  10. Family History • Father died of CHF at age 85 • Mother died at age 85; cause uncertain • One brother died of GI/Lung cancer • One brother alive in his 70’s

  11. Review of systems • Gen: Appetite unchanged. • CV: No increase in exertional dyspnea. No increase in exertional angina. No palpitations, presyncope, or syncope. No claudication. • Pulm: No URI sx’s. No pleuritic chest pain. • GI: No PUD/GIB/abdominal pain/diarrhea/ constipation. Some nausea with pain. • GU: No symptoms. • Neuro: No HA/focal symptoms.

  12. Physical Exam Vitals: T 37.7 HR 92-107, afib BP 84/51, RR 24, 99% on 6L Gen: In mild distress due to pain HEENT: PERRLA, EOMI, MMM, sclera non-icteric Neck: No bruits. No LAD/thyromegally. CVP 14-15 cm Resp: Crackles 1/3 up B/L. No wheezes. CV: Irregular. Distant HS. Soft 2/6 systolic murmur at LSB/apex. No S3/S4. Pulses symmetrically 2+. Abd: Soft, NT, no masses. NABS. Ext: + Heberden’s nodes in DIP joints of hands. No other joint swelling. LLE: Erythema, warmth and tenderness over anterior tibia extending towards thigh. No palpable cords. + varicosities. 2+ edema b/l. Neuro: Non-focal.

  13. Admission Labs 13.2 MCV 94.8138 101 65 9.4 131 RDW 14.44.4 24 2.4 38.3 Ca 9.3 Mg 0.89 Phos 4.2 PT 29.3 INR 4.1 PTT 41 D-dimer 376 Fibrinogen 410 ABG: 7.46 / 28 / 101 / 98.3% on 6L CPK 134 Trop 0.14 115 1.7

  14. Admission Tests • EKG: Atrial fibrillation with RVR (Vent rate 102), RBBB, Septal infarct (old). • CXR: Show

  15. ED Course • Pt felt significantly improved on arrival following IV furosemide • MSO4 2 mg IV for pain • Blood and urine cultures • Clindamycin 900 mg IV given • BP 61/40  started on dopamine • Admitted to CCU

  16. Assessment/Plan • LLE pain/erythema • Cellulitis v. thrombophlebitis v. osteomyelitis • Pulmonary edema • Likely due in part to restrictive cardiomyopathy. • Symptomatically improved with furosemide, MSO4 • Hypotension • Multifactorial – heart failure, ? Early sepsis, PE less likely

  17. Would you place a pulmonary artery catheter at this time? • Definitely • Maybe • Not sure • Probably not • No

  18. Assessment/Plan, continued • Acute renal failure • Prerenal azotemia secondary to CHF & hypotension • Urinalysis, urine lytes, osm • Renal ultrasound • Elevated troponin • ? Rate related ischemia – follow serial enzymes • ASA 81 mg po qd • Hold on ACE-inhibitor given renal failure • Rate control with ß-blocker given cautiously

  19. Assessment/Plan, continued • Atrial fibrillation • INR supratherapeutic • Wean dopamine/Beta blocker • Pulmonary Hypertension • Reassess with repeat Echocardiogram • Supratherapeutic INR • No evidence of active bleeding • Hold coumadin

  20. Hospital Day #1 • LLE erythema • Afebrile • Blood cultures x2 drawn • Switched to IV Nafcillin • X-rays done: No evidence of osteomyelitis • Pulmonary • Symptomatically improved; Pulse ox mid-90’s on 4L • Hypotension • NS fluid boluses – 1 liter total • Continued on Dopamine gtt 5-8 mcg/kg/min • Renal failure • Renal Ultrasound done

  21. LLE pain/erythema improved; afebrile Converted to NSR BP 70’s-80’s/40’s-50’s on dopamine Anuric Labs: 132 100 74 6.2 15 2.9 AG = 17 WBC 14.8 INR 6.2 PTT 51 Assessment/Plan: LLE Cellulitis: continue nafcillin Cardiac: Continue dopamine, plan for swan and echo Renal: Consult, treat hyperkalemia Coagulopathy: hold anticoagulants Hospital Day #2

  22. Echocardiogram

  23. Which of the following is associated with a poor prognosis in systemic amyloidosis? • Number of organ systems involved • Nephrotic syndrome • Presence of CHF • Time from onset of symptoms to diagnosis • Primary vs. secondary amyloidosis

  24. Number of organ systems involved • Nephrotic syndrome • Presence of CHF • Time from onset of symptoms to diagnosis • Primary vs. secondary amyloidosis

  25. Survival for 810 patients with systemic amyloidosis seen at the Mayo Clinic between 1/1/66 and 3/1/97 Kyle et al. Blood 93(3): 1062-66, February 1, 1999

  26. Subjectively improved. Anuric on dopamine. Now requiring 6L O2 Exam o/w unchanged. CI 1.8, PAOP 29, CVP 9-25 Labs: 135 102 92 5.6 18 4.2 PT >60 PTT 112 Tbili 8.7, Dbili 6.8, AlkP 72, AST 63, ALT 43 Cultures negative Assessment/Plan: Cardiac: continue dopamine gtt Renal: CVVH planned Coagulopathy: Vit K LLE Follow cultures, continue nafcillin End of life discussions Hospital Day #3

  27. Hospital Day #4 • Increasing oxygen requirement  100% facemask • Patient somnolent, but arousable, overnight • Patient brought outside

  28. Hospital Day #4, continued • Discussions with DPOA • Dopamine discontinued • Comfort measures only • Patient died comfortably • DPOA consented for autopsy

  29. Morbidity and MortalityA# 50082309-1Pathology Shaofeng Yan, M.D., Ph.D. November 21, 2001

  30. Heart - firm, dark brown

  31. Amyloid deposition in the heart Myocytes Amyloid Congo red stain H+E stain

  32. Amyloid deposition in the heart Apple-green birefringence with polarlized light Congo red stain

  33. Amyloid deposition in the lung Congo red stain H+E stain

  34. Amyloid deposition in the adrenal Congo red stain H+E stain

  35. Amyloid deposition in the kidney Congo red stain H+E stain

  36. Amyloid deposition in the liver Congo red stain H+E stain

  37. Amyloid deposition in the spleen Congo red stain H+E stain

  38. Tibial skin inflammation and amyloid deposition Congo red stain H+E stain

  39. Pseudomembranous colitis

  40. Final Pathologic Diagnosis I. Systemic amyloidosis: A. Amyloid cardiomyopathy 1. Biventricular dilatation and hypertrophy 2. Congestive heart failure 3. Atrial fibrillation (clinical) B. Lungs 1. Pulmonary hypertension 2. Pulmonary congestion C. Deposition also in kidneys, spleen, skin, GI, thyroid, pancreas, bladder II. Atherosclerosis : A. Mild CAD III. Pseudomembranous colitis IV. Acute subcutanous infection V. Benign prostate hyperplasia

  41. Treatment for Primary Amyloidosis • Treatment remains unsatisfactory. • Melphalan based therapy has been the treatment of choice for decades. • One randomized trial: Blood, 1978 • Melphalan - Prednisone (MP) vs. placebo. • Findings: • Patients receiving MP treated for a longer period before code broken (4.5mo vs 6.5 mo) • Trend toward improvement in proteinuria • Trend toward reduction in marrow plasma cells • No survival benefit found.

  42. Treatment for Primary Amyloidosis MP C MPC Kyle et al. NEJM 366 (17) : 1202 –07. April 24, 1997

  43. Treatment for Primary Amyloidosis • Ablative chemotherapy with bone marrow or stemcell transplantation. • Strict eligibility criteria • High mortality with treatment • Impressive longterm survival for those tolerating transplantation • No randomized controlled trials done todate

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