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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

Morbidity and MortalityA# 50082309-1

Elizabeth Kellogg Wolfe, M.D.

November 21, 2001


What day is it today
What day is it today?

  • Monday

  • Tuesday

  • Wednesday

  • Thursday

  • Friday


History of the present illness 10 01
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.


80 yo man with acute on chronic sob lle pain hpi continued

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


80 yo man with acute on chronic sob lle pain past medical history
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


80 yo man with acute on chronic sob lle pain pmhx continued
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


80 yo man with acute on chronic sob lle pain pmhx continued1
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


Medications
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


Social history
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


Family history
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


Review of systems
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.


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.


Admission labs
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


Admission tests
Admission Tests

  • EKG: Atrial fibrillation with RVR (Vent rate 102), RBBB, Septal infarct (old).

  • CXR: Show


Ed course
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


Assessment plan
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


Would you place a pulmonary artery catheter at this time
Would you place a pulmonary artery catheter at this time?

  • Definitely

  • Maybe

  • Not sure

  • Probably not

  • No


Assessment plan continued
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


Assessment plan continued1
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


Hospital day 1
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


Hospital day 2

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



Which of the following is associated with a poor prognosis in systemic amyloidosis
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


  • Number of organ systems involved in systemic amyloidosis?

  • Nephrotic syndrome

  • Presence of CHF

  • Time from onset of symptoms to diagnosis

  • Primary vs. secondary amyloidosis


Survival for 810 patients with systemic amyloidosis in 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


Hospital day 3

Subjectively improved. in systemic amyloidosis?

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


Hospital day 4
Hospital Day #4 in systemic amyloidosis?

  • Increasing oxygen requirement  100% facemask

  • Patient somnolent, but arousable, overnight

  • Patient brought outside


Hospital day 4 continued
Hospital Day #4, continued in systemic amyloidosis?

  • Discussions with DPOA

    • Dopamine discontinued

    • Comfort measures only

    • Patient died comfortably

    • DPOA consented for autopsy


Morbidity and Mortality in systemic amyloidosis?A# 50082309-1Pathology

Shaofeng Yan, M.D., Ph.D.

November 21, 2001


Heart - firm, dark brown in systemic amyloidosis?


Amyloid deposition in the heart in systemic amyloidosis?

Myocytes

Amyloid

Congo red stain

H+E stain


Amyloid deposition in the heart in systemic amyloidosis?

Apple-green birefringence

with polarlized light

Congo red stain


Amyloid deposition in the lung in systemic amyloidosis?

Congo red stain

H+E stain


Amyloid deposition in the adrenal in systemic amyloidosis?

Congo red stain

H+E stain


Amyloid deposition in the kidney in systemic amyloidosis?

Congo red stain

H+E stain


Amyloid deposition in the liver in systemic amyloidosis?

Congo red stain

H+E stain


Amyloid deposition in the spleen in systemic amyloidosis?

Congo red stain

H+E stain


Tibial skin inflammation and amyloid deposition in systemic amyloidosis?

Congo red stain

H+E stain


Pseudomembranous colitis in systemic amyloidosis?


Final Pathologic Diagnosis in systemic amyloidosis?

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


Treatment for Primary Amyloidosis in systemic 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.


Treatment for Primary Amyloidosis in systemic amyloidosis?

MP

C

MPC

Kyle et al. NEJM 366 (17) : 1202 –07.

April 24, 1997


Treatment for Primary Amyloidosis in systemic 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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