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Three days of dyspnea and increased edema.
History of Present Illness
The patient was a 47-year-old woman with a past medical history significant for cirrhosis, diabetes, and chronic pancreatitis. She never received a liver biopsy, but because of a long history of alcohol abuse was presumed to have alcoholic cirrhosis. In 2/2004, she was first admitted to The Johns Hopkins Hospital for esophageal varices and ascites. She underwent successful transjugular intrahepatic portal shunt (TIPS) placement. During this admission, she developed acute renal failure and an episode of bradycardic arrest. In 3/2004, the patient had an echocardiography, which showed an ejection fraction of 60%. In 7/2007, she was diagnosed with hepatocellular carcinoma. Shortly thereafter, she was admitted to The Johns Hopkins Hospital and underwent successful hepatic chemoembolization.
The patient continued to have worsening liver function, and she was placed on the transplant recipient list. On 2/21/2008, she was admitted to The Johns Hopkins Hospital and underwent successful orthotopic liver transplantation. During this admission, echocardiography identified decreased systolic function with an ejection fraction of 25%. Her explanted liver showed advanced micronodular cirrhosis with marked hepatocellular and bile duct hemosiderin. At the chemoembolization site there was focal necrosis and fibrosis, no evidence of malignancy. Her post-operative course was unremarkable and she recovered well and was discharged from Hopkins. On follow- up in clinic, she developed hyperkalemic renal insufficiency and edema. She was readmitted to the Johns Hopkins Hospital in March and also in April of 2008 for failure to thrive, renal insufficiency, and leg edema. Echocardiography at that time identified distal septal and apical hypokinesis with an ejection fraction of 35%.
On 6/16/2008, the patient was transferred to The Johns Hopkins Hospital from an outside institution following three days of dyspnea and increased edema. In the outside hospital, she was hypotensive with systolic pressures in the 80s and diastolic pressures in the 50s. She was also hypothermic with temperatures as low as 33°C with hypoglycemia.
Past Medical History
1. ESLD s/p TIPS in 2/08, history of variceal bleeding, hepatic encephalopathy,
hepatic
hydrothorax s/p R pleurodesis
2. HCC diagnosed 2007 s/p RFA
3. Orthotopic liver transplant on 2/21/2008 (recipient and donor CMV negative).
4. Post-transplant course complicated by acute renal failure in 3/2008 (Cr
in the 2 range) due to Prograf, Klebsiella oxytoca bacteremia 4/12/2008,
5. Cardiomyopathy-recent diagnosis
6. Hypothyroidism- >5 years
7. Diabetes- Type II; >5 years
Family History
There is no prior known history of liver disease or Cardiomyopathy. Her father had diabetes and hypertension.
Social History
She smokes 1/2 pack per day but says she is trying to quit. She was a heavy alcohol drinker up until 02/04. She has stopped completely ever since. She had tried pot at a younger age but has not used illicits otherwise. She worked for a restaurant up until her hospitalization in 02/04. She is a widow and she has 2 healthy children. She lived with her children in Jessup, but now lives with her sister who is her primary caretaker and aide. She has no pets and has not travelled recently.
Medications (6/22)
Levothyroxine
Pantoprazole
Hydrocortisone
DRIPS:
Levophed (2mcg/Kg/min)
Vasopressin (0.04 - stable)
Dobutamine (100 mcg/Kg/min)
ANTIBIOTICS:
Ambisome 300 mg q24 (5mg/Kg/q24) 6/20 - now
Vancomycin dosed by levels now 6/16 - now
Cefepime 500 mg q12 6/21 - now
(On admission the patient was on prophylactic fluconazole, dapsone and
valcyte)
IMMUNOSUPPRESSION:
Sirolimus 0.5 mg daily
Allergies
Codeine
Review of Systems
Could not be obtained
Physical Exam (6/22)
General: intubated, alert, follows commands, no acute distress
Vitals: Tc 35.6, Tmax 35.6, BP 80s/60s, HR 100-120s, RR 24, O2 sat 97%, Weight
58 Kg (has gained 9
lbs since admission)
HEENT: ETT in place, icteric, pale conjuctiva, no sinus tenderness, L IJ CVC
clean, supple neck,
moist mucous membranes
Pulmonary: coarse breath sounds bilaterally, no wheezing, no rales
Cardiac: tachycardic, regular, normal S1 and S2, systolic murmur in apex, S3
present
Abdomen: soft, distended, nontender, bowel sounds decreased, abdominal wall
edema, no Murphy
sign
Extremities: anasarca, extremities cool with thread pulses, distal fingers/toes
slightly
blue
Lymph nodes: no lymphadenopathy
Laboratory Values (6/22)
WBC: 7.7 (85% N)
Hgb: 7.8
PLTs: 18,000
Fibrinogen: 139
PT/PTT/INR: 35.1/44/4
ABG: 7.32/31/126
BuN/Cr: 31/1.2 (Cr peaked at 2.8 - was 2.1 on admission)
CO2: 15
Anion gap: 22
AST: 7141
ALT: 1236
ALP: 409
TBili: 2.2
Alb: 1.9
Ammonia: 65
Lactic acid: 9.6
AfP < 1
CK: 432
Troponin: 1
TSH: 0.7
CMV PCR- negative
DRUG LEVELS:
Rapamycin: 8.1
MICRO LABS:
all cultures negative
EKG
SINUS TACHYCARDIA
RIGHT BUNDLE BRANCH BLOCK
LEFT POSTERIOR FASICULAR BLOCK
BIFASCICULAR BLOCK
Radiologic Studies
See Images
Clinical Course
After admission to the Johns Hopkins Hospital Surgical ICU, she was given antibiotics for possible sepsis, and then was transferred to the floor. All cultures remained negative. However, her blood pressure remained low. On 06/19/08, a cardiology consult did a bedside echo showing a large left pleural effusion and an ejection fraction of 10%. She was then transferred to the CCU for hypotension and cardiogenic shock. A PA catheter demonstrated low cardiac output and high right atrial pressure. A pulmonary capillary wedge pressure could not be obtained. During the rest of her hospitalization, she was routinely hypotensive requiring pressor support. On 6/21/08 she had an episode of cardiac arrest due to PEA/V-tach. She was resuscitated and intubated. Following this episode, she developed severe renal failure and was started on continuous venovenous hemodialysis. Despite these measures, her condition rapidly deteriorated. On 6/27/2008, she had a second PEA cardiac arrest. Cardiopulmonary resuscitative efforts this time were unsuccessful, and she expired at 1641.
Questions
1. What caused her progressive heart failure?
Video link of Images 13
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| See Answer to CPC |
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