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Clinico-Pathological Conference
Case Study

CPC #4: Tuesday, December 17, 2002
Hurd Hall, The Johns Hopkins Hospital
12:00 Noon

Clinical Discussant:   Edward Kasper, MD
Pathologist Resident:   Walter Klein, MD PhD
Pathologist:   Rene Rodriguez, MD
Moderator:   Charles Weiner, MD

Chief Complaint:
A 46-year-old male with nausea and vomiting status post heart transplant who collapsed in his bathroom.

History of Present Illness:
The patient was a 46-year-old male with a long history of diabetes mellitus type II and multiple myocardial infarctions between 1993 and 2000. In July 2001 he was diagnosed with ischemic dilated cardiomyopathy. In August 2001 he was admitted for an acute exacerbation of congestive heart failure and received a left ventricular assist device. A cardiac biopsy at the time demonstrated severe hypertrophy and interstitial fibrosis consistent with a transmural healed infarct (Figure 1). Shortly thereafter, he developed acute renal failure requiring continuous veno-venous hemodialysis and also suffered a cerebrovascular accident in the watershed areas of the frontal and parietal
cortex (left>right).

Figure 1
Figure 1

On 11/27/01, the patient underwent orthotopic heart transplantation. His post-transplant course was complicated by wound dehiscence, sepsis, aspiration, gastritis, pleural effusions, acute renal failure, and an upper gastrointestinal bleeding requiring embolization of a branch of the gastroduodenal artery. His post-operative echocardiogram (12/26/01) showed an ejection fraction of 70-75% with mild left ventricular hypertrophy. Multiple cardiac biopsies demonstrated only mild grade IA rejection (12/17/01, 12/27/01, 2/1/02, 2/14/02). The biopsy from 2/14/02, demonstrating mild (ISHLT grade 1A) rejection and Quilty type B infiltrates, is shown in Figure 2. He was discharged for rehabilitation on 2/2/02. An echocardiogram on 2/16/02 showed a decreased ejection fraction of 35-40%, along with mild concentric left ventricular hypertrophy, inferoseptal and anteroseptal hypokinesis.

Figure 2a

Figure 2b

The patient completed rehabilitation on 3/5/02. On 3/17/02, he had an episode of syncope in his bathroom along with intractable nausea and vomiting. He was taken to an outside hospital. At the outside hospital, his arterial blood gas revealed a pH 7.06, pCO2 14 mmHg, pO2 160 mmHg, and bicarbonate 4 mmol/l. Blood cultures were drawn and the patient was given broad-spectrum antibiotics (Levoquin) and intravenous fluids. He was transferred to JHH for further management.

Past Medical History:
Long standing diabetes mellitus type II, hypertension, coronary artery disease with four myocardial infarctions (1993-2000), s/p cerebrovascular accident (9/01) after placement of left ventricular assist device, s/p orthotopic heart transplant (11/27/01).

Social History:
Tobacco use, former executive.

Family History:
Unavailable upon transfer.

Allergies:
Penicillin, horse serum.

Physical Examination:
Patient is agitated with abdominal pain. Vital signs: BP 90/47, HR 114, RR 33, T 34.1°C. HEENT- anicteric sclerae, extraocular movements intact, well healed sternotomy scar, JVP increased (9 cm). Chest - decreased breath sounds left side, no crackles. Cardiac - RRR, no murmurs, gallops or rubs. Abdomen - good bowel sounds, increased tenderness over right upper quadrant, no guarding. Extremities – cool, cyanotic, no edema. Neurological – non-focal, able to move all four extremities, agitated but alert and oriented.

Laboratory Studies:
Sodium 137 mEq/L, Potassium 6.0 mEq/L, Chloride 94 mEq/L, Bicarbonate 5 mEq/L, creatinine 3.8 mg/dL, BUN 73 mg/dL, glucose 96 mg/dL, anion gap 44 mEq/dl, lactate 20.1 mmol/L, ALT 459 IU/L, AST 744 U/L, Alk phos 156 IU/L, total bilirubin 2.0 mg/dL, prothrombin time 27.1s, INR 5.3

CBC: WBC 18,240/mm3, Hgb 12.3 g/dL, Hematocrit 41.6%, Platelets 294,000/mm3.

Hospital Course:
A liver duplex ultrasound taken at the time of admission demonstrated normal blood flow in the hepatic artery, portal veins and hepatic veins and homogenous liver parenchyma without focal masses. The patient was intubated for respiratory distress, and a chest X-ray demonstrated atelectasis in the left lower lobe with a small left pleural effusion and moderate cardiomegaly. A spiral CT of the abdomen demonstrated bilateral pleural effusions and minimal ascites. Despite aggressive treatment with intravenous antibiotics, fluids, and pressors, the patient deteriorated with severe hypotension that was refractory to vasopressors and expired one day after admission to JHH.

What is the most likely cause of the patient's demise?

See Answer to CPC #4

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