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Clinico-Pathological Conference
Case Study

CPC #5: Tuesday, January 04, 2005
Hurd Hall, The Johns Hopkins Hospital
Pulmonary at 12:00 PM

Clinical Discussant: Steve Yang, MD
Radiologist: Jack Scatarige, MD
Pathology Resident: Chung Shum, MD, PhD
Pathologist: William Westra, MD
Moderator: Charles Wiener, MD
Chief Complaint

A 71-year-old man with a 4-month history of worsening shortness of breath was transferred to The Johns Hopkins Hospital from an outside hospital.

History of Present Illness

The patient was a 71 year old Caucasian male with a 100-pack-year history of tobacco abuse, hypertension, coronary artery disease, congestive heart failure, and chronic obstructive lung disease who presented to an outpatient clinic at an outside hospital with a four-month history of worsening symptoms of congestive heart failure. He noted increased dependent edema in both of his legs and progressively worsening shortness of breath with orthopnea and paroxysmal nocturnal dyspnea. He specifically denied a history of previous myocardial infarction or angina.

On that day, the patient refused to be admitted to the hospital for treatment of his symptoms; however, he relented the following day and was admitted to the outside hospital with a presumptive diagnosis of biventricular congestive heart failure. On a two-dimensional echocardiogram, a 5.2 x 1.9 cm mass, presumed to be an atrial myxoma, was found occupying 80% of the left atrium. The patient was transferred to The Johns Hopkins Hospital for resection of this tumor.

Past Medical History

Hypertension
Coronary artery disease
Congestive heart failure
Chronic obstructive pulmonary disease

Past Surgical History

appendectomy (20 years ago)
hernia repair (2002)

Medications

(at home prior to admission to JHH)
diltiazem, metoprolol

Social History

Quit smoking 20 years ago after 100-pack-years of tobacco use.

Family History

Non-contributory

Allergies

NKDA

Physical Exam on Admission

General: 84.5 kg, alert, affable, no acute distress
Vital Signs: 138/66, HR 86, RR 24, O2 sat 98% 3L NC
HEENT: PERRL, EOMI, oropharynx clear, small oral aperture
Neck: JVP to angle of jaw, 2+ carotid pulses, full range of motion
Cardiac: RRR, normal S1 S2, distant heart sounds
Chest: I:E 1:4, diffuse end-expiratory wheeze, decreased breath sounds right base
Abdomen: bowel sounds present, firm, nontender, nondistended, no hepatosplenomegaly
GU: redness and “fungal looking” rash on penis and scrotum
Skin: epidermolysis of extremities, erythematous and edematous to shins; nonpalpable pulses in feet but warm; moves without pain
Neurological: CNII-XI grossly intact, strength intact throughout

Laboratory Values on Transfer

Na 135, Cl 85, K 4.6, HCO3 41, BUN 15, Cr 0.8, Glu 88, Ca 9.1
pH 7.38, pCO2 81, pO2 43, calculated HCO3 48 (room air)
TP 5.6, Alb 3.3, Bili 0.5, LFTs nl
WBC 9090, Hgb 13.9, Hct 46.6, Plt 324, MCV 75.4, RDW 20.5
Coags nl, UA nl

Radiology

Echocardiogram- 5.2 x 1.9 cm mass occupying 80% of the left atrium
CXR- right pleural effusion (Image 1)
Chest CT- The positive findings include right pleural effusion, right lower lobe atelectasis, right paratracheal lymphadenopathy, subcarinal mass, and small left pleural effusion (Image 2 and 3). Thoracic computed tomography image 5 cm caudal to Image 3 reveals occlusion of bronchus intermedius, collapsed right lower lung, and a filling defect in the left atrium (Image 4).

Electrocardiogram

A left atrial abnormality, right axis deviation, incomplete right bundle branch block, and possible right ventricular hypertrophy. An anteroseptal infarct of indeterminate age could not be ruled out.

Ultrasound

No evidence for deep vein thromboses were found.

Cardiac Catheterization

Elevated right heart and wedge pressure (35 mm Hg), 30-50% ostial left main artery narrowing with a 70% occlusion in the mid-left anterior descending artery (LAD), LAD with a marginal distribution and one large diagonal. First left circumflex (LCX) marginal artery with proximal 70% occlusion, LCX itself free of disease. Small non-dominant right coronary artery. Good left ventricular function with ejection fraction of 50-55%, no wall motion abnormalities, no left ventricular dilation, no mitral regurgitation, mild mitral valve prolapse.

Clinical Course

A thoracentesis was performed to remove the fluid from the large right pleural effusion. The thoracentesis produced 700 cc of bloody fluid which contained 570 white blood cells per cubic millimeter, innumerable red blood cells, a neutrophil to monocyte ratio of 41% to 59%, protein 2.0 g/dl, pH 7.47, albumin 1.3 g/dl, amylase 48 u/l, and lactate dehydrogenase 71 IU/l. No bacteria were seen microscopically, and cytopathology results showed no malignancy in the pleural fluid.

Shortly after the thoracentesis, a chest x-ray was taken and a 2.5 cm pneumothorax at the right apex was found. Later that evening, the patient was cyanotic and had pulseless electrical activity. Cardiopulmonary resuscitation was attempted with intubation and placement of a chest tube that drained 2 L of bloody fluid. Despite vigorous efforts at resuscitation, an echocardiogram showed no cardiac wall motion. The patient expired and, with permission, complete autopsy was performed.

Questions

What was the cause(s) of the patient's lung and heart findings?
What was the most likely cause of death?

Images Click on an image below to enlarge.

Figure 1
Figure 2
Figure 3
Figure 4

See Answer to CPC

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