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Clinico-Pathological Conference
Case Study

CPC #5: Tuesday, January 13, 2009
Hurd Hall, The Johns Hopkins Hospital
Cardiac at 12:00 PM

Clinical Discussant: Steven Jones, M.D.
Radiologist: Jens Vogel-Claussen, MD
Pathology Resident: Andrea Subhawong, MD
Pathologist: Charles Steenbergen, MD PhD
Moderator: Charles Wiener, MD
Chief Complaint

Progressive shortness of breath


History of Present Illness

The patient is a 50 year old Caucasian male who began to develop shortness of breath and
worsening exercise tolerance in 2000. His medical history was most significant for
Hodgkin's lymphoma treated with mediastinal radiation in childhood, as well as asthma
and hypercholesterolemia. A cardiac catherization in July of 2000 revealed single vessel
coronary artery disease with 60% proximal right coronary artery stenosis, mild to
moderate aortic stenosis, and normal left ventricular function.

His symptoms were treated medically until 2003 when his exercise intolerance became
exacerbated and he began to notice fluid retention in his legs. A repeat cardiac
catheterization at that time showed 60-70% stenosis of the left main coronary artery, 70%
stenosis of the circumflex at the ostium, and 70% proximal right coronary artery stenosis,
mild to moderate aortic stenosis, and an ejection fraction of 50% which was less than at
his prior catheterization.

He underwent triple coronary artery bypass and an aortic valve replacement in 2004, with
improvement in symptoms. In early 2007, he once again began to notice fluid retention
and increasing fatigue. An echocardiogram in June 2007 showed severe dilation of the
right ventricle with estimated right ventricle systolic pressure of 75 mm Hg, severe mitral
valve calcification with regurgitation, and moderate pulmonic valve regurgitation. In the
week prior to admission he reported that he had difficulty climbing one flight of stairs
due to shortness of breath. He used 2-3 pillows to sleep and did note difficulty lying flat.
He denied any rest or exertional chest pain, palpitations, heartburn, lightheadedness,
cough, or loss of consciousness. He was admitted to the JHH for diuresis and further
evaluation and management.


Past Medical History

Hypothyroidism
Gastroesophageal Reflux
Osteoporosis
Gout
Adrenal Insufficiency


Family History

The patient's mother died of breast cancer. His father is alive with hypertension and
coronary artery disease.


Social History

The patient works as a pharmacist. He lives with his wife, who is a physician, and two
cats. They have no children. The patient does not smoke, and rarely uses alcohol.


Medications

(prior to current admission)

Coumadin 2.5 mg daily
Fluticasone twice daily
Spironolactone 25 mg daily
Lipitor 40 mg daily
Synthyroid 112 mcg daily
Furosemide 40 mg in morning and 80 mg in evening
Ranitidine 300 mg daily
Carvedelol 3.125 mg twice daily
Aspirin 81 mg daily
Alendronate 70 mg weekly


Allergies

No known drug allergies


Review of Systems

The patient reports a recent 5 pound weight gain and increase in lower extremity edema.
He has worsening fatigue recently. He denies fevers, night sweats, chills, sputum
production, or epistaxis.


Physical Exam

  • T:36.5 BP:106/70 P:90 RR:10 SaO2: 88% on room air, 96% on 4L NC
  • General: Obese Caucasian male in no acute distress, awake and alert.
  • HEENT: Normocephalic, atraumatic, extra-ocular movements intact, sclera
    anicteric, mucosal membranes moist. Marked jugular venous distension to mid-
    upper neck, appreciable even when sitting upright.
  • CV: Regular rhythm with mechanical S2 and a 3/6 systolic murmur heard best
    along the left sternal border radiating to the apex.
  • Lungs: No audible crackles wheezes or rhonchi
  • Abdomen: Soft, distended, non-tender, with a slight fluid wave. Liver palpable
    and pulsatile 3-5 cm below right costal margin. No fluid wave or shifting
    dullness.
  • Extremities: No clubbing or cyanosis. 3+ pitting edema of bilateral lower
    extremities to the thighs. Sluggish capillary refill.
  • Neuro: Alert and oriented x 3, cranial nerves II-XII intact, sensation intact to fine
    touch, normal muscle bulk and tone, coordination grossly normal.


Laboratory Values

  • Na 129, K 3.8, Cl 89, HCO3 25, BUN 47, Cr 1.3, Glu 131, Calc 9.8, INR 1.5
  • WBC 8640, Hct 34.7%, Plt 264


ECG


Clinical Course

During the initial days of admission, despite increasing doses of intravenous diuretics, it
was difficult to maintain increased urinary output and his creatinine rose from 1.3 to 2.5
mg/dl. A Swan-Ganz catheter was placed and milrinone was added to his diuretic
regimen. While on milrinone (0.25 mcg/kg/min), he had:

RA pressure                                                    27 mmHg
RV pressure                                                    67/29 mmHg
PA pressure                                                    67/31
Pulmonary capillary wedge pressure         31 mmHg (mean)
Systemic arterial pressure                           95/70 mmHg
Cardiac output                                                4.4 L/min
Cardiac index                                                  2.4 L/min/m2
SvO2                                                                 60%

On that regimen the patient diuresed approximately 8-10 kg, his creatinine returned to
baseline, and his exertional dyspnea improved slightly. He was transferred out of the
cardiac care unit when the Swan-Ganz catheter was removed. The day prior to planned
discharge he complained of increasing shortness of breath and was found unresponsive in
bed. He was found to have pulseless electrical activity and resuscitation was
unsuccessful.


Radiologic

See Images 1-6

Image 1: 2003 Axial CT: Aortic root and LAD calcifications
Image 2: 2003 Axial CT: main and left main pulmonary artery calcifications
Image 3: 2003 MIP CT: Aortic root and arch calcifications
Image 4: 2003 Short axis cine cardiac MRI: Moderate aortic valve stenosis and regurgitation
Image 5: 2003 Short axis cine cardiac MRI: Moderate mitral valve regurgitation
Image 6: 2004 Post op: Aortic valve repair and CABG.


Questions

What are possible causes of the patient's increasing peripheral edema and worsening exercise tolerance?
What is the most likely cause of the increase in creatinine?
What factors contributed to the patient's coronary artery disease?


Images Click on an image below to enlarge.

Image 1
Image 2
Image 3
Image 4
Image 5
Image 6

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