2017 Annual Meeting

Evening Specialty Conference
Cardiovascular Pathology

The Cardiovascular System in Systemic Diseases

March 7 2017, 7:30pm to 9:30pm

Moderators:
Henry Tazelaar, MD and Robert Padera, MD
Panelists:
Gayle Winters, MD - Brandon Larsen, MD, PhD - Mike Seidman, MD, PhD - Marc Halushka, MD, PhD

Pre-Meeting Materials

Case 1

Faculty:
Gayle Winters, MD

Clinical History:

The patient is a 44 year old woman who presented with a 1-day history of nausea, fatigue, and fever. She denied chest pain, dyspnea, cough, orthopnea, or lower-extremity edema. An ECG was normal. She never smoked. Her medical history included obesity, depression, and hyperlipidemia and she had been scheduled for elective gastric bypass surgery. The following day she developed aching pain in her chest and jaw. She was tachycardic and dyspneic. Her ECG now had ST-segment elevation in inferior and apical leads that was new compared to her ECG 24 hours earlier. Her troponin I level was 19 ng/ml. Cardiac catheterization revealed normal epicardial coronary arteries. Echocardiogram demonstrated multiple wall motion abnormalities with an ejection fraction of 30%. Cardiac MRI revealed biventricular systolic dysfunction (LV ejection fraction ~20%) with diffuse myocardial necrosis. A myocardial biopsy was performed. Three days later, due to progressive hemodynamic collapse, bilateral ventricular assist devices were inserted as a bridge to recovery or transplantation.

Morphological Findings (click to enlarge):

Figure 1 Right Ventricular Endomyocardial Biopsy
Figure 2 Right Ventricular Endomyocardial Biopsy
Figure 3 Right Ventricular Endomyocardial Biopsy
Figure 4 LVAD Core - LV Apex
Figure 5 LVAD Core - LV Apex
Figure 6 Right Atrium

Case 2

Faculty:
Brandon Larsen, MD, PhD

Clinical History:

A 61-year-old previously healthy man presented with a 4-month history of progressive fatigue, lower extremity edema, and dyspnea. After admission to the hospital, an imaging workup revealed a moderate-sized pericardial effusion, bilateral pleural effusions, and interlobular septal thickening in the lung bases, and pericardial, lung, and pleural biopsies were obtained.

Morphological Findings (click to enlarge):

Figure 1 Lung 12.5x
Figure 2 Lung 40x
Figure 3 Pericardium 20x
Figure 4 Pericardium 100x
Figure 5 Pericardium 200x
Figure 6 Pericardium 400x
Figure 7 Pericardium CD1a IHC 100x
Figure 8 Pericardium CD68 IHC 100x
Figure 9 Pericardium Factor XIIIa IHC 100x
Figure 10Pericardium S100 IHC 100x

Case 3

Faculty:
Mike Seidman, MD, PhD

Clinical History:

A 60 year old Caucasian man was referred to cardiac surgery for findings of enlarged aortic root (68 mm), enlarged ascending aorta (49 mm), aortic insufficiency, left ventricular dilation, and functional mitral regurgitation, resulting in a left ventricular ejection fraction of 30 percent with symptoms of heart failure. These were discovered during a workup several months prior, originally ascribed (incorrectly) to pneumonia. He had no evidence of significant coronary artery disease. The patient's past medical history was notable for osteoarthritis, psoriasis (not on any oral medications), and a remote history of urothelial carcinoma of bladder status post surgery and BCG therapy. For almost 20 years, the patient's cystoscopy and cytology had been negative for malignancy, although he had been diagnosed with a chronic eosinophilic cystitis that was persistent. The patient was on appropriate heart failure medications and a proton pump inhibitor for dyspepsia. The patient worked as an artist. He was an active 30 pack-year smoker (at time of evaluation smoking half a pack per day), and formerly had 2-4 drinks of alcohol per week but was now abstaining in light of his heart failure diagnosis. He had no history of illicit drug use. His parents died in their old age, and there was no extended family history of significant cardiovascular or other major systemic disease. The patient was divorced, with three living adult children, all of whom were quite tall. The patient and his children had been investigated for Marfan syndrome or similar, but these were excluded by multiple specialists. The patient underwent a valve sparing David procedure aortic root replacement and mitral valve annuloplasty. Findings from the aortic specimen are shown.

Pertinent Laboratory Data:

  • WBC 8.0 x 109/L (ref range 4-11)
  • RBC 3.44 x 1012/L (ref range 4.2-5.8)
  • Hgb 98 g/L (ref range 135-170 Hct 0.31 (ref range 0.4-0.5)
  • Plts 358 x 109/L (ref range 150-400)
  • INR 1.2 (ref range 0.9-1.2)
  • APTT 34 s (ref range 25-38)
  • BNP 765 ng/L (ref range <40)
  • CRP 5.6 mg/L (ref range <3.1)
  • blood culture negative
  • urine culture negative
  • PPD negative
  • T. pallidum antibody EIA negative
  • IgG 21.90 g/L (ref range 7.00 -16.00)
  • IgG1 8.61 g/L (ref range 2.80 - 8.00)
  • IgG2 10.10 g/L (ref range 1.15 - 5.70)
  • IgG3 1.08 g/L (ref range 0.24 - 1.25)
  • IgG4 0.62 g/L (ref range 0.05 - 1.25)
  • ANA 1.2 U (ref range <1.0)
  • RF 94 IU/mL (ref range 0-15)
  • anti-CCP negative
  • ANCA negative
  • C3 normal
  • C4 normal

Morphological Findings (click to enlarge):

Figure 1 Low power H&E
Figure 2 High power H&E focused on the adventitia
Figure 3 Movat pentachrome stain
Figure 4 CD68 staining, media
Figure 5 CD68 staining, adventitia
Figure 6 IgG4 staining, low power
Figure 7 IgG4 staining, high power, media
Figure 8 IgG4 staining, high power, adventitia

Case 4

Faculty:
Marc Halushka, MD, PhD

Clinical History:

A 46 year old white female presents to the operating room with an aortic aneurysm and a bicuspid aortic valve with severe stenosis by echocardiogram. She previously had subaortic stenosis which was operated on ~35 years prior. The patient is seen frequently by her primary care physician. At surgery a recurrent subaortic membrane was noted and resected. Then the ascending aorta and aortic valve were removed and replaced with a St. Jude composite graft.

Morphological Findings (click to enlarge):

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