Moderator:

Gayle L. Winters
Brigham and Women's Hospital
Boston, Massachusetts

Case 1

submitted by:
Allen P. Burke
Armed Forces Institute of Pathology
Washington, D.C.

Clinical Summary:

A 60 year old white male, 5'11", 180 lbs., complained of chest pain one day prior to being found unresponsive on the ground in front of his home. He had a history of thyroid problems, asthma, and recent sinus infection. He was taking anti-allergy medicines [Advair Discus (fluticasone/salmeterol), Allegra-D (fexofenadine & pseudoephedrine), Atrovent (ipratropium bromide), Nasonex (mometasone furoate), and Singulair (montelukast)], an antibiotic [Cefzil (cefprozil)], and Synthroid (levothyroxine). At autopsy, his heart weighed 400 grams; the foramen ovale was probe patent; left ventricular chamber dimensions were normal; there was mild right ventricular hypertrophy (6 mm thick at posterior wall); and there were focal myocardial scarring and hyperemia in the posteroseptal left ventricle and posterior right ventricle. A section of the proximal right coronary artery is available for review.

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Case 2

submitted by:
Bruce M. McManus
University of British Columbia
Vancouver, British Columbia

Clinical Summary - Case A:

A 54-year-old female dentist had a 5-day history of 'flu-like' symptoms (including cough, fatigue, shortness of breath chills and malaise). On admission to hospital for shortness of breath and an inability to sleep, she was found to have left ventricular failure, cardiogenic shock and marked hypoxemia. She then developed uncontrollable cardiac arrhythmias, became profoundly hypotensive, terminating in ventricular tachycardia and death on the same day.

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Clinical Summary - Case B:

A 27-year-old female was admitted to hospital with a 5 day history of exertional chest pain and dyspnea. Her initial ECG demonstrated ST elevation in the anteroseptal leads, ST depression laterally, complete atrioventricular block and frequent premature ventricular complexes. She deteriorated clinically during the first 48 hours, with worsening of hypotension and heart failure requiring inotropic support and intubation. Novel therapies resulted in improvement of the patient's condition by 6 days after initial presentation.

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Case 3

submitted by:
Henry D. Tazelaar
Mayo Clinic
Rochester, Minnesota

Clinical Summary:

A 79 year-old woman with reactive airways disease and systemic hypertension presented with increasing dyspnea, fever and cough. A chest radiograph showed bilateral apical infiltrates and a dilated aorta. Sputum cultures grew H. influenzae. Subsequent transesophageal echo showed a 5.3 cm diameter ascending aortic aneurysm and marked aortic insufficiency. She underwent surgery at which time a large aortic aneurysm involving the aortic root and ascending aorta was identified. The aorta was normal distally. The wall was not obviously thickened, the aneurysm was resected and a graft placed. The specimen received in pathology was an opened portion of aorta measuring 9.4 x 5.1 x 0.1 cm. The endothelial surface was unremarkable.

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Case 4

submitted by:
Richard N. Mitchell
Brigham and Women's Hospital
Boston, Massachusetts

Clinical Summary:

The patient was a 34 year old male with a history of congenital heart disease (D-transposition of the great arteries); a Mustard repair procedure at age 4 was complicated by right phrenic nerve damage and right diaphragmatic paralysis. He subsequently developed progressive failure of his systemic ventricle, and underwent cardiac transplantation in 1990 (at the age of 22); his post-transplant immunosuppression consisted of cyclosporine A, azothioprine, and corticosteroids. His course was notable for two episodes of acute rejection in the first year following transplantation, treated with solumedrol boluses. In addition, within 4 months of transplant, he developed a post-transplant lymphoma in a left submandibular node. This was resected, and he experienced no further recurrence after his immunosuppressive therapy was slightly tapered. Other complications included obesity (eventually developing Pickwickian symptomatology), hypertension, osteoporosis, and hyperlipidemia.

The patient had annual angiographic evaluation of his coronaries; beginning approximately 10 years after transplant, diffuse coronary artery disease was first seen, most apparent in the smaller coronary arteries. Intravascular ultrasound also documented moderate-severe diffuse epicardial vessel disease with up to 85% chronic stenoses. Beginning 11 years after transplant, the patient developed progressive, severe exertional dyspnea and biventricular failure (R>L), although ejection fractions were largely maintained; moderate pulmonary hypertension was documented. PFT's documented profound restrictive and obstructive lung disease; although he was found to be a carrier for cystic fibrosis, his findings were felt overall to be most consistent with central neurogenic hypoventilation.

The patient presented terminally with right lower abdomenal discomfort; CT evaluation showed only mild thickening of the distal ileum and he was discharged to be worked up electively. He subsequently suffered a cardiac arrest at home, and could not be resuscitated. Slides available for review are a representative coronary artery and myocardium from the failed allograft.

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