—  SPECIALTY CONFERENCE  — Pediatric Pathology Sunday, February 27, 2011, 7:30 PM CC 101 A/B Pediatric Cardiac Biopsies Moderators: DAVID PARHAM University of Oklahoma Health Sciences Center College of Medicine, Oklahoma City, OK and BAHIG SHEHATA Children’s Heathcare of Atlanta, Atlanta, GA Disclosure: In accordance with ACCME guidelines regarding disclosure, the USCAP policy requires that faculty members who have a significant financial or other relationship with a commercial company, entity, or service (which will be discussed in this Symposium) must disclose this to attendees. The Academy also requires that speakers disclose any products that are not labeled for the use under discussion. The speakers listed below have indicated they have nothing to disclose. Panelists: David Parham, University of Oklahoma Health Sciences Center, Oklahoma, OK Bahig Shehata, Children’s Healthcare of Atlanta, Atlanta, GA Glenn P. Taylor, Hospital for Sick Children, Toronto, Ontario, Canada Aliya N. Husain, University of Chicago Hospitals, Chicago, IL Clinical histories are displayed below. For the fastest viewing of virtual slides, click: under each thumbnail image below. You must have Aperio ImageScope installed on your PC. If you do not already have Aperio ImageScope, Windows users with administrator privileges may download and install a free version in order to view USCAP Virtual Slides. Click the icon on the right to get your free copy:   Or, click on slide thumbnail images to view each slide in a Web-based slide viewer, which is somewhat slower. If you have any difficulties viewing these slides, email or call George Clay at +1.724.449.1137. Case 1 Submitted by: David Parham - Clinical Summary: The patient is a 2 year, 3 month old African American male admitted for apneic episodes during sleep. On physical examination, vital signs included a heart rate of 123 beats/min, respiratory rate 32 breaths/min, and blood pressure 100/70 mm Hg. The only physical finding of note was a liver border 2-3 cm below the right costal margin. Echocardiography revealed cardiomegaly with left ventricular diastolic and systolic dysfunction, biatrial dilatation, pulmonary venous and venocaval diastolic flow reversal, moderate tricuspid stenosis, tricuspid regurgitation, and a small pericardial effusion. Cardiac catheterization found markedly elevated right and left ventricular pressures. Following biopsy and diagnosis, heart transplantation was performed, with removal of an explant consisting of right and left ventricle and the lower portions of dilated right and left atria. Case history and special stains courtesy of Dr. Ali Saad, Arkansas Children’s Hospital. Case 1 - Figure 1 Case 1 - Figure 2 Case 1 - Figure 3 Case 1 - Figure 4 Case 1 - Figure 5 Case 1 - Figure 6 Case 1 - Figure 7 Case 2 Submitted by: Bahig Shehata - Clinical Summary: A 7 month old previously healthy born at term Caucasian male infant presented to the emergency room due to bloody loose stool, loss of appetite, and lethargy. A work up for intussusceptions was negative. An abdominal CT scan demonstrated ascites and mesenteric lymphadenopathy. The superior cut of the CT scan showed pericardial effusion. An echocardiogram was performed and demonstrated large pericardial effusion with signs of early tamponade (Figure 1). The bloody clear pericardial fluid was drained. A follow up echo showed a large left ventricular mass protruding beneath the mitral valve, bulging into the pericardial sac (Figure 2). Cytology of the fluid was not definitive and therefore a trucut biopsy of the mass was obtained. The majority of the specimen was necrotic, however in some areas a spindle-cell lesion was identified (Figures 3-5). Case 2 - Figure 1 Case 2 - Figure 2 Case 2 - Figure 3 Case 2 - Figure 4 Case 2 - Figure 5 Case 3 Submitted by: Glenn Paul Taylor - Clinical History: A 15 year-old boy was seen by a neurologist for the complaint of leg weakness that was characterized as episodes of his “knees giving away”. This had developed in the past 2 or 3 months. Prior, he had no health problems. Examination revealed mild to moderate proximal muscle weakness, but no other significant findings. Family review disclosed that the boy’s mother had developed dilated cardiomyopathy in her late thirties and had recently received an implantable cardioverter-defibrillator. This information prompted the boy’s urgent referral to a cardiologist. An echocardiogram showed marked concentric hypertrophic cardiomyopathy and a large pericardial effusion. He was admitted to hospital for pericardiocentesis and additional investigations. Endomyocardial biopsies and biopsy of vastus lateralis muscle were performed. The virtual slide and other images are from the endomyocardial biopsy. Case 3 - Slide 1 Click to view with ImageScope Click to view with a Web-Based Viewer Case 3 - Figure 1 Elastic-Trichrome Case 3 - Figure 2 H&E Case 3 - Figure 3 PAS Case 3 - Figure 4 PASD Case 3 - Figure 5 Case 3 - Figure 6 Case 4 Submitted by: Aliya N. Husain - Clinical Summary: The patient is a 3-year-old male born with complex heart anomalies, status post Norwood and Fontan repairs admitted to the University of Chicago Hospitals for heart transplantation. Post-operatively the patient was supported with ECMO due to poor cardiac function. The first endomyocardial biopsy was performed at 7 days post- transplant images from which are submitted for your review. The immunohistochemical stain for C4d is submitted for scanning as virtual slide. In addition, digital images are submitted as follows: Case 4 - Figure 1 H&E Case 4 - Figure 2 H&E Case 4 - Figure 3 H&E Case 4 - Figure 4 C4d Handouts for all Specialty Conferences will be accessible via the "Educational Materials" section on the homepage the morning after each respective conference. Printed copies of the handout will not be available at the meeting.