13 SUSPECTED VASCULITIS IN AN ADOLESCENT FEMALE WITH CARDIOMYOPATHY, MYOCARDIAL INFARCTION, AND HEART FAILURE REQUIRING HEART TRANSPLANTATION. (1st March 2005) - Nabatian, S.
Abstract : A 15 yr old white female gymnast developed a fever, dry cough, and "red eyes". She was seen at a local emergency room on 5/03 and was diagnosed with bilateral pneumonia. Her fever and cough improved after completing her antibiotics; however, she developed exertional dyspnea. In August 2003, she presented jaundiced to a local hospital with abdominal pain, emesis, and shortness of breath. A dilated cardiomyopathy with an ejection fraction of 10% was diagnosed. In the PICU, she developed respiratory distress and was intubated, treated with diuretics and maintained on inotropic support. Echocardiography confirmed a dilated cardiomyopathy with an ejection fraction of 10%, mild tricuspid, moderate mitral and mild aortic regurgitation. EKG showed sinus tachycardia (125 beats/min), with T wave inversions in V5-V6. She had a CK of 55 (Nl<150) and troponin I of 1.24 (Nl<0.1). An EKG 2 days later demonstrated ST elevation in the inferolateral leads, CK had risen to 667 (MB 21) and troponin I to 15.9. Coronary angiography showed widespread aneurysmal dilatation of all coronary arteries, severely depressed LV function (LVEDP 35 mm Hg). An intra-aortic balloon pump was placed and arrangements were made to transfer the patient to Children's Hospital for cardiac transplantation. She underwent a successful transplant 16 days later. Pathology of the explanted heart revealed cardiomegaly with biventricular dilatation, diffuse aneurysmal dilatation of all epicardial coronary vessels, moderate to severe myointimal proliferative lesions involving small and large vessels, and a large area (3 × 2 cm) of transmural discoloration of the mid posterior-lateral wall of the left ventricular myocardium, grossly consistent with acute myocardial infarction. The pathological changes seen in the epicardial coronary vessels however were not consistent with Kawasaki. Our patient's initial presumptive diagnosis was Kawasaki disease. There is no specific laboratory testing; a high index of suspicion is required. Many patients with inflammatory disease have overlapping characteristics that do not precisely fit into any one classification. This subgroup of patients is referred to as the polyangiitis overlap syndrome and is truly a systemic vasculitis.