Coronary artery thrombus resulting in sudden cardiac death in an infant with Kawasaki disease and giant coronary artery aneurysms
A six-month-old Hispanic male presented to the ER with seven-day history of fever, cough and congestion and he was treated with antibiotics for possible pneumonia. Two days later due to persistent fevers, irritability and gastrointestinal symptoms, he was admitted for septic work up. He was continued on antibiotics for possible urinary tract infection and aseptic meningitis (had elevated white cell counts in the urine and CSF). He continued to have recurrent fevers, and seventeen days into the illness he was noted to have increasing white cell count (29.5 K mm3), high platelet count (1061 K mm3), elevated CRP (138 mg/L), high ESR (>100 mm/hr) with mild peeling of his fingertips which prompted an echocardiogram. He had a transient conjunctivitis, dry lips and skin rash early in the course of his illness by history but had a normal exam at presentation. Echocardiogram showed extensive, multiple, giant CAA of the right coronary artery (RCA), fusiform shape, the largest measuring 19 m × 20 mm [], the proximal left anterior descending artery (LAD) measuring 22 mm × 24 mm [, [Videos and ].\nHe had a large pericardial effusion, which was drained. He was treated with IVIG and Abciximab. Due to risk of coronary artery thrombus and for myocardial protection, he was treated with enoxaparin, Clopidogrel, Aspirin, Captopril, and Propranolol, which were continued at the time of discharge. He was closely followed up as outpatient with serial echocardiograms. At his last follow up four months later, he was doing well, with stable giant CAA with no thrombus and normal biventricular function by echocardiography. A cardiac catheterization was planned in next 1-2 months.\nA month later he presented to the ER with nonspecific gastrointestinal symptoms and irritability, he was presumed to have a viral illness, and was discharged. The next day he died suddenly at home. Autopsy showed giant fusiform CAA of the RCA measuring 20 mm in diameter and 65 mm in length with occluding thrombus []. The giant LAD aneurysm was oval and measured 20 mm in diameter and 40 mm in length with occluding thrombus []. The myocardium showed multifocal grayish scar formation particularly of the inter-ventricular septum suggestive of infarction. Microscopic cross section showed a completely occluding mural thrombus in RCA aneurysm with intimal wall thickening.