Intracardiac thrombus causing systemic embolism in a child with idiopathic ventricular tachycardia and heterozygous activated protein C resistance

Systemic embolism in childhood is rare but often disastrous. Most often the concomitant occurrence of more than one prothrombotic factor is responsible for the acute event. We report on a child in whom an intracardiac thrombus embolized into the descending aorta resulting in subtotal occlusion. Causative for thrombus formation was an idiopathic ventricular tachycardia and a heterozygous activated protein C resistance, both previously unknown. Immediate surgical thrombectomy was successful without sequelae. Antithrombotic and antiarrhythmispioproptylactic treatment was started afterwards. We suggest that in cases of longstanding or repeated tachycardia and in children after thromboembolic events diagnostic work-up for thrombophilia should be undertaken.