P0420 - Ventricular Tachycardia as the Initial Presentation of Missed Kawasaki Disease in a Teenager

Introduction:
Ventricular Tachycardia (VT) can be a life threatening condition and is not typically seen in otherwise healthy children. We report the first case, to our knowledge, of an otherwise healthy teenager with sustained VT as the presenting symptom for missed Kawasaki Disease (KD).

Case presentation:
A 14 year old, healthy male was brought by ambulance to the emergency department due to sudden onset chest pain and palpitations at rest. Physical exam revealed an oriented, conversant teen without murmurs, crackles or hepatomegaly. His EKG demonstrated wide complex tachycardia with a right bundle branch block morphology and superior axis at a rate of 213 bpm (Figure 1) consistent with VT from the left ventricle. He was an active soccer player with no prior history of palpitations, chest pain, syncope, recent illness or illicit drug use. Two doses of adenosine were administered without effect. A limited echocardiogram revealed normal appearing systolic function in the setting of tachycardia. The possibility of Belhassen’s VT, a rare but typically benign form of VT that is responsive to calcium channel blockers and most commonly seen in otherwise healthy teenagers, was considered. Two doses of Verapamil were administered without effect. Next, concern arose for papillary muscle VT. Two boluses of lidocaine were administered without effect. During an IV bolus of sotalol he became unresponsive and hemodynamically unstable requiring a synchronized cardioversion to restore sinus rhythm. A complete echocardiogram in sinus rhythm demonstrated dilated coronary arteries (CA), regional wall motion abnormalities and low normal function concerning for KD. Extensive questioning for history of possible KD was negative. However, CT imaging demonstrated mild diffuse right CA dilation with multiple focal aneurysms, a large, calcified left main CA aneurysm impinging on the LAD origin, and a diffusely hypoplastic left circumflex CA with restricted flow (Figure 2), and cardiac MRI revealed dilated and aneurysmal proximal CAs and a large transmural infarct of the basal anterior and anterolateral left ventricle, consistent with a remote history of KD. His VT was determined to be secondary to scar associated with infarct. He underwent an implantable loop recorder placement (family refused an ICD) and was discharged home on beta blocker and anticoagulation therapy.

Discussion:
KD is a well described ailment of childhood known to cause a vasculitis preferentially affecting the coronary arteries. With prompt and appropriate treatment, outcomes are favorable; however, when not recognized, patients may develop serious sequelae secondary to CA aneurysms. This is the first case of VT as the presenting symptom for missed KD in a teenager, increasing our knowledge of KD presentation and should also remind clinicians to broaden the differential diagnosis in new onset ventricular tachycardia.