Recurrent Syncope in a Child with a Murmur
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A 7-year-old male presents to the emergency department with another pre-syncopal event. He had been on the anti-epileptic medication levetiracetam due to two episodes of loss of consciousness over the past month. He describes a prodrome of pallor, diaphoresis, and lightheadedness, and has developed new-onset fatigue and exercise intolerance, enabling him to bike for only about 5-10 minutes. He denies chest pain, palpitations, or edema.
There is a family history of systemic hypertension and dilated cardiomyopathy, but no congenital heart disease or sudden death in childhood.
On physical exam, note is made of a physiologically split S2 with a prominent pulmonary component (P2) and a grade III/IV short, diastolic, high-pitched murmur (Graham Steell murmur) along the left upper sternal border. An electrocardiogram (ECG) (Figure 1), chest X-ray, and transthoracic echocardiogram are obtained.
Figure 1: Electrocardiogram
Figure 2: Chest X-Ray
Figure 3: Transthoracic Echocardiogram Doppler Across the Pulmonary Valve in the Parasternal Short-Axis View
Video 1: Transthoracic Echocardiogram: Parasternal Long-Axis
Video 2: Transthoracic Echocardiogram: Parasternal Short-Axis
Which of the following is the patient's most likely diagnosis?