Objectives

A PREVIOUSLY WELL 2-YEAR-OLD BOY WITH NO known cardiac disease presented with a 5-day history of fever and acute cardiac failure. On physical examination he was found to be tachycardic with wide pulse pressure, collapsing femoral pulses, and an early diastolic murmur. Transthoracic echocardiogram showed acute endocarditis of the aortic valve with severe regurgitation, an aortic root abscess, and mitral regurgitation. Preoperative transoesophageal echocardiogram in the long-axis Figure 1. Pre-operative transoesophageal echocardiogram demonstrating long-axis view of the aortic valve, aortic root abscess, and fistulous connection to the left atrium.

An 11-year-old boy with familial dysautonomia presented with palpitations. Continuous 24-h Holter monitoring revealed intermittent high-grade atrioventricular block and asystole. The unopposed parasympathetic tone in patients with dysautonomia may make them susceptible to bradycardia and atrioventricular block. We recommend routine 24-h Holter monitoring screening and, when indicated, consideration of pacemaker implantation to reduce the high risk of sudden death phenomena in this patient population.

No abstract