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A 10 day old asymptomatic baby girl was evaluated for a to-and-fro systolic and diastolic murmur grade III/VI, audible along the left sternal border. The echocardiogram showed a valvar pulmonary stenosis with a maximum systolic gradient of 55 mm Hg across a thick, poorly mobile pulmonary valve, and an aortico-right ventricular tunnel between the left aortic sinus and the right ventricle. The aortic orifice of the tunnel originated distal from the expected origin of the left coronary artery. Echocardiography (below left) and magnetic resonance imaging (MRI) (below right) revealed an abnormally large right coronary artery and only a tiny left anterior descending (LAD) coronary artery. The further course of the tunnel revealed an aneurysmatic dilatation and several stenoses. Because of the association of the tunnel with pulmonary stenosis no heart failure developed, which made it possible to postpone the treatment beyond the neonatal period. At 5 months of age, the baby remained asymptomatic, but the maximum systolic gradient across the pulmonary stenosis increased to 75 mm Hg. The to-and-fro flow within the tunnel showed a systolic gradient of the right ventricle to the aorta of 5 mm Hg, and a diastolic gradient of the aorta to the right ventricle of 34 mm Hg. Because of an unsatisfactory relief of pulmonary stenosis with balloon valvuloplasty and failed coil closure of the tunnel, a surgical correction was performed. All findings were confirmed during surgery.
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