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Right aortic arch with a left retroesophageal innominate artery (type D double aortic arch) is rare. The diagnosis is made by aortography. The present case is the first known patient to undergo a magnetic resonance imaging study that outlined the anomaly clearly.

An unique case of left innominate vein compression by a leftward origin of a brachiocephalic artery in conjunction with an aberrant right subclavian artery anomaly occurred in a young patient. Aortography and magnetic resonance imaging were invaluable in arriving at a diagnosis.

A patient with pulmonary atresia and a ventricular septal defect is described in whom an arterial branch from the distal ascending aorta supplied segments of both lungs. The branch is considered to represent a persistent fifth aortic arch. The possible morphogenesis and differential diagnosis of a communication between the ascending aorta and the pulmonary artery in pulmonary atresia with ventricular septal defect are discussed.

Rare types of aortic arch malformations include isolation of the right or left subclavian arteries in conjunction with a left or right aortic arch respectively. Isolation of the innominate or carotid arteries and an aberrant left innominate artery may exist in association with a right aortic arch. Other anomalies are persistent fifth aortic arch, subclavian artery as the first branch of the aortic arch, pulmonary artery, and ductus arteriosus sling. The angiocardiographic features and embryology of each of these malformations are discussed.

This study is presented to identify and characterize the spectrum of the cardiovascular anomalies in children presenting with Williams-Beuren syndrome and cardiovascular anomalies at The Hospital for Sick Children, Toronto from 1966 to 1988. Forty-nine children were diagnosed and followed. The female to male ratio was 1.2:1. The age ranged from 1 month to 14 years at the time of diagnosis (mean 39 months), and follow-up periods were from 9 months to 20 years (mean 10 years). All patients having the typical features were also evaluated by geneticists. Based on cardiovascular findings four groups were identified. Group 1 had isolated supravalvular aortic stenosis (SVAS) (28 patients). There was follow-up in 24 of these children. Six had worsening of supravalvular narrowing and underwent surgery. One showed an increased gradient from 10-40 mmHg during 7 years. Seventeen had mild narrowing and showed no progression over a period of 75 months. Group 2 had isolated pulmonary artery branch stenosis (8 patients). Seven had mild narrowing which remained unchanged over a mean period of 16 months and one underwent surgery. Group 3 had combined lesions (11 patients). Six showed increased left-side narrowing, while right-side obstruction remained static or improved. Five showed improvement in narrowing in both outflow tracts. Five underwent surgery. Additional cardiovascular anomalies included peripheral artery stenosis in two patients, coronary artery abnormalities in three, mitral valve prolapse in three, and coarctation of the aorta in two. Group 4 had isolated lesions. One patient had isolated coarctation of the aorta and one isolated mitral prolapse.(ABSTRACT TRUNCATED AT 250 WORDS)

Changes in the mobility of the pulmonary valve were determined by a retrospective review of right ventricular cineangiograms from 25 balloon pulmonary valvotomy (BV) procedures in 23 infants and children. The angiographic changes were compared with the post-BV catheter and Doppler pressure gradients across the right ventricular outflow tract. Angiographic features felt to indicate valve tearing were present following 17 of 25 procedures and included increased excursion or straightening of leaflets, localized change in leaflet motion (flail leaflet), and the presence of an additional contrast jet through the valve. There was no statistically significant relationship between any of the angiographic parameters and the pressure data. Most patients with marked increase in angiographic valve mobility had low residual right ventricular to pulmonary artery gradients. However, the absence of angiographic change was not always associated with a high residual gradient.

The atrioventricular junction of 40 patients with univentricular heart was evaluated by two-dimensional echocardiography. The apical 4 chamber view optimally imaged the atrioventricular junction, and allowed determination of the type of atrioventricular connection: double inlet, common atrioventricular orifice, and absent right or left atrioventricular connection. When double inlet to 1 ventricle was demonstrated, the 4 chamber view allowed immediate comparison of the form and function of the right and left atrioventricular valves. Because anomalies of the atrioventricular valves frequently complicate the univentricular heart, two-dimensional echocardiographic assessment is a most important adjunct to the preoperative investigation of these patients.

This report describes the use of cross-sectional imaging--echocardiography and contrast-enhanced computed tomography--to confirm the presence of a foramen-type defect of the left pericardium with herniation of the left atrial appendage.

A patient in whom there was a double aortic arch with atresia of the right dorsal arch segment between the right subclavian artery and right ductus arteriosus is presented. The radiographic and aortographic features are demonstrated and the embryology is discussed.

Tears involving the anterior supravalvar annulus developed in 2 children and an infant following percutaneous balloon pulmonary valvotomy using oversized balloons. The 3 patients had angiographic features of three different types of stenotic valves: usual pulmonary valve stenosis in 1, a form of dysplastic pulmonary valve with supravalvar narrowing in a second, and a doming valve in a neonate. All had a successful reduction in right ventricular outflow tract gradient following the procedure. The pulmonary arterial tears were not associated with balloon rupture or clinical symptoms. It is postulated that the relative deficiency of elastic fibers in the supravalvar commissure makes this site relatively vulnerable to intimal tearing.