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      The Rare Association of Truncus Arteriosus with a Cervical Double Aortic Arch Presenting with Left Main Bronchial Compression

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          Truncus arteriosus, a double aortic arch, and a cervical aortic arch are all rare cardiovascular anomalies. We experienced a unique female newborn with the rare combination of truncus arteriosus with a cervical double aortic arch, which probably resulted from abnormal persistence of the bilateral 2nd or 3rd rather than the 4thembryonic aortic arches and failure of regression of the right 8th somitic segment of the right dorsal aorta. She presented with respiratory distress soon after birth, which was initially attributed to the vascular ring and hypertensive pulmonary arteries. Our inability to relieve her respiratory compromise by surgical division of the vascular ring and main pulmonary artery banding prompted the diagnosis of left main bronchial compression caused by a posteriorly displaced dilated ascending aorta that compressed the right pulmonary artery and left main bronchus against the descending aorta. The patient then underwent successful left main bronchus stent implantation. We speculate the cervical double aortic arch is redundant in nature and is a loose ring that may not cause tracheal compression. Nevertheless, a posteriorly displaced dilated ascending aorta in patients with truncus arteriosus may compress the right pulmonary artery and the main bronchus on the side of the aortic arch against the descending aorta.

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          Most cited references 9

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          Two-dimensional echocardiographic determination of aortic and pulmonary artery sizes from infancy to adulthood in normal subjects.

          The aorta, right pulmonary artery and pulmonary trunk were measured from the 2-dimensional echocardiogram (2-D echo) of 110 normal subjects aged 1 day to 18 years. The vessel diameters were measured from the parasternal short-axis view, the suprasternal long-axis view and the suprasternal short-axis view. Measurements were made at end-systole and at end-diastole and in both an axial and lateral direction where possible. When analyzed with respect to body surface area (BSA), the echocardiographic measurements were linearly related to the square root of the BSA, and there was inequality of variance around the relation. To establish a range of normal values for each vessel dimension, a weighted regression analysis was used to produce estimates of the regression line and a set of tolerance intervals. The systolic vessel dimension was larger than the diastolic vessel dimension and the measurement of a vessel in an axial direction was larger than the measurement of the same vessel in a lateral direction. In general, when a vessel was measured in several views, the largest diameter was obtained using the view that imaged the vessel in cross section. These data on normal values for the echocardiographic measurement of the aorta and pulmonary arteries at different BSAs should be useful for identifying patients with abnormalities in arterial size and for the serial assessment of arterial size in children who have undergone surgical or medical therapy.
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            Clinical,angiocardiographic, and pathologic findings in 100 patients

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              Persistent truncus arteriosus with double aortic arch.

               A Bhan,  M. Gupta,  M Kumar (2006)
              We report a rare association of persistent truncus arteriosus with double aortic arch in a 34-day old neonate.

                Author and article information

                S. Karger AG
                July 2008
                01 February 2008
                : 111
                : 1
                : 16-20
                Departments of aPediatrics, bRadiology and cSurgery, Chang-Gung Children’s Hospital, Chang-Gung University College of Medicine, Taoyuan, Taiwan
                113421 Cardiology 2008;111:16–20
                © 2008 S. Karger AG, Basel

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                Page count
                Figures: 5, References: 11, Pages: 5
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