Although cardiac catheterization and angiography may be required to assess pulmonary arterial pressure, pulmonary vascular resistance, and the distal pulmonary arterial anatomy in truncus arteriosus, 2-dimensional echocardiography can be used to correctly establish the morphologic diagnosis of truncus arteriosus in infants. Background: Truncus arteriosus (TA) with interrupted aortic arch (IAA) is a rare combination of complex anomalies. These echocardiographic images were contrasted with and discriminated from those of an infant with aorticopulmonary window with intact ventricular septum. Truncus arteriosus (TA), a rare complex congenital cardiac disease in which systemic pulmonary and coronary circulations originate from a common vessel. Subcostal coronal and sagittal views imaged the common truncus and the ventricular septal defect. Genetic Considerations: Approximately 50 of newborns with truncus arteriosus have associated genetic disorder, most commonly the chromosome 22q11.2 deletion syndrome (20 of the cases). 20), of which type B is the most frequent (5070). Type A4: This type is defined by presence of aortic arch hypoplasia, coarctation or interrupted aortic arch and a large PDA. Therefore, in about half of the patients with DiGeorge syndrome the major anomaly was one that is rare. Depending on the site of discontinuity, IAA is classified into three types (see Fig. Interrupted aortic arch type B was the major anomaly in 48 patients and persistent truncus arteriosus in 37. Transverse imaging sections at the base of the heart facilitated identification of the pulmonary artery origin of truncus arteriosus type I. Interrupted aortic arch (IAA) is a structural heart defect characterized anatomically by a discontinuity (interruption) along the aortic arch. Methods A retrospective cohort study of children who underwent repair of TA-IAA between 20 at 12 tertiary care referral centers within the United States was performed. Contemporary multicenter data are limited. The suprasternal notch image facilitated identification of the left- or right-side aortic arch and the origin of the pulmonary arteries from the truncus. Truncus arteriosus with interrupted aortic arch (TA-IAA) is a rare congenital heart defect with historically poor outcomes. The parasternal long-axis view demonstrated the great vessel-ventricular septal override and the origin of the pulmonary arteries from the posterior aspect of the ascending truncus. Multiple imaging views were utilized to confirm the diagnosis. Two infants had truncus arteriosus type I, 3 patients had truncus arteriosus type II, 1 infant had truncal valve stenosis with an interrupted aortic arch, and 1 had type IV truncus arteriosus with pulmonary hypertension. Two-dimensional echocardiographic prospective diagnosis of truncus arteriosus was made in 7 infants.
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