We report a neonate with aortic arch hypoplasia and coarctation, in whom patency of the arterial duct could not be re-established and who was too ill to undergo primary surgical correction safely. This patient was treated in two stages: 1) angioplasty and stenting, 2) surgical correction. The safe period for surgical correction may be 2–3 weeks after angioplasty and stenting.

Treatment of the aneurysms comprising the aortic arch is challenging. Surgical reconstruction usually requires aortic cross-clamping, cardiac arrest, and even deep hypothermia for a bloodless field. In this report, we present our surgical technique providing normothermic ascending aorta, aortic arch, and proximal descending aorta replacement with selective cannulation and perfusion of the whole body.

Left aortic arch with right descending aorta is a rare congenital anomaly. We describe the clinical presentation of this unusual anomaly associated with cardiorespiratory compromise from severe aortic obstruction and left main bronchus compression. The anatomical peculiarities, embryological basis, and surgical solutions are presented.

Purpose: The aim of this study was to investigate the frequency and gender distribution of variations in the aortic arch branching pattern using 64-slice computed tomograhic angiography. Materials and methods: A total of 1000 patients with a normal left-sided aortic arch who underwent computed tomographic angiography for various reasons were analysed retrospectively for the frequency of variation of aortic arch branching; the variations were categorised into seven types. Results: Of the 1000 patients, 79.2% had a normal aortic arch branching pattern – type 1 – and 20.8% had variations. The frequency of type 2 variation – brachiocephalic and left common carotid arteries arising from the aortic arch in a common trunk – was 14.1%, that of type 3 – left vertebral artery originating from the aortic arch – was 4.1%, that of type 4 – coexistence of type 2 and type 3 – was 1.2%, that of type 5 – aberrant right subclavian artery – was 0.6%, that of type 6 – coexistence of aberrant right subclavian artery and bicarotid trunk – was 0.7%, and that for type 7 – thyroidea ima artery arising from the aortic arch – was 0.1%. The incidences of the variations of aortic arch branching were similar among males and females (20% versus 22.1%). The incidence of an aberrant right subclavian artery – type 5 and type 6 – was higher among females compared with males (2.5% versus 0.5), whereas the frequencies of the other variations were either equal or similar in both genders. Conclusion: Recognition of variations of aortic arch branching is important because they may cause symptoms due to tracheoesophageal compression or complications during surgical or endovascular interventional procedures of the aorta and its branches.

Deletion of chromosome 22 and DiGeorge syndrome are associated with inappropriate development of the neural crest and abnormalities of the ventricular outflow tracts. Bis-diamine induces comparable cardiac anomalies by inhibiting the normal development of the neural crest. In order to clarify the effect of damage on the development of the neural crest and its relation to the cardiac anomalies, the effects of giving a small dose of bis-diamine were compared with the results of a large dose of bis-diamine. A small dose of bis-diamine (20 mg), therefore, was administered to 40 pregnant rats on the 10th day of pregnancy, and 330 fetal hearts were studied on the 21st day with the rapid whole-body freezing method. Intracardiac anomalies were rare, being found in only 55 fetuses. The anomalies discovered were: aberrant origin of the subclavian artery in 88 fetuses, right aortic arch in 26 fetuses, interruption of the aortic arch in 23 fetuses, and high location of the aortic arch in 8 fetuses. Anomalies of the arterial duct were also common; with a vascular ring formed by the aortic arch and contra-lateral duct being seen in 21 fetuses, isolation of the right subclavian artery associated with either bilateral ducts (9 fetuses) or a right duct and absent left duct (9 fetuses), absent duct with tetralogy of Fallot in 7 fetuses, and with common arterial trunk in one fetus. In conclusion, a small dose of bis-diamine induced extra cardiac anomalies of the aortic arch and arterial duct, indicating a greater susceptibility of the aortic arch and the duct to inappropriate formation of the neural crest cells in the early developing embryo. Clinically, these anomalies of the aortic arch and the duct should be searched for in all patients suspected of having deletion of their 22nd chromosome.

Isolation of the right subclavian artery from the aortic arch is a rare malformation. When isolated, the artery originates from the right pulmonary artery via a right-sided arterial duct, which may be patent or closed at the time of presentation. Intracardiac or other anomalies of the great vessels are commonly associated with this lesion. We present a case of isolation of the right subclavian artery which was initially diagnosed as persistent patency of a left-sided arterial duct. Surgical correction was eventually achieved by disconnecting the right subclavian artery from the right pulmonary artery and translocating it to the ascending aorta.

Cervical aortic arch is a rare congenital anomaly. We present a case of left-sided cervical aortic arch, found in a patient with mitral regurgitation, mitral stenosis, and a regurgitant bicuspid aortic valve. There was atypical obstruction proximally within the arch. The obstructive segment was resected, and corrected by performing an end-to-side anostomosis. Mitral valvoplasty was performed in the same surgical procedure. To the best of our knowledge, a cervical arch has not previously been described with such atypical obstruction, and in association with multiple lesions involving the left heart.