title: Neonatal repair of right interrupted aortic arch with cerebro-myocardial perfusion technique.
abstract: Right interrupted aortic arch and descending aorta is exceedingly rare and most likely cause respiratory presentation, since patent ductus arteriosus (PDA) courses over the right mainstem bronchus. We report a case of successful neonatal biventricular repair of a right interrupted aortic arch (type B), with an aberrant right subclavian artery ventricular septal defect (VSD) in a 2.7 kg term neonate with DiGeorge syndrome. Patient presented in severe respiratory distress and acidosis at one day old. Two-dimensional (2D) echocardiography revealed aortic arch interruption beyond the common carotid arteries with large perimembranous outlet VSD. Aortic annulus diameter was 4.8 mm and there was no left ventricle (LV) outflow tract obstruction. Three-dimensional (3D) CT-scan confirmed these findings and identified a right-sided ductal arch that continued over the right mainstem bronchus into a right-sided descending aorta and aberrant right subclavian artery. Brachiocephalic perfusion and ductal perfusion was employed for cooling during cardiopulmonary bypass. Under deep hypothermia (27 °C rectal temperature), selective cerebro-myocardial perfusion was used for successful aortic arch repair without sacrificing the aberrant right subclavian artery. A direct tension-free anastomosis was attained. Her postoperative course was uneventful and her respiratory symptoms disappeared postoperatively. Early surgical correction is mandatory for these patients with unique anatomy and presentation.
authors.affiliation: Division of Cardiovascular Surgery and Department of Pediatrics, Japanese Red Cross Medical Center, 4-1-22, Hiroo, Shibuya, Tokyo, Japan. koutakeuchi-circ@umin.ac.jp
authors.forename: Koh
authors.initials: K
authors.lastname: Takeuchi
authors.forename: Akihiro
authors.initials: A
authors.lastname: Masuzawa
authors.forename: Jotaro
authors.initials: J
authors.lastname: Kobayashi
authors.forename: Keiji
authors.initials: K
authors.lastname: Tsuchiya
mesh_terms: D000015:Abnormalities, Multiple; D001013:Aorta, Thoracic / Q000002:abnormalities / Q000000981:diagnostic imaging / Q000601:surgery*; D006348:Cardiac Surgical Procedures; D002560:Cerebrovascular Circulation; D003326:Coronary Circulation; D004374:Ductus Arteriosus, Patent / Q000150:complications; D004452:Echocardiography; D005260:Female; D006330:Heart Defects, Congenital / Q000150:complications / Q000175:diagnosis / Q000503:physiopathology / Q000601:surgery*; D006345:Heart Septal Defects, Ventricular / Q000150:complications / Q000601:surgery; D006801:Humans; D007231:Infant, Newborn; D010477:Perfusion / Q000379:methods*; D012127:Respiratory Distress Syndrome, Newborn / Q000209:etiology; D013348:Subclavian Artery / Q000002:abnormalities; D014057:Tomography, X-Ray Computed; D016896:Treatment Outcome