īoth our patients have an aberrant subclavian artery arising from the descending aorta, making a total of 11 patients with an anomalous origin of the subclavian artery historically. That patient underwent a successful surgical repair with direct termino-lateral anastomosis between the ascending and descending aorta, and central pulmonary artery band as a neonate. As reported previously, interruptions of right-sided aortic arches are usually type B interruptions except for a single case report in which the patient who was positive for 22q11 chromosomal microdeletion had a right-sided type A IAA with a complete atrio-ventricular canal. Right-sided IAA is an extremely rare anomaly with no known true incidence. Inclusion of our two patients increases the total reported patients with right-sided IAA in the literature to 25. She was doing well on her recent follow-up. She later underwent a repeat surgery with a 20 mm PTFE RV-PA conduit replacement and repair of branch PAs using a bovine pericardial patch. Five months later, the child presented with a shock-like picture and was found to have a severe RV-PA conduit stenosis at the site of the insertion of the conduit into the branch PA's by cardiac catheterization, and she had minimal response by balloon angioplasty. Following the second surgery, the patient's condition stabilized and she was subsequently discharged home. Her postoperative course was complicated by a prolonged intensive care unit course and a late redo sternotomy with neoaortic valve repair. Following stabilization, she underwent a modified Yasui procedure via a median sternotomy that included a Damus-Kaye-Stansel type aortic arch reconstruction, LeCompte manoeuvre, intraventricular rerouting from the left ventricle to the pulmonary arterial valve through a right ventriculotomy using a Dacron® patch and reconstruction of the right ventricular outflow tract using a 12-mm right ventricle to pulmonary artery (RV-PA) PTFE conduit. A magnetic resonance angiography was performed that confirmed the echocardiographic findings (Fig. The patient was hypocalcaemic at presentation and later tested positive for 22q11 chromosomal microdeletion. An echocardiogram was performed upon arrival that showed a right-sided IAA type B with bilateral carotid arteries arising from the ascending aorta and bilateral subclavian arteries coming off the descending aorta distal to the ductal insertion, bicuspid aortic valve (valve annulus = 3.5 mm, z-score −6.6), large posterior malaligned VSD and hypoplastic ascending aorta that measured 6 mm ( z-score −3.5). The child was started on prostaglandins at presentation and required mechanical ventilation with inotropic support. On her recent follow-up, the patient remained asymptomatic with mild supravalvar aortic and pulmonary stenosis related to her previous surgeries.Ī 2-week old full-term infant girl weighing 3.2 kg presented to us with cyanosis. She continued to do well and underwent an additional 16-mm interposed graft that was chosen by the surgeon during the operation and was placed via the right chest through the right side of the pericardium, as the descending aorta could not be approached via a median sternotomy due to the presence of the oesophagus directly anterior to the descending aorta, because of a left pulmonary artery repair at the age of 4 years. She developed significant right ventricular outflow tract obstruction related to the previous band and pulmonary valve adhesion to the VSD patch and underwent transannular patch repair 3 years after the initial surgery (Fig. She underwent staged repair at 4 months of age that included Dacron® patch closure of VSD, polytetrafluoroethylene (PTFE) patch closure of the atrial septal defect, PTFE patch aortoplasty and main pulmonary artery debanding. Her genetic work-up was positive for the DiGeorge syndrome. Staged palliation was chosen for this patient due to the unusual arrangement of the great vessels, even though our institutional practice had changed to primary complete repair of IAA in 2000. ![]() The patient initially remained on prostaglandins and underwent an 8-mm interposed graft placement between the ascending and descending aorta, which the surgeon chose based on his assessment at the time of the surgery, main pulmonary artery banding via right thoracotomy and ductal ligation on the 7th day. These findings were confirmed by cardiac catheterization. An echocardiogram showed a right-sided interrupted aortic arch (IAA) type B, large posterior malaligned ventricular septal defect (VSD), small secundum atrial septal defect, bicuspid aortic valve, aortic valve annulus measured at 7 mm and right-sided ductus arteriosus. A full-term female weighing 3.27 kg was referred due to poor perfusion and desaturations 24 h after birth.
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