Ann Thorac Surg 2006;82:1126
© 2006 The Society of Thoracic Surgeons
Images in cardiothoracic surgery
Unusual Vascular Anatomy in Interrupted Aortic Arch With Ventricular Septal Defect
Marco Ricci, MD*,
Steven Schultz, MD,
Eliot Rosenkranz, MD
Holtz Children's Hospital, University of Miami Miller School of Medicine, Miami, Florida
* Address correspondence to Dr Ricci, Division of Cardiothoracic Surgery, University of Miami, Holtz Center 3072, (R-114), 1611 NW 12th Ave, Miami, FL 33136 (Email: mricci{at}med.miami.edu).
A 5-day-old boy weighing 3.3 kg presented with type B interrupted aortic arch, ventricular septal defect, and absent femoral, as well as radial pulses. An umbilical arterial line revealed no waveform with an adequate mean pressure of 40 to 45 mm Hg. The boy had no metabolic acidosis and satisfactory renal function. A 2-dimensional echocardiogram revealed severe biventricular hypertrophy (Fig 1A; LA = left atrium; LV = left ventricle), an unusually large pulmonary artery, and absent ductus arteriosus. The left ventricular outflow was unobstructed. Further evaluation revealed an aberrant right subclavian artery originating from the descending thoracic aorta. Doppler interrogation showed forward flow into both common carotid arteries and reversal of flow in both subclavian arteries (Fig 1B; LCCA = left common carotid artery; LSCA = left subclavian artery). Transcranial ultrasound revealed antegrade flow in the internal carotids and circle of Willis, with reversal of flow in the vertebro-basilar system (Fig 2). Lower body perfusion occurred through the carotid arteries, circle of Willis, subclavian arteries, and the descending aorta, as shown in the illustration (Fig 3; Ao = aorta; LCCA = left common carotid artery; LSCA = left subclavian artery; LVA = left vertebral artery; MPA = main pulmonary artery; RCCA = right common carotid artery; RSCA = right subclavian artery; RVA = right vertebral artery; VSD = ventricular septal defect).
Surgical repair entailed cardiopulmonary bypass through the right carotid (using a 3.5-mm polytetrafluoroethylene vascular graft) and the right atrium. During cooling (20°C, pH-stat), adequate lower body perfusion occurred through both subclavian arteries. Closure of the ventricular septal defect was carried out through the pulmonary outflow. The ligamentum was divided. The aberrant right subclavian was also divided to facilitate mobilization. Low-flow antegrade cerebral perfusion was used to anastomose the arch to the descending aorta, augmenting the anastomosis with a homograft patch. The postoperative course was uneventful.
Absence of a patent ductus arteriosus in the setting of interrupted aortic arch is very rare [1]. In this patient, ductal closure might have occurred late in gestation, as the mother used anti-inflammatory medications during her third trimester for a dental abscess. The most unusual feature of this case is that there was an aberrant right subclavian (so that both right and left subclavian arteries originated from the descending thoracic aorta), in addition to type B interruption and nonpatent ductus. This might have caused increased afterload on the left ventricle, as demonstrated by the ventricular hypertrophy, as the entire systemic output was forced through the ascending aorta and carotid arteries. Adequate perfusion of the lower body occurred through the circle of Willis and subclavian arteries.
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- Williams RV, Ritter S, Minich LA, Pagotto LT, Tani LY. Doppler evidence of subclavian steal in neonates with interrupted aortic arch using transthoracic echocardiography Am J Cardiol 2000;15(85):1501-1503.
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