Successful salvage of late-presenting isolated type B arch interruption

The catastrophic presentation, intriguing pathophysiology, and tailored management of a baby with late-presenting isolated interrupted aortic arch type B are presented herein. A 4-month-old baby presented with shock, arrested, and was resuscitated. She had severe left ventricular dysfunction, the ductus arteriosus (DA) was closed, and the ventricular septum was intact. Multiorgan dysfunction precluded emergency surgery. Inotropes were escalated, accepting upper limb hypertension, thereby facilitating collateral-dependent end-organ perfusion at higher perfusion pressures. Gradually, organ functions improved. On computerized tomography, the descending aorta was largely perfused via a sizable left vertebral artery that communicated with a dilated left subclavian artery, which was fed by the circle of Willis. Surgical correction involved extensive mobilization of the descending aorta and direct anastomosis to the arch. The postoperative course was uneventful, with recovery of LV function over 48 h. The baby is well, 7 months later. An accurate definition of the anatomy and physiology enabled stabilization and surgery.