THE NORMAL aortic arch is an intrathoracic structure derived embryologically from the left fourth branchial arch. In rare individuals, the aortic arch appears to be derived from the right third branchial arch. In such instances, the aortic arch extends well into the neck and the pattern of the brachiocephalic arteries is altered. This unusual anomaly manifests certain characteristic clinical findings. Report of a CaseA white female infant, delivered without incident after an uneventful, full-term gestation, appeared normal at birth and weighed 3,090 gm (6 lb 13 ounces). At 1 month of age, her weight was 3,508 gm (7 lb 11\m=3/4\ounces). The only abnormality found at that time, an inconstant systolic murmur heard along the left sternal border, was also heard at each monthly examination during the first year of life. At age 5 months, blood pressure was 116/60 mm Hg in the right arm and 122/84 mm Hg in the left San Francisco 94115 (Dr. Kerth).leg. Roentgenogram of the chest at 6 months was within normal limits. At lO1/^months, an electrocardiogram showed no abnormality other than incomplete right bundle branch block. The infant was suspected of having either a small atrial septal defect of the secundum type or mild stenosis of the pulmonic valve.The patient was lost to observation for more than a year. When she was seen again, at age 2% years, growth and development had con¬ tinued within normal limits, except that the left scapula had now become prominent and some winging could be detected; also, her neck appeared to be webbed. Roentgenograms showed lack of definition of the cervical spine and pos¬ sible spina bifida, compatible with Klippel-Feil anomaly. Buccal smears were positive for sex chromatin. The previously heard murmur was present as a grade 2 soft systolic murmur at the base; in addition, a pulsating mass was now detected over the base of the right carotid artery. A carotid aneurysm was suspected. The ECG findings at this time were normal. A chest roentgenogram showed a rather prominent aortic arch and persistent trachéal shift to the left in the cervical region (Fig 1 and 2). By age 3 years 3 months, a thrill was detected over the mass at the base of the neck on the right side; the underlying murmur was con¬ tinuous. When the pulsating mass was occluded by pressure, the pulse rate dropped from 120 to 60 beats per minute. Blood pressures were 100/50 mm Hg in the right arm, 95/70 mm Hg in the left arm, and 115/80 mm Hg in the left leg. One examiner detected a slight Horner's Downloaded From: http://archpedi.jamanetwork.com/ by a UQ Library User on 06/17/2015
Organ transplantation has become the treatment of choice for selected patients with end-stage failure of the heart, liver or kidneys. The expanding role for organ transplantation, however, has led to a corresponding increase in the complexity of patient management. In response to these changes, University Hospital, London, Ont., has established an interdisciplinary multiorgan transplant service (MOTS). MOTS coordinates donor organ procurement and patient management. Donor organs have been retrieved from as far south as Dalton, Georgia, as far west as Calgary and as far east as Halifax. As of Dec. 31, 1985, 485 transplants had been performed, including 387 kidney transplants, 51 heart transplants, 3 heart/lung transplants, 43 liver transplants (in adults and children) and 1 pancreas transplant. With current immunosuppressive protocols MOTS projects 1-year patient survival rates of 95% after kidney transplantation, 88% after heart transplantation and 81% after liver transplantation. Patient rehabilitation has been excellent.Chez des malades choisis, la greffe d'organe est devenue le traitement prefOre d'une defaillance du coeur, du foie ou des reins arrivee au stade
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