Hypoplastic aortic arch with aortic coarctation: Surgical correction

Volume 89

Brief communications

Number 3 March, 1985

broma of vagus nerve. J THORAC SURG 31:632-634, 1956 II Penido JRF, Dodge HW Jr, Clagett OT, Starr GF: Tumors of the vagus nerve. Mayo Clin Proc 32:239-249, 1957 12 Davis C Jr, Brown G: Intrathoracic neurofibroma of vagus nerve associated with diaphragmatic hernia. J THORAC SURG 33:532-536, 1957 13 Pampari D, Lacerenza C, Emilia R: A case of neurofibroma of the intrathoracic vagus nerve in a woman with Recklinghausen's disease. Surgery 45:470-474, 1959 14 Carey LS, Ellis FH Jr, Good CA, Woolner LB: Neurogenic tumors of the mediastinum. A clinicopathological study. AJR 84: 189-205, 1960 15 Oberman HA, Abel MR: Neurogenic neoplasm of the mediastinum. Cancer 13:882-898, 1960 16 Byron, cited by Spain DM: Diagnosis of Tumors of the Chest, New York, 1960, Grune & Stratton, Inc., p 162 17 Ecker RR, Times JJ, Miscall L: Neurogenic tumor of intrathoracic vagus nerve. Arch Surg 86:222-229, 1963 18 Gayola G, Janis M, Weil PH: Intrathoracic nerve sheath tumor of vagus. J THORAC CARDIOVASC SURG 49:412-418, 1965 19 Saegesser F, Boumghar M: Tumeurs neurogenes endothoraciques chez l'enfant et chez I' adulte. Thoraxchirurgie 14:307-322,1966 20 Besznyak I, Padanyi A, Pinter E: Intrathoracales VagusNeurinom. Thoraxchirurgie 16:210-214, 1968 21 Moehling RC, Jarkowski TL: Neurilemoma of the vagus nerve in the posterior mediastinum. J Mich State Med Soc 63:442-443, 1964 22 Tomita M, Uchida Y, Nakamura Y, Kusaba H, Matsuo M, Nakagawa G, Ideda E, Watanabe Y, Handa S, Kubota M, Shibata K, Hadama T, Yazima K, Ayabe A, Shiraishi M, Adachi A, Kitazato S, Ohe E, Tsuji Y: A case of mediastinal tumor derived from vagus. Nippon Kyobu Rinsho 28:748-751,1969 23 Schwierenga J, cited by Hohmann H, Gebhardt C24 24 Hohmann H, Gebhardt C: Intrathoracale Neurofibrome des Nerves vagus. Zentralbl Chir 95:986-993, 1970 25 Newmann A, So SK: Bilateral neurofibroma of the intrathoracic vagus associated with von Recklinghausen's disease. AJR 112:389-392, 1971 26 Baba N, Hitomi S, Abe R, cited by Osada et al" 27 Strickland B, Wolverson MK: Intrathoracic vagus nerve tumors. Thorax 29:215 c222, 1974 28 Sarin GL, Benett MH, Jackson JW: Intrathoracic neurofibroma of the vagus nerve. Br J Dis Chest 68:46-50, 1974 29 Prohazka J, Kovar J: Schwierigkeiten bei der Diagnose von neurogenen Mediastinaltumoren. Z Erkr Atmungsorgane 142:233-240, 1975 ' 30 Shirakusa T, Yoshida T, Miyazaki N, Shigematsu N, Ishimaru S: A case of neurilemoma arising from intrathoracic vagal nerve. Kyobu Geka 28:827-830, 1975 31 Okaniwa G, Fujiwara M, Hashimoto L, Nakada T: A case of neurilemoma originating from the left vagus in the

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mid-superior mediastinum. Kyobu Geka 29:754-757, 1976 Zorbas JA, Kreatsas GK: Neurofibroma of the intrathoracic vagus nerve in man with Recklinghausen's disease. Case report. Milit Med 142:384-385, 1977 Lazar C, Chifan M, Dolinescu C, Niculescu D: A case of mediastinal neurinoma of the vagus nerve. Rev Med Chir Soc Med Nat Iasi 82:695-696, 1978 Tanaka S, Furutani S, Ohisi K, Ikeda T: A case of mediastinal schwannoma originating from intrathoracic vagal nerve. Nippon Kyobu Geka Gakkai Zasshi 27:889893, 1978 Osada H, Funaki S, Okada T, Kawada T, Mizutani K, Hiekata T, Noguchi T: A case report of intrathoracic vagal schwannoma. Kyobu Geka 32:303-306, 1979 Blades B: The Mediastinum, Surgery of the Chest, JH Gibbon Jr, ed., Philadelphia, 1962, W. B. Saunders Company, p 284 Linder E, Schamaun M: Die primaren Mediastinaltumoren. Literaturiibersicht und Analyse von 150 eigenen Fallen. Thoraxchirurgie 11:391-421,1964 Feldmann M, Morrison S: Vagotomy. Experimental and clinical appraisal of effect of section of vagus nerves at different levels. Rev Gastroenterol 17:344-347, 1950

Hypoplastic aortic arch with aortic coarctation: Surgical correction J. G. Vincent, M.D., O. Daniels, M.D., A. van Oort, M.D., and L. K. Lacquet, M.D., Nijmegen, The Netherlands From the Institute of Thoracic and Cardiovascular Surgery and the Department of Pediatric Cardiology, Sint Radboud University Hospital. Nijmegen, The Netherlands.

This report describes an alternative operation for hypoplastic aortic arch. The technique conserves the vascularization of the left arm and avoids the need of using flaps or free patches. The operative procedure is documented in three patients.

In most patients with congenital hypoplasia of the aortic arch, severe coarctation of the aorta is also present. Some of these patients have additional congenital anomalies of the heart. Especially in small infants, the first concern frequently is to correct the coarctation because of severe left heart decompensation. There' is stilI no consensus on the "best" means of coarctation correction-resection with end-to-end anastomosis or use of a left subclavian flap.!" In a few patients, even after successful correction of the coarctation, the left ventricular strain is not completely relieved because of remaining aortic arch hypoplasia. In most patients, the hypoplastic segment is restricted to the segment between

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The Journal of Thoracic and Cardiovascular Surgery

Fig. 1. The line of incision is shown extending from well out on the left subclavian artery, along the distal part of the aortic arch, and into the proximal portion of the left common carotid artery. The upperright drawing shows the reconstruction and the newly created left-sided brachiocephalic trunk. The lower drawing shows the improved cross-sectional area of the reconstructed channel.

the left common carotid artery and the left subclavian artery. Three infants were successfully operated upon by the following surgical technique. T.he result was an appropriate diameter of the aortic arch, without the need of sacrificing the subclavian artery. Surgical technique. The thoracic approach is the same as used for a coarctation of the aorta. The descending aorta is exposed for the coarctation resection and the aortic end-to-end anastomosis. The aortic arch is exposed to allow aortic clamping proximal to the left common carotid artery. The left common carotid artery and the left subclavian artery are mobilized along their entire intrathoracic course. The descending aorta is clamped distal to the coarctation and the aortic arch proximal to the left carotid artery, together with the distal portion of the left carotid artery right at its exit from the thoracic cavity. Finally, the left subclavian artery is clamped also close to its thoracic exit. The ductus arteriosus is clamped close to the pulmonary artery junction. The coarctation in the area of the ductus is resected and the pulmonary stump of the ductus is sutured. Then the concavity formed by the hypoplastic arch and the left subclavian artery is incised longitudinally on both sides over corresponding lengths (Fig. I). A continuous suture with 5-0 or 6-0 monofilament material joins the incised dorsal and ventral walls of the

two vessels. This suture line will bring the orifice of the subclavian artery cephalad to the left common carotid artery. In fact, a left-sided brachiocephalic trunk is created. The effect of this reconstruction is a large aortic channel in place of the narrow hypoplastic area (Fig. 1). The end-to-end anastomosis with the distal portion of the descending aorta restores the aortic continuity and completes the operation. Case reports. CASE 1. A IS-day-old infant weighing 3,230 gm had severe cyanosis, suggesting a congenital heart anomaly. Echocardiography showed transposition of the great arteries with two ventricular septal defects, severe coarctation of the aorta with aortic arch hypoplasia, and a large ductus arteriosus. Angiography confirmed the diagnosis, showing the hypoplastic arch and the typical "elongation" of the hypoplastic segment between the left common carotid and subclavian arteries (Fig. 2). A Rashkind procedure was done. Because of continued decompensation, operation was considered for coarctation resection, ductus closure, and banding of the pulmonary artery. At operation a descending aorta 8 mm in diameter, a ductus of the same size, a subclavian artery 4 mm in diameter, and an aortic arch 3 mm in diameter were found. The technique described herein was attempted as a possible solution to the problem. Pulmonary artery banding resulted in a pressure drop from 90 mm Hg in the right ventricle to 40 mm Hg in the pulmonary artery. The final diameter of the arch became 6

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Brief communications 4 6 7

March, 1985

Fig. 3. Echocardiographic recording demonstrating the resulting aortic channel and the common left brachiocephalic trunk. The arrow indicates a hanging thrombus at the level of the coarctation resection.

Fig. 2. Left heart and aortic arch angiography. Note the elongated, hypoplastic segment of aorta distal to the left carotid artery.

mm. The patient tolerated the operation and the early postoperative period well. Unfortunately, on the third day he experienced severe bradycardia during weaning from the respirator, caused by a tracheal tube obstruction. After this episodethe infant never fully recovered and finally died on the thirteenth postoperative day. At autopsy the reconstructed aortic segment was wide open. CASE 2. A l2-day-old infant weighing 3,380 gm had heart failure. The echocardiographic examination verified the diagnosis of aortic coarctation and aortic arch hypoplasia. Severe oliguria was initially managed with diuretics and digoxin. Prostaglandin E[ was given to widen the ductus and to enhance circulation to the lower part of the body. Because the conservative therapy afforded partial improvement, surgical correction was proposed. The described operative procedure. resulted in a pressure gradient between the right arm and the legs of 5 mm Hg. At night the femoral pulses disappeared. Echocardiography discloseda thrombus hanging on the end-to-end aortic anastomosis (Fig. 3). At reoperation the thrombus was removed. Renal insufficiency was briefly controlled by peritoneal dialysis. Echocardiography confirmed the successful enlargement of the reconstructed arch and descending aorta. The child was dismissed from the hospital in fairly good general condition and is being closely observed because of his impaired renal function. CASE 3. A 15-day-old infant weighing 4,450 gm had heart

failure. Neither the angiographic nor the echocardiographic examination clearly documented the extent of hypoplasia or interruption at the isthmus. An important coarctation, a large ductus arteriosus, and a large.ventricular septal defect (monoventricular type) were noted. Operation was performed the day of admission. Extreme distal arch and isthmic hypoplasia were found. The described operative procedure was performed. The left vertebral artery originated directly from the hypoplastic arch and was left in place, so that after the procedure the left carotid and subclavian arteries originated from the aorta proximal to the origin of the vertebral artery. Banding of the pulmonary artery completed the surgical procedure. The postoperative course was uneventful. Later echocardiographic studies confirmed an excellent correction of the malformation. No pressure gradient was found between the arms and legs. The baby was referred in good condition for repair of palatoschisis.

Comments. In our opinion, the proposed technique offers another possibility for the successful surgical treatment of small infants with severe heart failure caused by aortic arch hypoplasia and coarctation. The procedure allows good expansion of aortic size in comparison to other "patch" correction techniques and at the same time conserves the aortic origin of the left subclavian artery. 7, 8 REFERENCES Gross RE, Hufnagel CA: Coarctation of the aorta. Experimental studies regarding its surgical correction. N Engl J Med 233:287-293, 1945 2 Crafoord C, Nylin G: Congenital coarctation of the aorta and its surgical treatment. J THORAC SURG 14:347-361, 1945 3 Waldhausen JA, Nahrwold DL: Repair of coarctation of

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the aorta with subclavian flap. J THORAC CARDIOVASC SURG 51:532-533, 1966 4 Bergdahl LAL, Blackstone EH, Kirklin JW, Pacifico AD, Bargeron LM Jr: Determinants of early successes in repair of aortic coarctation in infants. J THORAC CARDIOVASC SURG 83:736-742, 1982 5 Hamilton DI, Di Eusanio G, Sandrasagra FA, Donnelly RJ: Early and late results of aortoplasty with the left subclavian flap for coarctation of the aorta in infancy. J THORAC CARDIOVASC SURG 75:699-704, 1978

The Journal of Thoracic and Cardiovascular Surgery

6 Koerfer R, Meyer H, Mehring J, Birks W: Early and late results after operation of coarctation of thoracic aorta in the first three months of life. Circulation 66:Suppl 2:196, 1982 7 Campbell DN, Paton BC, Wiggins JW, Wolfe RR, Clark DR: Infant coarctation of the aorta. Alternatives to subclavian flap repair. Pediatr Cardiol 3:139-142, 1982 8 Hart JC, Waldhausen JA: Reversed subclavian flap angioplasty for arch coarctation of the aorta. Ann Thorac Surg 36:715-717,1983

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