Asian Cardiovasc Thorac Ann 2007;15:482-485
© 2007 Asia Publishing EXchange Ltd
Surgical Experience of Coarctation of the Aorta in Infants and Young Children
Jing-Hao Zheng, MD,
Jin-Fen Liu, MD,
Zhi-Wei Xu, MD,
Zhao-Kang Su, MD,
Wen-Xiang Ding, MD
Department of Pediatric Thoracic and Cardiovascular Surgery, Shanghai Childrens Medical Center, Xin Hua Hospital and Shanghai Second Medical University, Shanghai, China
For reprint information contact: Jing-Hao Zheng, MD, Tel: 86 21 5873 2020, Fax: 86 21 6515 3984, Email: haohao6554{at}hotmail.com, Department of Pediatric Thoracic and Cardiovascular Surgery, Shanghai Childrens Medical Center, Xin Hua Hospital and Shanghai Second Medical University, Shanghai 200127, China.
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ABSTRACT
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We evaluated the effectiveness of surgical treatment for coarctation of the aorta in infants and young children, based on our experience over 7 years. From March 1999 to December 2005, 168 cases of coarctation of the aorta in patients aged 21 days to 3 years were treated by end-to-end, extended end-to-end, or extended end-to-side ascending aorta and aortic arch anastomosis. The mortality rate was 0.6%. In 138 (82%) patients, the pressure in the lower extremity was higher than in the upper extremity. During 6–24 months of follow-up, hoarseness appeared in 6 patients. Echocardiography revealed stenosis of the end-to-end anastomosis in only 2 patients. We suggest that the best option for surgical treatment of coarctation of the aorta with associated cardiac malformations is a one-stage procedure using a median sternotomy approach. It is better to perform extended end-to-end anastomosis or anastomosis between the distal descending aorta and the left wall of the ascending aorta, or to extend this anastomosis to the transverse arch.
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INTRODUCTION
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Coarctation of the aorta (CoA) is congenital narrowing of the descending thoracic aorta, usually (but not always) occurring just distal to the left subclavian artery and adjacent to the site of insertion of the ductus arteriosus. It is often associated with other congenital heart defects including patent ductus arteriosus (PDA), ventricular septal defect (VSD), and mitral valve abnormalities. The surgical management of CoA has evolved over the years, with the intent of preventing recoarctation. We evaluated the effectiveness of surgical treatment for coarctation of the aorta in infants and children, based on our recent experience over 7 years.
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PATIENTS AND METHODS
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From March 1999 to December 2005, 168 cases of coarctation of the aorta in patients aged 21 days to 3 years were treated in our hospital. The patients weighed 2.9–13.5 kg. The diagnosis was isolated CoA or CoA with PDA in 32 patients, CoA with VSD in 121, and the other 15 had CoA with other associated cardiac anomalies such as complete or partial atrioventricular canal, double-outlet right ventricle, or subaortic stenosis. Among these 168 patients, 8 neonates were in a shock state because of severe CoA and PDA, requiring infusion of prostaglandin E1 to maintain the patency of the ductus arteriosus. Many patients were essentially asymptomatic but presented with proximal systemic hypertension and inadequate lower extremity perfusion, leading to congestive heart failure in 38%, tachypnea and tachycardia in 26%, and absent lower extremity pulses in 88%. The chest radiographs of all patients demonstrated cardiomegaly and evidence of congestive heart failure. Color Doppler echocardiography was adopted for diagnosis in all patients. Catheterization was also performed in 97 patients, and 75 were diagnosed with arch hypoplasia.
All anastomoses were carried out using 6/0 fine absorbent monofilament sutures. In 27 of the 32 patients with isolated CoA or CoA with PDA, repair was undertaken through a left posterolateral thoracotomy incision, body temperature was kept between 33°C and 35°C, and the mean distal aortic pressure was maintained above 40 mm Hg. Resection and end-to-end anastomosis was performed in 18 patients, prosthetic patch aortoplasty in 7, and subclavian flap aortoplasty in 2. A median sternotomy approach was used in the other 5 patients because of arch hypoplasia; they had an extended end-to-end anastomosis. Of the other 136 patients with CoA and VSD or other associated cardiac anomalies, the 14 operated on in 1999 were first placed in the right lateral decubitus position and the CoA was corrected through a left posterolateral thoracotomy incision before being switched to the horizontal position and undergoing a median sternotomy.
Since 2000, the other 122 patients have undergone concomitant repair in the horizontal position via a median sternotomy during deep hypothermic cardiac arrest, deep hypothermic low flow, or normothermic cardiopulmonary bypass. Resection and end-to-end anastomosis was performed in 38 patients, extended end-to-end anastomosis in 45 (Figure 1
), and extended end-to-side ascending aorta and aortic arch anastomosis in 39 (Figures 2
and 3
). The overall cardiopulmonary bypass time was 63.61 ± 20.66 min (range, 58–109 min), the circulatory arrest time was 32.13 ± 11.72 min (range, 21–48 min), and aortic cross clamp time was 43.25 ± 6.87 min (range, 37–58 min).
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RESULTS
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One patient died from postoperative pulmonary hypertensive crisis and arrhythmia; the mortality rate was 0.6%. Lower extremity pressure was higher than upper extremity pressure in 138 (82%) patients, it was nearly equal to upper extremity pressure in 25 (15%), and lower than upper extremity pressure in 5 (3%). The intensive care unit stay was 3–11 days (mean, 5.36 ± 2.06 days) and ventilation was required for 11–256 hours (mean, 98.51 ± 6.68 hours). There were no other severe complications such as paraplegia, hemorrhage, aneurysm formation, or chylothorax in the other patients. Postoperative systemic hypertension was noted in 65 patients, subsiding within 2–3 days. Sodium nitroprusside was administered intravenously to titrate the blood pressure; however, the use of nitroprusside was often associated with wide swings in blood pressure, and more recently, intravenous esmolol (100 to 500 µg·kg–1·min–1) has been adopted to treat these patients. During 6 to 24 months of follow-up, hoarseness appeared in 6 patients; 5 had been treated through a median sternotomy and 1 through a left posterolateral thoracotomy incision. All patients underwent an echocardiographic study during follow-up, which revealed stenosis of the end-to-end anastomosis in only 2 patients. Their pressure gradients were 29 and 36 mm Hg. The pressure gradients in the other patients were all below 10 mm Hg.
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DISCUSSION
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With continuing improvements in surgical techniques, cardiopulmonary bypass, and postoperative intensive care, surgical treatment of CoA in infancy and early childhood is more widely undertaken. Surgical management at an early age may be preferred because the occurrence of postoperative pulmonary hypertension is strongly related to age at operation. In patients < 3 years of age, pulmonary hypertension is less likely.1 In this series, the youngest patient was 21-days old and weighed 2.9 kg; pulmonary hypertension subsided sharply after one-stage correction of VSD with PDA. Moreover, early surgery is indicated to avoid congestive heart failure that often occurs with a large VSD or double-outlet right ventricle. As well as these benefits, it is easy to perform extended end-to-end anastomosis between the descending aorta and aortic arch in a small baby, which is also beneficial to the growth of the vessel. Therefore, we suggest that CoA, especially if associated with other cardiac abnormalities, should be surgically managed as early as possible.2
In 1999, isolated CoA or CoA with PDA was handled through a left posterolateral thoracotomy incision in our hospital. If CoA was complicated with arch hypoplasia or other cardiac malformation, the CoA was corrected first through a left posterolateral thoracotomy, and a median sternotomy was used for correction of other cardiac malformations. This resulted in a prolonged operation time with increased chance of injury to the baby. Via a median sternotomy approach, all malformations including CoA can be corrected at the same time. What is neglected but worth mentioning is that we should pay more attention to aortic arch hypoplasia in isolated CoA, otherwise treatment of CoA through a median sternotomy has to be adopted to diminish the chance of CoA recurrence.3 In this series, 5 patients had extended end-to-end anastomosis through a median sternotomy instead of a left posterolateral thoracotomy because of arch hypoplasia.4 The results were satisfactory. If CoA is associated with cardiac anomalies, especially VSD, a one-stage procedure via a median sternotomy is usually applied. Spontaneous closure of a VSD is not easy to achieve, and a re-operation cannot always be avoided. Moreover, the residual abnormal hemodynamics will lead to lung congestion with circulatory and cardiac dysfunction, which affects the prognosis. Thus, it is inappropriate to separate the operation into two stages; the best option for surgical treatment of CoA with associated cardiac malformations is a one-stage procedure by a median sternotomy approach.
Keeping the body temperature at 33°C–35°C is the key to treating CoA via a left posterolateral thoracotomy approach. It is also necessary to keep the lower extremity pressure higher than 40–50 mm Hg, and the cross clamp time less than 30–35 min.5 During aortic cross clamping, it is important to maintain proximal aortic pressure quite high to provide adequate mean aortic arterial blood pressure distal to the clamp to prevent paraplegia. Sodium nitroprusside should never be used during cross clamping for coarctation repair. Large collateral intercostal vessels often enter the descending aorta distal to the coarctation. They should be mobilized carefully and either encircled with vessel loops or ligated and divided to fully mobilize the area of the coarctation. Fine monofilament suture should be used instead of silk sutures to avoid laryngeal nerve injury. It is necessary to resect all ductal tissues that may extend into the area of apparently normal aorta. The key in prosthetic patch aortoplasty is to use a large patch and place the widest portion of the patch at the level of the aortic constriction.
The preferred option to treat CoA with associated cardiac malformations is a one-stage procedure via a median sternotomy. Due to the high rate of recoarctation with arch hypoplasia, the technique of extended end-to-end anastomosis was used, and the results were encouraging.6 It is most important to fully mobilize the descending aorta to the level of the thoracic aorta to avoid distortion of the anastomosis because of high vessel wall tension. The benefits of extended end-to-end anastomosis include resection of all ductal tissues which might extend into the area of apparently normal aorta, simultaneous correction of arch hypoplasia, a low rate of recurrence of CoA, and potential for growth due to homologous tissue.7 The disadvantage is the possibility of hemorrhage due to high vessel wall tension at the anastomosis. However, if the transverse aortic arch is hypoplastic or small, extended end-to-end anastomosis is difficult to perform, and it is better to site the anastomosis between the distal descending aorta and the left wall of the ascending aorta with a normal diameter. If the patch on the aortic arch is small, it is better to extend this anastomosis to the transverse arch. As a result, this anastomosis will not only connect to the descending aorta but also enlarge the patch on the aortic arch (Figure 3
). The anastomosis should not allow such high tension that hemorrhage might occur in the rear wall of the aorta, which is always difficult to repair.
The results with this approach were encouraging, and no recoarctation occurred during the short to mid-term follow-up.8 Determination of the optimal surgical treatment for pediatric CoA associated with other cardiac anomalies requires long-term follow-up, a larger number of patients, and input from multiple research centers.
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