Modified Van Praagh's operation for interrupted aortic arch with severe subaortic stenosis in a neonate

A 26 day old neonate with Type B interrupted aortic arch, a ventricular septal defect and severe subaortic stenosis underwent a successful modified Van Praagh's operation, consisting of insertion of a graft between the main pulmonary artery and descending aorta, ligation of the patent ductus arteriosus and main pulmonary artery banding distal to the graft. This palliative procedure was effective and easily performed without the aid of cardiopulmonary bypass even in this severely ill neonate with such a complex defect.     

Spontaneous closure of ductus arteriosus in interrupted aortic arch with ventricular septal defect

A 2-month-old boy diagnosed with interrupted aortic arch type B was treated with a two-stage procedure. His ductus arteriosus had closed spontaneously. Collaterals via both vertebral arteries developed. A 15-mm stenotic segment existed between the left subclavian artery and the descending aorta. The direct anastomosis between the common carotid artery and the descending aorta was performed as a first palliation at the age of 3 months. The left subclavian artery was reconstructed by end-to-side anastomosis to the descending aorta. The postoperative course was uneventful. The closure of ventricular septal defect and pulmonary artery debanding were performed as a second operation 4 months after the first palliation. The patient is alive and well 7 months after the second operation.     

流出道室间隔缺损合并主动脉瓣关闭不全的外科处理

目的总结流出道室间隔缺损（VSD）合并主动脉瓣关闭不全（AI）的外科处理经验。方法回顾分析94例VSD合并AI的临床资料、VSD补片修补和主动脉瓣处理方法。结果无手术死亡,术后低心排6例（6．4％）。在主动脉瓣脱垂伴轻度AI60例中,出院时超声检查（UCG）14例仍有轻度AI,随访中2例加重需再次手术。在主动脉瓣成形术25例中,UCG提示23例主动脉瓣轻度返流,2例轻微返流,2～5年后3例返流加重。9例主动脉瓣置换术中1例术后1年死于感染性心内膜炎。结论流出道VSD伴有AI,应根据主动脉瓣病理改变而选择恰当的方法。     

主动脉弓中断合并室间隔缺损及肺动脉高压的一期手术矫治

目的 总结主动脉弓中断合并室间隔缺损及肺动脉高压的一期手术矫治经验.方法 2003年2月至2007年8月,一期手术矫治主动脉弓中断合并室间隔缺损及肺动脉高压病婴6例,男、女各3例.手术年龄18 d～14个月,平均(8.3±4.5)个月;平均体重(8.5 ±2.2)kg.A型4例,B型2例.所有病例术前均有反复呼吸道感染史.手术均采用胸骨正中切口.结果 全组病例均生存.随访3～36个月,恢复良好.结论 一期手术矫治主动脉弓中断合并室间隔缺损及肺动脉高压效果良好,需同时加强对围术期肺动脉高压的管理.     

Surgical management of complex and tunnel-like subaortic stenosis

Background: Relief of primary or secondary subaortic stenosis (SAS) remains a surgical challenge. Heart block, aortic valve regurgitation and recurrent obstruction have been persistent problems. Methods: Forty six patients who underwent surgery for complex and tunnel-like SAS between January 1990 and November 1998 were reviewed. In 45 of the 46 patients SAS developed following repair of a primary congenital heart defect and only one patient presented with de novo tunnel-like SAS. Fifteen of the 45 patients had undergone repair of double-outlet right ventricle (DORV) and the remaining 30 had undergone repair of a variety of defects. The median age at the time of surgery was 5 years. The modified Konno procedure was performed in 15 patients, Konno procedure in three, Ross–Konno procedure in two and resection of the conal septum in 12 patients. Five patients with DORV underwent replacement of the intraventricular baffle and two patients underwent an aortic valve-preserving procedure in conjunction with mitral valve replacement. Results: There were no deaths. None of the patients had an exacerbation of aortic regurgitation and none developed complete heart block. The median follow-up was 3 years (range 1 month–8.5 years). Two patients developed recurrent SAS defined as a gradient of 40 mmHg or greater diagnosed by transthoracic echocardiography. Freedom from SAS at 1, 3 and 5 years was 100, 94 and 86%, respectively. Conclusions: We favor the modified Konno procedure and conal resection to the Konno or the Ross procedure, since insertion of a prosthetic valve or homograft is avoided and aortic valve function is preserved. Excellent relief of tunnel-like SAS can be achieved without damage to the conduction tissue.     

Surgical management of patients with interrupted aortic arch and severe subaortic stenosis

Ten patients underwent palliative surgery for interrupted aortic arch and severe subaortic obstruction due to posterior displacement of the conal septum. Their ages ranged between 4 and 28 days (mean, 11.0 +/- 7.7 days) and their weights, between 2.1 and 4.2 kg (mean, 2.85 +/- 0.6 kg). Preoperative echocardiography and cardiac catheterization were performed on all patients. The ratios of the left ventricular outflow tract diameters and the ascending aortic diameters to the descending aortic diameters were 0.56 +/- 0.03 and 0.56 +/- 0.06, respectively, compared with 0.81 +/- 0.12 and 0.95 +/- 0.17, respectively, in 20 patients with interrupted aortic arch but without obstruction (p less than 0.001). Four of the 10 patients underwent pulmonary artery banding and insertion of a bypass graft between the ascending and the descending aorta. All 4 died of low cardiac output soon after operation (100% operative mortality). The remaining 6 patients underwent banding and insertion of a graft between the main pulmonary artery proximal to the band, and the descending aorta. All of these patients survived, and all except 1 are doing well 3 months to 4 years postoperatively. The use of a pulmonary artery-descending aorta conduit and of distal pulmonary artery banding provides good palliation for patients with interrupted aortic arch and major subaortic stenosis.     

Surgical closure of an apical ventricular septal defect through a septal ventriculotomy

Here we report a 3-year and 7-month-old boy with a muscular ventricular septal defect at the apex. At age 7 months, pulmonary artery banding was performed. Surgical treatment consisted of double patch closure via septal ventriculotomy and a main pulmonary artery plasty by an end-to-end anastomosis with cardiopulmonary bypass. There was no residual leak of the ventricular septal defect or ventricular aneurysm and the postoperative course was good. This technique could be a useful surgical option for repair of apical muscular ventricular septal defects.     

Surgical treatment for graft stenosis after repair of an interrupted aortic arch: Report of two cases

We report herein two cases of patients who underwent successful reoperation for graft stenosis after repair of an interrupted aortic arch (IAA). The first patient was a 10-year-old girl who suffered from upper limb hypertension 9 years after her initial operation. Cardiac catheterization revealed a pressure gradient of 55 mmHg across the repaired arch. At reoperation, a left subclavian turndown anastomosis was performed, following which the hypertension resolved and a car-diac catheterization done 5 years later demonstrated sufficient growth of the restored arch with no significant gradient. The second patient was a 17-year-old boy who suffered from general fatigue and intermittent hypertension 12 years after his initial operation. Cardiac catheterization revealed a gradient of 60 mmHg across the repaired arch. He underwent an extraanatomic ascending to descending aortic bypass employing an additional 18-mm graft, and a postoperative cardiac catheterization showed no gradient between the ascending and descending aorta. Our experience has shown that IAA should be repaired without prosthetic grafts if possible. Although extraanatomic bypass is useful for reducing the operative risks at reoperation, a large graft should be used to avoid the need for a third operation. For young children expected to outgrow a second graft, performing an endogenous anastomosis, such as a left subclavian turndown anastomosis, should be considered as an alternative. Received: June 14, 1999 / Accepted: March 24, 2000     

230例婴幼儿室间隔缺损的外科治疗

目的 探讨外科手术治疗婴幼儿室间隔缺损（ＶＳＤ）的结果和经验。方法 对１９９０年２月至１９９７年１２月的２３０例婴幼儿ＶＳＤ修补术进行总结。年龄３￣３６个月,体重３．２￣１５．５ｋｇ。膜周部ＶＳＤ１９２例,干下型ＶＳＤ３６便,膜周部并肌部ＶＳＤ２例,术前中度以上肺动脉高压１５０例（６５．２％）。结果 全组手术死亡１３例,手术病死率为５．６５％。手术死亡的主要原因为肺动脉高压危象和严重心律失常。术后     

小婴儿巨大室间隔缺损的外科治疗

目的 报告３９例出生６个月以内小婴儿巨大型空间隔缺损的外科治疗经验。方法 体外循环下除４例经肺动脉切口外其余均经右房切口,行补片缝合修补空间隔缺损。其中合并动脉导管未闭５例、房间隔水平分流１２例和右室流出道狭窄２例均同期矫治。结果 ３９例病儿均痊愈出院。结论 巨大型空间隔缺损的小婴儿出现顽固性心力衰竭、药物治疗无明显效果或生长发育停滞及肺动脉高压时即应手术。尽可能完善的围术期处理是降低病死率的重要     

Primary repair of aortic arch obstruction with ventricular septal defect in preterm and low birth weight infants

Objective: Previous reports have suggested that prematurity and low birth weight are risk factors for definitive surgical intervention in congenital cardiac malformations. The following data review our experience with primary repair of the complex malformation of aortic arch obstruction with ventricular septal defect (VSD) in this patient population. Methods: Since 1988, 21 consecutive preterm (≤36 weeks) and/or low birth weight (<3000 g) infants with interrupted aortic arch (IAA; n=10), or aortic coarctation (n=11) with VSD, underwent primary arch repair and VSD closure. The mean weight at operation was 2310 g (range, 1200–2900 g), including 12 patients at ≤2500 g. The gestational age ranged from 30 to 41 weeks (mean, 36.4 weeks). Five patients with interrupted arch and two patients with coarctation also had severe subaortic stenosis, which was relieved by transatrial incision of the infundibular septum. Results: The overall hospital mortality was 14% (3/21). Death was related to low cardiac output in association with severe subaortic stenosis (n=2) and sepsis (n=1). Late mortality occurred in three patients, two of which were non-cardiac. The mean follow-up was 33 months. Two patients had significant recurrent arch obstruction, which was successfully relieved by balloon angioplasty and surgical correction in one each. The survival at 30 days, and at 1 and 3 years was 86, 76 and 70%, respectively. Conclusions: Complete primary repair of aortic arch obstruction with VSD can be achieved with good results, even in the preterm and low birth weight infant. Therefore, early surgical repair of this congenital malformation is recommended.     

End-to-side anastomosis for coarctation of the aorta and type a aortic arch interruption with hypoplastic aortic arch

Objective: We investigated the advantages of reconstructing the aortic arch in neonates and infants by end-to-side anastomosis (ESA) in aortic coarctation and type A aortic arch interruption. Subjects and Methods: The patients were 28 neonates and infants who underwent median full sternotomy for correction of aortic coarctation (CoA) and type A interrupted aortic arch (IAA), at our institute, from October 1997 to September 2002. The cases were divided into two groups: ESA was performed in 16 cases (group A) and extended end to end anastomosis in 12 cases (group B). All repairs were made using simple clamping of the descending aorta under cardiac arrest. We compared groups A and B with regard to duration of simple clamping, postoperative blood pressure in the upper and lower extremities, echocardiographic pressure gradient, and the presence or absence of tracheal stenosis as diagnosed by postoperative clinical symptoms and chest radiography. Results: No significant difference between the groups was found in age, gender, body weight, or the ratio of CoA to IAA. In addition, there was no defference between the two groups in the duration of simple clamping or the difference in blood pressure between the lower and upper extremities. However, the echocardiographic pressure gradient at the site of anastomosis in group A was significantly lower than that in group B. There were no cases with symptoms of bronchial stenosis in either group. Conclusion: ESA is a relatively simple method for treating CoA or IAA with hypoplastic aortic arch, and was found to be effective for the elimination of residual stenosis.     

室间隔缺损修补术后残余漏的外科治疗

目的　总结室间隔缺损 (室缺 )修补术后残余漏的外科治疗经验 ,探讨残余漏的易发部位。方法　 1979年 1月至 2 0 0 3年 5月对 37例室间隔缺损术后残余漏患者行手术治疗 ,单纯室间隔缺损术后残余漏 19例、法洛四联症术后室间隔残余漏 17例、右心室双出口术后室间隔残余漏 1例 ,占同期心脏手术的 0 2 1% (37/ 180 0 0 )。其中男 2 6例、女 11例 ,年龄 3个月～ 5 3岁 ,平均 (16± 12 )岁。全组以室缺术后再度出现心脏杂音并行超声心动图检查确诊。手术用补片修补残余漏 2 6例 ,直接缝合残余漏 11例。结果　手术死亡 2例 ,病死率 5 % (2 / 37) ;手术成功 35例 ,术后随访 3个月～ 15年 ,疗效满意。结论　室间隔缺损修补术后残余漏多见于三尖瓣隔瓣根部 ,其次为第二和第一转移针处 ;室间隔缺损残余漏二次手术效果良好。     

Closure of a ventricular septal defect in a patient with von Willebrand disease

Few reports of open heart surgery being performed in patients with von Willebrand disease (vWD) have been documented. We describe, herein the case of a 5-year-old girl with a ventricular septal defect (VSD) and vWD who underwent patch closure under cardiopulmonary bypass (CPB). The hematological parameters relating to vWD, namely, factor VIII, von Willebrand factor, and factor VIII-related antigen were monitored perioperatively. These laboratory findings were elevated after the termination of CPB, and consistently maintained within the normal range for 4 months postoperatively. The operation was successfully completed without the administration of factor VIII concentrates or homologous blood products.