Staged biventricular repair of Taussig-Bing anomaly with subaortic stenosis and coarctation of aorta

We present successful procedures for 2 infants who had the Taussig-Bing anomaly with subaortic stenosis and coarctation of the aorta. The initial procedure was coarctoplasty and the Damus-Kaye-Stansel procedure with modified Blalock-Taussig shunt. The second procedure was intraventricular repair (Kawashima procedure), Damus-Kaye-Stansel take-down and the reuse of native aortic and pulmonary valves 19 and 25 months later. Both patients survived the operations and postoperative hemodynamics were excellent at both 28 and 59 months follow-ups.     

Surgical treatment of persistent fifth aortic arch associated with interrupted aortic arch

This study describes two cases of the rare congenital anomaly, persistent fifth aortic arch. Both cases involve boys (1 at 9 years of age and another at 7 months of age). To detect persistent fifth aortic arch with interrupted aortic arch, the following methods were used: echocardiogram, angiocardiography, and magnetic resonance imaging. In both cases the blood pressure between the upper and lower limbs differed. To relieve the obstruction of blood flow, each case was surgically repaired using patching or conduit interposition. Postoperative courses were uneventful. Two-year to 5-year follow-up examinations were positive showing that the anastomosis was unobstructed and the velocity of blood flow to the descending aorta was normal.     

Truncus arteriosus with interrupted aortic arch: successful repair using modified cardiopulmonary bypass and surgical techniques

A neonate with truncus arteriosus (type 1) and interrupted aortic arch (type A) associated with an excessively large gap between interrupted aortic segments underwent successful reconstruction of the aortic arch with a partial subclavian flap aortoplasty plus bovine jugular patch (Contegra 200, Medtronic, Inc, Minneapolis, MN) using continuous selective low-flow cardiopulmonary bypass without circulatory arrest. The ventricular septal defect was closed with a Dacron (IMPRA, Inc, Tempe, AZ) patch, and a Contegra prosthesis was used to re-establish right ventricle to pulmonary artery continuity. At an 18-month follow-up, the patient was free of symptoms with normal development. Echocardiography revealed a widely patent aortic arch and an unobstructed right ventricle to pulmonary artery connection.     

Results of arterial switch operation for primary total correction of the Taussig-Bing anomaly

BACKGROUND: This study evaluates the results of the arterial switch operation for early total repair of double-outlet right ventricle with subpulmonary ventricular septal defect (the Taussig-Bing heart). METHODS: From 1986 through April 2003, 27 patients with Taussig-Bing anomaly underwent arterial switch operation. Twenty patients were neonates (n = 11) or infants younger than 3 months (n = 9). Obstruction of aortic arch (n = 19) or subaortic right ventricular outflow tract obstruction (n = 20) and unusual coronary artery patterns (n = 19) were common. Total correction as a single procedure was performed in 21 patients. Events are depicted by Kaplan-Meier curves. RESULTS: There was 1 patient hospital death at 2 months after repair. One patient died late that was not cardiac related. Survival was 92% +/- 6% at 8 months and remained constant thereafter. Four patients underwent reoperation (1 for residual aortic arch obstruction and 3 for subvalvular and valvular pulmonary stenosis). Freedom from reoperation decreased to stabilize at 83% +/- 8% after 2 years. The risk to have right ventricular outflow tract obstruction develop was 33% +/- 10% at 1 year, increasing slowly and leveling out at 57% +/- 12% at year 5 and thereafter. Statistical analysis revealed no significant risk factor for death or need for reoperation. CONCLUSIONS: The Taussig-Bing anomaly should be corrected in the neonatal period or in early infancy by arterial switch operation, closure of the ventricular septal defect, and simultaneous correction of associated cardiovascular anomalies as a one-stage procedure. Right ventricular outflow tract obstruction often complicates the postoperative course and is the main cause for reintervention.     

Transplantation in truncus arteriosus combined with interrupted aortic arch

We present the case of an infant with truncus arteriosus combined with a dysplastic truncal valve not amenable to repair and interrupted aortic arch. Due to the disappointing results of truncal valve replacement we decided to perform cardiac transplantation. The infant survived transplantation and was discharged 4 weeks after surgery.     

Taussig-Bing畸形合并主动脉弓病变一期外科治疗

目的总结一期手术治疗Taussig-Bing畸形合并主动脉弓病变的经验。方法2000年5月至2006年12月手术治疗12例Taussig-Bing畸形合并主动脉弓病变病儿，其中主动脉弓中断5例。采用大动脉调转（arterial switch operation，ASO）的手术方法矫治Taussig-Bing畸形，将胸主动脉与主动脉弓下缘端端吻合或与升主动脉端侧吻合矫治主动脉弓病变。结果住院死亡3例（25％），使用呼吸机平均（7．4±2．1）d，ICU滞留平均（11．7±4．6）d。生存病儿无因残余解剖问题再次手术者。结论Taussig-Bing畸形合并主动脉弓病变病理解剖比较复杂，手术方式的选择、手术技巧和合并主动脉弓病变对Taussig-Bing畸形的治疗效果有影响。     

Pulmonary agenesis with interrupted aortic arch.

This report describes a unique case of an infant with right pulmonary agenesis associated with interrupted aortic arch, ventricular septal defect, and aortic valvular stenosis. Blalock-Park arterial anastomosis and pulmonary arterial banding were performed without incidence. These techniques were useful for this complicated heart defect with pulmonary agenesis. The postoperative course of the patient was uneventful.     

Pulmonary root translocation for biventricular repair of double-outlet left ventricle

Double-outlet left ventricle is conventionally repaired with an extracardiac conduit when pulmonary stenosis is present. We report the use of pulmonary root translocation to the right ventricle to construct the posterior wall with autologous tissue and a porcine pericardial monocusp ventricular outflow patch anteriorly for 2 patients with double-outlet left ventricle. This technique allows minimization of pulmonary insufficiency, avoids coronary artery ligation with infundibulotomy, and has a major theoretical advantage for growth potential.     

Staged surgical repair for infants with interrupted aortic arch

BackgroundInterrupted aortic arch (IAA) is a rare congenital cardiac anomaly, which necessitates surgical treatment. There are several surgical strategies for corrective repair of IAA, such as one-stage repair, rapid two-stage repair and two-stage repair. Here, we reported our surgical result of staged-repair policy for the patients with IAA.MethodFrom November 2003 to July 2015, there were 14 patients (8 boys, 6 girls) with IAA treated by us. Except one teenager patient, we routinely used intravenous infusion of prostaglandin E1 for all the infant patients (n = 13) to keep adequate end organ perfusion before the first surgical intervention. Surgical repair was performed after the perfusion of end organs recovered.ResultTwo patients (1 teenager and 1 infant with one-stage surgery) were excluded from this study. At the time of the first surgery, we did the first-stage surgery with anastomosis in between aortic arch and descending aorta, division of patent ductus arteriosus and banding of pulmonary trunk through left thoracotomy. The overall surgical survival rate of the first surgery was 100% (12/12). At the time of the second surgery, corrective repair was done under cardiopulmonary bypass through median sternotomy. The surgical survival rate of the corrective surgery was also 100%. There is no late death during follow-up for 9 years (range 4.2–15.0 years).ConclusionOut of several surgical strategies for the infants with IAA, staged repair still could be a treatment option to achieve satisfied surgical result.     

主动脉弓中断的外科治疗

目的 总结主动脉弓中断及合并心脏畸形的外科治疗经验.方法 1997年1月至2008年1月,36例主动脉弓中断患者进行了外科手术治疗,其中男性22例,女性14例.儿童患者36例,年龄2个月～7岁,平均年龄2.8岁.成人患者1例,年龄31岁.33例合并心内畸形,其中31例正中开胸同时矫治主动脉弓中断和心内畸形;1例左侧切口矫治主动脉弓中断,正中开胸修补心内畸形;1例采用姑息手术.3例无心内畸形的患者2例采用左后外侧切口,1例采用正中胸部加上腹部切口.术式包括16例管道连接,9例直接吻合,9例直接吻合并补片成形,1例应用左锁骨下动脉翻转.在31例正中切口一期手术中,17例应用选择性脑灌注加下半身停循环,8例采用深低温低流量灌注,6例采用全身停循环.结果 住院死亡5例,3例死于肺部感染,1例死于肺动脉高压危象,1例死于术后低心排血量.术后早期有其他重要并发症7例.31例存活患者随访3个月～5年,无远期死亡,无需要再次手术的病例.结论 合并心内畸形的主动脉弓中断患者可采取选择性脑灌注加下半身停循环或深低温全身低流量下正中一期手术同时矫治.     

Pulmonary root translocation in transposition of great arteries repair

Anterior pulmonary root translocation is used as a new approach for anatomic repair of transposition complexes with ventricular septal defect and pulmonary stenosis. It is performed to construct the right ventricle outflow tract, after patch diversion of left ventricle to aorta. Since 1994, 3 infants underwent this procedure. The preliminary results indicate some growth of the pulmonary root and suggest that this technique could diminish reoperations in this group of patients.     

Associated factors and trends in outcomes of interrupted aortic arch

Background

Interrupted aortic arch (IAA) continues to be associated with important mortality, both before and immediately after repair, with ongoing morbidity during follow-up. We sought to determine trends in presentation, management, outcomes and associated factors.

Methods

We reviewed all consecutive patients (n = 119) presenting from 1975 to 1999, and data were collected regarding demographics, anatomy, management and outcomes.

Results

Significant trends over time for patients born in three consecutive periods (1975 to 1984, 1985 to 1993, and 1994 to 1999) demonstrated a smaller proportion of patients with presentation with circulatory collapse (65%, 51%, and 25%, respectively), greater use of prostaglandins (72%, 90%, 100%), fewer deaths without IAA repair (49%, 15%, 13%) and greater use of one-stage repair (68%, 75%, 100%). Independent risk factors for death without IAA repair (p < 0.001) included absence of ventricular septal defect, and the presence of noncardiac anomaly, complex cardiac anomaly, episode of acidosis and earlier birth cohort. Overall survival after repair was 50% at age 1 month, 35% at 1 year, and 34% at 5 years. Early and constant-hazard phases were noted, with incremental risk factors for early phase mortality being cyanosis at presentation, presence of truncus arteriosus or aortic stenosis, an episode of circulatory collapse before repair, earlier date of repair, and lower weight at repair. Greatest survival occurred in those patients with uncomplicated IAA who had repair since 1993 (5 year survival, 83%). Freedom from reintervention for arch obstruction was 60% at 5 years.

Conclusions

While improving, outcomes of IAA remain of concern, especially in patients with associated lesions.     

Early and medium-term results for repair of Ebstein anomaly

OBJECTIVES: We evaluated the early and medium-term single-center results for primary repair of Ebstein anomaly in both adults and children. METHODS: The records were reviewed of patients undergoing repair of Ebstein anomaly at the Children's Hospital of New York from September 1990 to September 2002. Functional, demographic, and echocardiographic parameters were studied both preoperatively and postoperatively, along with functional status and adverse events. The repair technique involved vertical plication of the atrialized ventricle and valve leaflet reimplantation after clockwise rotation. RESULTS: A total of 25 patients (19 children and 6 adults) underwent repair. The average age was 14.2 +/- 15.9 years, and the average follow-up was 4.1 +/- 3.4 years. Three patients required reoperation for right ventricular overload (1 child) and progressive, severe tricuspid regurgitation (2 adults); both adults received tricuspid valve replacements, one at 4 years and the other at 8 years post-repair. Three patients had radiofrequency ablation procedures performed intraoperatively. Ten patients (40%) had moderate-to-severe tricuspid regurgitation perioperatively. However, 18 children (95%) and 5 adults (83%) demonstrated significant improvement in exercise capacity late postoperatively. Two children died suddenly 11 months and 4 years after repair. DISCUSSION: Ebstein repair has good functional outcomes in children despite residual tricuspid regurgitation, likely because of reduction in right ventricular volume loading and relative annular and ventricular plasticity. Adult patients did not demonstrate the same durability of valve repair and frequently required tricuspid valve replacement. Intraoperative radiofrequency ablation represents an important adjunctive treatment for intractable arrhythmias, which may now represent relative indications for operative intervention.