Long-term neurodevelopmental outcomes in school-aged children after neonatal arterial switch operation

OBJECTIVE: Neurodevelopmental status of children between 8 and 14 years of age after neonatal arterial switch operation for transposition of the great arteries has not previously been systematically evaluated. METHODS: Within a longitudinal study, 60 unselected children operated on as neonates with combined deep hypothermic circulatory arrest and low-flow cardiopulmonary bypass were reevaluated at the age of 7.9 to 14.3 years (mean +/- SD 10.5 +/- 1.6 years). Clinical neurologic status and standardized tests to assess gross motor function, intelligence, acquired abilities, language, and speech were carried out, and the results were related to preoperative, perioperative, and postoperative status, to management, and to neurodevelopmental status at a mean age of 5.4 years. RESULTS: Neurologic and speech impairments were evidently more frequent (27% and 40%, respectively) than in the general population. Intelligence and socioeconomic status were not different (P =.29 and P =.11), whereas motor function, acquired abilities, and language were reduced (P < or =.04 for each). Overall rate of developmental impairment in one or more domains was 55%, compared with 26% at age 5.4 years. Multivariable analysis showed that severe preoperative acidosis and hypoxia predicted reduced motor function (mean deficit 52.7 points, P <.001), whereas longer bypass duration predicted both neurologic (odds ratio per 10 minutes of bypass duration 1.8, P =.04) and speech (odds ratio per 10 minutes of bypass duration 1.9, P =.02) dysfunction, and perioperative and postoperative cardiocirculatory insufficiency predicted neurologic (odds ratio 6.5, P =.04) and motor (mean deficit 6.8 points, P =.03) dysfunction. CONCLUSIONS: The neonatal arterial switch operation with combined circulatory arrest and low-flow bypass is associated increasingly with age, with reduced neurodevelopmental outcome but not with cognitive dysfunction. In our experience, the risk of long-term neurodevelopmental impairment after neonatal corrective cardiac surgery is related to deleterious effects of the global perioperative management and to special adverse effects of prolonged bypass duration. Severe preoperative acidosis and hypoxia and postoperative hemodynamic instability must be considered as important additional risk factors.     

Long-term results of arterial switch operation

Surgical results and late outcome in 202 patients who had undergone arterial switch operation from 1984 to 1997 were investigated. Actuarial survival was 90.6% at 10 years and 90.0% at 20 years. Fifty-two patients (25.7%) underwent reoperation for pulmonary stenosis and 7 patients (3.5%) had aortic valve replacement. Freedom from re-intervention was 71.9% at 10 years and 60.4% at 20 years. Using xeno-pericardial patch for pulmonary reconstruction was strong predictor for postoperative pulmonary stenosis. Coronary ischemic event was rare but some patients showed electorocardiogram (ECG) change on exercise and hypoplastic left coronary artery. Cardiopulmonary function was almost normal in long term survivors.     

Coronary revascularization after arterial switch operation.

We report two cases presenting bilateral coronary artery obstruction after arterial switch operation. The first patient underwent bilateral internal thoracic artery grafting to the left and right coronary arteries. The other patient, presenting a single coronary ostium, underwent surgical coronary ostial angioplasty in concomitance to proximal arterioplasty of both coronary arteries employing a single "pantaloon" shape autologous pericardial patch. Both patients survived and, at 1 year and 9 months after the coronary revascularization procedures, the coronary angiography demonstrated a good patency of the internal thoracic grafts and excellent ostial plasty results, respectively. A complete literature review of patients undergoing different coronary revascularization procedures after arterial switch operation is reported.     

Long-term survival and functional follow-up in patients after the arterial switch operation

BACKGROUND: For many years, the arterial switch operation (ASO) has been the therapy of choice for patients with transposition of the great arteries (TGA). Although excellent short- and mid-term results were reported, long-term results are rare. METHODS: Between May 1983 and September 1997, ASO was performed on 285 patients with simple TGA (n = 171), TGA with ventricular septal defect (VSD) (n = 85), and Taussig-Bing (TB) anomaly (n = 29). This retrospective study describes long-term morbidity and mortality over a 15-year period. RESULTS: Hospital mortality was 3.5% for simple TGA, 9.4% for TGA with VSD, and 13.8% for TB anomaly. Late death occured in 2 patients, 1 with simple TGA and 1 with TGA and VSD. The cumulative survival for all patients at 5 and 10 years is 93%, and at 15 years is 86%. Reoperations were required in 31 patients and were most common for stenosis of the right ventricular outflow tract (RVOT). However, no correlation was found between technical variations on pulmonary artery reconstruction and this type of complication. Forty-six patients underwent follow-up angiography, which revealed five cases with coronary occlusion or stenosis. Follow-up is complete in 96% of the patients from 1 to 15.2 years. Sinus rhythm is present in 97%; 88% of the patients show no limitations on exertion. CONCLUSIONS: The ASO can be performed with low early mortality, almost absent late mortality, and infrequent need for reoperation. The favorable long-term results demonstrate that the ASO can be considered as the optimal approach for patients with TGA and special forms of double-outlet right ventricle.     

Long-term extracorporeal membrane oxygenation in a newborn child after arterial switch operation

A 7-day-old newborn girl with d-transposition of the great arteries (d-TGA) and complex coronary anatomy developed global myocardial dysfunction after arterial switch operation despite establishment of an effective coronary circulation. Extracorporeal membrane oxygenation was used for circulatory support for 163h after the operation. On day 7 after surgery she was successfully weaned from extracorporeal membrane oxygenation and is currently symptom-free 10 months after the operation. This case illustrates the effectiveness of extracorporeal membrane oxygenation for circulatory support after the arterial switch operation. In such cases, establishing adequate coronary circulation during surgery is mandatory to ensure a successful postoperative myocardial recovery.     

Neo-coarctation after the arterial switch operation

Neo-coarctation following arterial switch operation (ASO) for transposition of the great arteries (TGA) is a complication that is not regularly described, but may occur. We describe five patients who developed a neo-coarctation after operation. They were diagnosed with TGA, either with or without ventricular septal defect without signs or symptoms of a coarctation. Except for one patient, all patients were reoperated for a neo-coarctation within one year after the ASO. Several explanations are discussed as a possible cause for this phenomenon.     

Intrapericardial diaphragmatic hernia after arterial switch operation

Intrapericardial diaphragmatic hernia after median sternotomy for cardiothoracic procedures is a rare complication. We describe an interesting case of diaphragmatic hernia in a 6-month-old girl presenting as cardiac tamponade, 4 months after an arterial switch operation. The diaphragmatic defect was iatrogenic and emphasizes the danger of inadvertent diaphragmatic injury during cardiac operations.     

Aortic valve repair after arterial switch operation

A patient with transposition of the great arteries and a ventricular septal defect underwent an arterial switch operation 15 months after pulmonary artery banding. At 12 years of age, severe neoaortic valve regurgitation, due to dilated aortic sinuses and poor leaflet coaptation, developed. Aortic valve repair involved placement of subcommissural sutures, elliptical excision and tailored reduction of two anterior aortic sinuses, with triangular patch expansion of the proximal ascending aorta. A good result was obtained.     

Long-term coronary artery outcome after arterial switch operation for transposition of the great arteries

Objective: To analyse the long-term patency of coronary arteries after neonatal arterial switch operation (ASO). Methods: A retrospective study of the operative reports, follow-up and postoperative catheterisation data of 119 patients, who underwent the great arteries (TGA) repair since 1991, has been carried out. Patient population: Among the 133 survivors of the 137 ASOs performed between 1991 and 2007, 119 patients have been studied by routine control cardiac catheterisation and form the study population. Median time between repair and the coronary angiography was 2.9 ± 1.9 years. A comparison between the eight patients (6.7% out of the entire study population), known to have postoperative coronary obstructions (group I) and the rest of the cohort with angiographic normal coronary vessels (group II) was performed by univariate analysis of variance and logistic regression models. One patient had surgical plasty of the left coronary main stem with subsequent percutaneous angioplasty, three patients had primary coronary stent implantation and four patients had no further intervention at all. In group I, all but one patient denied symptoms of chest pain and echocardiography failed to show any difference between the two groups in terms of left ventricular systolic function (ejection fraction group I 61 ± 2% vs 62 ± 6% of group II, p = 1.0). Results: The association of coronary obstruction with complex native coronary anatomy (Yacoub type B to E) was evident at both univariate (62% of group I vs 22% of group II, p = 0.04) and logistic regression (p = 0.007, odds ratio (OR) 8.1) models. The type of coronary reimplantation (i.e., coronary buttons on punch vs trap-door techniques) was similar between the two groups (punch reimplantation in 25% of patients of group I vs 31% of group II, p = 0.1) as was the relative position of the great vessels (aorta anterior in 100% of patients of group I vs 96% of group II; univariate, p = 0.1). Conclusions: The late outcome in terms of survival and functional status after ASO is excellent. Nevertheless, the risk of a clinically silent late coronary artery obstruction of the reimplanted coronary arteries warrants a prolonged follow-up protocol involving invasive angiographic assessment.     

Long-term results of cardiac and general health status in children after neonatal arterial switch operation

BACKGROUND: The purpose of this study was to assess cardiac and general health status 8 to 14 years after neonatal arterial switch operation for transposition of the great arteries. METHODS: Sixty unselected children with intact ventricular septum (78.3%) or ventricular septal defect (21.7%) without or with aortic isthmic stenosis (5.1%) were examined 10.5 +/- 1.6 (mean +/- SD) years after neonatal switch and 5.3 +/- 1.6 years after mid-term evaluation. Complete clinical examination, standard and 24-hour Holter electrocardiogram, M-mode, 2D-, Doppler, and color Doppler echocardiography were performed. Results were compared with normal values and to mid-term follow-up results. RESULTS: Rates of reoperation after arterial switch operation and operation to correct concomitant coarctation were 3.3% and 5.1%, respectively. No patient needed medication, and 93.3% had no limitation of physical activity. All children had normal height and weight; 31.6% had abnormal thoracic configuration after median sternotomy. Most patients (91.7%) were in sinus rhythm. Incidence of complete right bundle branch block (10.0%) was unchanged, as was prevalence of ectopic activity (occasional atrial ectopy 20.0%, ventricular ectopy: occasional 21.7%; frequent 1.7%). Left ventricular dimensions and shortening fraction did not change over time. Diameters of neo-aortic valve annulus and neo-aortic root did not increase, and z-scores decreased between mid-term and present evaluation. Incidence of neo-aortic insufficiency was 13.3% and remained unchanged in comparison with the pre-examination value. Neo-aortic stenosis was not seen. Compared with mid-term follow-up, incidence (41.6%) and degree of supravalvular pulmonary stenosis increased. CONCLUSIONS: Good cardiac results persist 10 years after neonatal arterial switch operation for transposition of the great arteries. Encouraging findings include preservation of left ventricular function, low incidence of rhythm disturbances, lack of further neo-aortic root dilatation, and unchanged incidence of neo-aortic insufficiency compared with mid-term follow-up. Increased incidence and degree of supravalvular pulmonary stenosis are of concern.     

Fate of the aortic root after arterial switch operation.

OBJECTIVE: Concerns have been voiced about possible dilation and insufficiency of the neo-aortic valve after the arterial switch operation (ASO). AIMS: To determine growth of the neo-aortic valve and the aortic anastomosis after ASO and the prevalence of insufficiency or stenosis. PATIENTS AND METHODS: Since 1977, 144 consecutive patients (pts) underwent ASO for transposition of the great arteries (TGA). Median follow-up was 8.65 years (0.1--22.5 years). Simple TGA was present in 97 pts and 47 had TGA with ventricular septal defect (VSD). Detailed echocardiography included measurements of aortic diameter at four levels. The 608 measurements were compared with published normal values. RESULTS: The mean aortic valve z-score was 1.5, without significant change with age (P=0.75). Under 4 months, mean valve z-score was 0.63+/-2.20, between 5 and 12 months 2.56+/-2.30 (P<0.0001). Gradual growth occurs thereafter. The aortic sinus follows an identical growth pattern. The aorta at the anastomosis, is initially smaller than normal (z-score -0.64). After 4 months the z-score is 0.83, followed by continued growth of 0.1 z-score per year. At the last visit, the aortic valve z-score was above 2 in 51 patients, between -2 and 2 in 72 and less than -2 in six patients, none of whom had a flow velocity above 2 m/s. z-score of patients with VSD remained above those without VSD (P<0.0001).Aortic insufficiency was grade 2/4 in three patients, grade 3/4 in one and grade 4/4 in one. No patient developed aortic stenosis. CONCLUSION: After ASO the neo-aortic valve and sinus are larger than normal, representing the natural size difference in the prenatal situation and influence of associated cardiac malformations. In the first year of life, rapid dilatation of the new aorta is observed, followed by growth towards normalization of the valve and sinus size. Stenosis at the anastomosis was not observed. Aortic dilatation by itself is rarely associated with significant insufficiency.     

Unusual presentation of myocardial ischemia after arterial switch operation.

Ischemic changes and acute or subacute cardiac failure after arterial switch operation generally results from imperfect transfer of coronary arteries to the neoaorta. Peroperative and early postoperative myocardial ischemia is the main cause of death in these patients. We present an unusual cause of myocardial ischemia and cardiac failure after arterial switch: a congenital coronary artery fistula between the circumflex artery and the right ventricle. The symptoms imitate coronary translocation failure. In differential diagnosis of the coronary perfusion problems encountered after the arterial switch operation, coronary artery fistula should not be forgotten, although it is rare.     

Long-term outcomes after upper limb arterial injuries

Objective

To assess long-term outcomes in multisystem trauma victims who have arterial injuries to upper limbs.

Design

A retrospective case series.

Setting

Tertiary care regional trauma centre in a university hospital.

Patients

All consecutive severely injured patients (Injury Severity Score greater than 15) with an upper limb arterial injury treated between January 1986 and January 1995. Demographic data and the nature and management of the arterial and associated injuries were determined from the trauma registry and the hospital records.

Outcome measures

Death rate, discharge disposition, residual disabilities and functional outcomes as measured by the Glasgow Outcome Scale.

Results

Twenty-five (0.6%) of 4538 trauma patients assessed during the study period suffered upper extremity arterial injuries. Nineteen of them were victims of blunt trauma. The death rate was 24%. There were 10 primary and no secondary amputations. An autogenous vein interposition graft was placed in 10 patients. Concomitant fractures or nerve injuries in the upper limb were present in 80% and 86% of the patients, respectively. Long-term follow-up data (mean 2 years) were obtained in 16 of the 19 who survived to hospital discharge. The residual disability rate was high. It included upper limb joint contractures, pain and persistent neural deficits (69%). Associated injuries in other body areas also contributed to overall disability. Only 21% of the patients recovered completely or had only minor disabilities.

Conclusions

Associated injuries, rather than the vascular injury, cause long-term disability in the multi-system trauma victim who has upper extremity involvement. Persistent neural deficits, joint contractures and pain are the principal reasons for long-term impairment of function.     

Internal mammary artery bypass after the arterial switch operation

Myocardial ischemia may ensure after coronary artery translocation during the arterial switch operation. We report the successful use of a right internal mammary artery to right coronary artery bypass graft in an infant with angiographic documentation of persistent graft patency 6 months postoperatively.     

大动脉调转术后早期循环支持

目的　总结分析复杂先天性心脏病患者行大动脉调转术后早期的循环功能特点及循环支持的经验。　方法　回顾性分析 2 0 0 0年 1月至 2 0 0 2年 12月本院收治的 32例患者临床资料 ,术后均经联合应用正性肌力药、适当利尿、短期肾脏替代治疗及延迟关胸等综合治疗 ,维持循环功能稳定。　结果　死亡 7例 ,存活 2 5例 ,其中安装永久性起搏器 2例。术后随访 6～ 36个月 ,存活患者心肺功能正常。　结论　术后早期有效的循环支持是提高术后存活率及患者远期生活质量的关键     

Successful application of pulmonary arterial patch for coronary angioplasty late after arterial switch operation.

Pulmonary artery is infrequently utilized as a material for coronary patch angioplasty in children. We applied a pulmonary arterial patch for coronary angioplasty on an 8-year-old boy with total occlusion of the left main coronary artery late after an arterial switch operation. The pulmonary arterial patch was easy to handle and the immediate result after the operation was satisfactory.