Two-dimensional and Doppler echocardiographic evaluation after arterial switch repair in infancy for complete transposition of the great arteries

The most recent postoperative echocardiographic examinations of all children who underwent arterial switch repair of transposition of the great arteries from August 1985 to December 1987 were reviewed. The patients included 35 children whose age at operation was 12 +/- 16 days and whose weight was 3.6 +/- 0.4 kg. Thirty-three patients are alive and well; 1 died intraoperatively and 1 died immediately postoperatively. The time of the follow-up echocardiographic examination ranged from 1 day to 2.5 years (mean 9.2 months) with 11 patients examined greater than 1 year after surgery. Complete examination of the repair site was possible in all patients. Echocardiographic visualization of distortion of the great arteries at the suture lines was seen in all patients; however, Doppler evidence of hemodynamically significant obstruction at the repair site was uncommon. On Doppler examination in the surviving 33 patients, 16 had no supravalvular pulmonary stenosis and 14 had mild to moderate supravalvular pulmonary stenosis with peak systolic pressure gradients ranging from 16 to 56 mm Hg (mean 31). Three patients had severe supravalvular pulmonary stenosis and peak systolic pressure gradients of 66, 74 and 77 mm Hg (2 have had reoperation, 1 is awaiting surgery). On Doppler examination, 4 patients had mild supravalvular aortic stenosis with peak systolic gradients ranging from 10 to 29 mm Hg. Doppler gradients were confirmed in 10 patients who had catheterization 12 +/- 3 months after surgery. Three patients had mild pulmonary regurgitation by Doppler examination, 5 had mild aortic regurgitation, 4 had mild tricuspid regurgitation and 2 had mild mitral regurgitation.(ABSTRACT TRUNCATED AT 250 WORDS)     

Long-term growth following neonatal anatomic repair of transposition of the great arteries

Despite generally normal prenatal growth, surviving infants with transposition of the great arteries (TGA) frequently develop severe and progressive growth impairment which is not always fully reversed by elective atrial repair within the first year of life. This study was undertaken to determine the effect of neonatal anatomic repair of TGA on long-term growth. Twenty-three children with uncomplicated TGA were followed for a mean of 60 (12–90) months after anatomic repair at a mean age of 11 (1–40) days. Standardized measurements of weight, height, and head circumference for both patients and normal siblings were expressed as percentiles as well as in Z scores (in standard deviations from the mean for age and sex) based on internationally recognized standards. At latest follow-up, 22 (96%) of the patients were above the 3rd percentile for weight and 21 (91%) for both height and head circumference, with 13 (57%), 11 (48%), and 13 (57%) above the 50th percentile for each respective parameter. The mean Z scores (± SD) for weight, height, and head circumference for the patient group were – 0.1 ± 1.2, – 0.2 ± 1.3, and – 0.1 ± 1.1, respectively, and did not differ significantly from those of the reference population (p > 0.05 for each comparison). Paired comparisons of mean Z scores for each growth parameter with those of 35 normal siblings demonstrated no significant difference for weight or height and a small but significant difference for head circumference. Age at surgical repair (within the first 6 weeks of life), duration of follow-up and the development of moderate supravalvar pulmonary stenosis were not statistically related to long-term growth. These results indicate that in patients without extracardiac abnormalities, neonatal anatomic repair of uncomplicated TGA results in normal long-term growth.     

Pulsed Doppler echocardiographic assessment of the pulmonary venous pathway after the Mustard or Senning procedure for transposition of the great arteries

Pulsed Doppler evaluation of pulmonary venous flow was performed in three groups of patients who had undergone either a Mustard or Senning procedure. Group I consisted of 43 patients in sinus rhythm who had undergone the Mustard procedure 9 months or more before the Doppler examination and 16 who were evaluated immediately after surgery. Group II consisted of 12 patients in sinus rhythm who had undergone the Senning procedure 9 months or more before the Doppler evaluation. Group III consisted of eight patients with mid baffle obstruction of the pulmonary venous atrium and seven with isolated stenosis of the pulmonary vein of which all but one had previously had associated mid baffle obstruction. In those without obstruction, the pulmonary venous flow pattern mirrored the left atrial pressure trace, with peak forward flow occurring during the x and y descent. Obstruction produced a specific high-velocity turbulent pattern, whether at the mid baffle or pulmonary venous level. This technique provides reliable noninvasive information about pulmonary venous flow after the Mustard or Senning procedure.     

Two-dimensional echocardiography in complete transposition of the great arteries

Sixty three patients with complete transposition of the great arteries (d-TGA) were studied by two dimensional echocardiography in order to assess: great artery relationships; ventriculo-arterial connections; presence and nature of the left ventricular outflow obstruction (LVOTO). From the parasternal short axis view at the great arteries level, four different relationships were imaged: the aorta was anterior and to the right in 35 patients, the two vessels were in a direct anterior-posterior position in 15 or side by side in 3 and the aorta was anterior and to the left in 10. Differently from normals, early pulmonary bifurcation of the artery arising from the left ventricle was imaged in 42 patients (84%) from the apical approach and in 36 (72%) from the subcostal longitudinal view. Left ventricular outflow tract obstruction was diagnosed in 29 patients (46%) and two distinct types of obstruction were differentiated: fixed and dynamic. We conclude that information derived from two-dimensional echocardiography is a valuable tool for the medical management, cardiac catheterization and surgical treatment of infants and children with d-TGA.     

Pregnancy after atrial repair for transposition of the great arteries

OBJECTIVE: To investigate the risk of pregnancy in patients with transposition of the great arteries (TGA) who have undergone atrial repair. DESIGN: Retrospective analysis (1962-94) of 342 TGA patients who underwent atrial repair. Of 231 known late survivors, 48 were women over 18 years old who were interviewed about possible reproductive plans and previous pregnancies. As a control, comparison was made with data of 57 500 women (mean age 26 years) obtained from the Swiss Statistical Bank in Bern. RESULTS: Mean follow up was 13.7 years; 66% remained asymptomatic, 29% had mild to moderate cardiac symptoms, and 5% suffered from severe cardiac symptoms (New York Heart Association grade III-IV). Thirty six of the 48 women wished to bear children and, to date, there have been 10 live births, two spontaneous first trimester abortions, and one induced abortion at 16 weeks. During pregnancy there was one case of cardiac deterioration and two cases of pneumonia. There was no evidence of congenital heart disease in the children. CONCLUSIONS: In this relatively small series the completion of pregnancy in women with TGA who had undergone atrial repair and who had normal functional cardiac status was uncomplicated     

Intracoronary ultrasound assessment late after the arterial switch operation for transposition of the great arteries

OBJECTIVES: This research was undertaken to assess the status of the coronary wall morphology late after the arterial switch operation (ASO) for transposition of the great arteries employing intravascular ultrasound (IVUS). BACKGROUND: Long-term patency of the reimplanted coronary arteries is a key issue after ASO. Follow-up studies have demonstrated coronary obstruction in up to 8% of patients that may be related to progressive fibrocellular intimal thickening. METHODS: Twenty-two asymptomatic children were enrolled at a median age of 9.5 years (range 5 to 22 years); IVUS images were obtained in 20 children at cardiac catheterization 5.0 to 21.6 years after the operation (in two cases IVUS study was not feasible due to technical constraints). Quantitative analysis was performed in 37 coronary arteries involving segments with a mean length of 28.4 +/- 1.8 mm. RESULTS: Thirty-three arteries (89%) displayed variable degrees of proximal eccentric intimal proliferation, with the maximal intimal thickening being 0.26 +/- 0.14 mm (range 0.06 to 0.71 mm) at the most thickened site. According to the Stanford classification, all children had coronary artery involvement with 50% having moderate-to-severe lesions (>0.3 mm). No risk factors for such abnormalities were encountered, including age, origin of the coronary arteries, hemodynamics, and follow-up duration after surgery. CONCLUSIONS: Intravascular ultrasound assessment late after the ASO revealed proximal eccentric intimal thickening in most of the studied vessels. This observation suggests the development of early atherosclerosis in the reimplanted coronary arteries, which may have a role in the genesis of late coronary events.     

Pregnancy outcomes after atrial repair for transposition of the great arteries

Increasingly, women born with complete transposition of the great arteries who have undergone atrial repair by either the Senning or the Mustard procedure are reaching childbearing age. This study reports on pregnancy outcomes after the atrial repair of transposition of the great arteries. Record review and standardized questionnaires were used to ascertain the outcomes of 70 pregnancies reported in 40 women (36 Mustard procedures, 4 Senning procedures). Of the 70 pregnancies, 54 resulted in 56 live births, 10 in miscarriages, and 6 in therapeutic abortions. At pregnancy, 31 women were in New York Heart Association class I, 8 were in class II, and 1 was in class III. Thirty-nine percent of the infants were delivered prematurely and weighed 2,714 +/- 709 g; 28% were delivered by cesarean section, 8 for cardiac indications. Maternal complications included arrhythmias in 5 women and hemoptysis in 2 women. Heart failure occurred in 6 women, developing during the second and third trimesters. Postpartum cardiac events developed 2 to 9 days postpartum: heart failure in 5 women, atrial fibrillation in 1 woman, and decreased oxygen saturation due to a new atrial baffle leak in 1 woman. Severe right ventricular (RV) failure led to cardiac transplantation after delivery in 1 woman; another developed heart failure and then died suddenly 1 month after delivery. There was 1 late death, 4 years after the patient's last pregnancy. In conclusion, pregnancy after atrial repair carries a moderate degree of risk and should be undertaken with caution.     

Intermediate-term clinical and hemodynamic results of the neonatal arterial switch operation for complete transposition of the great arteries.

We prospectively evaluated 49 consecutive hospital survivors of the arterial switch operation for complete transposition and intact ventricular septum by clinical examination, echocardiography, cardiac catheterization, 12-lead and 24-h Holter ECG. The mean length of follow-up was 40 +/- 18 months. Forty-six children are clinically asymptomatic without medication, 2 died due to coronary related left ventricular dysfunction 3 and 12 months after surgery, and 1 required reoperation because of severe bilateral pulmonary branch stenoses. Except for this case, cardiac catheterization (n = 23) revealed a mean gradient of only 17 +/- 8 mmHg between the right ventricle and distal pulmonary arteries. Left ventricular end-diastolic volume was within normal limits except for 2 cases with volumes slightly below normal, the mean ejection fraction was 78 +/- 5%, and end-diastolic and end-systolic ventricular shapes were normal. The mean cardiac index was 4.14 +/- 0.69 l/min/m2. Left ventricular end-systolic wall stress to velocity of fiber shortening relation was normal in all cases examined (n = 15), indicating normal myocardial contractility. Significant neoaortic valve insufficiency was never observed despite considerably enlarged aortic roots. Twenty-four-hour Holter ECG records (n = 46) provided no evidence of serious atrial arrhythmias, especially sinus node dysfunction. These encouraging intermediate-term results make the arterial switch operation the treatment of choice at present, for neonates with simple transposition.     

一期大动脉调转术治疗完全性大动脉转位

目的:探讨一期大动脉调转术治疗完全性大动脉转位(TGA)的手术适应证及手术效果.方法:2006-05-2007-08对15例患儿[年龄0.5～14(2.51±0.76)个月,体重3～9(3.73±0.86)kg]实施一期大动脉调转手术.15例中室间隔完整TGA(TGA/IVS)13例,均并发房间隔缺损(ASD),同时并发动脉导管未闭(PDA)11例;TGA 并发室间隔缺损(TGA/VSD)2例,均同时并发PDA和ASD.12例并发中度肺动脉高压,3例并发重度肺动脉高压,术前均使用前列腺素E1.手术在全麻、中低温、低流量体外循环下完成.在2大动脉瓣上方横断,将左、右冠状动脉开口移植至肺动脉近端.经肺动脉分叉下方,吻合肺动脉近端和升主动脉远端.用自体心包修复主动脉近端缺失部分,并在新主动脉开放后吻合主动脉近端和肺动脉远端.结果:3例TGA/IVS患儿术后肺部感染,2例(分别为13个月、3个月龄)患儿术后12 h内突发心室颤动死亡,病死率为13.3%.未发生与冠状动脉移植相关并发症.术前肺动脉高压患儿,特别是重度肺动脉高压,术后肺动脉压力均明显下降.13例术后顺利恢复出院.随诊3～12个月,全部患儿心功能恢复良好,无远期并发症和死亡.结论:一期大动脉调转手术对于TGA有较好早期效果.对于年龄超过4个月部分患儿,即使并发肺动脉压,仍可取得满意治疗效果.     

Criteria for two-staged arterial switch operation for simple transposition of great arteries

We analyzed hemodynamic variables in patients with transposition of the great arteries with intact ventricular septum (simple transposition) after pulmonary artery banding and Blalock-Taussig anastomosis to establish the criteria for two-staged arterial switch operation. The study included 35 patients who underwent the switch operation after banding and anastomosis; two died shortly after surgery, and one died of tachycardia 31 days after the operation. The left-to-right ventricular pressure ratio was above 0.83 in all surviving patients, 0.68 and 0.63 in the two operative deaths, and 0.84 in the other death. In the 32 surviving patients, angiographically determined left ventricular (LV) end-diastolic volume and ejection fraction averaged 147 +/- 46(SD)% of normal and 0.64 +/- 0.12, respectively. We also measured LV mass using angiographic LV semiaxes and end-diastolic LV posterior wall thickness obtained from an echocardiogram. These variables of the patients who died were not different from those of the surviving patients. The wall thickness was greater than 4 mm in the surviving patients, while it was 3.5 mm in the two patients who died early and 5 mm in the patient who died later. From the semiaxes, the wall thickness, and aortic diastolic pressure, all of which were obtained before the anatomic repair, we calculated the predictive LV wall stress. This wall stress would indicate the LV wall stress at the time of aortic valve opening immediately after the arterial switch operation. Predictive wall stress was less than 120 x 10(3) dynes/cm2 in all but one of the surviving patients, whereas it was 151, 153, and 186 x 10(3) dynes/cm2 in the three patients who died.(ABSTRACT TRUNCATED AT 250 WORDS)     

Doppler echocardiography after anatomical correction of transposition of the great arteries

Nineteen patients who had anatomical correction of transposition of the great arteries between the ages of one week and 10 years were investigated by pulsed and continuous wave Doppler echocardiography 1-98 months after operation. Peak blood flow velocities across the atrioventricular valves were significantly higher than normal, but no pathological regurgitation was seen. Systolic aortic velocities were normal in all but one patient, but mild aortic regurgitation was detected in ten. Increased flow velocities either at pulmonary valve level, at the pulmonary artery bifurcation, or in the left pulmonary artery were detected in 14 patients, the maximum calculated pressure drop being 36 mm Hg. Adequate Doppler sampling of the right pulmonary artery was not achieved in any of the cases, and sampling of the left pulmonary artery was possible in only ten. The principal limitation of the Doppler ultrasound method in this study was the inability to obtain flow velocities from the branch pulmonary arteries in most patients. Doppler echocardiography provides valuable information after the arterial switch operation and may reduce the need for repeat cardiac catheterisation.     

Echocardiography and Doppler study of congenitally corrected transposition of the great arteries

In order to determine the echocardiographic and Doppler characteristics of patients with congenitally corrected transposition of the great arteries we studied 5 patients with this condition. Mean age was 19 years, ranging from 6 to 57 years. All patients except the oldest were asymptomatic. In all the patients we could observe the typical pattern of ventricular inversion by two-dimensional echocardiography from the apical view. There was dilation of systemic ventricle in 2 patients and dilation of venous ventricle in every patient. The anterograde valvular flows were normal, and also the hepatic vein flow, showing no impediment in the filling of venous atrium and venous ventricle. We observed tricuspid regurgitation in all the patients probably due to valvular inadaptation to the high ventricular pressure. Two-dimensional echocardiography can easily show the alterations of patients with congenitally corrected transposition of the great arteries. The cardiac Doppler gets additional information about the valvular performance.     

Role of balloon atrial septostomy before early arterial switch repair of transposition of the great arteries.

Preoperative balloon atrial septostomy is the standard therapy for babies with uncomplicated cyanotic dextrotransposition of the great arteries despite the effectiveness of prostaglandin E1 infusion in alleviating systemic hypoxemia and the reported success of arterial switch repair during the 1st weeks after birth. The clinical records and echocardiographic findings of 23 infants (mean birth weight +/- SD 3.3 +/- 0.5 kg) with uncomplicated transposition of the great arteries were analyzed. Fifteen infants (Group I) did not undergo septostomy, and 8 (Group II) underwent septostomy. Before prostaglandin infusion, mean arterial oxygen tension (Po2) in Group I (26 mm Hg) did not differ from that in Group II. After prostaglandin infusion, Po2 increased significantly in Group I (43 +/- 8 mm Hg, p less than 0.001) but not in Group II despite a widely patent ductus and predominant left to right ductal shunt in all. After septostomy, Po2 increased significantly (43 +/- 4 mm Hg, p less than 0.03), and did not differ from that in Group I. Echocardiographic features generally demonstrated a nonrestrictive foramen ovale in Group I and a restrictive foramen ovale in Group II. The latter was associated with persistent hypoxemia after prostaglandin. Thus, the diameter of the foramen ovale was the primary factor influencing arterial oxygenation during prostaglandin infusion. Babies underwent the arterial switch operation at a mean age of 70 +/- 65 h with an overall survival rate of 96%; there was only one postoperative death (Group II). Absence of septostomy had no negative influence on any postoperative variable, including duration of ventilatory and inotropic support, time to discharge, or mortality.(ABSTRACT TRUNCATED AT 250 WORDS)