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     

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.     

Long-term results of anatomic repair of transposition of great vessels

The long-term complications after anatomical repair of transposition of the great arteries (TGA) were analysed in a prospective study of 30 successive patients, from August 1996 to October 1999, who were presumed asymptomatic and investigated 10 years after surgery. All underwent clinical examination, ECG, stress Thallium 201 myocardial scintigraphy, Doppler echocardiography, Holter ECG, pulmonary perfusion scintigraphy, right and left cardiac catheterization with selective coronary angiography. Five patients had coronary lesions (4 thromboses and 1 coronary-pulmonary artery fistula). The other abnormalities observed were mild bilateral stenosis of the two pulmonary arteries (1 case), grade 1 aortic regurgitation (6 cases), including 1 case of aortic root dilatation. Type B to E coronary circulations (Yacoub classification) were not significantly correlated with coronary artery disease in this series (p = 0.06). For the diagnosis of these lesions, myocardial scintigraphy and Doppler echocardiographic detection of wall motion abnormalities had a sensitivity of 50% and respective specificities of 88% and 35%. Long-term results after anatomical repair of TGA are satisfactory. However, the high incidence of coronary lesions makes regular follow-up and systematic coronary angiography necessary in all children.     

Balloon dilation of pulmonary venous pathway obstruction after Mustard repair for transposition of the great arteries

Percutaneous balloon dilation was attempted in three patients with mid baffle pulmonary venous pathway obstruction after the Mustard operation for transposition of the great arteries. The procedure was unsuccessful in a 3 year old boy. Evidence for relief of obstruction in the other two patients (7 and 14 years old, respectively) consisted of angiographic demonstration of improved caliber at the site of the pulmonary venous pathway narrowing, improvement in the Doppler spectral signal at this site from an "obstructed" to a "normal" pattern as well as symptomatic improvement. Balloon dilation was performed twice in one of these patients. The Doppler and symptomatic improvement were sustained in both patients at short-term follow-up (5 and 6 months, respectively). This technique may offer effective relief of pulmonary venous pathway obstruction in some patients with this complication of the Mustard operation.     

Long-term outcome after senning operation for transposition of the great arteries

BACKGROUND: The Senning operation for transposition of the great arteries (TGA) was first introduced by Senning in 1959 and was revived by Quaegebeur et al. in the late 1970s, thus becoming the intervention of choice for the correction of TGA in many centers. HYPOTHESIS: The purpose of this study was to evaluate the long-term follow-up of a group of patients undergoing surgery with the Senning procedure for TGA. METHODS: From November 1978 to November 1987, 73 consecutive patients underwent the Senning operation. The 70 survivors had an average follow-up of 19 years (16-25 years). RESULTS: (1) Cardiac rhythm: with time there was a progressive decrease in stable sinus rhythm (60% after 20 years) and a progressive increase of supraventricular tachyarrhythmias requiring therapy (10% after 20 years). (2) Right ventricular function: 20% of the patients had reduced ejection fraction. (3) Late mortality: in the last 12 years of follow-up years there were two sudden deaths (2.8%). (4) Functional status: 80% of patients were in NYHA class I, 17% in class II, and 3% in class III. CONCLUSIONS: Our results confirm that the patients who undergo the Senning procedure have a progressive loss of sinus rhythm, an increase in active arrhythmias, and other important adverse outcomes such as late sudden death and a decrease in right ventricular function; however, most patients (93% in our series) are alive and in good functional status.     

Corrected transposition of great arteries. Surgical results

Thirteen patients with congenitally corrected transposition of the great arteries underwent cardiac surgery between May 1964 and May 1984. Eleven cases underwent corrective surgery and two palliative treatment. Four cases had closure of the ventricular septal defect two had pulmonary valvotomy, two closure of atrial septal defect and three tricuspid valve replacement. Two patients developed atrioventricular dissociation, requiring pacemaker insertion. Two patients died, one in the immediate postoperative period in pulmonary edema, and another due to bacterial endocarditis 3 years after surgery. Our observations suggest that surgical treatment of corrected transposition can be achieved with acceptable risks. The long-term follow up in the survivors was excellent.     

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.     

Detection of caval obstruction by magnetic resonance imaging after intraatrial repair of transposition of the great arteries

Vena caval obstruction may cause significant morbidity after intraatrial repair of transposition of the great arteries (TGA). Two noninvasive methods of diagnosing vena caval obstruction were compared with cardiac catheterization. Echocardiographically gated magnetic resonance imaging (MRI) and echocardiographic evaluation (2-dimensional saline contrast echocardiography and pulsed Doppler flow measurement) were performed on 15 patients 0.7 to 13.5 years after intraatrial repair of TGA (8 Mustard, 7 Senning). At catheterization, complete superior vena cava or partial caval obstruction (gradient greater than 5 mm Hg from cava to systemic venous atrium) was present in 7 of 15 patients. Superior vena cava obstruction was directly visualized by MRI in both patients with catheterization-proved complete superior vena cava occlusion. A dilated azygous/hemiazygous venous complex (greater than or equal to 5 mm cross-sectional diameter) was seen by MRI in 5 of 7 patients with complex or partial vena caval obstruction and in no patient without vena caval obstruction. MRI showed superior vena caval dilatation (ratio of superior vena caval diameter to aortic diameter greater than 1.45) in 3 of 5 patients with partial vena caval obstruction and in 0 of 8 without vena caval obstruction. Direct visualization of narrowing within the atrium was unreliable for any MRI plane because of the 3-dimensional nature of the intraatrial baffle. Two-dimensional saline contrast echocardiography, successfully performed in 12 of 15 patients, detected complete superior vena caval obstruction only in the 2 patients with catheterization-proved complete superior vena cava occlusion. Contrast echocardiography failed to identify any of the 5 patients with partial vena caval obstruction.(ABSTRACT TRUNCATED AT 250 WORDS)     

Long-term outcome after the mustard repair for simple transposition of the great arteries: 28-year follow-up

Objectives. This study examines the late outcome in patients with simple transposition of the great arteries (TGA) after a Mustard operation.Background. Continuing medical follow-up for patients after the Mustard procedure, now extending to three decades, is required. The quality of life of adult survivors has not been well documented.Methods. Survival and quality of life among 113 hospital survivors of the Mustard operation performed for simple TGA between 1964 and 1982 were assessed by medical review and a lifestyle questionnaire. The incidence of right ventricular failure and echocardiographic right ventricular dysfunction (RVD) were determined. A measure of lifestyle, the ability index, was determined.Results. Actuarial survival was 90%, 80%, and 80% at 10, 20, and 28 years, respectively, with 76% of survivors being New York Heart Association class 1. Sudden death, with an incidence of 7% without identifiable risk factors, was the most common cause of late demise. RVD was identified in 18% of patients who had echocardiography, but there was right ventricular failure in only two patients. Seventy-five percent of current survivors lead a normal life, 20% have some symptoms or lifestyle modification, and 5% are unable to work.Conclusions. The survival of patients to 28 years with the Mustard repair has been good. Late sudden death is the most worrisome feature. There is a 97% freedom from right ventricular failure to date. The quality of life of late survivors is good, most achieving a normal level of education and employment.     

Double aortic arch and d-transposition of the great arteries

We present a rare case of D-transposition of the great arteries (D-TGA) and double aortic arch (DAA). The anatomy was prospectively and preoperatively diagnosed by echocardiography and confirmed by ultra-fast computed tomography. The patient underwent successful arterial switch operation and division of the vascular ring at a single procedure.     

Intrapulmonary channel for one-stage correction of aortic arch obstruction

There are several methods of surgical repair of aortic coarctation or interruption; the optimal technique is still controversial. The purpose of this study was to assess a new surgical method: intrapulmonary channel for one-stage repair of aortic coarctation or interruption associated with intracardiac anomalies. Between 1993 and 1995, 4 patients with aortic coarctation or interruption and intracardiac anomalies received one-stage surgical correction. Their ages ranged from 5 to 26 years (mean, 16 years). The aortic arch lesions were preductal coarctation in 2, and type B interruption in 2. Coexisting anomalies consisted of patent ductus arteriosus in 4, ventricular septal defect in 3, and aortopulmonary window in 1. An intrapulmonary channel was constructed in all patients, and co-existing anomalies were corrected simultaneously. There was no hospital death or late mortality. A cerebral complication occurred in one patient because of air embolism. Mean follow-up was 9.5 years (range, 8.5-11.5 years). There was no evidence of recoarctation or late aneurysm formation. For selected patients with aortic coarctation or interruption and intracardiac anomalies, an intrapulmonary channel might be an option for one-stage correction.     

Silent unilateral pulmonary venous obstruction. Occurrence after surgical correction of transposition of the great arteries.

An 11-year-old girl was found to have completely obstructed left pulmonary veins eight years following corrective surgery for transposition of the great arteries. The patient was acyanotic and asymptomatic. Retrograde flow of arterial blood from the affected left lung accounted for an angiographic appearance that mimicked occlusion of the left pulmonary artery and resulted from a failure of systemic venous development. Pulmonary venous anatomy could only be demonstrated by pulmonary arterial wedge angiographic studies. This experience emphasizes that complete unilateral pulmonary venous obstruction may occur in an asymptomatic patient and underlines the importance of investigating pulmonary venous anatomy in any patient with gross inequality of the distribution of pulmonary blood flow.     

Congenitally corrected transposition of the great arteries and aortic coarctation--an uncommon association.

Congenitally corrected transposition of the great arteries, L-TGA, is a rare abnormality accounting for less than 0.5% of clinically apparent congenital heart disease. Age at time of diagnosis and survival rate are variable and depend mostly on associated anomalies. The authors present a clinical case of a twenty-four-year-old woman in whom, in a routine echocardiogram, congenitally corrected transposition of the great arteries and aortic coarctation were diagnosed, an unusual association. They describe the results of complementary exams (echocardiography, chest X-ray, electrocardiogram and cardiac angiography) that they believe to be useful for the correct diagnosis of this clinical situation. Additionally, the authors make a brief review of the literature relevant to the case.