Long-term outcomes of the arterial switch operation.

OBJECTIVES: The arterial switch operation (ASO) has excellent early outcomes in the modern era. We sought to determine the long-term outcomes in patients who underwent an ASO at a single institution. METHODS: Patients who underwent an ASO between 1983 and 2015 were identified from the hospital database and retrospectively reviewed using hospital records. RESULTS: From 1983 to 2015, 844 patients with a biventricular circulation underwent an ASO. There were 28 (3.3%, 28/844) early deaths. Follow-up was available for 94% (729/774) of local patients after hospital discharge. Median follow-up was 15 years (interquartile range, 8-20 years). There were 187 (26%, 187/729) patients with more than 20 years of follow-up and 95 (13%, 95/729) patients with more than 25 years of follow-up. Overall survival was 95% (95% confidence interval [CI], 94%-97%) at 10 and 25 years after the ASO. At 25 years after ASO, freedom from overall reintervention was 77% (95% CI, 73%-81%), freedom from reoperation on the neoaortic root or neoaortic valve was 92% (95% CI, 88%-95%), and freedom from coronary reoperation was 99% (95% CI, 98%-99.7%). Left ventricular (LV) systolic function was normal in 595 of 609 (98%) of patients who had LV function quantified at latest follow-up. Of the 95 patients with more than 25 years of follow-up after ASO, 6 (6.3%) had at least moderate neoaortic valve regurgitation (AR) and 8 (8.4%) had undergone replacement of the neoaortic valve. CONCLUSIONS: Overall, survivors of ASO have excellent late survival and normal LV systolic function into adult life. However, AR and reoperation on the neoaortic valve remains an issue for older patients.

Prenatal Diagnosis of Transposition of the Great Arteries Reduces Postnatal Mortality: A Population-Based Study.

BACKGROUND: Transposition of the great arteries (TGA) may present as a life-threatening neonatal malformation. Although prenatal detection facilitates the perinatal management, the impact on outcome is controversial. METHODS: This study reviewed the differences in prenatal diagnosis of TGA from 2009 to 2014 among the 5 geographic areas in Ontario and compared the management, morbidity, and mortality among neonates with a prenatal (prenatal cohort; n = 70) vs a postnatal (postnatal cohort; n = 76) anomaly diagnosis. Cases were identified from prospective databases of the provincial cardiac tertiary centres and the coroner's office. RESULTS: Prenatal TGA detection rates varied significantly among areas (median: 50%; range: 14% to 72%; P = 0.03). Compared with the postnatal cohort, time from birth to tertiary care admission (1.4 vs 10.4 hours, P < 0.001), prostaglandin therapy (0.1 vs 5.3 hours; P < 0.001), balloon atrial septostomy (5.3 vs 14.9 hours; P <0.001), and arterial switch operation (6 vs 9 days, P = 0.002) was significantly shorter in the prenatal cohort. Although other preoperative variables-including the need of ventilation and mechanical support, morbidity score, and lowest pH and preductal oxygen saturations-were comparable, a prenatal diagnosis was associated with improved 1-year survival (odds ratio: 0.108; 95% confidence interval, 0.013-0.88; P = 0.0184). CONCLUSIONS: Prenatal diagnosis of TGA significantly shortened time intervals from birth to neonatal care and surgery and was associated with improved survival. The prenatal detection rate of TGA in Ontario was low (50% or less) outside of Metropolitan Toronto, suggesting the need for new strategies to further improve intraprovincial detection rates.

Outcomes of the arterial switch operation in patients with aortic arch obstruction.

OBJECTIVE: Transposition of the great arteries or Taussig-Bing anomaly with concomitant aortic arch obstruction is uncommon, with limited data on long-term outcomes after arterial switch operation and aortic arch obstruction repair. This study sought to determine outcomes of patients undergoing arterial switch operation and aortic arch obstruction repair at a single institution. METHODS: From 1983 to 2015, 844 patients underwent an arterial switch operation for biventricular repair at The Royal Children's Hospital. Eighty-three (9.8%, 83/844) patients underwent an arterial switch operation and aortic arch obstruction repair. RESULTS: Fifty-five (66%, 55/83) patients had transposition of the great arteries. and 28 (34%, 28/83) patients had Taussig-Bing anomaly. Fifty-nine (71%, 59/83) patients underwent arterial switch operation and aortic arch obstruction repair as a single-stage procedure, and 24 (29%, 24/83) patients underwent arterial switch operation and aortic arch obstruction repair as a 2-stage procedure. There were 5 early deaths (6.0%, 5/83). Follow-up was available for 74 (95%) of the 78 survivors. Median follow-up was 13.3 years (interquartile range, 7.3-19.3 years; range, 1-30 years). There were no late deaths. Freedom from reintervention was 77%, 71%, and 68% at 5, 10, and 20 years, respectively. Reintervention was more common compared with patients without aortic arch obstruction (P < .001). Reintervention for right-sided obstruction was more common compared with patients without aortic arch obstruction (P = .006). CONCLUSIONS: Patients with transposition of the great arteries or Taussig-Bing anomaly with associated aortic arch obstruction have a higher reintervention rate, especially for right-sided obstruction. Closer monitoring of this subgroup of patients is warranted.

Pregnancy outcomes and mid-term prognosis in women after arterial switch operation for dextro-transposition of the great arteries - Tertiary hospital experiences and review of literature.

OBJECTIVE: Arterial switch operation (ASO) for dextro-transposition of the great arteries (d-TGA) has gradually replaced the atrial switch operation and has become the standard operation. To date, the outcomes of pregnant women with d-TGA after this new operation have not been investigated. In this study, we investigated the impact of ASO on pregnant outcomes and mid-term prognosis in women with d-TGA and compared with the atrial switch operation through the literature review. METHODS AND RESULTS: There were 20 pregnancies in 10 women with d-TGA after ASO and 6 resulted in abortion. Among 14 successful pregnancies in 10 women, 11 pregnancies achieved the term delivery and 3 pregnancies, including 1 twin pregnancy, resulted in preterm labor. Maternal cardiovascular events occurred in 4 (heart failure and arrhythmias in 3 and arrhythmia in 1), and all were controllable with medications. Risk factors for the peripartum cardiac events were older age at ASO and delivery, and higher concentration of brain natriuretic peptide (BNP) at first trimester (p<0.05). In 7-60 month-follow-up after delivery, no case showed deterioration of functional class and systemic ventricular function. According to the literature review, women after ASO demonstrated a better prognosis than those after the atrial switch operation. CONCLUSIONS: The majority of women with d-TGA after ASO tolerated pregnancy and delivery well. The older age at ASO, an elderly pregnancy, and higher BNP levels at the first trimester were possibly risk factors of peripartum cardiovascular events among the group. The literature reviews and this study may indicate the advantage of systemic left ventricle compared with systemic right ventricle in long-term outcomes after delivery.

Long-term quality of life in adult survivors after the arterial switch operation.

OBJECTIVES: We sought to determine the quality of life after the arterial switch operation (ASO) using the Short Form 36 questionnaire in adult survivors. METHODS: All patients (n = 107) who underwent the ASO and were 18 years of age or older living in the Australian state of Victoria with a contact telephone number were identified from the hospital database. Fifty-one (48%) patients were 18-24 years old and 56 (52%) patients were 25-34 years old. Patients completed the Short Form 36 quality of life questionnaire via telephone. The results of the 8 domains of the Short Form 36 questionnaire and the derived health state summary score (Short Form 6-Dimension) were compared against mean scores from age-matched Australian population data. RESULTS: Compared with the Australian population age-matched data, 18- to 24-year-old ASO patients ranked their health higher in 3 of the 8 domains (P < 0.01). The 25-34 age group ranked their health higher in 4 of the 8 domains (P < 0.01). No statistically significant differences in the mean Short Form 6-Dimension scores were observed in the 18-24 age group (0.769 for ASO patients vs 0.772 for Australian population, P = 0.85) or the 25-34 age group (0.795 for ASO patients vs 0.780 for Australian population, P = 0.33). CONCLUSIONS: Young adult survivors of the ASO have similar outcomes to age-matched controls in quality of life measured by Short Form 6-Dimension.

Arrhythmias Following the Mustard and Senning Operations for Dextro-Transposition of the Great Arteries: Clinical Aspects and Catheter Ablation.

The atrial switch operations, the Mustard and Senning procedures, performed for dextro-transposition of the great arteries, have largely been supplanted by the arterial switch operation. As such, affected patients will only exist for approximately 30 more years. The main arrhythmias in these patients include sinoatrial node dysfunction, intraatrial reentry tachycardia, and sudden death. Device therapy for these patients is well-established, and catheter ablation for atrial tachycardias is highly efficacious. The application of meticulous procedural planning, customization of catheter courses, and electrophysiologic principles to this patient group may be extended to all postoperative complex congenital heart patients.

Outcomes of Patients After Arterial Switch Operation: 18 Years of Experience in a Single Medium-Volume Center.

The objective of this paper was to describe the outcomes in patients submitted to arterial switch operation and to analyze the predictors of in-hospital mortality and further need of re-operation at a single-center institution. Between September 1995 and January 2014, 128 consecutive arterial switch operations were performed. Surgical mortality during this period was analyzed retrospectively, and a follow-up analysis of the survivors was conducted. Surgical era, cardiopulmonary bypass time (p = 0.001), and diagnosis category (p = 0.025) influenced in-hospital mortality. The estimated overall survival for the 91 hospital survivors was 96.8, 96.4, and 96.2 % at 5, 10, and 15 years, respectively. The median follow-up time was 67 months (range 0.71-222 months). Three patients (5 %) presented severe aortic regurgitation. Right ventricle outflow tract systolic gradient by echocardiography was above 60 mmHg in 2 %. Late re-interventions occurred in 12 (13 %) patients with mean time of 64 ± 34 months after the initial procedure. Actuarial freedom from re-interventions at 5, 10, and 15 years was 96.4, 69.7, and 61.9 %, respectively. Arterial switch operation remains the procedure of choice in patients with transposition of great arteries. It can be performed even in middle-volume institutions, leading to the same middle- and long-term outcomes of high-volume institutions. Early high mortality rate may occur due not only to learning curve, but also to cardiopulmonary bypass time and ventricular septal defect closure.

Long-distance transport of neonates with transposition of the great arteries for the arterial switch operation: A 26-year Western Australian experience.

AIM: There is evidence that outcomes of complex paediatric cardiac procedures including the arterial switch operation (ASO) for transposition of the great arteries (TGA) are improved when performed at higher volume centres. While in utero transport for surgery is considered ideal, antenatal detection rates of TGA are low. Long-distance transport of post-natally diagnosed neonates has the potential to destabilise the patient's clinical condition. Since 1986, many neonates with TGA have been transported interstate from Perth to Melbourne or Brisbane for ASO surgery. The aim of this study was to review the Western Australian experience of interstate transport of newborns with TGA for ASO, noting transport complications and comparing the early mortality of these patients with published outcomes of the ASO from Royal Children's Hospital (RCH), Melbourne. METHOD: In this retrospective cohort study, we reviewed the neonatal and cardiology databases and medical records to identify infants with TGA born between 1986 and 2011 and requiring ASO surgery during the neonatal period. RESULTS: Over 26 years, 80 neonates were transferred interstate for ASO surgery. Twelve infants required ventilation, 36 needed prostaglandin (prostaglandin E1) infusion and 3 inotropic support. There was no mortality during transport and there was a single early post-operative death. This early mortality of 1.2% compares favourably with the RCH mortality of 2.8% from a recently published review of early outcomes for ASO. CONCLUSIONS: When in utero transport is not possible, long-distance transport of neonates with TGA can be safely undertaken, with no evidence of increased transport mortality/ major morbidity or higher early surgical mortality.