Associations Between Age at Arterial Switch Operation, Brain Growth, and Development in Infants With Transposition of the Great Arteries

Abstract

Background: Brain injury, impaired brain growth, and long-term neurodevelopmental problems are common in children with transposition of the great arteries. We sought to identify clinical risk factors for brain injury and poor brain growth in infants with transposition of the great arteries undergoing the arterial switch operation, and to examine their relationship with neurodevelopmental outcome. Methods: The brains of 45 infants with transposition of the great arteries undergoing surgical repair were imaged pre- and postoperatively using magnetic resonance imaging. Brain weight z scores were calculated based on brain volume and autopsy reference data. Brain injury scores were determined as previously described. Neurodevelopment was assessed at 18 months using the Bayley-III scores of infant development. The relationships between clinical variables, brain injury, perioperative brain growth, and 18-month Bayley-III scores were analyzed. Results: On preoperative imaging, moderate or severe white matter injury was present in 10 of 45 patients, whereas stroke was seen in 4 of 45. A similar prevalence of injury was seen on postoperative imaging, and we were unable to identify any clinical risk factors for brain injury. Brain weight z scores decreased perioperatively in 35 of 45 patients. The presence of a ventricular septal defect ( P =0.009) and older age at surgery ( P =0.007) were associated with impaired perioperative brain growth. When patients were divided into those undergoing surgery during the first 2 weeks of life (32/45) versus those being repaired later (13/45), infants repaired later had significantly worse perioperative brain growth (late repair postoperative brain weight z = –1.0±0.90 versus early repair z = –0.33±0.64; P =0.008). Bayley-III testing scores fell within the normal range for all patients, although age at repair ( P =0.03) and days of open chest ( P =0.03) were associated with a lower composite language score, and length of stay was associated with a lower composite cognitive score ( P =0.02). Conclusions: Surgery beyond 2 weeks of age is associated with impaired brain growth and slower language development in infants with transposition of the great arteries cared for at our center. Although the mechanisms underlying this association are still unclear, extended periods of cyanosis and pulmonary overcirculation may adversely impact brain growth and subsequent neurodevelopment.

Perinatal and perioperative factors associated with mortality and an increased need for hospital care in infants with transposition of the great arteries: A nationwide 11‐year population‐based cohort

“…17 The frequency of BAS varies greatly among the centers (48-93%). [4][5][6]16,17 Consistent with the latest multicenter studies, these nationwide data showed that infants with BAS had a shorter first-year LOS in general during the first year of life, 5 although BAS has previously been associated with higher morbidity. 18,19 However, the small sample size and the characteristics of infants who did not need septostomy can affect these results.…”

“…Every infant with d-TGA receives similar care in Finland; operations are centralized to a single tertiary center. Septostomy was performed often (94%), similar to cohorts from Canada 16 and France. 17 The frequency of BAS varies greatly among the centers (48-93%).…”

“…[25][26][27] Interestingly, we observed an association between maternal BMI and longer initial LOS (adjusted β 0.015) and first-year LOS (adjusted β 0.016) in d-TGA infants, meaning that a 10-unit higher prepregnancy BMI increases the initial LOS by 16% (exp(10 × 0.015) = 1. 16) and first-year LOS by 17% (exp(10 × 0.016) = 1.17). However, for initial LOS, it could be as little as 2% (0.4 days median) to as high as 32%…”

Attention-deficit/hyperactivity disorder symptoms in children with surgically corrected Ventricular Septal Defect, Transposition of the Great Arteries, and Tetralogy of Fallot

“…The underlying mechanisms are uncertain, but the prolonged period of cyanosis combined with pulmonary overcirculation may contribute to the impaired growth of the brain and thereby delayed neurodevelopment. 28 The prenatal screening programme was introduced in 2004 in Denmark with more than 95% of women scanned in their pregnancy, increasing the number prenatal diagnosis of CHD from 4.5% in 1996 to 71% in 2013. 29 This may have led to an optimised management with planned delivery at a specialised cardiac centre, enabling prompt administration of prostaglandin and balloon atrial septostomy (if required) for the Transposition of the Great Arteries, thereby minimising the risk of preoperative circulatory collapse and risk of cerebral damage.…”

Echocardiographic assessment of transposition of the great arteries and congenitally corrected transposition of the great arteries

“…In patients with a large VSD the surgery can be postponed for a few weeks or months based on surgical preference. Recent data however suggested that postponing surgery beyond 2 weeks of life may affect neurodevelopmental outcomes (16).…”