Table 1.

Overview of selected studies on neuropsychological and psychiatric outcomes for patients with d-TGA.

Reference	n, age (years), diagnosis	Neurocognitive or psychiatric assessment	Main results
Children
Bellinger et al. (12)	n = 158, 4 years, d-TGA	–WPPSI revised, –Peabody Developmental Motor Scales, –Grooved pegboard, –Test for auditory comprehension of language, –Receptive one-word picture vocabulary test, –Expressive one-word picture vocabulary test, –Illinois test of psycholinguistic abilities.	Lower than expected mean scores in general intelligence (IQ), expressive language, visual-motor integration, motor function, and oromotor control.
Bellinger et al. (13)	n = 155, 8 years, d-TGA	–WISC III, –WIAT, –Wide range assessment of memory and learning, –Developmental test of visual–motor integration, –Test of variables of attention, –Rey–Osterrieth complex figure, –Verbal fluency, –Wisconsin card sorting test, –Trail making test, –Formulated sentences subtest of the clinical evaluation of language fundamentals, –Controlled oral word association test, –Grooved pegboard.	Lower than expected scores in academic achievement, memory, visual-spatial skills, sustained attention, and higher order language skills. Higher than expected proportion with scores >1SD below normative values in executive functions (e.g., planning, cognitive flexibility).
Calderon et al. (24)	n = 21, 7 years, d-TGA	–Columbia Mental Maturity Scale, –Animal Stroop test, –Statue subtest from the NEPSY, –Tower of London, –Digit span, –Corsi block-tapping task, –False belief tasks (1st and 2nd order).	Patients’ mean IQ scores within the normal range. Compared to a control group, patients with d-TGA had lower scores in executive functions (i.e., inhibition, working memory, planning) and social cognition (i.e., theory of mind).
Calderon et al. (25)	n = 45, 5/7 years, d-TGA	–Columbia Mental Maturity Scale, –Comprehension subtest from the NEPSY, –Digit span, –Spatial span, –The hand game, –Hearts and flowers incongruent and mixed conditions, –Day and night, –Animal Stroop test, –Dimensional change card sorting test.	Patients’ mean scores lower than controls’ mean scores in receptive language, attention, and executive functions (i.e., inhibition, cognitive flexibility). Persistent impairments in cognitive inhibition and cognitive flexibility from ages 5 to 7.
Freed et al. (8)	n = 82, 1.5–2 years, d-TGA	BSID II	Most patients with scores > 1SD below normative values in cognitive, motor, and language function.
Hicks et al. (11)	n = 91, 2 years, d-TGA	BSID III	Higher than expected proportion of patients with scores >1SD or >2SD below normative values in language function.
Hövels-Gürich et al. (32)	n = 77, 3–9 years, d-TGA	–K-ABC, –Vocabulary subtest of the K-ABC, –Kiphard and Schilling body coordination test, –Denver developmental screening test, –Frostig developmental test of visual perception.	Patients’ mean IQ scores within the normal range. Lower than expected scores in motor function, vocabulary, and acquired abilities.
Hövels-Gürich et al. (17)	n = 60, 7–14 years, d-TGA	–K-ABC, –Kiphard and Schilling body coordination test, –Oral and speech motor control test, –Mayo tests of speech and oral apraxia, –Illinois test of psycholinguistic abilities, –Test of auditory analysis skills.	Speech, motor, and developmental impairments more frequent compared to the general population. Lower than expected scores in acquired abilities and language.
Hövels-Gürich et al. (42)	n = 60, 7–14 years, d-TGA	Achenbach child behavior checklist	Parent-reported psychosocial maladjustment more frequent than in the general population on all domains (i.e., internalizing, externalizing, social, and attention problems, and competences).
Karl et al. (19)	n = 74, 4–14 years, d-TGA	–WPPSI, –WISC III, –Movement Assessment Battery, –Achenbach child development checklist, –Achenbach teacher report form.	Patients’ mean IQ scores within the normal range. Lower scores in motor function. Parent- and teacher-reported psychosocial maladjustment more frequent than in a control group on domains including behavioral, speech, language, and learning ability problems.
McGrath et al. (16)	n = 135, 1/8 years, d-TGA	Evaluation at 1 year –BSID, –Fagan test of infant intelligence. Evaluation at 8 years –WISC III, –WIAT.	Most 1-year test scores were statistically but modestly associated with 8-year test scores. The majority of patients with scores >1SD below normative values at 8 years had displayed scores >1SD at 1 year.
Adolescents
Bellinger et al. (14)	n = 139, 16 years, d-TGA	–WIAT II, –General Memory Index of the Children’s Memory Scale, –Test of visual–perceptual skills, –Rey–Osterrieth complex figure, –Delis–Kaplan executive function system, –Behavior rating inventory of executive function (child, parent, and teacher versions), –Connors attention-deficit and hyperactivity disorder (parent version), –Reading the mind in the eyes test, revised.	Lower than expected scores on academic skills, visuo-spatial skills, memory, executive functions, and social cognition. Higher than expected proportion of patients with scores >1SD or >2SD below normative values in academic skills, memory and visuo-spatial skills. By parent reports, about 1 in 5 had attention or executive impairments in daily life.
Cassidy et al. (22)	n = 139, 8/16 years, d-TGA	–WISC III, –WIAT II, –Trail making test, –Test of variables of attention.	Processing speed associated with executive functions (i.e., working memory, inhibition, and shifting) and academic skills at 8 and 16 years.
DeMaso et al. (45)	n = 139, 16 years, d-TGA	–Schedule for affective disorders and schizophrenia for school-aged children, –Children’s Global Assessment Scale, –Brief Psychiatric Rating Scale for Children, –Children’s Depression Inventory, –Revised Children’s Manifest Anxiety Scale, –Child stress disorders checklist, –Posttraumatic Stress Diagnostic Scale, –Conners’ attention-deficit/hyperactivity disorder rating scales, –Conduct Disorder Scale.	Patients were more likely than controls to meet criteria for a lifetime psychiatric diagnosis. Higher lifetime and current prevalence of attention-deficit/hyperactivity disorder. Psychosocial functioning was within a pathological range for 15% of patients. Parent- and self-reports identified high depressive, anxiety, and posttraumatic stress symptoms.
Heinrichs et al. (31)	n = 60, 14–21 years, d-TGA	–Hamburg-Wechsler intelligence test, –Analytical thinking subtests of the Leistungsprüfsytem nach Horn, –Mannheimer Rechtschreib test.	Patients’ mean IQ scores within the normal range. Higher than expected proportion of patients with IQ scores ≥2. Lower than expected scores on orthography.
Adults
Klouda et al. (53)	Total mixed cohort, n = 48, 18–49 years n = 24, moderate CHD, n = 24, complex CHD (including d-TGA, n = 9).	CNS vital signs	Patients with critical CHD had lower than expected scores on multiple domains: psychomotor speed, processing speed, sustained and executive attention, and on the overall, neurocognitive index.
Tyagi et al. (54)	Total mixed cohort, n = 310, 18–76 years n = 80, d-TGA, n = 81, TOF, n = 65, SV anatomy, n = 84, mild CHD.	–Controlled oral word association test, –Grooved pegboard, –Rey auditory verbal learning test, –Stroop test, –Symbol digit modalities test, –Trail making test, –Wisconsin card sorting test, –Wechsler Adult Intelligence Scale.	d-TGA patients scored worse than those with mild CHD on an overall neuropsychological index. Proportion with scores >1.5 SD below normative values on at least 3 tests was higher in the d-TGA group compared to both the mild CHD group and the expected frequency in the general population.
van Rijen et al. (56)	Total mixed cohort, n = 349, 20–46 years, n = 55, d-TGA, n = 72, TOF, n = 37 pulmonary stenosis, n = 185, mild CHD.	–Young adult self-report, –Young adult behavior checklist.	Diagnosis of d-TGA was associated with higher risks of behavioral problems, particularly externalizing problems (e.g., intrusive and aggressive behavior).

BSID, Bayley Scales of Infant Development; CHD, congenital heart defects; d-TGA, dextro-transposition of the great arteries; IQ, intelligence quotient; K-ABC, Kaufman assessment battery for children; SV, single ventricle; TOF, tetralogy of Fallot; WIAT, Wechsler individual achievement test; WISC, Wechsler intelligence scale for children; WPPSI, Wechsler preschool and primary scale of intelligence.