![]() Common problems seen in these patients are impulsivity, distractibility, perseveration, and hyperactivity. During childhood, the majority of the children has learning problems, especially in the area of reading comprehension, arithmetic and problem-solving. Speech difficulties related to velo-palatal abnormalities may become clearly manifest late, particularly when speech has fully developed. This delayed speech development is prevalently due to the concomitance of velopharyngeal insufficiency and developmental delay. Difficulties in expressive language are generally evident in preschooler children. Psychomotor therapy is therefore recommended from an early age. 44, 45 Delayed motor development is mainly attributed to the hypotonia present in more than half of the patients with del22. 16, 31 Right or cervical aortic arch may be associated.ĭevelopmental delays can be present in children with del22, including delays in the motor, linguistic and cognitive domains. Ventricular septal defect: The type of VSD in patients with Del22 is prevalently subarterial doubly committed. 26 – 30 In these cases the infundibular septum is often hypoplastic or absent and is deviated posteriorly and to the left the VSD results in a subarterial position doubly committed with the pulmonary and aortic valves. Interrupted aortic arch: Del 22 is particularly common in patients with IAA type B. ![]() 23 – 25 Additional CHDs to TA are: 1) interruption or right aortic arch 2) discontinuity, stenosis, or crossing of the pulmonary arteries 3) severe dysplasia of the truncal valve 4) origin of the TA from the right ventricle. Truncus arteriosus: Del22 is prevalent in patients with TA with nonconfluent pulmonary arteries (Type 3 of van Praagh), in which the right pulmonary artery arises from the TA near to the truncal valve, and the left pulmonary artery is supplied by the ductus arteriosus. 19 – 22 Additionally, a high prevalence of right aortic arch and of discontinuity and defects of arborization of the pulmonary arteries can be found. In children with pulmonary atresia with ventricular septal defect and Del22 the pattern of pulmonary blood supply provided by the MAPCA is prevalent. Pulmonary atresia with ventricular septal defect: Considering the pattern of pulmonary blood supply, among children with pulmonary atresia with ventricular septal defect two major groups of patients can be recognized: 1) children with a single ductus arteriosus that usually presents confluent and well formed pulmonary arteries (also called tetralogy of Fallot with pulmonary atresia), and 2) children with major aortopulmonary collateral arteries (MAPCA) frequently associated with discontinuity, hypoplasia or absence of the central pulmonary arteries. 17, 18 The associated cardiac defects include: 1) right or cervical aortic arch with or without aberrant left subclavian artery 2) hypoplasia or absence of the infundibular septum 3) absence of the pulmonary valve 4) discontinuity and diffuse hypoplasia of the pulmonary arteries. Tetralogy of Fallot: Additional cardiac defects are found in the half of the patients with this TF and Del22. 15 In fact, patients with conotruncal heart defect and Del22 have often additional CHDs as a distinctive recognizable pattern. The study of the specific “cardiac phenotype” in patients with Del22 shows that a particular cardiac anatomy can be identified.
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