Velopharyngeal insufficiency in tetralogy of Fallot: A report of four cases

Abstract

SUMMARY . This report describes 4 children with tetralogy of Fallot and velopharyngeal insufficiency, but not cleft palate . Since there may be an association between velopharyngeal insufficiency and tetralogy of Fallot, careful attention should be paid to the speech of children with tetralogy . KEY WORDS : Velopharyngeal insufficiency -Tetralogy of Fallot -Speech disordersNormal nasal resonance and the proper production of most speech sounds require adequate velopharyngeal closure . Such closure is achieved by a coordinated muscle system which functions as a sphincter with both velar and pharyngeal components [23,24] . The velum (soft palate) lifts and moves posteriorly whereas the pharyngeal walls may move medially and/or anteriorly . When there is inadequate closure (velopharyngeal insufficiency), excess air enters the nasal cavity and affects articulation and the resonance of voice (hypernasality) . This condition is most frequently associated with cleft or submucous cleft palate ; it can also occur with a foreshortened palate, a capacious pharynx, or a neuromuscular deficiency of the soft palate and/or pharyngeal musculature . In patients whose adenoidal tissue thickens the posterior pharyngeal wall, thus aiding in velopharyngeal closure, removal of the adenoids may unmask velopharyngeal insufficiency [8, 271 . Velopharyngeal insufficiency has also been reported in children who have tetralogy of Fallot or other types of congenital heart disease [7, 251 . This report describes 4 patients with velopharyngeal insufficiency but no obvious cleft palate in whom tetralogy of Fallot had been diagnosed . A 4 .5-year-old boy was referred for assessment of velopharyngeal insufficiency in 1975 . Neonatal anoxic spells led to a diagnosis of tetralogy of Fallot with pulmonary atresia and a right aortic arch . A left Blalock-Taussig shunt had been performed at age I year and at 2 .5 years, a Waterston shunt because of persistent severe cyanosis . A tonsillectomy and adenoidectomy was performed at age 4 years because of frequent respiratory infections . Postoperatively, velopharyngeal insufficiency became apparent .The patient's early speech development had been delayed . At assessment, his speech was characterized by moderate hypernasal resonance . Although articulation was commensurate with the child's age, he had difficulty producing the sibilant sounds (sun, zoo, chair, shoe) .On videofluoroscopic examination during speech, velopharyngeal closure was not obtained . The anterior-posterior dimension of the pharynx was increased [26] and neuromuscular weakness of the soft palate was suspected .Treatment of the velopharyngeal insufficiency-pharyngealflap surgery [51 at age 5 .3 years, and speech therapy-improved vocal resonance and articulation significantly . However, 2 years later, his exercise tolerance decreased and he died after pulmonary arterioplasty and right-ventricular-outflow reconstruction . Articulation was defective, and there was hypernasal resonance and flaring of the nares during speech . The child had l...