ISSN - 0973-0958

Pediatric Oncall Journal View Article

Pediatric Atypical Shone`s complex
Faten Tinsa, Mohamed Riadh Boukhris, Samia Hamouda, Khadija Boussetta.
Department of Pediatrics B, Childrens Hospital Bechir Hamza of Tunis, Universite Tunis Elmanar, Tunis, Tunisia.
Shone’s complex is an extremely rare and severe congenital heart disease with about fifty cases reported in literature. It is characterized by great clinical variability and difficulties in diagnosis and management. Shone’s complex consists on the association of obstructive defects at many levels of the left ventricular outflow tract. The full or typical Shone’s complex, in its first description, corresponds to 4 levels of obstruction - supravalvular mitral membrane, valvular mitral stenosis due to parachute mitral valve, subaortic stenosis, and coarctation of the aorta. Since then, several Shone’s complex descriptions with three or two levels of obstruction have been reported and called incomplete Shone’s complex or atypical. We report the case of a 57-day-old boy transferred to pediatric department for persistent dyspnea. The diagnosis of atypical Shone’s complex with three levels of obstruction was made. Surgical treatment was performed at 60th day of life with favorable outcome.

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