[Total anomalous pulmonary venous connection associated with Klippel-Feil syndrome: a case report]

Abstract

Klippel-Feil syndrome is characterized by congenital fusion of the cervical vertebrae. Anomalies in other organs sometimes combine. Many kinds of congenital cardiac anomalies, most frequently ventricular septal defect, considerably associate with this syndrome. Few cases of surgical corrections of cardiac anomalies with Klippel-Feil syndrome have been reported up to today. Therefore surgical problems with this syndrome have not been solved yet, completely. We have experienced a very rare case of a 18-year-old female of total anomalous pulmonary venous connection (Darling Ia type) associated with Klippel-Feil syndrome, that was repaired successfully. No difficulty in the surgical and anesthesiological management referring to the fusion of cervical vertebrae was encountered. The result of surgical correction of congenital cardiac anomalies in Klippel-Feil syndrome mainly depends on the severity of the heart disease and not on Klippel-Feil syndrome itself, as literature and the present case suggest.