Introduction
UCSS is a group of rare, complex cardiac malformations caused by the
absence of left atrial venous plica during the development of the
embryo, that is, the partial or complete defect of coronary sinus (CS)
septum, which lead to direct communication between CS and the LA. UCSS
is often associated with LSVC (80% to 90%), whose overall incidence is
about 0.3% to 0.5%, and incidence in congenital heart diseases is
about 3% to 10%[1]. Preoperative diagnosis used to be difficult
because of the lack of specific clinical manifestations and examination
methods of UCSS. From May 1998 to May 2019, we treated 68740 cases of
congenital heart diseases, including 159 cases of UCSS (0.23%), which
treated by different methods of operation and achieved good effects.
This paper is to summarize the clinical characteristics and surgical
treatment experience, and to provide reference for the preoperative
diagnosis and treatment of such diseases in the operation, in order to
improve the curative effect