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Ann Thorac Surg 2006;81:e16-e17
© 2006 The Society of Thoracic Surgeons

Case report

Coronary Sinus Orifice Atresia With Left Superior Vena Cava in Patients With Univentricular Heart

Jukka T. Salminen, MD, PhD a , * , Tapio Hakala, MD, PhD a , Jaana Pihkala, MD, PhD b , Ilkka Mattila, MD, PhD a , Juha Puntila, MD a , Heikki Sairanen, MD, PhD a

a Department of Pediatric Cardiac Surgery, Hospital for Children and Adolescents, University of Helsinki, Helsinki, Finland
b Department of Pediatric Cardiology, Hospital for Children and Adolescents, University of Helsinki, Helsinki, Finland

Accepted for publication January 17, 2006.

* Address correspondence to Dr Salminen, Division of Pediatric Surgery and Pediatric Cardiac Surgery, Hospital for Children and Adolescents, University of Helsinki, POB 281, Helsinki, 00029 HUS Finland (Email: jukka.salminen{at}hus.fi).

Two patients with hypoplastic left heart syndrome had coronary sinus orifice atresia with persistent left superior vena cava. Both patients underwent successfully coronary sinus unroofing. One underwent surgery at the time of the bidirectional Glenn procedure and the other before creation of a total cavopulmonary connection. According to our population-based database, 10.3% of patients with univentricular heart have persistent left superior vena cava, and 2.3% have associated coronary sinus orifice atresia. Our cases highlight the importance of recognizing this anomaly in patients with univentricular heart to avoid high coronary venous pressure, which is potentially lethal.




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