ISSN : 1301-5680
e-ISSN : 2149-8156
TURKISH JOURNAL OF
THORACIC AND
CARDIOVASCULAR SURGERY
Turkish Journal of Thoracic and Cardiovascular Surgery     
Absent right superior vena cava with persistent left superior vena cava and normal atrial arrangement
Özben Ceylan, Senem Özgür, Utku Arman Örün, Vehbi Doğan, Murat Koç, Osman Yılmaz, Mahmut Keskin, Selmin Karademir
1Departments of Pediatric Cardio, Dr. Sami Ulus Obstetrics and Gynecology, Children’s Health and Diseases Training and Research Hospital, Ankara, Turkey
2Departments of Pediatric Cardiovascular Surgery, Dr. Sami Ulus Obstetrics and Gynecology, Children’s Health and Diseases Training and Research Hospital, Ankara, Turkey
DOI : 10.5606/tgkdc.dergisi.2014.8215
Background: This study aims to investigate the role of echocardiography in the detection of venous anomalies of large thoracic veins in patients with large coronary sinus.

Methods: Between January 2010 and March 2012, six patients (3 boys, 3 girls; mean age 4.1; range 3 days - 9 years) were diagnosed with an absent right superior vena cava (RSVC) accompanied by persistent left superior vena cava (PLSVC) in our clinic. The diagnosis of the absent RSVC was established by echocardiography and angiography.

Results: Diagnosis of the absent RSVC was confirmed by echocardiography in four patients. The diagnosis was based on cardiac catheterization and angiography in two patients. Additional cardiac anomalies included peripheral pulmonary stenosis, patent ductus arteriosus, tetralogy of Fallot, atrial septal defect, ventricular septal defect and interrupted aortic arch type B. One patient had Trisomy 9p syndrome, while another one was the baby of a diabetic mother. Three patients were followed clinically, while other three patients were operated.

Conclusion: Absent RSVC should be thoroughly investigated in cases with a large coronary sinus along with increased coronary sinus flow and PLSVC, as evidenced by echocardiography.

Keywords : Absent right superior vena cava; congenital heart disease; infant of diabetic mother; persistent left superior vena cava; Trisomy 9p syndrome
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