Prenatal Diagnosis of Persistent Left Superior Vena Cava and its Clinical Significance

Year 2014, Volume: 2014 Issue: 1, 50 - 54, 07.08.2014

Aytül Çorbacıoğlu Esmer Atıl Yüksel Halime Çalı Mehmet Özsürmeli Rukiye Eker Ömeroğlu İbrahim Kalelioğlu Recep Has

https://doi.org/10.5152/balkanmedj.2014.9426

Abstract

Background: Persistent left superior vena cava (PLSVC) is a variant of systemic venous return which is observed in 0.3% of autopsies in the general population and in 4-8% of patients with congenital heart disease. Aims: To evaluate associated cardiac, extracardiac and chromosomal anomalies in prenatally diagnosed cases of PLSVC and to review their outcome. Study Design: Retrospective comparative study. Methods: The data of patients with a prenatal diagnosis of PLSVC between May 2008 and January 2013 were reviewed retrospectively. Results: Data of 31 cases were reviewed. Fifteen (48.4%) cases were associated with cardiac defects and 17 (54.8%) cases had associated extracardiac sonographic or postpartum findings. Two fetuses had karyotype anomalies. Outcome was significantly more favorable in cases not associated with cardiac defects in comparison to those associated with cardiac anomalies (84.6% vs. 33.3%, p=0.009). All cases with isolated PLSVC survived, while among the cases associated with extracardiac anomalies, with cardiac anomalies and with both extracardiac and cardiac anomalies, the survival rate was 75%, 50% and 22.2%, respectively. The most frequent group of cardiac anomalies associated with PLSVC was septal defects and VSD was the most common heart defect individually, being observed in nine fetuses. Conclusion: Prenatally diagnosed PLSVC is associated with cardiac and extracardiac anomalies in the majority of cases. Outcome is significantly worse if PLSVC is associated with a cardiac defect, and the prognosis is excellent in isolated cases.

Keywords

Cardiac anomaly, congenital anomaly, persistent left superior vena cava, prenatal diagnosis, ultrasound

Prenatal Diagnosis of Persistent Left Superior Vena Cava and its Clinical Significance

Abstract

References

• Jouannic JM, Picone O, Martinovic J, Fermont L, Dumez Y, Bonnet D. Diminutive fetal left ventricle at mid-gastation associated with persistent left superior vena cava and coronary sinus dilatation. Ultrasound Obstet Gynecol 2003;22:527-30. [CrossRef]
• Berg C, Knüppel M, Geipel A, Kohl T, Krapp M, Knöpfle G, et al. Prenatal diagnosis of persistent left superior vena cava and its associated congenital anomalies. Ultrasound Obstet Gynecol 2006;27:274-80. [CrossRef]
• Pasquini L, Belmar C, Seale A, Gardiner HM. Prenatal diagnosis of absent right and persistent left superior vena cava. Prenat Diagn 2006;26:700-2. [CrossRef]
• Kalache KD, Romero R, Conoscenti G, Qureshi F, Jacques SM, Chaiworapongsa T, et al. Prenatal diagnosis of dilated coronary sinus with persistent left superior vena cava in a fetus with trisomy 18. Prenat Diagn 2003;23:108-10. [CrossRef]
• Thompson C. Congenital cardiac malformations in relation to central venous access. Br J Nurs 2006;15:276-81.
• Galindo A, Gutierrez-Larraya F, Escribano D, Arbues J, Velasco JM. Clinical significance of persistent left superior vena cava diagnosed in fetal life. Ultrasound Obstet Gynecol 2007;30:152-61. [CrossRef]
• Barea C, Ovaert C, Moniotte S, Biard J-M, Steenhaut P, Bernard P. Prenatal diagnosis of abnormal cardinal systemic venous return without other heart defects: a case series. Prenat Diagn 2011;31:380-8. [CrossRef]
• Chaoui R, Heling KS, Kalache KD. Caliber of the coronary sinus in fetuses with cardiac defects with and without left persistent superior vena cava and in growth-restricted fetuses with heart-sparing effect. Prenat Diagn 2003;23:552-7. [CrossRef]
• Nsah EN, Moore GW, Hutchins GM. Pathogenesis of persistent left superior vena cava with a coronary sinus connection. Pediatr Pathol 1991;11:261-9. [CrossRef]
• Freund M, Stoutenbeek P, ter Heide H, Pistorius L. ‘Tobacco pipe’ sign in the fetus: patent left superior vena cava with absent right superior vena cava. Ulrasound Obstet Gynecol 2008;32:592-7. [CrossRef]
• Guarnieri GF, Romano F, Cleico L, Balducci G. Absent right and persistent keft superior vena cava: Fetal and neonatal echocardiographic diagnosis. Pediatr Cardiol 2006;27:646-8. [CrossRef]