Prenatal diagnosis of persistent left superior vena

Prenatal diagnosis of persistent left superior vena
cava and its associated congenital anomalies
C. BERG*, M. KN¨ UPPEL*, A. GEIPEL*, T. KOHL*, M. KRAPP†, G. KN ¨ OPFLE‡, U. GERMER§,M. HANSMANN* and U. GEMBRUCH*
*Department of Prenatal Medicine and Obstetrics, University of Bonn, and ‡Department of Pathology, Rheinische
Friedrich-Wilhelms-Universit ¨ at, Bonn, †Division of Prenatal Medicine, Department of Obstetrics and Gynecology, University Hospital
Schleswig-Holstein, Campus L¨ ubeck and §Department of Prenatal Medicine, University of Regensburg, Germany

ABSTRACT

Objective To evaluate the associated conditions and the
outcome of persistent left superior vena cava (PLSVC)
detected in fetal life.
Methods This was a retrospective review of all cases
of PLSVC detected prenatally between 1998 and 2004
in two tertiary referral centers in Germany. Patient
charts, ultrasound video recordings and still frames of
all cases were reviewed for associated conditions and
outcome.
Results Eighty-two cases of PLSVC were detected in the
study period. Thirty-seven cases (45%) were associated
with heterotaxy syndromes, 19 (23%) with isolated cardiac
malformations, seven (9%) with aneuploidy, six
(7%) with complex malformation syndromes and six
(7%) with isolated extracardiac malformations. Seven
cases (9%) had no associated condition. Eighty-three percent
of the fetuses in this series had associated cardiac
malformations; the most frequent cardiac malformations
in those with heterotaxy syndromes were complete atrioventricular
septal defect (75%) and right outflow tract
obstruction (58%). After exclusion of cases with heterotaxy,
most congenital heart defects were ventricular
septal defects (41%) and coarctation (34%). The outcome
of PLSVC was determined solely by the associated
conditions. After exclusion of terminated cases, heterotaxy
syndromes as well as complete atrioventricular
septal defects were associated significantly with perinatal
and infant death. In contrast, all cases with isolated
PLSVC or associated correctable extracardiac malformations
survived and were doing well at the time of
writing.
Conclusions PLSVC detected in fetal life has to be
followed by a meticulous inspection of the fetal anatomy
as it is frequently associated with heterotaxy syndromes,
other cardiac/non-cardiac malformations and aneuploidy
that determine the outcome. Isolated PLSVC is a benign
vascular anomaly and may not affect the outcome.

Copyright  2006 ISUOG. Published by John Wiley & Sons, Ltd.

Ultrasound Obstet Gynecol 2006; 27: 274–280

 Published online 2 February 2006 in Wiley InterScience ().