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Prenatal diagnosis of persistent left superior vena cava and its associated congenital anomalies
Author(s) -
Berg C.,
Knüppel M.,
Geipel A.,
Kohl T.,
Krapp M.,
Knöpfle G.,
Germer U.,
Hansmann M.,
Gembruch U.
Publication year - 2006
Publication title -
ultrasound in obstetrics and gynecology
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 3.202
H-Index - 141
eISSN - 1469-0705
pISSN - 0960-7692
DOI - 10.1002/uog.2704
Subject(s) - heterotaxy , medicine , persistent left superior vena cava , aneuploidy , fetus , fetal echocardiography , heart disease , prenatal diagnosis , cardiology , pregnancy , radiology , coronary sinus , biochemistry , chemistry , genetics , biology , chromosome , gene
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.