Prenatal diagnosis of abnormal cardinal systemic venous return without other heart defects: a case series.
OBJECTIVE
To describe fetal spectrum and echocardiographic characteristics of anomalous systemic venous return (ASVR, cardinal veins) without other structural heart defects (isolated ASVR), evaluate associated extracardiac and genetic anomalies and review neonatal outcome.


METHODS
From 2003 to 2009, 369 consecutive fetuses were diagnosed with cardiac malformation, including 27 cases with an isolated ASVR. The following variables were collected: type of ASVR, extracardiac anomalies, karyotype and short-term outcome.


RESULTS
The most common forms of ASVR were persistent left superior vena cava (SVC) (n = 15) and interrupted inferior vena cava (IVC) (n = 6). The other ASVR types were a persistent left SVC without a right SVC (n = 4), bilateral agenesis of the SVC with cephalic venous drainage toward the IVC through the azygos system (n = 1) and total ASVR to the coronary sinus (n = 1). Genetic disorders were confirmed in five cases, all with a persistent left SVC, with extracardiac anomalies present in four of them. Ten other patients had extracardiac anomalies detected prenatally or postnatally. When available, ASVR diagnosis was confirmed postnatally in all cases.


CONCLUSIONS
Isolated ASVRs can be accurately diagnosed prenatally and are frequently associated with extracardiac and genetic anomalies. A detailed morphological ultrasound and fetal karyotype should be suggested. Neonatal outcome was mainly related to the associated anomalies.
Pediatric Cardiology Unit, Department of Pediatrics, Cliniques universitaires Saint Luc, UCL, Brussels, Belgium. catherine.barrea@uclouvain.be
Catherine
C
Barrea
Caroline
C
Ovaert
Stéphane
S
Moniotte
Jean-Marc
JM
Biard
Patricia
P
Steenhaut
Pierre
P
Bernard
D000015:Abnormalities, Multiple / Q000000981:diagnostic imaging / Q000235:genetics; D016022:Case-Control Studies; D002869:Chromosome Aberrations / Q000706:statistics & numerical data; D015331:Cohort Studies; D054326:Coronary Sinus / Q000000981:diagnostic imaging / Q000473:pathology; D004452:Echocardiography; D005260:Female; D005865:Gestational Age; D006330:Heart Defects, Congenital / Q000000981:diagnostic imaging* / Q000235:genetics / Q000401:mortality; D006801:Humans; D007231:Infant, Newborn; D011247:Pregnancy; D011256:Pregnancy Outcome / Q000453:epidemiology; D012189:Retrospective Studies; D016216:Ultrasonography, Prenatal; D014680:Veins / Q000002:abnormalities* / Q000000981:diagnostic imaging