Prenatal diagnosis of anomalous systemic venous return: a case series

  • Dr Catherine Barrea, University Clinic Saint Luc, Belgium
  • Dr Stéphane Moniotte, University Clinic Saint Luc, Belgium
  • Dr Corinne Hubinont, University Clinic Saint Luc, Belgium
  • Dr Pierre Bernard, University Clinic Saint Luc, Belgium

    • OBJECTIVE : Increasing experience in fetal ultrasound leads to more frequent diagnoses of isolated anomalous systemic venous return (ASVR). Our objectives were to describe the spectrum and the echocardiographic features of ASVR not associated with other structural heart defect, and to evaluate associated extracardiac and chromosomal anomalies.

    METHODS : From 2003 to 2009, 340 consecutive fetuses were diagnosed with a cardiac malformation, including 25 cases of isolated ASVR. The following variables were collected: gestational age (GA) et diagnosis, type of ASVR, associated extracardiac anomalies, karyotype and outcome.

    RESULTS: The diagnosis was obtained at a mean GA of 27+3 weeks. The most common forms of ASVR were: 1) persistence of a left superior vena cava (SVC) draining into the coronary sinus with the presence of a normal right SVC (N=15) and 2) presence of an interrupted inferior vena cava (IVC) with azygos continuation (N=5). The other fetuses had a persistent left SVC without a right SVC (N=3), bilateral agenesis of the SVC with cephalic venous drainage towards the IVC through the azygos (N=1) or total ASVR to the coronary sinus (N=1). Genetic disorders were confirmed in 4 fetuses, all with a persistent left SVC, with associated extracardiac anomalies in 3 of them. Seven other fetuses had associated extracardiac anomalies. When available, cardiac diagnosis was confirmed postnatally in all but one. Echographic findings are summarized in the Table.

    CONCLUSIONS: Detailed diagnosis of isolated ASVR can be accurately obtained prenatally. The majority of fetuses with a genetic anomaly presented also with extracardiac malformation.

    Abnormal ‘3-vessel view’ Dilated coronary sinus Retro-aortic vessel Abnormal RSVC –IVC view
    Persistent LSVC to coronary sinus + (4 vessels, LSVC on the left) + - -
    Interrupted IVC with azygos continuation - - + (feet-to-head) + (no IVC continuation)
    Persistent LSVC to coronary sinus without RSVC + (3 vessels, large LSVC on the left) ++ - -
    Bilateral absent SVC + (2 vessels anterior without SVC, (hemi)-azygos posterior) - + (head-to-feet) + (dilated IVC)
    Total ASVR to coronary sinus + (3 vessels, large LSVC on the left) +++ - -