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Ultraschall Med 2017; 38(02): 206-211
DOI: 10.1055/s-0034-1399695
Original Article
© Georg Thieme Verlag KG Stuttgart · New York

Levoatrial Cardinal Vein in a Series of Five Prenatal Cases with Hypoplastic Left Heart Syndrome and Intact Atrial Septum

Laevoatriale Kardinalvene bei fünf Feten mit hypoplastischem Linksherzsyndrom und intaktem atrialen Septum
Astrid Hellmund
1   Department of Obstetrics and Prenatal Medicine, University Hospital Bonn, Germany
,
Christoph Berg
1   Department of Obstetrics and Prenatal Medicine, University Hospital Bonn, Germany
2   Division of Prenatal Medicine and Gynecologic Sonography, University Medical School Cologne, Köln, Germany
,
Ulrike Herberg
3   Department of Pediatric Cardiology, University Hospital Bonn, Germany
,
Annegret Geipel
1   Department of Obstetrics and Prenatal Medicine, University Hospital Bonn, Germany
,
Andrea Kempe
1   Department of Obstetrics and Prenatal Medicine, University Hospital Bonn, Germany
,
Ulrich Gembruch
1   Department of Obstetrics and Prenatal Medicine, University Hospital Bonn, Germany
› Author Affiliations
Further Information

Publication History

19 June 2014

12 March 2015

Publication Date:
17 June 2015 (online)

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Abstract

Purpose The rare finding of a levoatrial cardinal vein (LACV) represents a pulmonary-systemic connection providing an alternative egress from the left atrium for pulmonary venous blood in fetal cardiac malformations with severe left heart obstruction and intact atrial septum (IAS). The purpose of the study was the description of the various sonographic and Doppler findings, the peripartal management and neonatal outcome of this rare cardiovascular anomaly.

Materials and Methods Retrospective review of 53 967 echocardiograms in our fetal database between 2002 and 2013 for cases with an LACV. The various sonographic findings of the LACV, the associated cardiac findings and the perinatal management were assessed.

Results The presence of a decompressing LACV was documented in 5/27 (18.5 %) of all fetuses with hypoplastic left heart syndrome and intact atrial septum. All five fetuses were diagnosed with an LACV originating from the left atrium and draining either into the innominate vein (two cases), the azygos vein (one case), or directly into the vena cava superior (two cases). Elevated pressure in the pulmonary veins was present in the three cases with obstruction of the LACV. Two pregnancies were terminated, two neonates received compassionate care and one neonate died despite atrioseptectomy.

Conclusion LACV in the presence of HLHS and IAS is a rare condition with various forms of pathway and degree of obstruction and generally has an unfavorable prognosis. The presence of obstruction can be diagnosed by spectral Doppler of the LACV. Therefore, in cases of HLHS and IAS, careful sonographic evaluation for aberrant vessels is mandatory.

Zusammenfassung

Ziel Eine laevoatriale Kardinalvene (LACV) ist ein seltener Befund, der sich in der fetalen Echokardiografie bei Feten mit hypoplastischem Linksherzsyndrom (HLHS) und intaktem atrialen Septum (IAS) findet. Dieser stellt eine pulmonal-systemische Gefäßverbindung dar, über die das Blut aus dem pulmonal-venösen Gefäßbett über den linken Vorhof in die Systemvenen abfließt. Ziel der Studie war es, Ultraschall- und Dopplerbefunde dieser seltenen Gefäßanomalie, das peripartale Management und das kindliche Outcome zu beschreiben.

Material und Methoden Retrospektive Analyse von 53 967 in unserer Ambulanz für Pränatale Medizin durchgeführten fetalen Echokardiografien zwischen 2002 und 2013 bezüglich einer LACV. Ultraschall- und Dopplerbefunde einer LACV, assoziierte kardiale Fehlbildungen und das peripartale Management wurden erfasst.

Ergebnisse Eine LACV wurde in 5/27 Fällen (18,5 %) von Feten mit HLHS und IAS gefunden. In allen 5 Fällen entsprang die LACV dem linken Vorhof und drainierte entweder in die Vena anonyma (2 Fälle), die Vena azygos (1 Fall) oder direkt in die Vena cava superior (2 Fälle). In den drei Fällen einer Obstruktion der LACV fand sich eine erhöhte Pulsatilität des Blutflussgeschwindigkeitsprofils der Venae pulmonales. Ein Schwangerschaftsabbruch wurde auf Wunsch der Eltern in zwei Fällen durchgeführt, zwei weitere Neugeborene verstarben nach palliativer Versorgung und ein Neugeborenes verstarb perioperativ trotz Atrioseptostomie.

Schlussfolgerung Eine LACV ist ein seltener Befund bei Feten mit HLHS und IAS. Der Verlauf dieser Vene ist variabel, das Outcome dieser Kinder ist aufgrund der Assoziation mit dem schweren Herzfehler sehr ungünstig. Eine Obstruktion der LACV kann mithilfe des Spektraldopplers erfasst werden. Eine sorgfältige Suche nach einer LACV sollte im Rahmen der fetalen Echokardiografie bei HLHS und intaktem atrialen Septum immer durchgeführt werden.

Key words

fetus - levoatrial cardinal vein - hypoplastic left heart syndrome - intact atrial septum
 

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