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Ultraschall Med 2023; 44(04): e184-e190
DOI: 10.1055/a-1823-0821
Original Article

Aorto-Left Ventricular Tunnel – Prenatal Diagnosis and Outcome

Aorto-links ventrikulärer Tunnel – pränatale Diagnose und Outcome
Eva Christin Weber
1   Department for Prenatal Medicine and Gynecologic Ultrasound, University Hospital Cologne, Köln, Germany
2   Department for Obstetrics and Prenatal Medicine, University Hospital Bonn, Germany
,
Florian Recker 
2   Department for Obstetrics and Prenatal Medicine, University Hospital Bonn, Germany
,
Ulrike Herberg
3   Department of Pediatric Cardiology, University Hospital Bonn, Germany
,
Renate Oberhoffer
4   Department of Pediatric Cardiology and Congenital Heart Disease, Deutsches Herzzentrum München, Germany
5   Preventive and Rehabilitative Sports Medicine, Technische Universität München, Germany
,
Andrii Kurkevych
6   Fetal Cardiology Unit, Ukrainian Children’s Hospital, Kyiv, UA, Kyiv, Ukraine
,
Roland Axt-Fliedner
7   Obstetrics and Gynecology, University Hospital of Giessen and Marburg Campus Marburg, Germany
,
Annegret Geipel
8   Department of Obstetrics and Prenatal Medicine, University Hospital Bonn, Germany
,
Ulrich Gembruch
8   Department of Obstetrics and Prenatal Medicine, University Hospital Bonn, Germany
,
Christoph Berg
1   Department for Prenatal Medicine and Gynecologic Ultrasound, University Hospital Cologne, Köln, Germany
8   Department of Obstetrics and Prenatal Medicine, University Hospital Bonn, Germany
,
Ingo Gottschalk
1   Department for Prenatal Medicine and Gynecologic Ultrasound, University Hospital Cologne, Köln, Germany
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Abstract

Purpose Aorto-left ventricular tunnel (ALVT) is an extremely rare, albeit prenatally detectable, extracardiac channel that connects the ascending aorta to the cavity of the left ventricle.

Materials and Methods All ALVTs diagnosed prenatally (2006–2020) in five tertiary referral centers were retrospectively assessed for prenatal ultrasound findings, intrauterine course, postnatal outcome, and surgical treatment. We focused on the size of the tunnel and alterations of perfusion of the left ventricular outflow tract and aortic arch.

Results 11 fetuses were diagnosed with ALVT at a mean gestational age of 24.8 weeks. All cases were associated with severe dilatation of the left ventricle and a to-and-fro flow in the left outflow tract. Signs of congestive heart failure were present in five fetuses, four of which were terminated and one of which died in the neonatal period. One fetus died in utero at 34 weeks without prior signs of cardiac failure. Of the five survivors, two underwent the Ross procedure. In both cases the prenatal left ventricular outflow was exclusively via a large tunnel. The remaining three neonates underwent patch closure of the tunnel. In these cases, the prenatal outflow of the left ventricle was via the aortic valve and simultaneously over the tunnel.

Conclusion Prenatal diagnosis of ALVT should be considered in the presence of left ventricular hypertrophy, dilatation of the aortic root, and to-and-fro flow in the aortic outflow tract. Signs of heart failure are associated with an unfavorable outcome. Large tunnels, particularly in combination with the absence of flow over the aortic valve, may be an unfavorable predictor of surgical repair.

Zusammenfassung

Ziel Der aorto-links ventrikuläre Tunnel (ALVT) ist ein sehr seltener, aber pränatal diagnostizierbarer, extrakardialer Tunnel, der die Aorta ascendens mit dem linken Ventrikel verbindet.

Material und Methode Retrospektive Analyse aller ALVT aus 5 Referenzzentren für Pränataldiagnostik (2006–2020) hinsichtlich pränataler Ultraschallbefunde, intrauterinem Verlauf, postnatalem Outcome und operativer Versorgung. Als potenziell prognostische Marker wurden die Größe des Tunnels und die Perfusionsparameter des Aortenbogens untersucht.

Ergebnisse Bei 11 Feten wurde ein ALVT diagnostiziert (Median 24,8 SSW). Alle Fälle zeigten eine linksventrikuläre Hypertrophie und einen Pendelfluss im linksventrikulären Ausflusstrakt. Fünf Feten hatten Zeichen einer Herzinsuffizienz; 4 dieser Schwangerschaften wurden abgebrochen und ein Neugeborenes verstarb nach Palliativtherapie. Ein Fet ohne Herzinsuffizienz-Zeichen verstarb intrauterin mit 34. SSW. Zwei der 5 überlebenden Kinder wurden mit einer Ross-Operation versorgt. In beiden Fällen erfolgte der pränatale linksventrikuläre Auswurf ausschließlich über einen großen Tunnel. Die anderen 3 Überlebenden erhielten einen Patch-Verschluss des ALVT. In diesen Fällen war der pränatale linksventrikuläre Ausfluss nicht nur über den Tunnel, sondern auch über die Aortenklappe vorhanden.

Schlussfolgerungen Die pränatale Diagnose eines ALVT sollte bei linksventrikulärer Hypertrophie und Pendelfluss im linksventrikulären Ausflusstrakt vermutet werden. Herzinsuffizienz-Zeichen sind mit einem ungünstigen Outcome assoziiert. Große ALVT, insbesondere in Kombination mit fehlendem Auswurf über die Aortenklappe, verschlechtern die Korrekturoptionen.

Key words

congenital heart defect - aortic disease - paravalvular channel - aorto-left ventricular tunnel


Publication History

Received: 31 January 2022

Accepted: 03 April 2022

Article published online:
05 May 2022

© 2022. Thieme. All rights reserved.

Georg Thieme Verlag KG
Rüdigerstraße 14, 70469 Stuttgart, Germany

 

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