Zusammenfassung
Die Transposition der großen Gefäße (TGA) ist mit einer Häufigkeit von ca. 3–5% aller angeborenen Herzfehler eine seltene Erkrankung. Die Aorta entspringt ganz oder zum größten Teil aus dem morphologisch rechten und die Pulmonalarterie aus dem morphologisch linken Ventrikel. Es besteht also eine ventrikuloarterielle Diskordanz. Geht diese mit einer ventrikuloatrialen Konkordanz einher, spricht man von einer kompletten bzw. D-TGA, liegt zusätzlich auch eine ventrikuloatriale Diskordanz vor, von kongenital korrigierter oder L-TGA. Die D-TGA ist mit dem Leben nur bei gleichzeitigem Vorliegen eines Shunts vereinbar, der evtl. postnatal durch das so genannte Rashkind-Manöver auf Vorhofebene geschaffen werden muss.
Heute wird primär die frühe anatomische Korrektur mit Hilfe der arteriellen „Switchoperation“ angestrebt. Bis in die 80er Jahre wurde zur Korrektur einer D-TGA auch die Vorhofumkehroperation, der „atrial switch“ nach Senning/Mustard durchgeführt. Bei der Bildgebung wird neben der Echokardiographie v. a. die MRT zur Klärung der komplexen anatomischen Verhältnisse, der Volumetrie, Vitalität und Funktionsanalyse sowie der Flussmessung eingesetzt. Ist die MRT kontraindiziert oder steht die Klärung der Anatomie im Vordergrund, insbesondere die Koronararteriendarstellung, ist die Multidetektorcomputertomographie (MDCT) die bildgebende Methode der Wahl.
Abstract
Transposition of the great arteries (TGA) is a rare disease representing not more than 3–5% of all congenital heart diseases. TGA is a cardiac anomaly in which the aorta arises entirely or largely from the morphological right ventricle and the pulmonary artery from the morphological left ventricle. This is called a ventriculo-arterial discordant connection and when accompanied by an atrio-ventricular concordant connection it is called a complete or D-transposition (D-TGA). The terms congenitally corrected TGA (ccTGA) or L-TGA describe an atrio-ventricular discordant connection. In D-TGA survival can only be achieved if additional shunting is simultaneously present, which possibly has to be created post-natal by the so-called Rashkind maneuver.
Nowadays, an early anatomic correction using the arterial switch operation is the treatment of choice. Up to the 1980s, an atrial switch operation according to Senning/Mustard was performed. Apart from echocardiography the imaging modality of choice is usually MRI to assess the complex postoperative anatomy, viability of the myocardium and to perform a volumetric and functional assessment, including MR flow measurements. Multidetector computed tomography (MDCT) is used if there are contraindications to MRI or if an assessment of cardiac and especially coronary anatomy is the main interest.
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Interessenkonflikt
Der Autor Prof. Dr. M. Gutberlet ist als Referent für die Firmen BayerSchering Pharma, Bracco, Philips und Siemens tätig.
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Die Autoren MG, AF, SS, PB, HA wurden durch das Kompetenznetz „Angeborene Herzfehler“ unterstützt.
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Gutberlet, M., Hoffmann, J., Künzel, E. et al. Prä- und Postoperative Bildgebung bei Patienten mit Transposition der großen Gefäße. Radiologe 51, 15–22 (2011). https://doi.org/10.1007/s00117-010-1996-7
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DOI: https://doi.org/10.1007/s00117-010-1996-7
Schlüsselwörter
- Transposition der großen Gefäße (TGA)
- Magnetresonanztomographie (MRT)
- Multidetektorcomputertomographie (MDCT)
- Arterielle Umkehroperation
- Vorhofumkehroperation