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Late outcome, therapy and systemic ventricular function in patients with a systemic right ventricle: data of the German National Register for Congenital Heart Defects

Published online by Cambridge University Press:  18 October 2021

Corinna Lebherz Open the ORCID record for Corinna Lebherz [Opens in a new window] ,
Martin Gerhardus ,
Astrid Elisabeth Lammers ,
Paul Helm ,
Oktay Tutarel ,
Ulrike Bauer ,
Tanja Bülow ,
Gunter Kerst Open the ORCID record for Gunter Kerst [Opens in a new window] ,
Gerhard-Paul Diller  and
Nikolaus Marx
Corinna Lebherz*
Affiliation:
Department of Cardiology, University Hospital Aachen, Aachen, Germany
Martin Gerhardus
Affiliation:
Department of Cardiology, University Hospital Aachen, Aachen, Germany
Astrid Elisabeth Lammers
Affiliation:
Department of Cardiology III - Adult Congenital and Valvular Heart Disease, University Hospital Muenster, Muenster, Germany
Paul Helm
Affiliation:
Competence Network for Congenital Heart Defects, Berlin, Germany National Register for Congenital Heart Defects, DZHK (German Center for Cardiovascular Research), Berlin, Germany
Oktay Tutarel
Affiliation:
Department of Congenital Heart Disease and Paediatric Cardiology, German Heart Centre Munich, Technical University of Munich, Munich, Germany DZHK (German Center for Cardiovascular Research), Partner Site Munich, Munich, Germany
Ulrike Bauer
Affiliation:
Competence Network for Congenital Heart Defects, Berlin, Germany National Register for Congenital Heart Defects, DZHK (German Center for Cardiovascular Research), Berlin, Germany
Tanja Bülow
Affiliation:
Institute of Medical Statistics, RWTH Aachen, Aachen, Germany
Gunter Kerst
Affiliation:
Department of Pediatric Cardiology, University Hospital Aachen, Aachen, Germany
Gerhard-Paul Diller
Affiliation:
Department of Cardiology III - Adult Congenital and Valvular Heart Disease, University Hospital Muenster, Muenster, Germany
Nikolaus Marx
Affiliation:
Department of Cardiology, University Hospital Aachen, Aachen, Germany
*
Author for correspondence: Priv-Doz. Dr C. Lebherz, Department of Cardiology, University Hospital Aachen, Aachen, Germany. Tel: +49-241-8035774; Fax: +49-241-8082131. Email: clebherz@ukaachen.de
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Abstract

Background:

Adults with systemic right ventricle have a significant risk for long-term complications such as arrhythmias or heart failure.

Methods:

A nationwide retrospective study based on the German National Register for Congenital Heart Disease was performed. Patients with transposition of the great arteries after atrial switch operation or congenitally corrected TGA were included.

Results:

Two hundred and eight-five patients with transposition of the great arteries after atrial switch operation and 95 patients with congenitally corrected transposition of the great arteries were included (mean age 33 years). Systolic function of the systemic ventricle was moderately or severely reduced in 25.5 % after atrial switch operation and in 35.1% in patients with congenitally corrected transposition. Regurgitation of the systemic atrioventricular valve was present in 39.5% and 43.2% of the cases, respectively. A significant percentage of patients also had a history for supraventricular or ventricular arrhythmias. However, polypharmacy of cardiovascular drugs was rare (4.5%) and 38.5 % of the patients did not take any cardiovascular medication. The amount of cardiovascular drugs taken was associated with NYHA class as well as systemic right ventricular dysfunction. Patients with congenitally corrected transposition were more likely to receive pharmacological treatment than patients after atrial switch operation.

Conclusion:

A significant portion of patients with systemic right ventricle suffer from a relevant systemic ventricular dysfunction, systemic atrioventricular valve regurgitation, and arrhythmias. Despite this, medication for heart failure treatment is not universally used in this cohort. This emphasises the need for randomised trials in patient with systemic right ventricle.

Keywords

Systemic right ventricleheart failuretCHDtransposition of the great arteriescongenitally corrected transposition of the great arteries
Type
Original Article
Information
Cardiology in the Young , Volume 32 , Issue 8 , August 2022 , pp. 1235 - 1245
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Copyright
© The Author(s), 2021. Published by Cambridge University Press

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Footnotes

On behalf of the German Competence Network for Congenital Heart Defects Investigators (please refer to the list in the acknowledgement).

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