Hostname: page-component-78c5997874-mlc7c Total loading time: 0 Render date: 2024-11-04T21:51:09.437Z Has data issue: false hasContentIssue false
  • English
  • Français

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: [email protected]
Get access Rights & Permissions [Opens in a new window]

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
Check for updates
Copyright
© The Author(s), 2021. Published by Cambridge University Press

Access options

Get access to the full version of this content by using one of the access options below. (Log in options will check for institutional or personal access. Content may require purchase if you do not have access.)

Footnotes

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

References

Moons, P, Gewillig, M, Sluysmans, T, et al. Long term outcome up to 30 years after the Mustard or Senning operation: a nationwide multicentre study in Belgium. Heart 2004; 90: 307–313.CrossRefGoogle ScholarPubMed
Piran, S, Veldtman, G, Siu, S, Webb, GD, Liu, PP. Heart failure and ventricular dysfunction in patients with single or systemic right ventricles. Circulation 2002; 105: 1189–1194.CrossRefGoogle ScholarPubMed
Schwerzmann, M, Salehian, O, Harris, L, et al. Ventricular arrhythmias and sudden death in adults after a Mustard operation for transposition of the great arteries. Eur Heart J 2009; 30: 1873–1879.CrossRefGoogle ScholarPubMed
Kammeraad, JA, van Deurzen, CH, Sreeram, N, et al. Predictors of sudden cardiac death after Mustard or Senning repair for transposition of the great arteries. J Am Coll Cardiol 2004; 44: 1095–1102.CrossRefGoogle ScholarPubMed
Graham, TP Jr., Bernard, YD, Mellen, BG, et al. Long-term outcome in congenitally corrected transposition of the great arteries: a multi-institutional study. J Am Coll Cardiol 2000; 36: 255–261.CrossRefGoogle ScholarPubMed
Beauchesne, LM, Warnes, CA, Connolly, HM, Ammash, NM, Tajik, AJ, Danielson, GK. Outcome of the unoperated adult who presents with congenitally corrected transposition of the great arteries. J Am Coll Cardiol 2002; 40: 285–290.CrossRefGoogle ScholarPubMed
Helm, PC, Koerten, MA, Abdul-Khaliq, H, Baumgartner, H, Kececioglu, D, Bauer, UM. Representativeness of the German National Register for Congenital Heart Defects: a clinically oriented analysis. Cardiol Young 2016; 26: 921–926.CrossRefGoogle ScholarPubMed
Woudstra, OI, Kuijpers, JM, Meijboom, FJ, et al. High burden of drug therapy in adult congenital heart disease: polypharmacy as marker of morbidity and mortality. Eur Heart J Cardiovasc Pharmacother 2019; 5: 216–225.CrossRefGoogle ScholarPubMed
Cuypers, JA, Eindhoven, JA, Slager, MA, et al. The natural and unnatural history of the Mustard procedure: long-term outcome up to 40 years. Eur Heart J 2014; 35: 1666–1674.CrossRefGoogle ScholarPubMed
Couperus, LE, Vliegen, HW, Zandstra, TE, et al. Long-term outcome after atrial correction for transposition of the great arteries. Heart 2019; 105: 790–796.CrossRefGoogle ScholarPubMed
Dennis, M, Kotchetkova, I, Cordina, R, Celermajer, DS. Long-term follow-up of adults following the atrial switch operation for transposition of the great arteries - a contemporary cohort. Heart Lung Circ 2018; 27: 1011–1017.CrossRefGoogle ScholarPubMed
Khairy, P. Sudden cardiac death in transposition of the great arteries with a Mustard or Senning baffle: the myocardial ischemia hypothesis. Curr Opin Cardiol 2017; 32: 101–107.CrossRefGoogle ScholarPubMed
Kral Kollars, CA, Gelehrter, S, Bove, EL, Ensing, G. Effects of morphologic left ventricular pressure on right ventricular geometry and tricuspid valve regurgitation in patients with congenitally corrected transposition of the great arteries. Am J Cardiol 2010; 105: 735–739.CrossRefGoogle ScholarPubMed
Helsen, F, De Meester, P, Van Keer, J, et al. Pulmonary outflow obstruction protects against heart failure in adults with congenitally corrected transposition of the great arteries. Int J Cardiol 2015; 196: 1–6.CrossRefGoogle ScholarPubMed
Stauber, A, Wey, C, Greutmann, M, et al. Left ventricular outflow tract obstruction and its impact on systolic ventricular function and exercise capacity in adults with a subaortic right ventricle. Int J Cardiol 2017; 244: 139–142.CrossRefGoogle ScholarPubMed
Deal, BJ. Late arrhythmias after surgery for transposition of the great arteries. World J Pediatr Congenit Heart Surg 2011; 2: 32–36.CrossRefGoogle ScholarPubMed
Kapa, S, Vaidya, V, Hodge, DO, et al. Right ventricular dysfunction in congenitally corrected transposition of the great arteries and risk of ventricular tachyarrhythmia and sudden death. Int J Cardiol 2018; 258: 83–89.CrossRefGoogle ScholarPubMed
van Dissel, AC, Winter, MM, van der Bom, T, et al. Long-term clinical outcomes of valsartan in patients with a systemic right ventricle: follow-up of a multicenter randomized controlled trial. Int J Cardiol 2019; 278: 84–87.CrossRefGoogle ScholarPubMed
Skoglund, K, Heimdahl, J, Mandalenakis, Z, et al. Effect of medical treatment in patients with systemic right ventricle. Scand Cardiovasc J 2020; 54: 300–305.CrossRefGoogle ScholarPubMed
Zaragoza-Macias, E, Zaidi, AN, Dendukuri, N, Marelli, A. Medical therapy for systemic right ventricles: a systematic review (part 1) for the 2018 AHA/ACC Guideline for the management of adults With congenital heart disease: a report of the American College of Cardiology/American Heart Association Task Force on Clinical Practice Guidelines. J Am Coll Cardiol 2019; 73: 1564–1578.CrossRefGoogle Scholar
Baumgartner, H, De Backer, J, Babu-Narayan, SV, et al. 2020 ESC Guidelines for the management of adult congenital heart disease. Eur Heart J 2020; 42: 563–645.CrossRefGoogle Scholar