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CC BY-NC-ND 4.0 · Thorac Cardiovasc Surg 2021; 69(S 03): e68-e75
DOI: 10.1055/s-0041-1740070
Pediatric and Congenital Cardiology

Long-Term Outcome Up To 40 Years after Single Patch Repair of Complete Atrioventricular Septal Defect in Infancy or Childhood

Stefanie Reynen‡   *
1   Department of Surgery for Congenital Heart Disease, RWTH Aachen University, Aachen, Germany
2   Department of Internal Medicine II (Cardiology), University Hospital Regensburg, Regensburg, Germany
,
Hedwig H. Hövels-Gürich‡   *
3   Department of Pediatric Cardiology, RWTH Aachen University, Aachen, Germany
,
Jaime F. Vazquez-Jimenez
1   Department of Surgery for Congenital Heart Disease, RWTH Aachen University, Aachen, Germany
,
Bruno J. Messmer
1   Department of Surgery for Congenital Heart Disease, RWTH Aachen University, Aachen, Germany
,
Joerg S. Sachweh
1   Department of Surgery for Congenital Heart Disease, RWTH Aachen University, Aachen, Germany
› Author Affiliations Funding This research received no specific grant from any funding agency, commercial, or not-for-profit sectors.
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Abstract

Objectives Patients with repaired complete atrioventricular septal defect (CAVSD) represent an increasing portion of grown-ups with congenital heart disease. For repair of CAVSD, the single-patch technique has been employed first. This technique requires division of the bridging leaflets, thus, among other issues, long-term function of the atrioventricular valves is of particular concern.

Methods Between 1978 and 2001, 100 consecutive patients with isolated CAVSD underwent single-patch repair in our institution. Hospital mortality was 11%. Primary endpoints were clinical status, atrioventricular valve function, and freedom from reoperation in long term. Follow-up was obtained contacting the patient and/or caregiver, and the referring cardiologist.

Results Eighty-three patients were eligible for long-term follow-up (21.0 ± 8.7, mean ± standard deviation [21.5; 2.1–40.0, median; min–max] years after surgical repair). Actual long-term mortality was 3.4%. Quality of life (QoL; self- or caregiver-reported in patients with Down syndrome) was excellent or good in 81%, mild congestive heart failure was present in 16%, moderate in 3.6% as estimated by New York Heart Association classification. Echocardiography revealed normal systolic left ventricular function in all cases. Regurgitation of the right atrioventricular valve was mild in 48%, mild–moderate in 3.6%, and moderate in 1.2%. The left atrioventricular valve was mildly stenotic in 15% and mild to moderately stenotic in 2%; regurgitation was mild in 54%, mild to moderate in 13%, and moderate in 15% of patients. Freedom from left atrioventricular-valve-related reoperation was 95.3, 92.7, and 89.3% after 5, 10, and 30 years, respectively. Permanent pacemaker therapy, as an immediate result of CAVSD repair (n = 7) or as a result of late-onset sick sinus syndrome (n = 5), required up to six reoperations in single patients. Freedom from pacemaker-related reoperation was 91.4, 84.4, and 51.5% after 5, 10, and 30 years, respectively.

Conclusion Up to 40 years after single-patch repair of CAVSD, clinical status and functional results are promising, particularly, in terms of atrioventricular valve function. Permanent pacemaker therapy results in a life-long need for surgical reinterventions.

Keywords

complete atrioventricular septal defects - surgical repair - long-term results

* Both authors contributed equally to this work.




Publication History

Received: 18 April 2021

Accepted: 23 August 2021

Article published online:
25 December 2021

© 2021. The Author(s). This is an open access article published by Thieme under the terms of the Creative Commons Attribution-NonDerivative-NonCommercial License, permitting copying and reproduction so long as the original work is given appropriate credit. Contents may not be used for commercial purposes, or adapted, remixed, transformed or built upon. (https://creativecommons.org/licenses/by-nc-nd/4.0/)

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