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. 2023 Mar 23:12:100452.
doi: 10.1016/j.ijcchd.2023.100452. eCollection 2023 Jun.

Outcome of Down patients with repaired versus unrepaired atrioventricular septal defect

Mathies Daene  1 , Lore De Pauw  1 , Pieter De Meester  1   2   3 , Els Troost  1   2   3 , Philip Moons  1   4   5   6 , Marc Gewillig  1   2   7 , Filip Rega  1   2   8 , Alexander Van De Bruaene  1   2   3 , Werner Budts  1   2   3
Affiliations

Outcome of Down patients with repaired versus unrepaired atrioventricular septal defect

Mathies Daene et al. Int J Cardiol Congenit Heart Dis. .

Erratum in

Abstract

Background and aims: Patients with Down Syndrome (DS) are frequently born with an atrioventricular septal defect (AVSD). Surgical repair of the defect aims to minimize mortality and morbidity. However, a surgical intervention, specifically in DS patients, is not without risk and a subgroup of patients underwent only conservative non-surgical treatment. Outcome data of these different approaches are scarce. The aim of this retrospective study was to compare the long-term outcome of DS patients with and without surgery for AVSD.

Methods: DS patients registered with AVSD in the hospital's database from January 1980 till December 2020 were selected. Patient characteristics, peri-operative if appropriate, and follow-up data were obtained from the medical files.

Results: In total, 72 unrepaired (36 male, 50%) and 134 repaired patients (61 male, 46%) were included. After a maximum of 60 years of follow-up, the all-cause mortality was 45.8% and 17.1%, respectively. Thirty-six percent and 13%, respectively, were labeled as non-cardiovascular death. Mean survival time for unrepaired AVSD was 40.7 years (95% CI 36.1-45.2) and for repaired AVSD 38.5 years (95% CI 35.3-41.6) (Log rank p = 0.465). However, the survival rate 35 years after birth was 62.1% for unrepaired patients versus 81.7% for repaired patients. Mortality rates were the highest the first months after surgical repair.

Conclusions: The mean survival rate of Down patients, born with an AVSD, did not differ between repair or not. However, long-term survival rate was higher in patients who underwent surgical repair. Mortality was highest the first months after surgery.

Keywords: Atrioventricular canal; Atrioventricular septal defect; Down syndrome; Eisenmenger syndrome; Outcome; Surgery.

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Conflict of interest statement

The authors declare that they have no known competing financial interests or personal relationships that could have appeared to influence the work reported in this paper.

Figures

Fig. 1
Fig. 1
Kaplan Meier survival curve: all cause death, repaired versus non-repaired patients. (Patient with longest follow-up in the non-operated group censored).
Fig. 2
Fig. 2
Kaplan Meier redo surgery free curve in repaired patients.
See this image and copyright information in PMC

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