Esophageal atresia and tracheoesophageal fistula associated with tetralogy of Fallot: a review of mortality

Abstract

Introduction

Oesophageal atresia ± tracheoesophageal fistula (EA/TEF) associated with congenital heart disease (CHD) carries a worse prognosis than EA/TEF alone. Though the Spitz classification takes major CHD into account, there are no data regarding survival with the specific combination of EA/TEF and Tetralogy of Fallot (TOF). With advances in postnatal care, we hypothesised that, survival is improving in these complex patients. This study reports morbidity and mortality outcomes of newborns with oesophageal atresia and TOF cardiac malformations

Methods

All patients with EA/TEF and TOF treated at Alder Hey Children’s Hospital between the years 2000–2020, were identified. Data sets regarding gestation, birth weight, associated anomalies, operative intervention, morbidity, and mortality were analysed.

Results

Of a total of 350, EA/TEF patients 9 (2.6%) cases had EA/TEF associated with TOF (M:F 4:5). The median gestational age was 35/40 (range 28–41 weeks) with a median birth weight of 1790 g (range 1060–3350 g). Overall survival was 56% (5/9 cases) and all survivors remain under follow up (range 37–4458 days). Surgical strategies for managing EA/TEF with Fallot’s tetralogy included 6/9 primary repairs and 3/9 cases with TEF ligation only (+ gastrostomy ± oesophagostomy).

Conclusions

This study reports outcome data from one of the largest series of EA TEF patients with Fallot’s tetralogy. Whilst outcomes may be challenging for this unique patient cohort, survival metrics provide important prognostic information that can be widely shared with health care teams and parents.