Esophageal atresia and tracheoesophageal fistula associated with tetralogy of Fallot: a review of mortality
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ORIGINAL ARTICLE
Esophageal atresia and tracheoesophageal fistula associated with tetralogy of Fallot: a review of mortality K. M. O’Shea1 · M. L. Griffiths1 · K. L. King1 · P. Losty1 · M. Jones1 · J. Minford1 · F. Murphy1 Accepted: 12 August 2020 © Springer-Verlag GmbH Germany, part of Springer Nature 2020
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. Keywords Oesophageal atresia · Tracheoesophageal fistula · Tetralogy of Fallot · Congenital heart disease · Spitz classification · Survival · Outcomes
Introduction Oesophageal atresia (EA) is a rare congenital anomaly, with an incidence of 0.3 per 1000 live births [1] and there is a well-recognized association of newborns also having congenital heart disease (CHD). The Spitz classification system (1994) provides a robust prognostic scoring tool that shows a survival correlation with infant birth weight and presence or absence of congenital heart disease [2, 3]. Survival for isolated EA is almost 98%; however, when EA TEF is associated with a major cardiac anomaly and low birth weight, less than 50% of patients experience hospital discharge [3]. * K. M. O’Shea [email protected] 1
Department of Paediatric Surgery, Alder Hey Children’s Hospital NHS Foundation Trust, University of Liverpool, Liverpool, UK
Outcomes for Tetralogy of Fallot (TOF) have significantly improved over the past 20 years, with notable advances in cardiac surgery, and such that survival beyond adolescence is reported in around 80% of cases [4].
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