Congenital: Transposition of the Great Arteries
Emergency arterial switch: Rescue therapy for life-threatening hypoxemia in infants with transposition of great arteries with intact intraventricular septum

https://doi.org/10.1016/j.jtcvs.2022.11.014Get rights and content

Abstract

Objective

A small percentage of infants with d-loop transposition of the great arteries with intact intraventricular septum have life-threatening refractory hypoxemia often due to coexistent persistent pulmonary hypertension of the newborn. In this case series we describe the outcomes of a “rescue” emergency arterial switch operation (ASO).

Methods

We undertook a retrospective medical record analysis of infants with d-loop transposition of the great arteries with intact intraventricular septum who underwent an ASO in New Zealand from January 1, 1996, to April 30, 2017. Data were compared for those who received an emergency ASO and those with a nonemergency ASO for descriptive purposes. An emergency ASO was defined as one that was undertaken for life-threatening refractory hypoxemia when the only alternative stabilization strategy was preoperative extracorporeal life support. Primary outcome measures were 30-day postoperative mortality and abnormal neurodevelopmental outcome in the survivors. Secondary outcomes were low cardiac output, arrhythmia, renal dysfunction, postoperative seizures, and length of stay. Other known risk factors for morbidity and mortality were also assessed.

Results

Two hundred seventy-two infants underwent an ASO with 25 (9%) who received an emergency ASO. No infants received preoperative extracorporeal life support. The emergency group had greater 30-day postoperative mortality (8.0% vs 0.4%; P = .01) with no difference in abnormal neurodevelopmental outcome among the survivors (17.4% vs 13.8%; P = .35). The emergency group had more therapies for low cardiac output syndrome, more postoperative seizures, and a longer length of stay.

Conclusions

An emergency ASO is a definitive rescue therapy that can be undertaken with acceptable mortality and neurodevelopmental outcome with consideration of the preoperative clinical state.

Section snippets

Study Population

This retrospective study included all infants with TGA/IVS who received an ASO in Auckland, New Zealand from January 1, 1996, to April 30, 2017. Those with additional major cardiac anatomical abnormalities other than a patent ductus arteriosus, atrial septal defect, or patent foramen ovale were excluded.

New Zealand has a single pediatric cardiothoracic center, which is based in Auckland. This was at Green Lane Hospital until 2003 when it relocated to Starship Children's Hospital. In addition to

Results

During the study period, 279 infants with TGA/IVS underwent an ASO. Seven, who all had a nonemergency ASO, were excluded from analysis: 6 had other cardiac anomalies including a dysplastic pulmonary valve, subpulmonary valve stenosis, left pulmonary artery stenosis, and dysplastic mitral valve; and in 1 case the medical record was unavailable. Of the remaining 272 patients, 25 (9%) received an emergency ASO.

The emergency group tended to be born at a later gestational age. The rate of postnatal

Discussion

The main finding of this report is that infants with TGA/IVS and severe hypoxemia can be treated successfully with a “rescue” emergency ASO. The early mortality rate was higher compared with the nonemergency group but this was not excessive considering the clinical state. The cause of death reflects the morbidity profile; 1 of the deaths occurred in an infant who had an intact atrial septum and restrictive arterial duct in utero—a scenario known to be associated with significant pulmonary

Conclusions

In this large single-center case series we described the outcomes of an emergency ASO to treat infants with TGA/IVS complicated by life-threatening refractory hypoxemia most likely from coexistent PPHN. The emergency ASO is a definitive strategy and can be undertaken with a low mortality rate and neurodevelopmental morbidity considering the preoperative condition.

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