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

Published:November 21, 2022DOI:https://doi.org/10.1016/j.jtcvs.2022.11.014

      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.

      Graphical abstract

      Key Words

      Abbreviations and Acronyms:

      ASO (arterial switch operation), BAS (balloon atrial septostomy), CPB (cardiopulmonary bypass), ECLS (extracorporeal life support), LCOS (low cardiac output syndrome), maxVIS (maximum vasoactive-inotropic score), PGE1 (prostaglandin E1), PPHN (persistent pulmonary hypertension of the newborn), TGA/IVS (d-loop transposition of the great arteries with intact intraventricular septum)
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      References

        • Fricke T.A.
        • d'Udekem Y.
        • Richardson M.
        • Thuys C.
        • Dronavalli M.
        • Ramsay J.M.
        • et al.
        Outcomes of the arterial switch operation for transposition of the great arteries: 25 years of experience.
        Ann Thorac Surg. 2012; 94: 139-145
        • Cain M.T.
        • Cao Y.
        • Ghanayem N.S.
        • Simpson P.M.
        • Trapp K.
        • Mitchell M.E.
        • et al.
        Transposition of the great arteries–outcomes and time interval of early neonatal repair.
        World J Pediatr Congenit Heart Surg. 2014; 5: 241-247
        • Vida V.L.
        • Zanotto L.
        • Zanotto L.
        • Triglia L.T.
        • Bellanti E.
        • Castaldi B.
        • et al.
        Arterial switch operation for transposition of the great arteries: a single-centre 32-year experience.
        J Card Surg. 2019; 34: 1154-1161
        • Luciani G.B.
        • Chang A.C.
        • Starnes V.A.
        Surgical repair of transposition of the great arteries in neonates with persistent pulmonary hypertension.
        Ann Thorac Surg. 1996; 61: 800-805
        • Roofthooft M.T.
        • Bergman K.A.
        • Waterbolk T.W.
        • Ebels T.
        • Bartelds B.
        • Berger R.M.
        Persistent pulmonary hypertension of the newborn with transposition of the great arteries.
        Ann Thorac Surg. 2007; 83: 1446-1450
        • Trittenwein G.
        • Furst G.
        • Golej J.
        • Frenzel K.
        • Burda G.
        • Hermon M.
        • et al.
        Preoperative ECMO in congenital cyanotic heart disease using the AREC system.
        Ann Thorac Surg. 1997; 63: 1298-1302
        • Jaillard S.
        • Belli E.
        • Rakza T.
        • Larrue B.
        • Magnenant E.
        • Rey C.
        • et al.
        Preoperative ECMO in transposition of the great arteries with persistent pulmonary hypertension.
        Ann Thorac Surg. 2005; 79: 2155-2158
        • Bautista-Hernandez V.
        • Thiagarajan R.R.
        • Fynn-Thompson F.
        • Rajagopal S.K.
        • Nento D.E.
        • Yarlagadda V.
        • et al.
        Preoperative extracorporeal membrane oxygenation as a bridge to cardiac surgery in children with congenital heart disease.
        Ann Thorac Surg. 2009; 88: 1306-1311
        • Sallaam S.
        • Natarajan G.
        • Aggarwal S.
        Persistent pulmonary hypertension of the newborn with D-transposition of the great arteries: management and prognosis.
        Congenit Heart Dis. 2016; 11: 239-244
        • Said A.S.
        • McBride M.E.
        • Gazit A.Z.
        Successful preoperative bridge with extracorporeal membrane oxygenation in three neonates with D-transposition of the great vessels and pulmonary hypertension.
        Cardiol Young. 2018; 28: 1175-1177
        • Lim J.M.
        • Porayette P.
        • Marini D.
        • Chau V.
        • Au-Young S.H.
        • Saini A.
        • et al.
        Associations between age at arterial switch operation, brain growth, and development in infants with transposition of the great arteries.
        Circulation. 2019; 139: 2728-2738
        • Yam N.
        • Chen R.H.
        • Rocha B.A.
        • Lun K.S.
        • Yung T.C.
        • Au T.W.
        Preoperative venovenous extracorporal membrane oxygenation for transposition of great arteries with severe pulmonary hypertension in a newborn.
        Ann Thorac Surg. 2020; 109: e329-e330
      1. Extracorporeal Life Support Organization (2022). ECLS Registry report. International summary–October 2022. Accessed December 8, 2022. https://www.elso.org/registry/internationalsummaryandreports/internationalsummary.aspx

        • Gaies M.G.
        • Jeffries H.E.
        • Niebler R.A.
        • Pasquali S.K.
        • Donohue J.E.
        • Yu S.
        • et al.
        Vasoactive-inotropic score is associated with outcome after infant cardiac surgery: an analysis from the Pediatric Cardiac Critical Care Consortium and Virtual PICU System Registries.
        Pediatr Crit Care Med. 2014; 15: 529-537
        • Armishaw J.
        • Gentles T.L.
        • Calder A.L.
        • Raudkivi P.J.
        • Kerr A.R.
        Transposition of the great arteries: operative outcome in the current era.
        N Z Med J. 2000; 113: 456-459
        • Maeno Y.V.
        • Kamenir S.A.
        • Sinclair B.
        • van der Velde M.E.
        • Smallhorn J.F.
        • Hornberger L.K.
        Prenatal features of ductus arteriosus constriction and restrictive foramen ovale in d-transposition of the great arteries.
        Circulation. 1999; 99: 1209-1214
        • Jouannic J.M.
        • Gavard L.
        • Fermont L.
        • Le Bidois J.
        • Parat S.
        • Vouhé P.R.
        • et al.
        Sensitivity and specificity of prenatal features of physiological shunts to predict neonatal clinical status in transposition of the great arteries.
        Circulation. 2004; 110: 1743-1746
        • Di Donato R.M.
        • Fujii A.M.
        • Jonas R.A.
        • Castañeda A.R.
        Age-dependent ventricular response to pressure overload. Considerations for the arterial switch operation.
        J Thorac Cardiovasc Surg. 1992; 104: 713-722
        • Polito A.
        • Barrett C.S.
        • Rycus P.T.
        • Favia I.
        • Cogo P.E.
        • Thiagarajan R.R.
        Neurologic injury in neonates with congenital heart disease during extracorporeal membrane oxygenation: an analysis of extracorporeal life support organization registry data.
        ASAIO J. 2015; 61: 43-48
        • Peer S.M.
        • Emerson D.A.
        • Costello J.P.
        • Shu M.K.
        • Zurakowski D.
        • Jonas R.A.
        • et al.
        Intermediate-term results of extracorporeal membrane oxygenation support following congenital heart surgery.
        World J Pediatr Congenit Heart Surg. 2014; 5: 236-240
        • Garcia Guerra G.
        • Robertson C.M.
        • Alton G.Y.
        • Joffe A.R.
        • Moez E.K.
        • Dinu I.A.
        • et al.
        Health-related quality of life in pediatric cardiac extracorporeal life support survivors.
        Pediatr Crit Care Med. 2014; 15: 720-727
        • Fleck T.P.
        • Dangel G.
        • Bächle F.
        • Benk C.
        • Grohmann J.
        • Kroll J.
        • et al.
        Long-term follow-up on health-related quality of life after mechanical circulatory support in children.
        Pediatr Crit Care Med. 2017; 18: 176-182
        • Cloete E.
        • Bloomfield F.H.
        • Sadler L.
        • de Laat M.W.M.
        • Finucane A.K.
        • Gentles T.L.
        Antenatal detection of treatable critical congenital heart disease is associated with lower morbidity and mortality.
        J Pediatr. 2019; 204: 66-70
        • Blyth M.
        • Howe D.
        • Gnanapragasam J.
        • Wellesley D.
        The hidden mortality of transposition of the great arteries and survival advantage provided by prenatal diagnosis.
        BJOG. 2008; 115: 1096-1100
        • Calderon J.
        • Angeard N.
        • Moutier S.
        • Plumet M.H.
        • Jambaque I.
        • Bonnet D.
        Impact of prenatal diagnosis on neurocognitive outcomes in children with transposition of the great arteries.
        J Pediatr. 2012; 161: 94-98.e1
        • Gopalakrishnan A.
        • Sasidharan B.
        • Krishnamoorthy K.M.
        • Sivasubramonian S.
        • Dharan B.S.
        • Mathew T.
        • et al.
        Left ventricular regression after balloon atrial septostomy in d-transposition of the great arteries.
        Eur J Cardio Thorac Surg. 2016; 50: 1096-1101
        • Hiraishi S.
        • Fujino N.
        • Saito K.
        • Oguchi K.
        • Kadoi N.
        • Agata Y.
        • et al.
        Responsiveness of the ductus arteriosus to prostaglandin E1 assessed by combined cross sectional and pulsed Doppler echocardiography.
        Br Heart J. 1989; 62: 140-147
        • Rudolph A.M.
        High pulmonary vascular resistance after birth: I. Pathophysiologic considerations and etiologic classification.
        Clin Pediatr (Phila). 1980; 19: 585-590
        • Beca J.
        • Gunn J.
        • Coleman L.
        • Hope A.
        • Whelan L.C.
        • Gentles T.
        • et al.
        Pre-operative brain injury in newborn infants with transposition of the great arteries occurs at rates similar to other complex congenital heart disease and is not related to balloon atrial septostomy.
        J Am Coll Cardiol. 2009; 53: 1807-1811
        • Bellinger D.C.
        • Wypij D.
        • Kuban K.C.
        • Rappaport L.A.
        • Hickey P.R.
        • Wernovsky G.
        • et al.
        Developmental and neurological status of children at 4 years of age after heart surgery with hypothermic circulatory arrest or low-flow cardiopulmonary bypass.
        Circulation. 1999; 100: 526-532
        • Bellinger D.C.
        • Wypij D.
        • duPlessis A.J.
        • Rappaport L.A.
        • Jonas R.A.
        • Wernovsky G.
        • et al.
        Neurodevelopmental status at eight years in children with dextro-transposition of the great arteries: the Boston Circulatory Arrest Trial.
        J Thorac Cardiovasc Surg. 2003; 126: 1385-1396
        • Bellinger D.C.
        • Wypij D.
        • Rivkin M.J.
        • DeMaso D.R.
        • Robertson Jr., R.L.
        • Dunbar-Masterson C.
        • et al.
        Adolescents with d-transposition of the great arteries corrected with the arterial switch procedure: neuropsychological assessment and structural brain imaging.
        Circulation. 2011; 124: 1361-1369
        • Gaynor J.W.
        • Jarvik G.P.
        • Gerdes M.
        • Kim D.S.
        • Rajagopalan R.
        • Bernbaum J.
        • et al.
        Postoperative electroencephalographic seizures are associated with deficits in executive function and social behaviors at 4 years of age following cardiac surgery in infancy.
        J Thorac Cardiovasc Surg. 2013; 146: 132-137

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      • Commentary: Emergent arterial switch for critically ill babies with transposition
        The Journal of Thoracic and Cardiovascular Surgery
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          Among the most important advances over the past 2 decades in the field of congenital heart surgery has been the development and refinement of emergency extracorporeal membrane oxygenation (ECMO). Whether dealing with cardiac arrest after surgery or arrival in the middle of the night of an undiagnosed neonate, generic arteriovenous ECMO smoothly instituted by a coordinated experienced team has become the hallmark of every high quality, busy congenital program. ECMO allows for stabilization, thoughtful and accurate diagnosis, and time to develop a longer-term plan.
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