Gazit, Avihu Z

McBride, Mary E

Said, Ahmed S

English

Digital Commons@Becker

Washington University School of MedicineDigital Commons@BeckerOpen Access Publications2018Successful preoperative bridge with extracorporealmembrane oxygenation in three neonates with D-transposition of the great vessels and pulmonaryhypertensionAhmed S. SaidMary E. McBrideAvihu Z. GazitFollow this and additional works at: https://digitalcommons.wustl.edu/open_access_pubsSuccessful preoperative bridge withextracorporeal membrane oxygenation inthree neonates with D-transposition of thegreat vessels and pulmonary hypertensionAhmed S. Said1, Mary E. McBride2 and Avihu Z. Gazit11Department of Pediatrics, Division of Critical Care, Washington University in St. Louis, St. Louis, MO, USA and2Department of Pediatrics, Divisions of Cardiology and Critical Care, Northwestern University Feinberg Schoolof Medicine, Chicago, IL, USAAbstractPulmonary hypertension with transposition of the great arteries is associated with significantmorbidity and mortality. At the worst end of the spectrum are patients who undergoextracorporeal support perioperatively. We describe our experience with three patients whoreceived preoperative extracorporeal support and separated from cardiopulmonary bypasssuccessfully on conventional postoperative care, with no significant deficits on follow-up.Dextro-transposition of the great arteries and intact ventricular septum associated with per-sistent pulmonary hypertension is uncommon and associated with poor outcomes.1 Severaltherapeutic interventions have been described to improve systemic oxygen delivery and reduceoxygen consumption including balloon atrial septostomy, prostaglandin infusion to maintainductal patency, inotropic support,2 inhaled nitric oxide,3,4 epoprostenol,5 bosentan6, andextracorporeal membrane oxygenation.4,7,8 We present three patients with dextro-transposition of the great arteries, intact ventricular septum, and refractory persistent pul-monary hypertension of the newborn supported with extracorporeal membrane oxygenationpreoperatively, separated from bypass following their arterial switch operation and survived todischarge with favourable long-term follow-up.Case presentationsCase 1A term male newborn was diagnosed postnatally with dextro-transposition of the greatarteries and intact ventricular septum with a large atrial septal defect and patent ductusarteriosus. He remained hypoxaemic with evidence of low systemic oxygen delivery likelysecondary to pulmonary hypertension in spite of escalation in support. Given continuedevidence of cardiogenic shock, he underwent veno-arterial extracorporeal membrane oxyge-nation cannulation on day of life 1 and arterial switch operation on day 8 of support. He wasseparated from bypass successfully, albeit with evidence of pulmonary hypertension requiringsedation, neuromuscular blockade, mechanical ventilation, inhaled nitric oxide, and inotropicsupport. He was extubated on postoperative day 9 and discharged on postoperative day 12. Atthe most recent follow-up visit, he was 5 years old and doing well. He has no evidence ofadverse sequelae related to extracorporeal support.Case 2A near-term male infant – 37 weeks’ gestation – was diagnosed postnatally with dextro-transposition of the great arteries and intact ventricular septum and restrictive atrial septum.Following intubation and initiation of prostaglandin E1, balloon atrial septostomy was per-formed. In spite of initiation of inhaled nitric oxide and significant escalation in support, hedeveloped evidence of cardiogenic shock and underwent initiation of veno-arterial extra-corporeal membrane oxygenation on day of life 3. After an arterial switch operation onextracorporeal support day 3, he was separated successfully from bypass and returned to theICU with an open sternum, on inhaled nitric oxide and inotropic support. Following delayedsternal closure on postoperative day 12, the patient had ongoing respiratory failure, persistentchylothorax, and pulmonary hypertension. Thus, an open lung biopsy was performed, whichrevealed diffuse arterial wall and venous intima thickening, as well as pulmonary interstitialCardiology in the Youngcambridge.org/ctyBrief ReportCite this article: Said AS, McBride ME, Gazit AZ(2018) Successful preoperative bridge withextracorporeal membrane oxygenation in threeneonates with D-transposition of the greatvessels and pulmonary hypertension.Cardiology in the Young 28: 1175–1177.doi: 10.1017/S1047951118001038Received: 29 December 2017Revised: 7 May 2018Accepted: 18 May 2018Key words:ECMO; PPHN; D-TGA; arterial switch; intensivecareAuthor for correspondence:A. Z. Gazit, 660S. Euclid ave, Campus Box 8116,St. Louis, MO 63110, USA. Tel: +314 286 1246;Fax +13147478880. Email: Gazit_a@wustl.edu© Cambridge University Press 2018.https://www.cambridge.org/core/terms. https://doi.org/10.1017/S1047951118001038Downloaded from https://www.cambridge.org/core. Amigos Library Services, on 14 Aug 2018 at 00:07:22, subject to the Cambridge Core terms of use, available atglycogenosis. Given these findings, he was treated with a course ofsystemic steroids, extubated on postoperative day 40, and tran-sitioned from inhaled nitric oxide to sildenafil and bosentan.These medications were weaned gradually and discontinued5 months after discharge. At his last follow-up visit, he was 7 yearsold. He has mild right-ventricular outflow tract obstruction withno evidence of adverse sequelae related to extracorporeal support.Case 3A premature twin male infant – 35 weeks’ gestation – waspostnatally diagnosed with dextro-transposition of the greatarteries and intact ventricular septum with right to left flowacross a restrictive patent foramen ovale, as well as right to leftductus arteriosus flow (Fig 1). He was intubated and treated withsurfactant followed by initiation of prostaglandin E1, inhalednitric oxide, inotropic support, and balloon atrial septostomy. Onday of life 2 – because of evidence of cardiogenic shock – he wasurgently placed on veno-arterial extracorporeal membrane oxy-genation and developed posterior subdural haematoma while onsupport. He underwent an arterial switch operation on supportday 8 and was successfully separated from bypass. The post-operative course was favourable, allowing weaning and dis-continuation of inhaled nitric oxide, and inotropic supportfollowed by extubation on postoperative day 3. At his last follow-up visit, he was 8 years old, and asymptomatic with evidence ofmild pulmonary and aortic insufficiency. He has no evidence ofadverse sequelae related to extracorporeal support or the sub-dural haematoma.DiscussionTransient postnatal elevation of pulmonary vascular resistance iscommon and relatively benign. However, persistent pulmonaryhypertension of the newborn defined as failure to achieve orsustain the normal decrease in pulmonary vascular resistance atbirth may lead to severe hypoxaemia and need for aggressivecardiorespiratory support including – albeit less frequent fol-lowing introduction of inhaled nitric oxide – extracorporealoxygenation. Persistent pulmonary hypertension refractory tomedical management and – when indicated – balloon atrialseptostomy in neonates with dextro-transposition of the greatarteries and intact ventricular septum significantly increases themortality of these patients, with preoperative mortality rates of upto 28.6%9 as compared with the reported mortality rates of thetransposition of the great arteries population of <5%.10At the most severe end of the spectrum are patients whorequire extracorporeal oxygenation preoperatively because ofprogressive cardiogenic shock and risk for end-organ injury andcardiac arrest. These cases are uncommon, especially withintroduction of inhaled nitric oxide and other potent pulmonaryvasodilators. However, such patients require a thoughtfulapproach with regard to the timing and mode of support (veno-arterial vs. veno-venous), management on extracorporeal support,and timing of the arterial switch operation. Unfortunately, onlyfew reports are available on these patients, and none of themdescribe successful separation from bypass following arterialswitch operation with favourable long-term outcomes.4,7,8 Each ofour patients showed clinical and biochemical signs of progressiveshock leading to oxygen debt in spite of significant escalation ofsupport. To prevent irreversible end-organ injury and cardiacarrest with further escalation of conventional support, veno-arterial extracorporeal membrane oxygenation was initiated.Veno-arterial rather than veno-venous support was thought to beindicated in our patients because of the degree of cardiogenicshock. However, if oxygen delivery meets uptake needs, veno-venous support may be beneficial as it would lead to return ofoxygenated blood into the right atrium and from there mostlyinto the right ventricle and aorta while sparing the carotid artery.Timing of initiation of extracorporeal support is critical, asshown by Gill et al11 who studied neonates with meconiumaspiration syndrome cannulated for extracorporeal supportbetween 1989 and 1998. They found that delay in cannulationwhile assessing the effects of inhaled nitric oxide and high-frequency ventilation was associated with an increase in mortalityand prolongation of hospital stay.The pathophysiology of complete transposition of the greatarteries places neonates with persistent pulmonary hypertensionat a disadvantage in comparison with a neonate with normalcardiac anatomy because of high-volume physiological shuntingand the dependency of effective pulmonary blood flow on small-Figure 1. (a) Suprasternal notch long-axis colour flow Doppler view showing a patent ductus arteriosus (PDA) with evidence of pulmonary artery (PA) to descending aorta (DAo)flow. (b) Subcostal coronal colour flow Doppler view showing right atrium (RA) to left atrium flow across the patent foramen ovale (PFO). Both flow patterns may occur in thecontext of pulmonary hypertension.1176 A. S. Said et alhttps://www.cambridge.org/core/terms. https://doi.org/10.1017/S1047951118001038Downloaded from https://www.cambridge.org/core. Amigos Library Services, on 14 Aug 2018 at 00:07:22, subject to the Cambridge Core terms of use, available atvolume anatomical shunting via the patent ductus arteriosus andatrial septal defect.While correcting the anatomy in the operating room is theultimate goal, cardiopulmonary bypass and cross-clamping of theaorta may cause further increase in pulmonary vascular resis-tance, as well as myocardial inflammation and low cardiac outputsyndrome, leading to risk of further end-organ injury post-operatively. We believe that preoperative extracorporeal supportin these patients provides optimisation of end-organ function,facilitates protective mechanical ventilation and safe administra-tion of pulmonary vasodilators, and haemodynamic unloading ofthe pulmonary vascular tree before the unavoidable operation-related insults. The shortest duration of support that providesnormalisation of end-organ function, discontinuation of vaso-pressors, and weaning ventilator settings is preferred as a bridgeto the arterial switch operation to avoid complications related tothe support.ConclusionPatients with dextro-transposition of the great arteries and intactventricular septum and pulmonary hypertension with refractoryhypoxaemia and haemodynamic compromise may benefit from aperiod of stabilisation on extracorporeal support before theirarterial switch operation. An attempt should be made to separatesuch patients from cardiopulmonary bypass rather than transi-tioning back to extracorporeal support at the conclusion of asuccessful operation.Acknowledgements. The authors would like to acknowledge the criticalcare, cardiology, cardiothoracic surgery and perfusion divisions who suc-cessfully cared for these patients.Financial Support. This research received no specific grant from anyfunding agency or from commercial or not-for-profit sectors.Conflicts of Interest. None.Ethical Standards. This article does not contain any studies with humanparticipants performed by any of the authors.References1. Kirklin JW, Blackstone EH, Tchervenkov CI, Castaneda AR. Clinicaloutcomes after the arterial switch operation for transposition. Patient,support, procedural, and institutional risk factors. Congenital HeartSurgeons Society. Circulation 1992; 86: 1501–1515.2. Chang AC, Wernovsky G, Kulik TJ, Jonas RA, Wessel DL. Managementof the neonate with transposition of the great arteries and persistentpulmonary hypertension. Am J Cardiol 1991; 68: 1253–1255.3. El-Segaier M, Hellstrom-Westas L, Wettrell G. Nitric oxide in neonataltransposition of the great arteries. Acta Paediatr 2005; 94: 912–916.4. Jaillard S, Belli E, Rakza T, et al. Preoperative ECMO in transposition ofthe great arteries with persistent pulmonary hypertension. Ann ThoracSurg 2005; 79: 2155–2158.5. Kovach J, Ibsen L, Womack M, Steusse D, Law YM. Treatment ofrefractory pulmonary arterial hypertension with inhaled epoprostenol inan infant with congenital heart disease. Congenit Heart Dis 2007; 2: 194–198.6. Goissen C, Ghyselen L, Tourneux P, et al. Persistent pulmonaryhypertension of the newborn with transposition of the great arteries:successful treatment with bosentan. Eur J Pediatr 2008; 167: 437–440.7. Luciani GB, Chang AC, Starnes VA. Surgical repair of transposition of thegreat arteries in neonates with persistent pulmonary hypertension. AnnThorac Surg 1996; 61: 800–805.8. Trittenwein G, Furst G, Golej J, et al. Preoperative ECMO in congenitalcyanotic heart disease using the AREC system. Ann Thorac Surg 1997; 63:1298–1302.9. Roofthooft MT, Bergman KA, Waterbolk TW, Ebels T, Bartelds B, BergerRM. Persistent pulmonary hypertension of the newborn with transposi-tion of the great arteries. Ann Thorac Surg 2007; 83: 1446–1450.10. Khairy P, Clair M, Fernandes SM, et al. Cardiovascular outcomes after thearterial switch operation for D-transposition of the great arteries.Circulation 2013; 127: 331–339.11. Gill BS, Neville HL, Khan AM, Cox CS Jr, Lally KP. Delayed institution ofextracorporeal membrane oxygenation is associated with increasedmortality rate and prolonged hospital stay. J Pediatr Surg 2002; 37: 7–10.Cardiology in the Young 1177https://www.cambridge.org/core/terms. https://doi.org/10.1017/S1047951118001038Downloaded from https://www.cambridge.org/core. Amigos Library Services, on 14 Aug 2018 at 00:07:22, subject to the Cambridge Core terms of use, available at

Successful preoperative bridge with extracorporeal membrane oxygenation in three neonates with D-transposition of the great vessels and pulmonary hypertension

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Successful preoperative bridge with extracorporeal membrane oxygenation in three neonates with D-transposition of the great vessels and pulmonary hypertension

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