Beating-heart aortic arch surgery in neonates and infants

Beating-heart aortic arch surgery in neonates and infants Abstract OBJECTIVES Aortic arch repair has been shifted from deep hypothermia plus circulatory arrest to cerebral perfusion at tepid temperatures. A step forward is a simultaneous brain–coronary perfusion, allowing beating-heart arch surgery. METHODS A ‘Y’ cannula from the arterial line delivers oxygenated blood to brain and heart. The arch is repaired on a beating heart at 25°C. Intracardiac repair is performed after running cardioplegia through the root line. Fifty patients are classified into 3 groups: A, Norwood (8 neonates); B, aortic arch (14 children) and C, aortic arch plus intracardiac repair (28 patients). Associated anomalies in Group C are as follows: ventricular septal defect (10), arterial switch (5), atrial septal defect (4), cor triatriatum (3), aortic commissurotomy (2), comprehensive repair (2), ostium primum (1) and Yasui (1). RESULTS The mean bypass time was 161 ± 54.44 (range 93–312) min. Mean brain–coronary perfusion was 37.26 ± 10.54 (18–60) min. Mean coronary ischaemia was 31 ± 32.40 (0–160) min. The heart was not arrested in Group B patients. Follow-up was complete for a mean of 30 (1–48) months. Four patients died in the postoperative period. Two required angioplasty for recoarctation. CONCLUSIONS Selective brain–coronary perfusion is feasible and easy to switch to conventional cardioplegia delivery. Coronary ischaemia can be notably reduced and even 0 min in isolated arch surgery. Aortic arch, Beating heart, Hypothermia, Brain, Coronary, Perfusion INTRODUCTION Deep hypothermia plus circulatory arrest was the method of choice for aortic arch surgery some decades ago [1, 2] (and still the strategy in many well-known centres), relying on the reduction in metabolism and oxygen requirements for brain protection. Complex neonatal arch surgery with long periods of circulatory arrest was associated with seizures and choreoathetosis, as well as a likelihood of future neurological impairment. Antegrade cerebral perfusion (ACP) emerged as an adjunct to brain protection [3] by providing blood flow from the arterial line through the innominate artery. Somatic flow is expected to be guaranteed by collateral circulation at low temperature. Several reports compared both perfusion strategies [4–6], deep hypothermia plus circulatory arrest vs ACP, with regard to neurological outcomes and brain preservation at different temperatures [7]. On gaining experience, some papers elucidated the balanced circulation between both hemispheres [8, 9] to provide adequate brain perfusion by a single carotid (and vertebral) artery. A step forward in protection was to deliver flow to the coronary arteries while performing arch surgery [10]. This way, one could accomplish arch repair under tepid temperature [7] on a ‘beating-heart’ basis. We will describe the perioperative impact of ACP and selective myocardial perfusion at 25°C in newborns and infants undergoing arch reconstructions below. Herein, we report our experience and outcomes. METHODS Between January 2013 and December 2017, 50 patients (38 neonates and 12 infants) were prospectively recorded. Complex arch surgery on cardiopulmonary bypass (CPB) and moderate hypothermia (25°C) was performed, with ACP (50 ml/kg/min) via the innominate artery. The mean age was 49 ± 84.55 days (range 1 day to 11 months). Mean weight was 3.75 ± 1.62 (range 2.1–10.5) kg. Patients were classified into 3 main groups (Table 1): Table 1: Patient distribution Total Norwood (Group A) Arch (Group B) Arch+ (Group C) Patients enrolled (older than 1 month), n (%) 50 (12) 8 14 (6) 28 (6) Age (days) 49 ± 84.55 3 ± 1.07 68 ± 90.82 53 ± 90.34 Weight (kg) 3.75 ± 1.62 3.17 ± 0.47 4.4 ± 2.10 3.6 ± 1.49 CPB (min) 161 ± 54.44 182 ± 37.35 133 ± 49.86 170 ± 56.74 Clamp (min) 31 ± 32.40 50.75 ± 7.42 0 41 ± 34.29 Cerebral + myocardial perfusion (ACP) 37.26 ± 10.54 25 ± 6.56 40.5 ± 9.07 39 ± 9.90 Total Norwood (Group A) Arch (Group B) Arch+ (Group C) Patients enrolled (older than 1 month), n (%) 50 (12) 8 14 (6) 28 (6) Age (days) 49 ± 84.55 3 ± 1.07 68 ± 90.82 53 ± 90.34 Weight (kg) 3.75 ± 1.62 3.17 ± 0.47 4.4 ± 2.10 3.6 ± 1.49 CPB (min) 161 ± 54.44 182 ± 37.35 133 ± 49.86 170 ± 56.74 Clamp (min) 31 ± 32.40 50.75 ± 7.42 0 41 ± 34.29 Cerebral + myocardial perfusion (ACP) 37.26 ± 10.54 25 ± 6.56 40.5 ± 9.07 39 ± 9.90 Data are presented as mean ± standard deviation. ACP: antegrade cerebral perfusion; Arch: isolated arch surgery; Arch+: arch plus intracardiac repair; Clamp: coronary ischaemia (aortic cross-clamp); CPB: cardiopulmonary bypass. Table 1: Patient distribution Total Norwood (Group A) Arch (Group B) Arch+ (Group C) Patients enrolled (older than 1 month), n (%) 50 (12) 8 14 (6) 28 (6) Age (days) 49 ± 84.55 3 ± 1.07 68 ± 90.82 53 ± 90.34 Weight (kg) 3.75 ± 1.62 3.17 ± 0.47 4.4 ± 2.10 3.6 ± 1.49 CPB (min) 161 ± 54.44 182 ± 37.35 133 ± 49.86 170 ± 56.74 Clamp (min) 31 ± 32.40 50.75 ± 7.42 0 41 ± 34.29 Cerebral + myocardial perfusion (ACP) 37.26 ± 10.54 25 ± 6.56 40.5 ± 9.07 39 ± 9.90 Total Norwood (Group A) Arch (Group B) Arch+ (Group C) Patients enrolled (older than 1 month), n (%) 50 (12) 8 14 (6) 28 (6) Age (days) 49 ± 84.55 3 ± 1.07 68 ± 90.82 53 ± 90.34 Weight (kg) 3.75 ± 1.62 3.17 ± 0.47 4.4 ± 2.10 3.6 ± 1.49 CPB (min) 161 ± 54.44 182 ± 37.35 133 ± 49.86 170 ± 56.74 Clamp (min) 31 ± 32.40 50.75 ± 7.42 0 41 ± 34.29 Cerebral + myocardial perfusion (ACP) 37.26 ± 10.54 25 ± 6.56 40.5 ± 9.07 39 ± 9.90 Data are presented as mean ± standard deviation. ACP: antegrade cerebral perfusion; Arch: isolated arch surgery; Arch+: arch plus intracardiac repair; Clamp: coronary ischaemia (aortic cross-clamp); CPB: cardiopulmonary bypass. A: Norwood (8 neonates). B: aortic arch (14 patients; 6 patients older than 1 month). C: arch plus intracardiac repair (28 patients; 6 patients were older than 1 month). Age, weight and operative data are listed in Table 1. Surgical technique ACP was delivered via the innominate artery with the interposition of a 3.5-mm graft with flow rates of 40–60 ml/kg/min, maintaining a mean arterial pressure (25–55 mmHg) appropriate for the age of the child. During ACP, the arch branches were controlled using tourniquets or fine bull-dog clamps, and the descending thoracic aorta was clamped. Cerebral and somatic near infra red spectroscopy (NIRS) monitored regional oxygenation. The ascending aorta was clamped as usual, whereas a line coming from the arterial return was connected in a ‘Y’ fashion to the aortic root. A 3-way stopcock (or alternatively locking the Y line) allowed either blood (arch repair) or cardioplegia (for intracardiac repair) to be flushed in the aortic root and repeated every 30–45 min, at the surgeon’s discretion (Fig. 1). Figure 1: View largeDownload slide Drawing shows either cardioplegia or blood delivery to the root. Line arrangement with a ‘Y’ connection between main arterial return and root cannula. Oxygenated blood or cardioplegia can be delivered into the aortic root by simply switching lockers. Figure 1: View largeDownload slide Drawing shows either cardioplegia or blood delivery to the root. Line arrangement with a ‘Y’ connection between main arterial return and root cannula. Oxygenated blood or cardioplegia can be delivered into the aortic root by simply switching lockers. For the Norwood procedure, a fine clamp was placed in the proximal arch (between innominate and left carotid arteries) so as to deliver blood flow to the innominate artery and ascending aorta simultaneously while performing the distal arch repair. On completion, the fine clamp was shifted to the base of the innominate artery, and a soft-tipped cardioplegia cannula was slipped down into the ascending aorta to arrest the heart. The arch repair (mostly coarctation plus hypoplastic arch) was accomplished as follows: resection of ductal tissue, end-to-end anastomosis of back wall of the arch and descending aorta, plus anterior augmentation with glutaraldehyde-treated autologous pericardium patch. In cases where a true ridge was not present, straightforward patch enlargement was performed (Videos 1 and 2). After fulfilling the aortic arch repair in a beating heart, the patient either weaned off CPB (Group B) or had his/her heart arrested for the Norwood completion (Group A) or intracardiac repair accomplished (Group C). In the same period of time, 36 neonates with an isolated aortic coarctation were operated through a left thoracotomy. Video 1 Isolated aortic arch by clamping the ascending aorta and the descending aorta, plus excluding the supra-aortic vessels with tourniquets. Arterial return through a graft and Y connected to the aortic root. The arch is longitudinally opened, while the heart is beating. Video 1 Isolated aortic arch by clamping the ascending aorta and the descending aorta, plus excluding the supra-aortic vessels with tourniquets. Arterial return through a graft and Y connected to the aortic root. The arch is longitudinally opened, while the heart is beating. Close Video 2 The same scenario, stitching the pericardial patch in the arch. Video 2 The same scenario, stitching the pericardial patch in the arch. Close Ethical/institutional approval for the modification and indication was obtained, and informed consent was obtained from the parents. Data are expressed as mean ± standard deviation and range (minimum, maximum). Given the number of patients and low incidence of complications, additional statistical analysis was not meaningful clinically or statistically. RESULTS For the whole cohort, mean CPB was 161 ± 54.44 (range 93–312) min, coronary ischaemia was 31 ± 32.40 (range 0–160) min and mean selective brain/heart perfusion was 37.26 ± 10.54 (range 18–36) min. Data pertaining to the 3 different groups are displayed in Table 1. A.  Norwood patients (8) with the mean age of 3 ± 1.07 (1–5) days and mean weight of 3.17 ± 0.46 (2.7–4) kg. Mean CPB was 182 ± 37.35 (124–227) min, mean coronary ischaemia was 50.75 ± 7.42 (42–62) min and mean selective brain/heart perfusion was 25 ± 6.56 (18–36) min. Norwood–Sano was adopted as a surgical strategy, with the ‘dunk’ modification in the proximal anastomosis with a ringed conduit in the last 2 patients. B.  ‘Isolated’ arch repair in 14 patients (8 neonates and 6 infants). Mean age was 68 ± 90.82 days (4 days to 11 months), and mean weight was 4.4 ± 2.10 (2.1–10.5) kg. Mean CPB was 133 ± 49.86 (93–292) min. Coronary ischaemia was 0, as they were operated on a beating-heart basis. Mean brain/heart perfusion was 40.5 ± 9.07 (26–60) min. One of the latest patient in our series (the oldest one, aged 11 months and 10.5 kg in weight) underwent a ‘total body perfusion’ technique [11], with a ‘Y’ arterial cannula providing blood to the innominate artery (and coronary arteries, simultaneously) on 1 arm and to the descending aorta on the other arm. C.  Aortic arch plus intracardiac procedures in 28 children (22 neonates and 6 infants). Mean age was 49 ± 84.55 days (1 day–11 months), and weight was 3.75 ± 1.62 (range 2.1–10.5) kg. Associated procedures are detailed in Table 2: Table 2: Intracardiac repair along with arch surgery (Group C) Associated defects to arch repair n (28) Ventricular septal defect 10 Arterial switch 5  Simple 1  With ventricular septal defect 1  Taussig–Bing 1  Palliative switch 2 Atrial septal defect (ostium secundum) 4 Cor triatriatum 3 Aortic valve commissurotomy 2 Comprehensive II (Norwood + Glenn) 2 Atrial septal defect (ostium primum) 1 Yasui (Norwood + Rastelli) 1 Associated defects to arch repair n (28) Ventricular septal defect 10 Arterial switch 5  Simple 1  With ventricular septal defect 1  Taussig–Bing 1  Palliative switch 2 Atrial septal defect (ostium secundum) 4 Cor triatriatum 3 Aortic valve commissurotomy 2 Comprehensive II (Norwood + Glenn) 2 Atrial septal defect (ostium primum) 1 Yasui (Norwood + Rastelli) 1 Table 2: Intracardiac repair along with arch surgery (Group C) Associated defects to arch repair n (28) Ventricular septal defect 10 Arterial switch 5  Simple 1  With ventricular septal defect 1  Taussig–Bing 1  Palliative switch 2 Atrial septal defect (ostium secundum) 4 Cor triatriatum 3 Aortic valve commissurotomy 2 Comprehensive II (Norwood + Glenn) 2 Atrial septal defect (ostium primum) 1 Yasui (Norwood + Rastelli) 1 Associated defects to arch repair n (28) Ventricular septal defect 10 Arterial switch 5  Simple 1  With ventricular septal defect 1  Taussig–Bing 1  Palliative switch 2 Atrial septal defect (ostium secundum) 4 Cor triatriatum 3 Aortic valve commissurotomy 2 Comprehensive II (Norwood + Glenn) 2 Atrial septal defect (ostium primum) 1 Yasui (Norwood + Rastelli) 1 Ventricular septal defect closure in 10 children (including 2 Type B interrupted aortic arch cases). Arterial switch operation (ASO) in 5 neonates: 1 single switch, 1 ASO plus ventricular septal defect, 1 Taussig–Bing, 2 palliative ASO. Ostium secundum atrial septal defect closure in 4 patients Cor triatriatum in 3 children. Aortic valve commissurotomy in 2 neonates. Comprehensive II procedure (Norwood plus Glenn) in 2 infants. Ostium primum atrial septal defect repair in 1 patient. Yasui procedure (Norwood plus Rastelli) in 1 infant. Lactate levels at the end of the procedure were 5.4 (2.5–7.3) mmol/l. Delayed sternal closure was routine in all the Norwood patients (Group I), plus 2 patients in Group II and 3 patients in Group III, at the surgeon’s discretion. Neither neurological nor renal complications were recorded. Follow-up was complete for a mean of 30 (range 1–48) months. Four patients died in the postoperative period (Fig. 2): 2 Norwood due to overcirculation and low ejection fraction (Group A), 1 ‘isolated’ arch repair because of ventricular arrhythmia several days after repair (Group B) and 1 interrupted aortic arch on chylothorax and sepsis (Group C). Two patients (1 in Group B and 1 in Group C) underwent angioplasty for recoarctation within the first 6 months (gradient <20 mmHg on discharge). Figure 2: View largeDownload slide The Kaplan–Meier curves of survival and recoarctation freedom. Figure 2: View largeDownload slide The Kaplan–Meier curves of survival and recoarctation freedom. DISCUSSION The choice of performing aortic arch repair under deep hypothermia plus circulatory arrest or ACP is a matter of debate nowadays, with enthusiastic followers and detractors of both strategies [12, 13]. Good results with a classical technique prevent one to change, whereas innovation showing achievements pushes others to pursue evolving strategies. This literature provides strong evidence [1, 2, 4, 5, 14–16] of the accuracy and security of ACP for brain protection when performing aortic arch repair, providing even blood flow to both hemispheres. Tepid temperature [7, 17, 18] adjustment spares the drawbacks of deep hypothermia as reported. Relying on collateral circulation, at moderate or deep hypothermia, has shown adequate lower body protection [19], whereas some authors do not agree [20], and others suggest a ‘total body perfusion’ strategy [11]. In fact, regular coarctation repair through left thoracotomy is performed under normothermia with negligible side effects when ischaemia is no longer than 20 min. Simultaneous brain and heart perfusion provided by the same arterial line in a ‘Y’ fashion by connecting the Luer lock and the root cannula is feasible and easy to monitor. Bradycardia follows the drop in temperature, and no changes in the ECG are to be expected. If this occurs, subtle kinking in the root line must be checked. Any inconvenience can be overcome by switching the aortic root perfusion from arterial blood to cardioplegia, which is the next step to follow for the completion of the intracardiac repair. Our cohort of patients is divided into 3 groups to cluster them in categories alike (Table 1). As expected, CPB time is shorter in Group B, because only arch repair is performed under 0 coronary ischaemia (true beating-heart procedure; Videos 1 and 2). When comparing selective brain/heart perfusion CPB time, Groups B and C are similar (40.5 vs 39 min) but slightly longer than in Group A (the Norwood patients have the distal part or the arch repaired on a beating-heart basis only). Regarding Group B, one could speculate about the accuracy of approaching aortic coarctation plus hypoplastic arch through left thoracotomy versus midline sternotomy. In the same lapse of time, 36 neonates were operated by a left-side approach, whereas 14 children in this report were approached through sternotomy on CPB. Results are good in both groups, showing an appropriate indication. As the authors became more confident with the technique and strategy, more demanding procedures were included in our current practice. Thus, according to the severity of the intracardiac condition in Group C patients, ‘simple’ defects (ventricular septal defect, atrial septal defect, cor triatriatum and aortic commissurotomy) are distinguished from ‘complex’ repairs (switch, comprehensive procedures and Yasui). At the end, once the surgeon becomes proficient with the strategy, the aortic arch repair is the same, irrespective of the underlying intracardiac condition (Table 2). Even re-do procedures after a hybrid approach (ductal stent plus bilateral banding) have been attempted: 2 comprehensive procedures (Norwood plus Glenn) and 1 Yasui (Norwood plus Rastelli). The background and experience accumulated prompted us to give a step forward. After brain and heart protection, descending aorta cannulation to achieve ‘total body perfusion’ as described by Cesnjevar et al. [11] group in Erlangen, Germany, was attempted in one of the latest patients in our series (the oldest one, aged 11 months and 10.5 kg in weight). To make things simple, a ‘Y’ line was arranged for arterial return (so as for an interrupted arch repair) with an arm connected to the arterial duct for descending aorta perfusion, and the other arm was connected to a graft in the innominate artery (and the aortic root in the aforementioned strategy). Flow metre in both arms guaranteed appropriate flow, avoiding either hypoperfusion or hyperperfusion to the brain. Upon reaching the target temperature of 25°C, the side arm in the duct was switched to the descending aorta in a previously fashioned purse-string suture through the pericardium well (as described by the German group [11]). We intend to spread the technique to younger infants and neonates in the near future. CONCLUSIONS Our initial experience with the first 50 patients enables us to conclude that selective brain–coronary perfusion in aortic arch surgery is feasible and easy to switch to conventional cardioplegia delivery at tepid temperatures. Coronary ischaemia can be notably reduced (particularly appealing for long, complex cases), being even 0 min in isolated arch surgery. The set-up in neonates and infants is reproducible, with little changes in the usual arrangements for anaesthesiologists, perfusionists and surgeons. No drawbacks have been registered in our cohort of patients. Novel strategies, as total body perfusion allowing blood to the descending aorta, are welcome and ready to be implemented in our armamentarium. ACKNOWLEDGEMENTS The authors thank Gregorio P. Cuerpo for his statistical analysis and Rafael Cid for his drawings. Conflict of interest: none declared. 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Deep hypothermic circulatory arrest and antegrade selective cerebral perfusion during ascending aorta-hemiarch replacement: a retrospective comparative study . J Thorac Cardiovasc Surg 2003 ; 125 : 849 – 54 . Google Scholar CrossRef Search ADS PubMed 5 Goldberg CS , Bove EL , Devaney EJ , Mollen E , Schwartz E , Tindall S et al. A randomized clinical trial of regional cerebral perfusion versus deep hypothermic circulatory arrest: outcomes for infants with functional single ventricle . J Thorac Cardiovasc Surg 2007 ; 133 : 880 – 7 . Google Scholar CrossRef Search ADS PubMed 6 Algra SO , Jansen NJ , van der Tweel I , Schouten AN , Groenendaal F , Toet M et al. Neurological injury after neonatal cardiac surgery: a randomized, controlled trial of 2 perfusion techniques . Circulation 2014 ; 129 : 224 – 33 . Google Scholar CrossRef Search ADS PubMed 7 Salazar JD , Coleman R , Griffith S , McNeil J , Young H , Calhoon J et al. Brain preservation with selective cerebral perfusion for operations requiring circulatory arrest: protection at 25°C is similar to 18°C with shorter operating times . Eur J Cardiothorac Surg 2009 ; 36 : 524 – 31 . Google Scholar CrossRef Search ADS PubMed 8 Andropoulos DB , Stayer SA , McKenzie ED , Fraser CD. Regional low-flow perfusion provides comparable blood flow and oxygenation to both cerebral hemispheres during neonatal aortic arch reconstruction . J Thorac Cardiovasc Surg 2003 ; 126 : 1712 – 7 . Google Scholar CrossRef Search ADS PubMed 9 Rüffer A , Tischer P , Münch F , Purbojo A , Toka O , Rascher W et al. Comparable cerebral blood flow in both hemispheres during regional cerebral perfusion in infant aortic arch surgery . Ann Thorac Surg 2017 ; 103 : 178 – 85 . Google Scholar CrossRef Search ADS PubMed 10 Sano S , Mee RB. Isolated myocardial perfusion during arch repair . Ann Thorac Surg 1990 ; 49 : 970 – 2 . 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Published by Oxford University Press on behalf of the European Association for Cardio-Thoracic Surgery. All rights reserved. This article is published and distributed under the terms of the Oxford University Press, Standard Journals Publication Model (https://academic.oup.com/journals/pages/about_us/legal/notices) http://www.deepdyve.com/assets/images/DeepDyve-Logo-lg.png Interactive CardioVascular and Thoracic Surgery Oxford University Press

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Abstract

Abstract OBJECTIVES Aortic arch repair has been shifted from deep hypothermia plus circulatory arrest to cerebral perfusion at tepid temperatures. A step forward is a simultaneous brain–coronary perfusion, allowing beating-heart arch surgery. METHODS A ‘Y’ cannula from the arterial line delivers oxygenated blood to brain and heart. The arch is repaired on a beating heart at 25°C. Intracardiac repair is performed after running cardioplegia through the root line. Fifty patients are classified into 3 groups: A, Norwood (8 neonates); B, aortic arch (14 children) and C, aortic arch plus intracardiac repair (28 patients). Associated anomalies in Group C are as follows: ventricular septal defect (10), arterial switch (5), atrial septal defect (4), cor triatriatum (3), aortic commissurotomy (2), comprehensive repair (2), ostium primum (1) and Yasui (1). RESULTS The mean bypass time was 161 ± 54.44 (range 93–312) min. Mean brain–coronary perfusion was 37.26 ± 10.54 (18–60) min. Mean coronary ischaemia was 31 ± 32.40 (0–160) min. The heart was not arrested in Group B patients. Follow-up was complete for a mean of 30 (1–48) months. Four patients died in the postoperative period. Two required angioplasty for recoarctation. CONCLUSIONS Selective brain–coronary perfusion is feasible and easy to switch to conventional cardioplegia delivery. Coronary ischaemia can be notably reduced and even 0 min in isolated arch surgery. Aortic arch, Beating heart, Hypothermia, Brain, Coronary, Perfusion INTRODUCTION Deep hypothermia plus circulatory arrest was the method of choice for aortic arch surgery some decades ago [1, 2] (and still the strategy in many well-known centres), relying on the reduction in metabolism and oxygen requirements for brain protection. Complex neonatal arch surgery with long periods of circulatory arrest was associated with seizures and choreoathetosis, as well as a likelihood of future neurological impairment. Antegrade cerebral perfusion (ACP) emerged as an adjunct to brain protection [3] by providing blood flow from the arterial line through the innominate artery. Somatic flow is expected to be guaranteed by collateral circulation at low temperature. Several reports compared both perfusion strategies [4–6], deep hypothermia plus circulatory arrest vs ACP, with regard to neurological outcomes and brain preservation at different temperatures [7]. On gaining experience, some papers elucidated the balanced circulation between both hemispheres [8, 9] to provide adequate brain perfusion by a single carotid (and vertebral) artery. A step forward in protection was to deliver flow to the coronary arteries while performing arch surgery [10]. This way, one could accomplish arch repair under tepid temperature [7] on a ‘beating-heart’ basis. We will describe the perioperative impact of ACP and selective myocardial perfusion at 25°C in newborns and infants undergoing arch reconstructions below. Herein, we report our experience and outcomes. METHODS Between January 2013 and December 2017, 50 patients (38 neonates and 12 infants) were prospectively recorded. Complex arch surgery on cardiopulmonary bypass (CPB) and moderate hypothermia (25°C) was performed, with ACP (50 ml/kg/min) via the innominate artery. The mean age was 49 ± 84.55 days (range 1 day to 11 months). Mean weight was 3.75 ± 1.62 (range 2.1–10.5) kg. Patients were classified into 3 main groups (Table 1): Table 1: Patient distribution Total Norwood (Group A) Arch (Group B) Arch+ (Group C) Patients enrolled (older than 1 month), n (%) 50 (12) 8 14 (6) 28 (6) Age (days) 49 ± 84.55 3 ± 1.07 68 ± 90.82 53 ± 90.34 Weight (kg) 3.75 ± 1.62 3.17 ± 0.47 4.4 ± 2.10 3.6 ± 1.49 CPB (min) 161 ± 54.44 182 ± 37.35 133 ± 49.86 170 ± 56.74 Clamp (min) 31 ± 32.40 50.75 ± 7.42 0 41 ± 34.29 Cerebral + myocardial perfusion (ACP) 37.26 ± 10.54 25 ± 6.56 40.5 ± 9.07 39 ± 9.90 Total Norwood (Group A) Arch (Group B) Arch+ (Group C) Patients enrolled (older than 1 month), n (%) 50 (12) 8 14 (6) 28 (6) Age (days) 49 ± 84.55 3 ± 1.07 68 ± 90.82 53 ± 90.34 Weight (kg) 3.75 ± 1.62 3.17 ± 0.47 4.4 ± 2.10 3.6 ± 1.49 CPB (min) 161 ± 54.44 182 ± 37.35 133 ± 49.86 170 ± 56.74 Clamp (min) 31 ± 32.40 50.75 ± 7.42 0 41 ± 34.29 Cerebral + myocardial perfusion (ACP) 37.26 ± 10.54 25 ± 6.56 40.5 ± 9.07 39 ± 9.90 Data are presented as mean ± standard deviation. ACP: antegrade cerebral perfusion; Arch: isolated arch surgery; Arch+: arch plus intracardiac repair; Clamp: coronary ischaemia (aortic cross-clamp); CPB: cardiopulmonary bypass. Table 1: Patient distribution Total Norwood (Group A) Arch (Group B) Arch+ (Group C) Patients enrolled (older than 1 month), n (%) 50 (12) 8 14 (6) 28 (6) Age (days) 49 ± 84.55 3 ± 1.07 68 ± 90.82 53 ± 90.34 Weight (kg) 3.75 ± 1.62 3.17 ± 0.47 4.4 ± 2.10 3.6 ± 1.49 CPB (min) 161 ± 54.44 182 ± 37.35 133 ± 49.86 170 ± 56.74 Clamp (min) 31 ± 32.40 50.75 ± 7.42 0 41 ± 34.29 Cerebral + myocardial perfusion (ACP) 37.26 ± 10.54 25 ± 6.56 40.5 ± 9.07 39 ± 9.90 Total Norwood (Group A) Arch (Group B) Arch+ (Group C) Patients enrolled (older than 1 month), n (%) 50 (12) 8 14 (6) 28 (6) Age (days) 49 ± 84.55 3 ± 1.07 68 ± 90.82 53 ± 90.34 Weight (kg) 3.75 ± 1.62 3.17 ± 0.47 4.4 ± 2.10 3.6 ± 1.49 CPB (min) 161 ± 54.44 182 ± 37.35 133 ± 49.86 170 ± 56.74 Clamp (min) 31 ± 32.40 50.75 ± 7.42 0 41 ± 34.29 Cerebral + myocardial perfusion (ACP) 37.26 ± 10.54 25 ± 6.56 40.5 ± 9.07 39 ± 9.90 Data are presented as mean ± standard deviation. ACP: antegrade cerebral perfusion; Arch: isolated arch surgery; Arch+: arch plus intracardiac repair; Clamp: coronary ischaemia (aortic cross-clamp); CPB: cardiopulmonary bypass. A: Norwood (8 neonates). B: aortic arch (14 patients; 6 patients older than 1 month). C: arch plus intracardiac repair (28 patients; 6 patients were older than 1 month). Age, weight and operative data are listed in Table 1. Surgical technique ACP was delivered via the innominate artery with the interposition of a 3.5-mm graft with flow rates of 40–60 ml/kg/min, maintaining a mean arterial pressure (25–55 mmHg) appropriate for the age of the child. During ACP, the arch branches were controlled using tourniquets or fine bull-dog clamps, and the descending thoracic aorta was clamped. Cerebral and somatic near infra red spectroscopy (NIRS) monitored regional oxygenation. The ascending aorta was clamped as usual, whereas a line coming from the arterial return was connected in a ‘Y’ fashion to the aortic root. A 3-way stopcock (or alternatively locking the Y line) allowed either blood (arch repair) or cardioplegia (for intracardiac repair) to be flushed in the aortic root and repeated every 30–45 min, at the surgeon’s discretion (Fig. 1). Figure 1: View largeDownload slide Drawing shows either cardioplegia or blood delivery to the root. Line arrangement with a ‘Y’ connection between main arterial return and root cannula. Oxygenated blood or cardioplegia can be delivered into the aortic root by simply switching lockers. Figure 1: View largeDownload slide Drawing shows either cardioplegia or blood delivery to the root. Line arrangement with a ‘Y’ connection between main arterial return and root cannula. Oxygenated blood or cardioplegia can be delivered into the aortic root by simply switching lockers. For the Norwood procedure, a fine clamp was placed in the proximal arch (between innominate and left carotid arteries) so as to deliver blood flow to the innominate artery and ascending aorta simultaneously while performing the distal arch repair. On completion, the fine clamp was shifted to the base of the innominate artery, and a soft-tipped cardioplegia cannula was slipped down into the ascending aorta to arrest the heart. The arch repair (mostly coarctation plus hypoplastic arch) was accomplished as follows: resection of ductal tissue, end-to-end anastomosis of back wall of the arch and descending aorta, plus anterior augmentation with glutaraldehyde-treated autologous pericardium patch. In cases where a true ridge was not present, straightforward patch enlargement was performed (Videos 1 and 2). After fulfilling the aortic arch repair in a beating heart, the patient either weaned off CPB (Group B) or had his/her heart arrested for the Norwood completion (Group A) or intracardiac repair accomplished (Group C). In the same period of time, 36 neonates with an isolated aortic coarctation were operated through a left thoracotomy. Video 1 Isolated aortic arch by clamping the ascending aorta and the descending aorta, plus excluding the supra-aortic vessels with tourniquets. Arterial return through a graft and Y connected to the aortic root. The arch is longitudinally opened, while the heart is beating. Video 1 Isolated aortic arch by clamping the ascending aorta and the descending aorta, plus excluding the supra-aortic vessels with tourniquets. Arterial return through a graft and Y connected to the aortic root. The arch is longitudinally opened, while the heart is beating. Close Video 2 The same scenario, stitching the pericardial patch in the arch. Video 2 The same scenario, stitching the pericardial patch in the arch. Close Ethical/institutional approval for the modification and indication was obtained, and informed consent was obtained from the parents. Data are expressed as mean ± standard deviation and range (minimum, maximum). Given the number of patients and low incidence of complications, additional statistical analysis was not meaningful clinically or statistically. RESULTS For the whole cohort, mean CPB was 161 ± 54.44 (range 93–312) min, coronary ischaemia was 31 ± 32.40 (range 0–160) min and mean selective brain/heart perfusion was 37.26 ± 10.54 (range 18–36) min. Data pertaining to the 3 different groups are displayed in Table 1. A.  Norwood patients (8) with the mean age of 3 ± 1.07 (1–5) days and mean weight of 3.17 ± 0.46 (2.7–4) kg. Mean CPB was 182 ± 37.35 (124–227) min, mean coronary ischaemia was 50.75 ± 7.42 (42–62) min and mean selective brain/heart perfusion was 25 ± 6.56 (18–36) min. Norwood–Sano was adopted as a surgical strategy, with the ‘dunk’ modification in the proximal anastomosis with a ringed conduit in the last 2 patients. B.  ‘Isolated’ arch repair in 14 patients (8 neonates and 6 infants). Mean age was 68 ± 90.82 days (4 days to 11 months), and mean weight was 4.4 ± 2.10 (2.1–10.5) kg. Mean CPB was 133 ± 49.86 (93–292) min. Coronary ischaemia was 0, as they were operated on a beating-heart basis. Mean brain/heart perfusion was 40.5 ± 9.07 (26–60) min. One of the latest patient in our series (the oldest one, aged 11 months and 10.5 kg in weight) underwent a ‘total body perfusion’ technique [11], with a ‘Y’ arterial cannula providing blood to the innominate artery (and coronary arteries, simultaneously) on 1 arm and to the descending aorta on the other arm. C.  Aortic arch plus intracardiac procedures in 28 children (22 neonates and 6 infants). Mean age was 49 ± 84.55 days (1 day–11 months), and weight was 3.75 ± 1.62 (range 2.1–10.5) kg. Associated procedures are detailed in Table 2: Table 2: Intracardiac repair along with arch surgery (Group C) Associated defects to arch repair n (28) Ventricular septal defect 10 Arterial switch 5  Simple 1  With ventricular septal defect 1  Taussig–Bing 1  Palliative switch 2 Atrial septal defect (ostium secundum) 4 Cor triatriatum 3 Aortic valve commissurotomy 2 Comprehensive II (Norwood + Glenn) 2 Atrial septal defect (ostium primum) 1 Yasui (Norwood + Rastelli) 1 Associated defects to arch repair n (28) Ventricular septal defect 10 Arterial switch 5  Simple 1  With ventricular septal defect 1  Taussig–Bing 1  Palliative switch 2 Atrial septal defect (ostium secundum) 4 Cor triatriatum 3 Aortic valve commissurotomy 2 Comprehensive II (Norwood + Glenn) 2 Atrial septal defect (ostium primum) 1 Yasui (Norwood + Rastelli) 1 Table 2: Intracardiac repair along with arch surgery (Group C) Associated defects to arch repair n (28) Ventricular septal defect 10 Arterial switch 5  Simple 1  With ventricular septal defect 1  Taussig–Bing 1  Palliative switch 2 Atrial septal defect (ostium secundum) 4 Cor triatriatum 3 Aortic valve commissurotomy 2 Comprehensive II (Norwood + Glenn) 2 Atrial septal defect (ostium primum) 1 Yasui (Norwood + Rastelli) 1 Associated defects to arch repair n (28) Ventricular septal defect 10 Arterial switch 5  Simple 1  With ventricular septal defect 1  Taussig–Bing 1  Palliative switch 2 Atrial septal defect (ostium secundum) 4 Cor triatriatum 3 Aortic valve commissurotomy 2 Comprehensive II (Norwood + Glenn) 2 Atrial septal defect (ostium primum) 1 Yasui (Norwood + Rastelli) 1 Ventricular septal defect closure in 10 children (including 2 Type B interrupted aortic arch cases). Arterial switch operation (ASO) in 5 neonates: 1 single switch, 1 ASO plus ventricular septal defect, 1 Taussig–Bing, 2 palliative ASO. Ostium secundum atrial septal defect closure in 4 patients Cor triatriatum in 3 children. Aortic valve commissurotomy in 2 neonates. Comprehensive II procedure (Norwood plus Glenn) in 2 infants. Ostium primum atrial septal defect repair in 1 patient. Yasui procedure (Norwood plus Rastelli) in 1 infant. Lactate levels at the end of the procedure were 5.4 (2.5–7.3) mmol/l. Delayed sternal closure was routine in all the Norwood patients (Group I), plus 2 patients in Group II and 3 patients in Group III, at the surgeon’s discretion. Neither neurological nor renal complications were recorded. Follow-up was complete for a mean of 30 (range 1–48) months. Four patients died in the postoperative period (Fig. 2): 2 Norwood due to overcirculation and low ejection fraction (Group A), 1 ‘isolated’ arch repair because of ventricular arrhythmia several days after repair (Group B) and 1 interrupted aortic arch on chylothorax and sepsis (Group C). Two patients (1 in Group B and 1 in Group C) underwent angioplasty for recoarctation within the first 6 months (gradient <20 mmHg on discharge). Figure 2: View largeDownload slide The Kaplan–Meier curves of survival and recoarctation freedom. Figure 2: View largeDownload slide The Kaplan–Meier curves of survival and recoarctation freedom. DISCUSSION The choice of performing aortic arch repair under deep hypothermia plus circulatory arrest or ACP is a matter of debate nowadays, with enthusiastic followers and detractors of both strategies [12, 13]. Good results with a classical technique prevent one to change, whereas innovation showing achievements pushes others to pursue evolving strategies. This literature provides strong evidence [1, 2, 4, 5, 14–16] of the accuracy and security of ACP for brain protection when performing aortic arch repair, providing even blood flow to both hemispheres. Tepid temperature [7, 17, 18] adjustment spares the drawbacks of deep hypothermia as reported. Relying on collateral circulation, at moderate or deep hypothermia, has shown adequate lower body protection [19], whereas some authors do not agree [20], and others suggest a ‘total body perfusion’ strategy [11]. In fact, regular coarctation repair through left thoracotomy is performed under normothermia with negligible side effects when ischaemia is no longer than 20 min. Simultaneous brain and heart perfusion provided by the same arterial line in a ‘Y’ fashion by connecting the Luer lock and the root cannula is feasible and easy to monitor. Bradycardia follows the drop in temperature, and no changes in the ECG are to be expected. If this occurs, subtle kinking in the root line must be checked. Any inconvenience can be overcome by switching the aortic root perfusion from arterial blood to cardioplegia, which is the next step to follow for the completion of the intracardiac repair. Our cohort of patients is divided into 3 groups to cluster them in categories alike (Table 1). As expected, CPB time is shorter in Group B, because only arch repair is performed under 0 coronary ischaemia (true beating-heart procedure; Videos 1 and 2). When comparing selective brain/heart perfusion CPB time, Groups B and C are similar (40.5 vs 39 min) but slightly longer than in Group A (the Norwood patients have the distal part or the arch repaired on a beating-heart basis only). Regarding Group B, one could speculate about the accuracy of approaching aortic coarctation plus hypoplastic arch through left thoracotomy versus midline sternotomy. In the same lapse of time, 36 neonates were operated by a left-side approach, whereas 14 children in this report were approached through sternotomy on CPB. Results are good in both groups, showing an appropriate indication. As the authors became more confident with the technique and strategy, more demanding procedures were included in our current practice. Thus, according to the severity of the intracardiac condition in Group C patients, ‘simple’ defects (ventricular septal defect, atrial septal defect, cor triatriatum and aortic commissurotomy) are distinguished from ‘complex’ repairs (switch, comprehensive procedures and Yasui). At the end, once the surgeon becomes proficient with the strategy, the aortic arch repair is the same, irrespective of the underlying intracardiac condition (Table 2). Even re-do procedures after a hybrid approach (ductal stent plus bilateral banding) have been attempted: 2 comprehensive procedures (Norwood plus Glenn) and 1 Yasui (Norwood plus Rastelli). The background and experience accumulated prompted us to give a step forward. After brain and heart protection, descending aorta cannulation to achieve ‘total body perfusion’ as described by Cesnjevar et al. [11] group in Erlangen, Germany, was attempted in one of the latest patients in our series (the oldest one, aged 11 months and 10.5 kg in weight). To make things simple, a ‘Y’ line was arranged for arterial return (so as for an interrupted arch repair) with an arm connected to the arterial duct for descending aorta perfusion, and the other arm was connected to a graft in the innominate artery (and the aortic root in the aforementioned strategy). Flow metre in both arms guaranteed appropriate flow, avoiding either hypoperfusion or hyperperfusion to the brain. Upon reaching the target temperature of 25°C, the side arm in the duct was switched to the descending aorta in a previously fashioned purse-string suture through the pericardium well (as described by the German group [11]). We intend to spread the technique to younger infants and neonates in the near future. CONCLUSIONS Our initial experience with the first 50 patients enables us to conclude that selective brain–coronary perfusion in aortic arch surgery is feasible and easy to switch to conventional cardioplegia delivery at tepid temperatures. Coronary ischaemia can be notably reduced (particularly appealing for long, complex cases), being even 0 min in isolated arch surgery. The set-up in neonates and infants is reproducible, with little changes in the usual arrangements for anaesthesiologists, perfusionists and surgeons. No drawbacks have been registered in our cohort of patients. Novel strategies, as total body perfusion allowing blood to the descending aorta, are welcome and ready to be implemented in our armamentarium. ACKNOWLEDGEMENTS The authors thank Gregorio P. Cuerpo for his statistical analysis and Rafael Cid for his drawings. Conflict of interest: none declared. 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Published by Oxford University Press on behalf of the European Association for Cardio-Thoracic Surgery. All rights reserved. This article is published and distributed under the terms of the Oxford University Press, Standard Journals Publication Model (https://academic.oup.com/journals/pages/about_us/legal/notices)

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Interactive CardioVascular and Thoracic SurgeryOxford University Press

Published: Apr 2, 2018

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