Tutorial
Single-stage repair of Taussig-Bing anomaly and interrupted aortic arch-type A
The surgical repair of Taussig-Bing anomaly and associated lesions has evolved over the years from palliative procedures to complete repairs - either in two stages or in one single stage. We present a video illustrating our preferred surgical option in the treatment of Taussig-Bing anomaly, in this case, with an associated type A interrupted aortic arch.
A two-day old neonate was admitted to our centre from the local hospital following planned induction and delivery by emergency caesarean section. The child had been diagnosed prenatally as having a Double outlet right ventricle with sub-pulmonary VSD (Taussig-Bing anomaly) and a type A interrupted aortic arch.
Preoperative echocardiogram confirmed the diagnosis; showing mal-posed (side-by-side) great vessels, with a small aorta to the right and slightly posterior to a larger main pulmonary artery. The aortic valve measured 6.2mm (z = -1.73), whereas the pulmonary valve annulus measured 11mm (z = +1.31). The right and left pulmonary arteries were 5mm (z= +0.1) and 6mm (z = +1.5) respectively. The VSD was sub-pulmonary and measured about 7mm. There was also a small ASD, shunting left-to-right. The aortic interruption was distal to the left subclavian artery (type A) and there was a large patent arterial duct (PDA) with bi-directional flow. Both ventricles were functioning well.
Clinically, the child was stable, on 5ng/kg/min of prostaglandin E, with pre-and post-ductal oxygen saturations of 85 and 95 per cent respectively.
Following routine pre-operative work-up, the child was taken to the operating theatre on day 7 of life for a single stage complete repair. The weight at surgery was 3.1kg.
1 - Pre-operative echocardiogram (0:00)
The aorta and the main pulmonary artery were side-by-side and there was a sub-pulmonary ventricular septal defect.
2 - Institution of cardiopulmonary bypass (0:29)
Note the use of a Gore-tex tube extension for cannulation in order to minimise trauma to the small brachiocephalic artery. Surgical approach was via a midline sternotomy. Cardiopulmonary bypass was established via two arterial cannulas and a single venous cannula in the right atrium. One of the arterial cannulas (8Fr) was inserted into a 3.5mm goretex tube sutured unto the brachiocephalic artery, whereas the second cannula (6Fr) was directly inserted into the large PDA, to ensure lower body perfusion. Whilst the patient was cooled in readiness for circulatory arrest, the head vessels, aortic arch and the descending aorta were dissected out.
3 - Repair of aortic arch interruption (1:06)
A left subclavian artery flap was incorporated into the anastomosis to bridge the gap to the descending aorta following ductal tissue resection.
At a temperature of 18 degrees Celsius, the ductal cannula was removed; the duct was doubly ligated and divided. The descending aorta was further mobilised. Cold blood cardioplegia (St Thomas’ cardiopelgia solution, Harefield Hospital formulation (84mmol/l K+) was given at an initial dose of 30ml/kg. Subsequent doses of 15ml/kg were given at 20 minute intervals. The blood to crystalloid ratio was 4:1. The head vessels were snared and circulation arrested. The ascending aorta and the main pulmonary artery were both transected and the descending aorta was secured with a clamp. The remaining ductal tissue was completely resected from the descending aorta.
An incision was made in the distal ascending aorta running into the arch. The left subclavian artery was ligated and divided. The arch incision was extended into the proximal stump of the left subclavian artery, thus creating a flap, which was then anastomosed to the back wall of the descending aorta. The use of a subclavian flap extension was necessary due to the wide gap between the arch and the descending aorta. The entire under-surface of the arch was then reconstructed with a patch of pulmonary artery homograft, also using 7-0 polypropylene suture (Video 3).
4 - Closure of septal defects (4:03)
The VSD was easily accessed through the large pulmonary valve.
Following arch reconstruction, the head vessels were un-snared and circulation recommenced at low flow. The right atrium was incised and the ASD was closed directly. The VSD was closed with a bovine pericardial patch via the left sided pulmonary valve. Full bypass flow was then re-established.
5 - Arterial switch procedure (5:29)
The Lecompte manoeuvre was not necessary in this case due to the position of the great arteries.
Coronary artery buttons were carefully excised from the aortic wall (in this case, the right coronary and the left anterior descending arteries from sinus1, and the circumflex artery from sinus2 – 1LAD,RCA, 2Cx) and transferred into corresponding sinuses in the main pulmonary artery (neo-aorta) using trap-door incisions. The neo-ascending aorta was then anastomosed to the distal aorta. The Lecompte manoeuvre was not necessary here as the aorta and the main pulmonary artery lay side-by-side. The neo-pulmonary trunk was reconstructed with a patch of autologous pericardium and then anastomosed to the distal pulmonary bifurcation in an end-to-end fashion, without moving the bifurcation to the right.
6 - Weaning off cardiopulmonary bypass (9:56)
A left atrial pressure monitoring line was placed, temporary pacing wires were attached, patient was fully rewarmed and cardiopulmonary bypass was discontinued. Modified ultra-filtration was carried out. The effect of heparin was reversed with protamine, cannulas were removed and the chest was closed over two drains.
7 - Echocardiographic findings at late follow-up (10:13)
The immediate postoperative course was uneventful, with the pre-discharge echocardiogram showing no residual intra-cardiac shunts, no left ventricular outflow tract obstruction and laminar flow across the reconstructed aortic arch. There was however mild pulmonary stenosis (V= 3m/s) owing to the relatively small native aorta (neo-pulmonary artery). Patient was discharged home a couple of weeks after surgery in a very good clinical state.
Subsequent follow-ups revealed a gradual increase in gradient across the neo-pulmonary artery, rising to a maximum velocity of 6m/s two years after the initial surgery. Echocardiogram also showed a hypertrophied and dilated right ventricle, with moderate tricuspid regurgitation. The neo-pulmonary valve was hypoplastic (z=-4) and the branch pulmonary arteries were smallish, with the RPA measuring 6.5mm (z= -2) and the LPA 6.9mm (z= -1.4).8 - Echocardiographic findings after re-operation (10:31)
In view of these findings, a repeat surgery was carried out to address the RV outflow tract obstruction. The procedure entailed resection of the hypertrophied RVOT muscle bundles, incision of the neo-pulmonary valve commissures and construction of an RV-to-PA conduit using an 8mm PTFE (Gore-tex) tube to augment pulmonary blood flow. The latter procedure was necessary owing to the coronary anatomy, with the right coronary artery crossing the RV outflow tract. The immediate postoperative result was very good, with no residual RVOT obstruction on echocardiogram and a directly measured RV pressure of about 60% systemic.
In the period between January 2003 and December 2012, a total of 21 patients underwent a single stage repair of Taussig-Bing complex and associated anomalies. 13 (62%) patients had some degree of left heart obstruction comprising 3 (14%) cases of interrupted aortic arch, 6 (29%) cases of hypoplastic aortic arch and 4 (19%) cases of discrete coarctation of the aorta. The mean age at surgery was 15.5days (range: 2 to 206 days); with a mean weight of 3.49kg (range: 2.7 to 5.2 kg)
In all of the cases, the surgical procedure involved arterial switch operation, closure of the sub-pulmonary VSD and repair of any associated left heart lesion. There were two early and one late postoperative deaths, accounting for an overall survival of 86%. The two early deaths were in patients with complex coronary anatomy, who required ECMO due to inability to wean from cardiopulmonary bypass. The late death was due to complications relating to rhythm disturbance and poor myocardial function many weeks after discharge from hospital.
8 (38%) patients had some form of re-intervention over a follow-up period lasting between 3 and 13 years. 3 patients had balloon dilatation of recurrent aortic coarctation, whereas 1 patient had stenting of the aorta for the same reason. Relief of neo-RVOT obstruction and construction of additional RV-to-PA conduit (double barrel RVOT) was carried out in two patients. In both cases, one of the main coronary arteries was in close proximity to the RVOT.
The overall freedom from re-intervention was 94% at 3 years, 81% at 5 years and 61% at 10 years.
One-stage repair is increasingly becoming the surgical approach of choice in the treatment of Double outlet right ventricle with sub-pulmonary VSD (Taussig-Bing) and associated anomalies. Where correctly applied, the advantage of this approach lies not only in avoiding the risks associated with multiple surgical interventions, but also in providing these little patients with the right haemodynamic substrate for adequate development early in life.
Early and mid-term results of one-stage repair of Taussig-Bing anomaly has greatly improved over the years. In their recent study of patients undergoing arterial switch operation for Taussig-Bing anomaly, Schwarz et al (1) reported a survival rate of 88% over a follow-up period of 15 years. In a similar study involving 43 patients over a period of 21 years, Hayes et al (2) reported a7% in-hospital mortality and a 2% late mortality following total correction of Taussig-Bing anomaly. The above mentioned results are not at variance with our findings from a review of 21 patients. We recorded an early survival of 91% and an overall survival of 86% over a13-year follow-up period. The risk factors most commonly linked with early mortality in patients with Taussig-Bing were unusual coronary anatomy (3) and severe associated anomalies (4). However, a number of recent studies have not identified these as significant risk factors for death (1,2,5) It is worth pointing out though, that arrhythmias are a relatively common cause of sudden death in the early postoperative period in some patients after repair of Taussig-Bing anomaly (1,2) and these may not be entirely unrelated to the coronary anatomy.
With improving surgical techniques, the number of reported cases of re-intervention has fallen over the years. In our series we recorded an overall freedom from re-intervention of 94% at 3 years and 81% at 5 years. Most of the re-interventions were balloon dilatation or stenting of recurrent coarctation or pulmonary stenosis. Surgical reconstruction of RVOT following initial one-stage repair was necessary in two of our patients. Unsurprisingly these patients had significant pre-operative risk factors for development of late RVOTO such as size mismatch between the native pulmonary artery and the aorta, presence of sub-aortic RVOTO and side-by-side position of the great vessels (6). It has been suggested that vigorous resection of sub-aortic RVOTO at the time of initial surgery significantly reduces the incidence of late RVOT problems and the subsequent need for re-interventions (5,6,7). However, such extensive resection is not always feasible, as witnessed in two of our patients who had major coronary arteries crossing their RVOT. In these patients, it became necessary to augment pulmonary blood flow by inserting an RV-to-PA conduit in addition to the native outflow tract, thus creating a double-barrel outflow tract.
Compared to the RVOT, the incidence of LVOT obstruction following repair of Taussig-Bing anomaly is lower. Griselli et al (3) reported a 90% freedom from recurrent sub-aortic stenosis and coarctation as opposed to 80% freedom from recurrent RVOTO at 10 years of follow-up. There were no cases of sub-aortic stenosis in our series; however we recorded 4 cases of recurrent coarctation requiring either balloon dilation or stenting of the arch.
Approach to closure of the sub-pulmonary VSD has also varied. Schwarz et al (1) described their preference for a trans-atrial approach. This, in their opinion, enables a more extensive resection of the conal septum and provides better access to the anterior-lateral rim of the VSD. We adopt a more conventional approach; hence our decision is based on the intra-operative findings and does not exclude the trans-pulmonary approach, as shown in the video.
In conclusion, much progress has been made on the surgical repair of Taussig-Bing anomaly, with a shift in preference in favour of single-stage repair. Results of this approach are quite encouraging with low mortality and lower incidence of major re-operations. However, a multi-centre study of this group of patients over a longer period of time will shed more light on the efficacy of this surgical approach.
- Schwarz F, Blaschczok HC, Sinzobahamvya N, Sata S, Korn F, Weber A et al. The Taussig-Bing anomaly: long term results. Eur J Cardiothorac 2013;44:821–827.
PubMed Abstract | Publisher Full Text - Hayes D, Jones S, Quaegebeur J, Richmond ME, Andrews HF, Glickstein JS et al. Primary arterial switch operation as a strategy for total correction of Taussig-Bing anomaly. Circulation 2013;128(suppl 1):194–198.
PubMed Abstract | Publisher Full Text - Griselli M, McGuirk SP, Ko CS, Clarke AJ, Barron DJ, Brawn WJ. Arterial switch operation in patients with Taussig-Bing anomaly–influence of staged repair and coronary anatomy on outcome. Eur J Cardiothorac Surg 2007;31:229–235.
PubMed Abstract | Publisher Full Text - Comas JV, Mignosa C, Cochrane AD, Wilkinson JL, Karl TR. Taussig-Bing anomaly and arterial switch: aortic arch obstruction does not influence outcome. Eur J Cardiothorac Surg 1996;10:1114–1119.
PubMed Abstract | Publisher Full Text - Alsoufi B, Cai S, Williams WG, Coles JG, Caldarone CA, Redington AM et al. Improved results with single-stage total correction of Taussig-Bing anomaly. Eur J Cardiothorac Surg 2008;33:244–50.
PubMed Abstract | Publisher Full Text - Sinzobahamvya N, Blaschczok HC, Asfour B, Arenz C, Jussli MJ, Schindler E et al. Right ventricular outflow tract obstruction after arterial switch operation for the Taussig-Bing heart. Eur J Cardiothorac Surg 2007;31:873–878.
PubMed Abstract | Publisher Full Text - Masuda M, Kado H, Shiokawa Y, Fukae K, Kanegae Y, Kawachi Y et al. Clinical results of arterial switch operation for double-outlet right ventricle with subpulmonary VSD. Eur J Cardiothorac Surg 1999;15:283–288.
PubMed Abstract | Publisher Full Text - Huber C, Mimic B, Oswal N, Sullivan I, Kostolny M, Elliott M et al. Outcomes and re-interventions after one-stage repair of transposition of great arteries and aortic arch obstruction. Eur J Cardiothorac Surg 2011;39:213–220.
PubMed Abstract | Publisher Full Text - Lamers LJ, Frommelt PC, Mussatto KA, Jaquiss RDB, Mitchell ME, Tweddell JS. Coarctectomy combined with an interdigitating arch reconstruction results in a lower incidence of recurrent arch obstruction after the Norwood procedure than coarctectomy alone. J Thorac Cardiovasc Surg 2012;143:1098–102.
PubMed Abstract | Publisher Full Text | Free Full Text
The authors wish to thank Mr. Nicholas Geddes for processing the videos used in this article.
None declared.
Author
Martin Kostolny and Ikenna Omeje
Author Affiliations
Cardiothoracic Unit, Great Ormond Street Hospital for Children, NHS Foundation Trust, London, UK
Corresponding Author
Martin Kostolny
Cardiothoracic Unit, Great Ormond Street Hospital for Children, London WC1N 3JH, UK
Phone: +44-2074-059200, ext. 5734
Fax: +44-2074-301281
Email: kostom@gosh.nhs.uk
Keywords
© The Author 2016. Published by MMCTS on behalf of the European Association for Cardio-Thoracic Surgery. All rights reserved.
