Hostname: page-component-74656f9884-54qdv Total loading time: 0 Render date: 2026-11-09T23:32:36.688Z Has data issue: false hasContentIssue false
  • English
  • Français

Autologous vascularised pericardial flap tunneling technique in Scimitar syndrome repair with dextrocardia: lessons from the Senning procedure

Part of: Surgery

Published online by Cambridge University Press:  15 January 2021

Alain J. Poncelet Open the ORCID record for Alain J. Poncelet [Opens in a new window] ,
Karlien Carbonez  and
Jean E. Rubay
Alain J. Poncelet*
Affiliation:
Department of Cardiovascular and Thoracic Surgery, Cliniques Universitaires Saint-Luc, Bruxelles, Belgium
Karlien Carbonez
Affiliation:
Department of Pediatric Cardiology, Cliniques Universitaires Saint-Luc, Bruxelles, Belgium
Jean E. Rubay
Affiliation:
Department of Cardiovascular and Thoracic Surgery, Cliniques Universitaires Saint-Luc, Bruxelles, Belgium
*
Author for correspondence: A. Poncelet, MD, PhD, Cardio-Vascular Department, Cliniques Universitaires Saint-Luc, Avenue Hippocrate 10, B - 1200 Bruxelles, Belgium. Tel.: +32 2 7646107; Fax +32 2 764 8960. E-mail: alain.poncelet@uclouvain.be
Rights & Permissions [Opens in a new window]

Abstract

Scimitar syndrome is a rare variant (5%) of partial abnormal pulmonary venous return. Surgery is required when pulmonary overcirculation is present. Following repair, Scimitar vein stenosis occurs in approximately 20%. We applied a variant of the atrial switch technique using autologous pericardial flap in a patient with Scimitar syndrome and dextrocardia. This tunneling technique allowed tension-free anastomosis and minimal Scimitar vein rotation.

Keywords

Scimitar syndromecardiac congenital-surgery

Information

Type
Brief Report
Information
Cardiology in the Young , Volume 31 , Issue 5 , May 2021 , pp. 859 - 861
Check for updates
Copyright
© The Author(s), 2021. Published by Cambridge University Press

Scimitar syndrome is a rare congenital anomaly. In its complete form, the right hypoplasic lung drains totally into the systemic veins, whereas its lower lobe is supplied by a systemic arterial collateral branch arising from the thoracic or abdominal aorta. Reference Neill, Ferencz, Sabiston and Sheldon1 Hypoplasia of the pulmonary arteries and abnormal bronchial branching accompany this syndrome. Reference Neill, Ferencz, Sabiston and Sheldon1

In this brief report, we describe the repair of a Scimitar syndrome associated with dextrocardia using an autologous vascularised pericardial flap tunneling technique.

Materials and methods

Our patient is a 15-year-old Algerian female with a late diagnosis of Scimitar syndrome associated with dextrocardia.

On evaluation, her chest X-ray was suggestive of right-sided Scimitar vein. Cardiac echocardiography demonstrated moderate right ventricular dilatation with preserved function and the absence of atrial septal defect. The left pulmonary veins were normally located, the right Scimitar vein drained in the inferior caval vein with a mild restrictive flow (mean gradient 4mmHg).

At angio-computed tomography scan, the Scimitar vein connected to the inferior caval vein underneath the diaphragm, the right lung was hypoplasic. Its lower lobe was supplied by a systemic arterial branch arising from the thoracic aorta (Fig 1).

Figure 1.

3D computed tomography scan reconstruction (posterior view) illustrating the Scimitar vein draining into the distal inferior caval vein (supra-hepatic veins not shown)

Preoperative catheterisation confirmed the absence of pulmonary hypertension. The systemic arterial branch was coil-occluded during the same procedure.

At surgery, as a first step, a 5 cm length muscle-sparing right lateral thoracotomy was performed, allowing the dissection of the triangular ligament, the diaphragm, and the mobilisation of the Scimitar vein. Surgical repair was performed through a sternotomy, under normothermic cardiopulmonary bypass, and femoral venous cannulation for the inferior caval vein drainage.

To allow appropriate surgical exposure in the presence of dextrocardia, two stay sutures were placed on the apex of the left ventricle and on the right atrial wall to allow clockwise heart rotation.

On a beating heart, as originally described by Shumacker, Reference Shumacker2 a pericardial flap was created between the superior and inferior atrio-caval junctions to create a bowl-like pericardial baffle. The right phrenic nerve pedicle was mobilised from its pericardial attachment using bipolar coagulation.

Once the pericardial tunnel was partially created, cardioplegic arrest was performed.

Through a short right atriotomy, a trans-septal approach was used to delineate the precise location of the left atrium within the pericardial baffle. As in any Senning-type repair, the widest incision was made on the left atrial wall near the Sondegaard’s groove.

The Scimitar vein was divided at its insertion site on the inferior caval vein. The distal stump was oversawn. The proximal end was rotated toward the pericardial “well” to identify the site of anastomosis. A 2 cm elliptic portion of the pericardium was excised, and a termino-lateral anastomosis was performed The repair was completed by trimming the pedicled pericardial flap and suturing it to the epicardial surface of the right atria (Figs 2 and 3a–c).

Figure 2.

Illustration of the technical aspects to create a bowl-like pedicled autologous pericardial baffle to which the Scimital vein can be connected with a tension-free anastomosis (DP: diaphragm, IV: inferior caval vein, LA: left atrium, RA: right atrium, RPA: right pulmonary artery, SV: Scimitar vein, SVC: superior caval vein)

Figure 3.

( a ) The proximal end of the Scimitar vein was rotated toward the pericardial “well” to identify the site of anastomosis. ( b ) A 2 cm elliptic portion of the pericardium was excised. ( c ) a termino-lateral anastomosis of the Scimitar Vein was performed. ( d ) Post-operative transthoracic echocardiography demonstrating a wide channel towards the left atrium

Post-operative echocardiography demonstrated unrestricted and laminar flow from the Scimitar vein to the left atrium (Fig 3d). The patient was extubated on the first post-operative day. Her intensive care unit stay was 2 days.

Her final cardiac evaluation confirmed unobstructed pulmonary venous pathways.

Discussion

First reported by Neill et al in 1960, Scimitar syndrome is a rare association of congenital cardio-pulmonary anomalies. Reference Neill, Ferencz, Sabiston and Sheldon1 Its hallmark is an anomalous right pulmonary vein that drains all or part of the right lung into the inferior caval vein, with this vessel producing the Scimitar sign on the chest radiograph.

Several techniques have been described to redirect the Scimitar vein into the left atrium, including intra-atrial (right) baffling, Reference Zubiate and Kay3 direct reimplantation, Reference Tornvall, Jackson, Alvayay, Vargas, Koch and Zarate4 and more recently prosthesis interposition Reference Lam, Reemtsen, Starnes and Wells5 or pericardial flap tunneling. Reference Lugones and Garcia6

Though short-term results are excellent for both intra-atrial baffling and direct reimplantation techniques, long-term studies have demonstrated residual pulmonary vein or systemic venous pathway stenosis ranging from 17 to 50% requiring re-interventions (dilatation or stenting) or reoperations. Reference Vida, Padalino and Boccuzzo7,Reference Dusenbery, Geva and Seale8

The autologous pericardium has been successfully used in total cavo pulmonary connection, Reference Hvass, Pansard, Bohm, Depoix, Enguerrand and Worms9 as well as in simple Senning-type repair of late-presenting transposition of great arteries-intact interventricular septum, Reference Rubay, de Halleux and Jaumin10 and in double-switch procedures Reference Mee11 with excellent functional results and low incidence of tunnel stenosis.

We modified the technique described by Lugones et al in 2014, by using a termino-lateral anastomosis which requires only a 45° anterior rotation of the Scimitar vein on its long-axis.

The suboptimal long-term results of well-established techniques of Scimitar vein rerouting justify the search of alternative technique of repair to improve outcome and reduce the rate of re-intervention or reoperations.

Though short-term results appear to be very good with this new technique, additional patients and longer follow-up is required to validate its value as part of the armentarium to the cardiac congenital surgeon.

Acknowledgements

The authors thanks Dr Jérome Duisit for his drawing skills to generate the brilliant illustration of the technical aspect of the repair.

Financial support

This research received no specific grant from any funding agency, or from commercial or not-for-profit sectors.

Conflict of interest statement

None.

Ethical standards

All patient identifiers were removed from this case report.

Informed consent

Written informed consent was obtained from the patient’s parents.

References

Neill, CA, Ferencz, C, Sabiston, DC, Sheldon, H. The familial occurrence of hypoplastic right lung with systemic arterial supply and venous drainage “scimitar syndrome”. Bull Johns Hopkins Hosp 1960; 107: 1–21.Google ScholarPubMed
Shumacker, HB Jr. A new operation for transposition of the great vessels. Surgery 1961; 50: 773–777.Google ScholarPubMed
Zubiate, P, Kay, JH. Surgical correction of anomalous pulmonary venous connection. Ann Surg 1962; 156: 234–250.10.1097/00000658-196208000-00009CrossRefGoogle ScholarPubMed
Tornvall, SS, Jackson, KH, Alvayay, JC, Vargas, AC, Koch, W, Zarate, E. Anomalous drainage of the pulmonary veins into the inferior vena cava. Report of an operation. J Thorac Cardiovasc Surg 1961; 42: 413–417.10.1016/S0022-5223(20)31930-9CrossRefGoogle ScholarPubMed
Lam, TT, Reemtsen, BL, Starnes, VA, Wells, WJ. A novel approach to the surgical correction of Scimitar syndrome. J Thorac Cardiovasc Surg 2007; 133: 573–574.CrossRefGoogle ScholarPubMed
Lugones, I, Garcia, R. A new surgical approach to Scimitar syndrome. Ann Thorac Surg 2014; 97: 353–355.CrossRefGoogle ScholarPubMed
Vida, VL, Padalino, MA, Boccuzzo, G et al. Scimitar syndrome: a European Congenital Heart Surgeons Association (ECHSA) multicentric study. Circulation 2010; 122: 1159–1166.CrossRefGoogle ScholarPubMed
Dusenbery, SM, Geva, T, Seale, A et al. Outcome predictors and implications for management of Scimitar syndrome. Am Heart J 2013; 165: 770–777.10.1016/j.ahj.2013.01.016CrossRefGoogle ScholarPubMed
Hvass, U, Pansard, Y, Bohm, G, Depoix, JP, Enguerrand, D, Worms, AM. Bicaval pulmonary connection in tricuspid atresia using an extracardiac tube of autologous pediculated pericardium to bridge inferior vena cava. Eur J Cardiothorac Surg 1992; 6: 49–51.10.1016/1010-7940(92)90099-JCrossRefGoogle ScholarPubMed
Rubay, JE, de Halleux, C, Jaumin, P et al. Long-Term follow-up of the Senning operation for transposition of the great arteries in children under 3 months of age. J Thorac Cardiovasc Surg 1987; 94: 75–81.10.1016/S0022-5223(19)36320-2CrossRefGoogle ScholarPubMed
Mee, RBB. The double switch operation with accent on the Senning component. Semin Thorac Cardiovasc Surg Pediatr Card Surg Annu. 2005; 8: 57–65.CrossRefGoogle Scholar
Figure 0

Figure 1. 3D computed tomography scan reconstruction (posterior view) illustrating the Scimitar vein draining into the distal inferior caval vein (supra-hepatic veins not shown)

Figure 1

Figure 2. Illustration of the technical aspects to create a bowl-like pedicled autologous pericardial baffle to which the Scimital vein can be connected with a tension-free anastomosis (DP: diaphragm, IV: inferior caval vein, LA: left atrium, RA: right atrium, RPA: right pulmonary artery, SV: Scimitar vein, SVC: superior caval vein)

Figure 2

Figure 3. (a) The proximal end of the Scimitar vein was rotated toward the pericardial “well” to identify the site of anastomosis. (b) A 2 cm elliptic portion of the pericardium was excised. (c) a termino-lateral anastomosis of the Scimitar Vein was performed. (d) Post-operative transthoracic echocardiography demonstrating a wide channel towards the left atrium