Minimally Invasive Surgical Correction of Scimitar Syndrome

Scimitar Syndrome is a rare congenital anomaly with a bimodal presentation that requires surgical intervention. This video illustrates a safe and minimally invasive approach to surgical correction for this anomaly performed on an adult patient. Scimitar Syndrome is a rare congenital anomaly with a bimodal presentation (1-3).

The infantile manifestation is severe with significant mortality, and the child or adult form is generally less critical (3). The anomaly consists of partial to complete anomalous pulmonary venous drainage of the right lung to the inferior vena cava (4).

This anomaly has been associated with other anomalies such as anomalous systemic arterial supply to the right lung, hypoplastic right pulmonary artery, hypoplastic or sequestration of the lung, dextroposition, atrial septal defect and pulmonary hypertension (2, 4, 5).

Various surgical approaches have been described in the literature. Approaches include median sternotomy or right anterolateral thoracotomy with creation of intra-atrial baffles or reimplantaton of the scimitar vein into the left atrium with or without the use of cardiopulmonary bypass, and lung resection procedures (1, 2, 4, 5).

We present a minimally invasive approach for correction of the defect through a limited right anterior thoracotomy. The patient is a 45-year old man with progressive dyspnea and recurrent atrial tachyarrhythmias. Echocardiogram and CT scan revealed mesocardia, dextroposition with Scimitar syndrome, moderate tricuspid regurgitation and a patent foramen ovale (PFO). Surgery was performed via a limited right thoracotomy. Cardiopulmonary bypass with cardioplegic arrest was used via peripheral cannulation through the right femoral artery and vein, and right internal jugular vein. The Scimitar vein (SV) was identified draining into the inferior vena cava at the level of diaphragm. Accessory vascular supply to the right lower lobe from the diaphragm was identified, ligated and divided. The SV was transected and the inferior vena caval side was over sewn in two layers. The right atrium was incised to perform a PFO closure, a right atrial cryoablation Cox Maze III procedure, and tricuspid valve repair with a flexible annuloplasty band. The SV was brought through a generous posterior pericardial window and anastomosed end to side to the left atrium. The posterior layer was sutured continuously and the anterior suture line was interrupted to ensure a widely patent anastomosis. The patient recovered uneventfully and was discharged after 4 days.

CONCLUSION

We present a safe and effective minimally invasive approach for surgical treatment of Scimitar syndrome in an adult patient. The technique can be reproduced with central cannulation in infants and children and presents a pragmatic approach for surgical treatment of Scimitar syndrome.

REFERENCES

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Vida VL, Padalino MA, Boccuzzo G, Tarja E, Berggren H, Carrel T, et al. Scimitar syndrome: a European Congenital Heart Surgeons Association (ECHSA) multicentric study. Circulation. 2010;122(12):1159-66. Epub 2010/09/09.

Najm HK, Williams WG, Coles JG, Rebeyka IM, Freedom RM. Scimitar syndrome: twenty years' experience and results of repair. The Journal of thoracic and cardiovascular surgery. 1996;112(5):1161-8; discussion 8-9. Epub 1996/11/01.

Brown JW, Ruzmetov M, Minnich DJ, Vijay P, Edwards CA, Uhlig PN, et al. Surgical management of scimitar syndrome: an alternative approach. The Journal of thoracic and cardiovascular surgery. 2003;125(2):238-45. Epub 2003/02/13.

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