Volume 5, Issue 6, November 2019, Page: 80-83
Successful Surgical Repair of Scimitar Syndrome in a 38-Year-Old Adult
Rafael Meza, Department of Congenital Heart Surgery, Somer in Care Cardiovascular Center, Rionegro, Colombia
John Araujo, Department of Pediatric and Adult Congenital Heart Disease, Somer in Care Cardiovascular Center, Rionegro, Colombia
Alejandro Escobar, Department of Congenital Heart Surgery, Somer in Care Cardiovascular Center, Rionegro, Colombia
Alejandra Echeverri, Department of Pediatric and Adult Cardiovascular Anesthesiology, Somer in Care Cardiovascular Center, Rionegro, Colombia
Juan Turizo, Department of Congenital Heart Surgery, Somer in Care Cardiovascular Center, Rionegro, Colombia
Susana Cardona, Department of Congenital Heart Surgery, Somer in Care Cardiovascular Center, Rionegro, Colombia
Received: Nov. 30, 2019;       Accepted: Dec. 19, 2019;       Published: Jan. 7, 2020
DOI: 10.11648/j.ijcts.20190506.12      View  506      Downloads  162
Scimitar syndrome is an infrequent congenital malformation. It is a partial anomalous pulmonary venous drainage of the right lung into the inferior vena cava. Up to 25% of cases are associated with other heart diseases. The incidence in adults is reported to be 0.5-0.7%; classically, the adult form does not have pulmonary arterial hypertension, and may be unnoticed and asymptomatic for many years. It often presents after the second decade of life with nonspecific symptoms. Without surgical repair, clinical worsening, increased pulmonary pressure and sustained pulmonary arterial hypertension ultimately occur, when the syndrome is associated with other congenital heart diseases. We present the case of a 38-year-old woman diagnosed with scimitar syndrome associated with a large superior sinus venosus atrial septal defect, who underwent successful surgical repair using a lateral pericardial tunnel technique to redirect right pulmonary venous flow to the left atrium through a left atriotomy. This repair has shown good medium-term results. Possible complications such as tunnel thrombosis and stenosis will need to be explored in future assessments, and follow up must be lifelong.
Scimitar Syndrome, Partial Anomalous Pulmonary Venous Drainage, Adult Congenital Heart Disease, Pericardial Tunnel
To cite this article
Rafael Meza, John Araujo, Alejandro Escobar, Alejandra Echeverri, Juan Turizo, Susana Cardona, Successful Surgical Repair of Scimitar Syndrome in a 38-Year-Old Adult, International Journal of Cardiovascular and Thoracic Surgery. Vol. 5, No. 6, 2019, pp. 80-83. doi: 10.11648/j.ijcts.20190506.12
Copyright © 2019 Authors retain the copyright of this article.
This article is an open access article distributed under the Creative Commons Attribution License (http://creativecommons.org/licenses/by/4.0/) which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited.
Wu Y, Wu Z, Zheng J, Li Y, Zhou Y, Kuang H, et al. Sutureless technique versus conventional surgery in the primary treatment of total anomalous pulmonary venous connection: a systematic review and meta-analysis. J Cardiothorac Surg. 2018; 13 (1): 69.
Luna A, González G, Echeverry P. Scimitar syndrome and anesthetic implications. Rev Colomb Anestesiol. 2015; 43 (3): 245-49.
Youssef T, Mahmoud H, Ionescu N, Stoica D, Grigore C, Nicolescu A, et al. Scimitar syndrome associated with aberrant right subclavian artery, diaphragmatic hernia, and urinary anomalies - case report and review of the literature. Romanian J Morphol Embryol Rev Roum Morphol Embryol. 2018; 59 (2): 625-30.
Masrani A, McWilliams S, Bhalla S, Woodard P. Anatomical associations and radiological characteristics of Scimitar syndrome on CT and MR. J Cardiovasc Comput Tomogr. July 2018; 12 (4): 286-9.
Al Rukban H, Al Ghaihab M, Tamimi O, Al-Saleh S. Clinical spectrum of infantile scimitar syndrome: A tertiary center experience. Ann Pediatr Cardiol. 2014; 7 (1): 29-33.
Espinola N, Játiva S, Muñoz L, Zamora C. Aspectos clínicos y ecocardiográficos del síndrome de la cimitarra. Rev Esp Cardiol. 2006; 59 (3): 284-8.
Vida V, Padrini M, Boccuzzo G, Agnoletti G, Bondanza S, Butera G, et al. Natural History and Outcome of “Uncorrected” Scimitar Syndrome Patients: A Multicenter Studyof the Italian Society of Pediatric Cardiology. Rev Esp Cardiol. 2013; 66 (7): 556-60.
Stout K, Daniels C, Aboulhosn J, Bozkurt B, Broberg C, Colman J, et al. 2018 AHA/ACC Guideline for the management of adults with congenital heart disease: executive summary: a report of the American College of Cardiology/American Heart Association Task force on clinical practice guidelines. J Am Coll Cardiol. 2019; 73 (12): 1494-63.
Vida V, Padalino M, Boccuzzo G, Tarja E, Berggren H, Carrel T, e al. Scimitar syndrome A European Congenital Heart Surgeons Association (ECHSA) Multicentric Study. Circulation. 2010; 122: 1159-166.
Dusenbery S, Geva T, Seale A, Valente A, Zhou J, Sena L, et al. Outcome predictors and implications for management of scimitar síndrome. Am Heart J. 2013; 165 (5): 770-7.
Kahrom M, Kahrom H. Scimitar syndrome and evolution of managements. The Pan African Medical Journal. 2009; 3: 20.
Warnes C, Williams R, Bashore T, Bashore T, Child J, Conolly H, et al. ACC/AHA 2008 Guidelines for the managent of adults with congenital heart disease. J Am Coll Cardiol. 2008; 52 (23): 143-63.
Galie N, Manes A, Palazzini M, Negro L, Marinelli A, Gambetti S, et al. Management of pulmonary arterial hypertension associated with congenital systemic-to-pulmonary shunts and Eisenmenger's syndrome. Drugs 68: 1049-1066.
Galiè N, Humbert M, Vachiery J, Gibbs S, Lang I, Torbicki A, et al. 2015 ESC/ERS Guidelines for the diagnosis and treatment of pulmonary hypertension: The Joint Task Force for the Diagnosis and Treatment of Pulmonary Hypertension of the European Society of Cardiology (ESC) and the European Respiratory Society (ERS): Endorsed by: Association for European Paediatric and Congenital Cardiology (AEPC), International Society for Heart and Lung Transplantation (ISHLT). Eur Heart J. 2016; 37 (1): 67-119.
Sun Y, Zhang H, Liu J, Xu Z, Wang S, Zhu H. Pericardial tunnel technique in the surgical management of the vertical form of scimitar syndrome. Interact Cardiovasc Thorac Surg. 2018; 27 (3): 387-93.
Benito F, González A, Oliver J. Intraluminal dilation of inferior vena cava stenosis after repair of the scimitar syndrome in an adult patient. Rev Esp Cardiol 2002; 55 (2): 190-2.
Browse journals by subject