Successful Surgical Repair of Scimitar Syndrome in a 38-Year-Old Adult

Rafael Meza; John Araujo; Alejandro Escobar; Alejandra Echeverri; Juan Turizo; Susana Cardona

doi:doi:10.11648/j.ijcts.20190506.12

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Successful Surgical Repair of Scimitar Syndrome in a 38-Year-Old Adult

Rafael Meza, John Araujo, Alejandro Escobar, Alejandra Echeverri, Juan Turizo, Susana Cardona

Published in International Journal of Cardiovascular and Thoracic Surgery (Volume 5, Issue 6)

Received: 30 November 2019 Accepted: 19 December 2019 Published: 7 January 2020

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Abstract

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.

Published in	International Journal of Cardiovascular and Thoracic Surgery (Volume 5, Issue 6)
DOI	10.11648/j.ijcts.20190506.12
Page(s)	80-83
Creative Commons	This is an Open Access article, distributed under the terms of the Creative Commons Attribution 4.0 International License (http://creativecommons.org/licenses/by/4.0/), which permits unrestricted use, distribution and reproduction in any medium or format, provided the original work is properly cited.
Copyright	Copyright © The Author(s), 2024. Published by Science Publishing Group

Keywords

Scimitar Syndrome, Partial Anomalous Pulmonary Venous Drainage, Adult Congenital Heart Disease, Pericardial Tunnel

References

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[2]	Luna A, González G, Echeverry P. Scimitar syndrome and anesthetic implications. Rev Colomb Anestesiol. 2015; 43 (3): 245-49.
[3]	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.
[4]	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.
[5]	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.
[6]	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.
[7]	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.
[8]	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.
[9]	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.
[10]	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.
[11]	Kahrom M, Kahrom H. Scimitar syndrome and evolution of managements. The Pan African Medical Journal. 2009; 3: 20.
[12]	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.
[13]	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.
[14]	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.
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[16]	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.

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APA Style

Rafael Meza, John Araujo, Alejandro Escobar, Alejandra Echeverri, Juan Turizo, et al. (2020). Successful Surgical Repair of Scimitar Syndrome in a 38-Year-Old Adult. International Journal of Cardiovascular and Thoracic Surgery, 5(6), 80-83. https://doi.org/10.11648/j.ijcts.20190506.12

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ACS Style

Rafael Meza; John Araujo; Alejandro Escobar; Alejandra Echeverri; Juan Turizo, et al. Successful Surgical Repair of Scimitar Syndrome in a 38-Year-Old Adult. Int. J. Cardiovasc. Thorac. Surg. 2020, 5(6), 80-83. doi: 10.11648/j.ijcts.20190506.12

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AMA Style

Rafael Meza, John Araujo, Alejandro Escobar, Alejandra Echeverri, Juan Turizo, et al. Successful Surgical Repair of Scimitar Syndrome in a 38-Year-Old Adult. Int J Cardiovasc Thorac Surg. 2020;5(6):80-83. doi: 10.11648/j.ijcts.20190506.12

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@article{10.11648/j.ijcts.20190506.12,
  author = {Rafael Meza and John Araujo and Alejandro Escobar and Alejandra Echeverri and Juan Turizo and Susana Cardona},
  title = {Successful Surgical Repair of Scimitar Syndrome in a 38-Year-Old Adult},
  journal = {International Journal of Cardiovascular and Thoracic Surgery},
  volume = {5},
  number = {6},
  pages = {80-83},
  doi = {10.11648/j.ijcts.20190506.12},
  url = {https://doi.org/10.11648/j.ijcts.20190506.12},
  eprint = {https://article.sciencepublishinggroup.com/pdf/10.11648.j.ijcts.20190506.12},
  abstract = {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.},
 year = {2020}
}

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TY  - JOUR
T1  - Successful Surgical Repair of Scimitar Syndrome in a 38-Year-Old Adult
AU  - Rafael Meza
AU  - John Araujo
AU  - Alejandro Escobar
AU  - Alejandra Echeverri
AU  - Juan Turizo
AU  - Susana Cardona
Y1  - 2020/01/07
PY  - 2020
N1  - https://doi.org/10.11648/j.ijcts.20190506.12
DO  - 10.11648/j.ijcts.20190506.12
T2  - International Journal of Cardiovascular and Thoracic Surgery
JF  - International Journal of Cardiovascular and Thoracic Surgery
JO  - International Journal of Cardiovascular and Thoracic Surgery
SP  - 80
EP  - 83
PB  - Science Publishing Group
SN  - 2575-4882
UR  - https://doi.org/10.11648/j.ijcts.20190506.12
AB  - 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.
VL  - 5
IS  - 6
ER  -

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Author Information

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

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Plain Text BibTeX RIS

APA Style

Rafael Meza, John Araujo, Alejandro Escobar, Alejandra Echeverri, Juan Turizo, et al. (2020). Successful Surgical Repair of Scimitar Syndrome in a 38-Year-Old Adult. International Journal of Cardiovascular and Thoracic Surgery, 5(6), 80-83. https://doi.org/10.11648/j.ijcts.20190506.12

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ACS Style

Rafael Meza; John Araujo; Alejandro Escobar; Alejandra Echeverri; Juan Turizo, et al. Successful Surgical Repair of Scimitar Syndrome in a 38-Year-Old Adult. Int. J. Cardiovasc. Thorac. Surg. 2020, 5(6), 80-83. doi: 10.11648/j.ijcts.20190506.12

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AMA Style

Rafael Meza, John Araujo, Alejandro Escobar, Alejandra Echeverri, Juan Turizo, et al. Successful Surgical Repair of Scimitar Syndrome in a 38-Year-Old Adult. Int J Cardiovasc Thorac Surg. 2020;5(6):80-83. doi: 10.11648/j.ijcts.20190506.12

Copy | Download

@article{10.11648/j.ijcts.20190506.12,
  author = {Rafael Meza and John Araujo and Alejandro Escobar and Alejandra Echeverri and Juan Turizo and Susana Cardona},
  title = {Successful Surgical Repair of Scimitar Syndrome in a 38-Year-Old Adult},
  journal = {International Journal of Cardiovascular and Thoracic Surgery},
  volume = {5},
  number = {6},
  pages = {80-83},
  doi = {10.11648/j.ijcts.20190506.12},
  url = {https://doi.org/10.11648/j.ijcts.20190506.12},
  eprint = {https://article.sciencepublishinggroup.com/pdf/10.11648.j.ijcts.20190506.12},
  abstract = {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.},
 year = {2020}
}

Copy | Download

TY  - JOUR
T1  - Successful Surgical Repair of Scimitar Syndrome in a 38-Year-Old Adult
AU  - Rafael Meza
AU  - John Araujo
AU  - Alejandro Escobar
AU  - Alejandra Echeverri
AU  - Juan Turizo
AU  - Susana Cardona
Y1  - 2020/01/07
PY  - 2020
N1  - https://doi.org/10.11648/j.ijcts.20190506.12
DO  - 10.11648/j.ijcts.20190506.12
T2  - International Journal of Cardiovascular and Thoracic Surgery
JF  - International Journal of Cardiovascular and Thoracic Surgery
JO  - International Journal of Cardiovascular and Thoracic Surgery
SP  - 80
EP  - 83
PB  - Science Publishing Group
SN  - 2575-4882
UR  - https://doi.org/10.11648/j.ijcts.20190506.12
AB  - 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.
VL  - 5
IS  - 6
ER  -

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