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Scimitar syndrome and left pulmonary vein stenosis: A serious and rare association


1 Pediatric Heart Institute, University Hospital 12 de Octubre, Madrid, Spain
2 Research Institute Hospital 12 de Octubre (i+12), University Hospital 12 de Octubre, Madrid, Spain

Correspondence Address:
Dr. Alberto Mendoza
Pediatric Heart Institute, University Hospital 12 de Octubre, Madrid
Spain
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/apc.apc_53_21

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Year : 2022  |  Volume : 15  |  Issue : 1  |  Page : 80-83

 

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Scimitar syndrome is a rare constellation of cardiovascular anomalies consisting of partial right anomalous pulmonary venous drainage to the inferior vena cava, hypoplasia of the right lung, cardiac dextroposition, and in many cases, one or more aortopulmonary collaterals from the descending aorta to the hypoplastic lung. The “infantile form” commonly presents with significant heart failure and pulmonary hypertension. Stenosis of the left pulmonary veins associated with scimitar syndrome is a rare but well-described combination, usually associated with a poor prognosis. We describe two patients with this association in whom the left pulmonary vein stenosis manifested months after the initial diagnosis as a progressive lesion that complicated the course of the disease due to severe pulmonary hypertension. Both patients were successfully treated with a combination of surgical, percutaneous, and hybrid treatment.






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1 Pediatric Heart Institute, University Hospital 12 de Octubre, Madrid, Spain
2 Research Institute Hospital 12 de Octubre (i+12), University Hospital 12 de Octubre, Madrid, Spain

Correspondence Address:
Dr. Alberto Mendoza
Pediatric Heart Institute, University Hospital 12 de Octubre, Madrid
Spain
Login to access the Email id

Source of Support: None, Conflict of Interest: None


DOI: 10.4103/apc.apc_53_21

Rights and Permissions

Scimitar syndrome is a rare constellation of cardiovascular anomalies consisting of partial right anomalous pulmonary venous drainage to the inferior vena cava, hypoplasia of the right lung, cardiac dextroposition, and in many cases, one or more aortopulmonary collaterals from the descending aorta to the hypoplastic lung. The “infantile form” commonly presents with significant heart failure and pulmonary hypertension. Stenosis of the left pulmonary veins associated with scimitar syndrome is a rare but well-described combination, usually associated with a poor prognosis. We describe two patients with this association in whom the left pulmonary vein stenosis manifested months after the initial diagnosis as a progressive lesion that complicated the course of the disease due to severe pulmonary hypertension. Both patients were successfully treated with a combination of surgical, percutaneous, and hybrid treatment.






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