Scimitar syndrome: incidence, treatment, and prognosis

• Published in: European journal of pediatrics Vol. 167; no. 2; pp. 155 - 160
• Main Authors: Wang, Ching-Chia, Wu, En-Ting, Chen, Shyh-Jye, Lu, Frank, Huang, Shu-Chien, Wang, Jou-Kou, Chang, Chung-I, Wu, Mei-Hwan
• Format: Journal Article
• Language: English
• Published: Berlin/Heidelberg Springer-Verlag 01.02.2008; Springer; Springer Nature B.V
• Subjects: Biological and medical sciences; Cardiology. Vascular system; Child; Child, Preschool; Congenital heart diseases. Malformations of the aorta, pulmonary vessels and vena cava; Embolization, Therapeutic; Epidemiology; Female; Follow-Up Studies; General aspects; Heart; Humans; Imaging, Three-Dimensional; Incidence; Infant; Male; Medical sciences; Medicine; Medicine & Public Health; Original Paper; Pediatrics; Prognosis; Public health. Hygiene; Public health. Hygiene-occupational medicine; Radiography, Thoracic; Scimitar Syndrome - diagnostic imaging; Scimitar Syndrome - epidemiology; Scimitar Syndrome - therapy; Taiwan - epidemiology; Tomography, X-Ray Computed; Pulmonary veins; Congenital heart disease; Airway anomaly; Scimitar syndrome; Pediatrics; Prognosis; Respiratory disease; Cardiovascular disease; Respiratory system; Congenital disease; Epidemiology; Incidence; Vascular disease; Respiratory tract; Treatment; Pulmonary vessel; Malformation; Blood vessel; Anomaly; Circulatory system; Airway anomaly, Pulmonary veins; Congenital cardiopathy; Public health; Pulmonary vein
• ISSN: 0340-6199; 1432-1076
• DOI: 10.1007/s00431-007-0441-z

This study is based on a database of 16 years; we sought to define the incidence and outcome of scimitar syndrome. Of 8,771 patients, 5 (0.057%) with scimitar syndrome were identified and constituted the study population. Follow-up ranged from 1 to 16 years (median: 10 years). Diagnosis was assured by computed tomography in four patients and by cardiac catheterization in one. Two patients presented with respiratory distress soon after birth and required early pneumonectomy in one case and coil embolization of the abnormal feeding arteries to the right lower lung followed by surgical rerouting of the abnormal pulmonary vein and repair of the atrial septal defect in the other case. The former was supported by ventilator therapy for 3 years after pneumonectomy, but was finally weaned from the ventilator. Among the other three, two had repeated pneumonia that resolved after rerouting of the abnormal right pulmonary vein and cardiac repair. The asymptomatic child did not receive any intervention. In spite of the abnormal orientation of the airways, none of the four patients with detailed computed tomography imaging showed any significant compression of the airways. All five patients were doing well as of the last follow-up. In conclusion, scimitar syndrome is a very rare disease in this Asian country and the varied symptoms, such as tachypnea and repeated infection, could be improved after interventions.