The diagnosis and treatment of scimitar syndrome in 6 cases

Abstract

Objective Investigate the clinical features, diagnosis and treatment of the scimitar sydrome. Methods A retrospective analysis of clinical data of six children with scimitar syndrome from 2008 to 2014, including clinical symptoms and signs, chest X-ray, echocardiography, chest CT and cardiac catheterization, treatment outcome and follow-up and review of the literature. Results Six patients with scimitar syndrome were diagnosed between 2008 and 2014. There were 3 girls and 3 boys; 5 cases<6 kg in weight. Scimitar syndrome was suspected in 5 cases because of extroversion, and diagnosed by color Doppler echocardiography in all 6 when a scimitar vein was detected entering the inferior vena cava. Computed tomography confirmed the diagnosis in all patients. One patient had horseshoe lung, 3 had a unilobar right lung, 3 had a hypoplastic right lower lobe. Three patients had severe pulmonary arterial hypertension, 1 had moderate to severe pulmonary arterial hypertension, and 2 had moderate pulmonary arterial hypertension. All patients had lower respiratory tract infections, volume loss of the right lung, a normal or hyperinflated left lung, dextroversion of the heart, and scimitar arteries from the descending aorta. 4 cases of pulmonary venous drainage correction surgery, 3 underwent body - pulmonary embolism, 1 case of 9-year-old asymptomatic children without surgery, and 1 patient with heart failure, severe pulmonary hypertension, pulmonary infection, septicemia died before surgery. Conclusion Clinically, found children with heart of dextrocardia position, growth retardation, recurrent lung infections, unexplained right heart failure, pulmonary hypertension, should consider the possibility scimitar syndrome. Early diagnosis and early treatment of pulmonary venous drainage and pulmonary vascular embolization, can significantly improve the clinical symptoms in children and reduce mortality. Key words: Heart disease, congenital; Scimitar syndrome; Pulmonary veins; Pulmonary hypertension