Duck HE, Tavera RG

Language: Spanish
References: 12
Page: 37-41
PDF size: 108.21 Kb.

ABSTRACT

The scimitar syndrome accounts for 3 to 5% of diagnosed cases of anomalous return of pulmonary veins and has an incidence of 1 to 3 cases per 100,000 live births. The age of presentation is variable with a mean age of 7 months. The clinical picture depends on the severity of the dysgenesis and hypoplasia of the right lung, recurrent infections, the degree of shunting from left to right, and associated congenital heart disease or development of pulmonary hypertension. About half of patients are asymptomatic or with few symptoms at the time of diagnosis, despite varying degrees of pulmonary hypoplasia and pulmonary hypertension. Neonates have a worse prognosis and with more severe symptoms, while older children have recurrent respiratory infections or heart murmur. The prognosis for these patients is good with conservative treatment, surgical treatment is considered in symptomatic patients and those with associated cardiac abnormalities. The diagnosis is usually made by echocardiography, cardiac catheterization but is considered the gold standard for confirmation of diagnosis and is also used for embolization of systemic-pulmonary collateral artery to improve symptoms of heart failure and pulmonary hypertension. MRI is a new modality that has proved adequate in the diagnosis of pulmonary and systemic venous anomalies and identification of aortopulmonary collateral artery.

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