[Scimitar syndrome in the newborn child and infant].
We present 10 symptomatic patients with scimitar syndrome. Their age was below 1 year and four were newborn babies. In 5 patients there was secundum atrial septal defect and in one there was a patent ductus arteriosus. Sixty per cent of the patients had pulmonary sequestration. The right lung was moderately or severely hypoplasic in 8 cases and seven had frequent respiratory infections. The anomalous venous drainage produced a left-to right shunt at atrium level which in our series had a value of 2.87 +/- 0.5. Five patients had pulmonary hypertension either moderate or severe. The severity of this disease in early infancy is related to the pulmonary hypertension (40%), requiring a prompt surgical correction. Eight patients were operated upon, the drainage being connected to the left atrium. The six surviving patients are asymptomatic at 12 years of follow-up. Our series is the biggest published in our country and the second in the world literature in infancy.
A
A
Cabrera
J J
JJ
Sarrionandia
G
G
Idígoras
F
F
Mondragón
M
M
Rosell
J
J
Pilar
J M
JM
Galdeano
M
M
Chouza
E
E
Pastor
D000015:Abnormalities, Multiple; D006330:Heart Defects, Congenital; D006801:Humans; D006976:Hypertension, Pulmonary / Q000209:etiology; D007223:Infant; D007231:Infant, Newborn; D008168:Lung / Q000000981:diagnostic imaging; D011859:Radiography; D011877:Radionuclide Imaging; D012587:Scimitar Syndrome / Q000000981:diagnostic imaging / Q000473:pathology / Q000601:surgery*
El síndrome de la cimitarra en el recién nacido y en el lactante.