partial anomalous pulmonary venous drainage – scimitar syndrome
description
this is a condition where some or all of the right pulmonary veins drain anomalously below the diaphragm. there is usually hypoplasia of the right lung, dextroposition of the heart and hypoplasia of the right pulmonary artery. the right lung is frequently supplied directly by a collateral artery from the aorta. if the affected infant presents soon after birth, with evidence of failure to thrive and pulmonary hypertension, the outlook is poor. on the other hand if only a small portion of lung is involved the patient may not present until adult life. those detected in utero tend to be the most severe cases.
diagnosis
the heart is displaced into the right thorax, with the apex usually oriented directly anteriorly and the interventricular septum parallel to the midline. the venous drainage of the right lung cannot be seen entering the right atrium. the right pulmonary artery is usually smaller than normal or even undetectable.
differential diagnosis
the dextroposition seen in this condition is similar to that seen in diaphragmatic hernia or congenital cystic adenomatous malformation of the lung. the left lung however, is normal in a scimitar syndrome.
sonographic features
displacement of the heart into the right chest.
right pulmonary veins not draining to la.
small rpa