Prenatal diagnosis of fetal left pulmonary artery sling.

Left pulmonary artery (LPA) sling is a very rare anomaly in which the LPA arises distally, far from the right pulmonary artery on the right side of the distal trachea, turns sharply leftwards around the trachea and courses to the left lung hilum through the space between the trachea and esophagus. LPA sling is often associated with distal tracheal narrowing, due to either intrinsic stenosis or secondary compression by the anomaly itself. To our knowledge, prenatal diagnosis of LPA sling has not been reported so far. We report a case in which LPA sling was diagnosed during fetal ultrasound examination.

Iris hypoplasia and aorticopulmonary septal defect: a neurocristopathy.

BACKGROUND: Errors in neural crest development are responsible for a number of ocular disorders. In some cases, these ocular abnormalities may be associated with systemic disorders, some of which may be life threatening. METHODS: We reviewed the medical record of 3 children from 2 institutions with a similar constellation of ocular and cardiac abnormalities. RESULTS: All 3 children had iris hypoplasia with pupils 6-7 mm in diameter that had minimal or no response to light or pharmacological agents. The ocular examinations were otherwise normal. All 3 children also had large aorticopulmonary septal defects which were surgically repaired. CONCLUSIONS: We report an association between iris hypoplasia and aorticopulmonary septal defects. Like the iris stroma, the aorticopulmonary septum forms from neural crest cells during embryogenesis which divide the truncus arteriosus into the ascending aorta and pulmonary trunk. Children with iris hypoplasia should undergo an immediate cardiac evaluation. It is important that opthalmologists be aware of the association between these two disorders since an aorticopulmonary septal defect is a life-threatening disorder.

Defeito do septo atrioventricular forma total / Atrial and ventricular heart septal defect total form

Nesta monografia é abordada a experiência adquirida durante anos de prática cirúrgica no tratamento dos defeitos do septo atrioventricular, sendo apresentada a opinião dos autores sobre a sua divisão em diferentes formas (parcial, intermediária e total). São apresentados inicialmente, alguns fatos históricos sobre a doença, destacando-se nomes e contibuições para o diagnóstico e o tratamento cirúrgico desta entidade tão complexa. O desenvolvimento embriológico do sistema cardiocirculatório é seguido pelo posicionamento com relação à gênese do aparecimento deste defeito cardíaco e pela noção de que as diferentes formas fazem parte de uma única doença em diferentes fases evolutivas...

Anatomy and pathogenesis of aorticopulmonary septal defect.

The pathologic anatomic features and associated cardiac anomalies of 13 patients with aorticopulmonary septal defect (APSD) and of 236 previously reported cases were analyzed. Morphologically, 3 types of APSD were distinguished: a defect with a more or less circular border located about halfway between the arterial valves and the bifurcation of the pulmonary trunk; a similarly located defect in which the border represents a helix; and a usually large defect in which there is no posterior (distal) border. The appearance of these 3 types of defects suggests a different developmental mechanism for each. Approximately half of the cases of APSD are not associated with other cardiovascular anomalies. Of the anomalies that do occur, anomalous origin of a coronary artery from the pulmonary trunk and interruption of the aortic arch (IAA) type A or severe preductal coarctation are seen far more often than expected. An association with DiGeorge syndrome, frequently noted with persistent truncus arteriosus (TA) and IAA type B, was not seen. Certain cardiac anomalies often seen with TA are rarely seen with APSD, and defects often seen with APSD are rarely seen with TA. These observations indicate that APSD and TA are pathogenetically unrelated even though located in the same region of the heart and, unlike TA and IAA type B, APSD is probably not due to a developmental error involving the neural crest.