The impact of interruption of anomalous systemic arterial supply on scimitar syndrome presenting during infancy

We sought to evaluate the impact of interruption of anomalous systemic arterial supply (ASAS) on clinical course and management outcome of scimitar syndrome (SS) presenting during infancy.

No systematic study has been reported so far on this subject, although there are conflicting sporadic reports indicating variable effect.

Out of 23 children diagnosed to have SS during the past 25 years, 16 symptomatic infants had ASAS. After interrupting ASAS (coil embolization -14, surgical ligation -2), they were prospectively followed up to define their clinical course and management outcome.

All 16 infants had sizable ASAS and 9 had variable scimitar vein (SV) stenosis. Fifteen (94%) had pulmonary hypertension and significant left to right shunt. Post intervention, there was variable reduction of shunt in 14 and pulmonary artery pressure in 15 cases. All showed varying clinical improvement. One died of septicemia shortly afterwards. Definitive surgery was deferred for optimal results in seven children for a mean period of 8 months (range 1 month to 3 years). Remaining eight children did not require definitive surgery. One among them had closure of stenosed partial SV by Amplatzer duct occluder ("physiologic correction"). Two children on short term and six children on long term follow-up (mean 5.2 years; range 3.3-10.3 years) are doing well.

Interruption of ASAS helps to avoid or defer definitive surgery for SS during infancy. Therefore, we recommend coil embolization of ASAS as initial palliation, and long term surveillance to assess need for further intervention.