Successful Repair of Infantile Scimitar Syndrome with Contralateral Pulmonary Vein Stenosis in an Infant

Braz J Cardiovasc Surg. 2021 Feb 1;36(1):133-136. doi: 10.21470/1678-9741-2019-0228.

Abstract

Infantile scimitar syndrome (SS) is a rare congenital heart disease and has high mortality. Guidelines have not been established, but surgery is indicated in symptomatic patients. Despite the various surgical approaches, outcomes continue to be disappointing. We present our surgical experience with an infantile SS patient who had stenotic pulmonary veins contralateral to the hypoplastic lung with complicated anatomy. There are few cases with this complex pathology in the literature. Moreover, our patient was the first transplant-free survivor with this complexity in the literature.

Keywords: Heart Defects, Congenital; Pulmonary Syndrome; Pulmonary Veins; Respiratory System Abnormalities; Survivors.

Publication types

  • Case Reports

MeSH terms

  • Constriction, Pathologic
  • Heart Defects, Congenital*
  • Humans
  • Infant
  • Pulmonary Veins* / diagnostic imaging
  • Pulmonary Veins* / surgery
  • Scimitar Syndrome* / complications
  • Scimitar Syndrome* / diagnostic imaging
  • Scimitar Syndrome* / surgery
  • Stenosis, Pulmonary Vein* / diagnostic imaging
  • Stenosis, Pulmonary Vein* / etiology
  • Stenosis, Pulmonary Vein* / surgery