Ultrasound findings in neonates with alveolar capillary dysplasia with misalignment of the pulmonary veins: report of two cases

J Int Med Res. 2022 Sep;50(9):3000605221126876. doi: 10.1177/03000605221126876.

Abstract

Alveolar capillary dysplasia with misalignment of the pulmonary veins (ACDMPV) is a rare congenital pulmonary disease that affects newborns. Most patients with ACDMPV are born at full term and are healthy. The main clinical manifestations are refractory pulmonary hypertension and pulmonary failure with gastrointestinal, urinary, or cardiac malformations. ACDMPV often progresses rapidly, but no conventional biological or imaging tests other than genetic testing are available for its diagnosis. Lung biopsy is currently the gold standard for diagnosis. We herein report two cases of ACDMPV confirmed by pathological examination and discuss their ultrasonographic findings.

Keywords: Alveolar capillary dysplasia with misalignment of the pulmonary veins; case report; lung ultrasound; neonate; pathologic examination; persistent pulmonary hypertension of the newborn.

Publication types

  • Case Reports

MeSH terms

  • Forkhead Transcription Factors / genetics
  • Humans
  • Infant, Newborn
  • Persistent Fetal Circulation Syndrome* / diagnostic imaging
  • Persistent Fetal Circulation Syndrome* / genetics
  • Pulmonary Alveoli / abnormalities
  • Pulmonary Alveoli / diagnostic imaging
  • Pulmonary Veins* / abnormalities
  • Pulmonary Veins* / diagnostic imaging
  • Pulmonary Veins* / pathology

Substances

  • Forkhead Transcription Factors

Supplementary concepts

  • Alveolar capillary dysplasia