Alveolar capillary dysplasia with misalignment of pulmonary veins (ACD/MPV)—awareness prevents extended or futile ECMO use

  • Ullas Angadi
  • Vishnuvardhan Meedimale
  • Simon Robinson
Original Article
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Abstract

Background

Alveolar capillary dysplasia with misalignment of pulmonary veins (ACD/MPV) is a rare, fatal, congenital lung disorder involving abnormal development of the capillary vascular system around the alveoli of the lungs, which clinically presents as persistent pulmonary hypertension of the newborn (PPHN) refractory to treatment. It has been linked to the gene FOXF1 on chromosome 16q24.1–q24.2. Histopathological examination by lung biopsy is the gold standard for diagnosis.

Materials and methods

We present four cases of ACD/MPV who were referred for ECMO support with a diagnosis of PPHN with no apparent congenital anomalies.

Results

All the newborns had an overwhelming course, with PPHN and hypoxemia refractory to treatment. The diagnosis of ACD/MPV was established by ante-mortem lung biopsy in all cases. Intensive care treatment was withdrawn post diagnosis, with none of the four surviving.

Conclusions

Early lung biopsy for a histological diagnosis allows expensive and ineffective treatment to be avoided. Lung biopsy can be performed with low risk and high-diagnostic yield for alveolar capillary dysplasia.

Keywords

Pulmonary veins Lung biopsy Alveolar capillary dysplasia 

Notes

Acknowledgments

The authors would like to thank Dr. O’neill (histopathologist).

Conflicts of interest

None

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Copyright information

© Indian Association of Cardiovascular-Thoracic Surgeons 2014

Authors and Affiliations

  • Ullas Angadi
    • 1
  • Vishnuvardhan Meedimale
    • 1
  • Simon Robinson
    • 1
  1. 1.Paediatric Intensive Care UnitUniversity Hospitals of LeicesterLeicesterUK

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