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Left Atrial Stenosis Induced Pulmonary Venous Arterialization and Group 2 Pulmonary Hypertension in Rat
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Alveolar Capillary Dysplasia with Misalignment of Pulmonary Veins (ACD/MPV): A Case Series

Joana Miranda1, Gustavo Rocha2, Henrique Soares1

  • 1Division of Neonatology, Hospital of São João, Faculty of Medicine, University of Porto, Porto, Portugal.

Case Reports in Critical Care
|May 16, 2014
PubMed

Insights

Alveolar capillary dysplasia with misalignment of pulmonary veins (ACD/MPV) is a rare, fatal lung disorder. This study reviews three cases, highlighting its typical presentation as treatment-resistant persistent pulmonary hypertension of the newborn.

Area of Science:

  • Pediatric Pulmonology
  • Developmental Biology
  • Medical Genetics

Background:

  • Alveolar capillary dysplasia with misalignment of pulmonary veins (ACD/MPV) is a rare and often fatal developmental lung disorder.
  • ACD/MPV typically manifests as persistent pulmonary hypertension of the newborn (PPHN) that is unresponsive to conventional treatments.

Purpose of the Study:

  • To present the clinical experience with three cases of ACD/MPV over a 15-year period.
  • To underscore the diagnostic challenges and clinical course of ACD/MPV.

Main Methods:

  • Retrospective case series review.
  • Analysis of clinical data from three neonates diagnosed with ACD/MPV.

Main Results:

  • All three cases presented with severe PPHN refractory to standard therapies.
  • The study details the clinical course and outcomes for these patients.

Conclusions:

  • ACD/MPV is a severe condition requiring high clinical suspicion.
  • Early recognition and potential future therapeutic strategies are critical for managing this disorder.

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