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Late presentation of alveolar capillary dysplasia in an infant

Shankar, Venkat MD, MBA; Haque, Anwarul MBBS; Johnson, Joyce MD; Pietsch, John MD

Pediatric Critical Care Medicine: March 2006 - Volume 7 - Issue 2 - p 177-179
doi: 10.1097/01.PCC.0000202570.58016.67
Case Reports

Objective: To report a case of late presentation of alveolar capillary dysplasia and misalignment of pulmonary veins.

Design: Descriptive case report.

Setting: Pediatric intensive care unit of a tertiary care children's hospital.

Patient: Seven-week-old female infant with severe hypoxemic respiratory failure.

Conclusion: Alveolar capillary dysplasia with misalignment of pulmonary veins is a cause of primary pulmonary hypertension in newborns that can rarely present past the newborn period. Early lung biopsy can be helpful in establishing the diagnosis and avoiding ineffective, futile, and expensive therapeutic interventions.

From the Divisions of Pediatric Critical Care Medicine (VS, AH), Pathology (JJ), and Surgery (JP), Monroe Carell Jr. Children's Hospital at Vanderbilt, Nashville, TN.

None of the authors have received anything of value, either directly or indirectly, from a commercial or other party related directly or indirectly to the subject of this article submission.

©2006The Society of Critical Care Medicine and the World Federation of Pediatric Intensive and Critical Care Societies