Alveolar Capillary Dysplasia with Misalignment of Pulmonary Veins (ACD/MPV): A Case Series

Alveolar capillary dysplasia with misalignment of pulmonary veins (ACD/MPV) is a rare, fatal, developmental lung disorder, which usually presents as persistent pulmonary hypertension of the newborn (PPHN) unresponsive to treatment. The authors present their own experience with three cases admitted during the last 15 years.

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Congenital Misalignment of Pulmonary Veins with Alveolar Capillary Dysplasia Causing Persistent Neonatal Pulmonary Hypertension: Report of Two Affected Siblings

Pediatric and Developmental Pathology ◽

10.1007/s100249910035 ◽

2000 ◽

Vol 3 (3) ◽

pp. 271-276 ◽

Cited By ~ 44

Author(s):

Carmen Gutierrez ◽

Angeles Rodriguez ◽

Sylvia Palenzuela ◽

Carolina Forteza ◽

Jose L. Diaz Rossello

Keyword(s):

Pulmonary Hypertension ◽

Pulmonary Veins ◽

Duodenal Atresia ◽

Persistent Pulmonary Hypertension ◽

Pulmonary Vessels ◽

Alveolar Capillary Dysplasia ◽

Pulmonary Abnormality ◽

Alveolar Capillary

Misalignment of pulmonary vessels with alveolar capillary dysplasia is a rare cause of persistent pulmonary hypertension of the newborn. Most of the reported cases have been sporadic. We present two consecutive affected siblings with this disorder. This is the fifth reported family occurrence of this condition. In addition to the pulmonary abnormality, one of our cases had duodenal atresia.

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Nitric oxide inhalation therapy for an infant with persistent pulmonary hypertension caused by misalignment of pulmonary veins with alveolar capillary dysplasia

Journal of Pediatric Surgery ◽

10.1016/s0022-3468(97)90105-6 ◽

1997 ◽

Vol 32 (1) ◽

pp. 99-100 ◽

Cited By ~ 24

Author(s):

Yasuhiro Kitayama ◽

Shinkichi Kamata ◽

Hirohomi Okuyama ◽

Noriaki Usui ◽

Toshio Sawai ◽

...

Keyword(s):

Nitric Oxide ◽

Pulmonary Hypertension ◽

Pulmonary Veins ◽

Persistent Pulmonary Hypertension ◽

Inhalation Therapy ◽

Alveolar Capillary Dysplasia ◽

Nitric Oxide Inhalation ◽

Alveolar Capillary

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Congenital Alveolar Capillary Dysplasia: Rare Cause of Persistent Pulmonary Hypertension

Fetal and Pediatric Pathology ◽

10.3109/15513819709168760 ◽

1997 ◽

Vol 17 (6) ◽

pp. 959-975 ◽

Cited By ~ 1

Author(s):

Sibylle Haraida ◽

Hannelore Lochbuhler ◽

A. Heger ◽

A. Nerlich ◽

J. Diebold ◽

...

Keyword(s):

Pulmonary Hypertension ◽

Persistent Pulmonary Hypertension ◽

Alveolar Capillary Dysplasia ◽

Alveolar Capillary

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Incidence of alveolar capillary dysplasia with misalignment of pulmonary veins in infants with unexplained severe pulmonary hypertension: The roles of clinical, pathological, and genetic testing

Early Human Development ◽

10.1016/j.earlhumdev.2021.105323 ◽

2021 ◽

Vol 155 ◽

pp. 105323

Author(s):

Tetsuo Onda ◽

Takuma Akimoto ◽

Itaru Hayasaka ◽

Masahiko Ikeda ◽

Yuta Furuse ◽

...

Keyword(s):

Pulmonary Hypertension ◽

Genetic Testing ◽

Pulmonary Veins ◽

Severe Pulmonary Hypertension ◽

Alveolar Capillary Dysplasia ◽

Alveolar Capillary

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Alveolar capillary dysplasia with misalignment of the pulmonary veins: clinical, histological, and genetic aspects

Pulmonary Circulation ◽

10.1177/2045894018795143 ◽

2018 ◽

Vol 8 (3) ◽

pp. 204589401879514 ◽

Cited By ~ 10

Author(s):

Evelien Slot ◽

Gabriëla Edel ◽

Ernest Cutz ◽

Arno van Heijst ◽

Martin Post ◽

...

Keyword(s):

Transcription Factor ◽

Molecular Mechanism ◽

Pulmonary Veins ◽

Clinical Aspects ◽

Lung Disorder ◽

Genetic Studies ◽

Ongoing Research ◽

Alveolar Capillary Dysplasia ◽

Alveolar Capillary ◽

Severe Lung

Alveolar capillary dysplasia with misalignment of the pulmonary veins (ACD/MPV) is a rare and lethal disorder mainly involving the vascular development of the lungs. Since its first description, significant achievements in research have led to a better understanding of the underlying molecular mechanism of ACD/MPV and genetic studies have identified associations with genomic alterations in the locus of the transcription factor FOXF1. This in turn has increased the awareness among clinicians resulting in over 200 cases reported so far, including genotyping of patients in most recent reports. Collectively, this promoted a better stratification of the patient group, leading to new perspectives in research on the pathogenesis. Here, we provide an overview of the clinical aspects of ACD/MPV, including guidance for clinicians, and review the ongoing research into the complex molecular mechanism causing this severe lung disorder.

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