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A Case of the TOF with APV Complicated with Polyhydramnios and Severe Bronchomalacia

Absent pulmonary valve syndrome (APVS) is a rare congenital heart disease with severe pulmonary insufficiency, characterized with aneurysmal dilation in the pulmonary artery and one or both of its branches. We presented a rare case with APVS and literature review in this letter. Prenatal USG examina...

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Detalles Bibliográficos
Autores principales: Seven, Ali, Yalinbas, Emine Esin, Ozdemir, Rahmi
Formato: Online Artículo Texto
Lenguaje:English
Publicado: Hindawi Publishing Corporation 2016
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC4992531/
https://www.ncbi.nlm.nih.gov/pubmed/27579200
http://dx.doi.org/10.1155/2016/3641453
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author Seven, Ali
Yalinbas, Emine Esin
Ozdemir, Rahmi
author_facet Seven, Ali
Yalinbas, Emine Esin
Ozdemir, Rahmi
author_sort Seven, Ali
collection PubMed
description Absent pulmonary valve syndrome (APVS) is a rare congenital heart disease with severe pulmonary insufficiency, characterized with aneurysmal dilation in the pulmonary artery and one or both of its branches. We presented a rare case with APVS and literature review in this letter. Prenatal USG examination of the fetus at the 26th week of gestation revealed severe polyhydramnios, dilatation at right ventricle, and abnormal appearance of the heart. At the 31st gestational week, the baby was born with cesarean section. The newborn had right heart failure but had no hydrops fetalis. Therefore, severe respiratory distress observed in the infant has been associated with pulmonary complications. The infant, who had respiratory acidosis according to blood gas analysis, was intubated and attached to mechanical ventilator. Despite progressively increased respiratory support and other interventions, the infant died on the 3rd day of admission. Compression against bronchial tree and esophagus due to dilated pulmonary artery and its branches may inevitably lead to bronchomalacia and polyhydramnios. In conclusion, presence of polyhydramnios and the possibility of severe bronchomalacia should be kept in mind; and due to the risk of early neonatal mortality, delivery should be performed in a center where pediatric heart surgery is available.
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spelling pubmed-49925312016-08-30 A Case of the TOF with APV Complicated with Polyhydramnios and Severe Bronchomalacia Seven, Ali Yalinbas, Emine Esin Ozdemir, Rahmi Case Rep Obstet Gynecol Case Report Absent pulmonary valve syndrome (APVS) is a rare congenital heart disease with severe pulmonary insufficiency, characterized with aneurysmal dilation in the pulmonary artery and one or both of its branches. We presented a rare case with APVS and literature review in this letter. Prenatal USG examination of the fetus at the 26th week of gestation revealed severe polyhydramnios, dilatation at right ventricle, and abnormal appearance of the heart. At the 31st gestational week, the baby was born with cesarean section. The newborn had right heart failure but had no hydrops fetalis. Therefore, severe respiratory distress observed in the infant has been associated with pulmonary complications. The infant, who had respiratory acidosis according to blood gas analysis, was intubated and attached to mechanical ventilator. Despite progressively increased respiratory support and other interventions, the infant died on the 3rd day of admission. Compression against bronchial tree and esophagus due to dilated pulmonary artery and its branches may inevitably lead to bronchomalacia and polyhydramnios. In conclusion, presence of polyhydramnios and the possibility of severe bronchomalacia should be kept in mind; and due to the risk of early neonatal mortality, delivery should be performed in a center where pediatric heart surgery is available. Hindawi Publishing Corporation 2016 2016-08-07 /pmc/articles/PMC4992531/ /pubmed/27579200 http://dx.doi.org/10.1155/2016/3641453 Text en Copyright © 2016 Ali Seven et al. https://creativecommons.org/licenses/by/4.0/ This is an open access article distributed under the Creative Commons Attribution License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited.
spellingShingle Case Report
Seven, Ali
Yalinbas, Emine Esin
Ozdemir, Rahmi
A Case of the TOF with APV Complicated with Polyhydramnios and Severe Bronchomalacia
title A Case of the TOF with APV Complicated with Polyhydramnios and Severe Bronchomalacia
title_full A Case of the TOF with APV Complicated with Polyhydramnios and Severe Bronchomalacia
title_fullStr A Case of the TOF with APV Complicated with Polyhydramnios and Severe Bronchomalacia
title_full_unstemmed A Case of the TOF with APV Complicated with Polyhydramnios and Severe Bronchomalacia
title_short A Case of the TOF with APV Complicated with Polyhydramnios and Severe Bronchomalacia
title_sort case of the tof with apv complicated with polyhydramnios and severe bronchomalacia
topic Case Report
url https://www.ncbi.nlm.nih.gov/pmc/articles/PMC4992531/
https://www.ncbi.nlm.nih.gov/pubmed/27579200
http://dx.doi.org/10.1155/2016/3641453
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