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Case Reports in Obstetrics and Gynecology
Volume 2016, Article ID 3641453, 3 pages
Case Report

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

1Department of Obstetrics and Gynecology, Dumlupinar University School of Medicine, 43040 Kutahya, Turkey
2Department of Pediatrics, Dumlupınar University School of Medicine, 43040 Kutahya, Turkey
3Department of Pediatric Cardiology, Dumlupınar University Kutahya Evliya Çelebi Training and Research Hospital, Kutahya, Turkey

Received 12 March 2016; Revised 26 June 2016; Accepted 30 June 2016

Academic Editor: Svein Rasmussen

Copyright © 2016 Ali Seven et al. 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.


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.