Tetralogy of Fallot With Absent Pulmonary Valve and Nonconfluent Pulmonary Arteries: A Management Conundrum

Tetralogy of Fallot with absent pulmonary valve syndrome is a rare form of congenital heart disease. Among the different variations with this rare anomaly is nonconfluent pulmonary artery branches with anomalous origin of the left pulmonary artery from the ductus arteriosus. The authors present one...

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Bibliographic Details
Published in:World journal for pediatric & congenital heart surgery 2020-07, Vol.11 (4), p.NP168-NP171
Main Authors: Swaminathan, Sethuraman, Agarwal, Arpit, Infante, Juan C., Rosenkranz, Eliot
Format: Article
Language:English
Subjects:
Ductus Arteriosus, Patent - diagnosis
Ductus Arteriosus, Patent - embryology
Echocardiography - methods
Fatal Outcome
Female
Humans
Infant, Newborn
Male
Prenatal Diagnosis
Pulmonary Artery - abnormalities
Pulmonary Artery - diagnostic imaging
Pulmonary Artery - embryology
Pulmonary Atresia - diagnosis
Pulmonary Atresia - embryology
Pulmonary Valve - abnormalities
Pulmonary Valve - diagnostic imaging
Pulmonary Valve - embryology
Tetralogy of Fallot - diagnosis
Tetralogy of Fallot - embryology
Tomography, X-Ray Computed
Young Adult
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Summary:Tetralogy of Fallot with absent pulmonary valve syndrome is a rare form of congenital heart disease. Among the different variations with this rare anomaly is nonconfluent pulmonary artery branches with anomalous origin of the left pulmonary artery from the ductus arteriosus. The authors present one such case which was diagnosed prenatally to have tetralogy of Fallot with absent pulmonary valve and identified postnatally to have nonconfluent pulmonary artery branches in addition. We discuss the conundrum of respiratory management in this patient pre- and postoperatively due to a unique ventilation perfusion mismatch problem, which varies between the two lungs.
ISSN:2150-1351
2150-136X
DOI:10.1177/2150135118775661