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Absent Pulmonary Valve Syndrome with Left Hemitruncus

Absent pulmonary valve syndrome (APVS) is a rare congenital heart defect, usually associated with tetralogy of Fallot (TOF), although other associations have been described. A pregnant woman was referred to fetal echocardiography clinic from the Maternal Fetal Medicine department due to abnormal fin...

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Autores principales: Aly, Ashraf M., Reid, Deborah A., Jiwani, Amyn K., Desai, Pooja H.
Formato: Online Artículo Texto
Lenguaje:English
Publicado: Thieme Medical Publishers 2012
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC3653514/
https://www.ncbi.nlm.nih.gov/pubmed/23946907
http://dx.doi.org/10.1055/s-0032-1316464
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author Aly, Ashraf M.
Reid, Deborah A.
Jiwani, Amyn K.
Desai, Pooja H.
author_facet Aly, Ashraf M.
Reid, Deborah A.
Jiwani, Amyn K.
Desai, Pooja H.
author_sort Aly, Ashraf M.
collection PubMed
description Absent pulmonary valve syndrome (APVS) is a rare congenital heart defect, usually associated with tetralogy of Fallot (TOF), although other associations have been described. A pregnant woman was referred to fetal echocardiography clinic from the Maternal Fetal Medicine department due to abnormal findings on routine antenatal ultrasound, showing a pulsatile cystic mass above the left atrium and a suspected TOF. A fetal echocardiogram confirmed the presence of TOF/APVS. The pulsatile cystic mass was the aneurysmally dilated main pulmonary artery. The exact origin of the left pulmonary artery (LPA) was not clearly established prenatally. A postnatal echocardiogram of the neonate showed an abnormal origin of the LPA from the ascending aorta (hemitruncus). The neonate subsequently underwent surgical repair with a good outcome. We present a novel case of a TOF/APVS associated with an abnormal origin of the LPA from the ascending aorta.
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spelling pubmed-36535142013-08-14 Absent Pulmonary Valve Syndrome with Left Hemitruncus Aly, Ashraf M. Reid, Deborah A. Jiwani, Amyn K. Desai, Pooja H. AJP Rep Article Absent pulmonary valve syndrome (APVS) is a rare congenital heart defect, usually associated with tetralogy of Fallot (TOF), although other associations have been described. A pregnant woman was referred to fetal echocardiography clinic from the Maternal Fetal Medicine department due to abnormal findings on routine antenatal ultrasound, showing a pulsatile cystic mass above the left atrium and a suspected TOF. A fetal echocardiogram confirmed the presence of TOF/APVS. The pulsatile cystic mass was the aneurysmally dilated main pulmonary artery. The exact origin of the left pulmonary artery (LPA) was not clearly established prenatally. A postnatal echocardiogram of the neonate showed an abnormal origin of the LPA from the ascending aorta (hemitruncus). The neonate subsequently underwent surgical repair with a good outcome. We present a novel case of a TOF/APVS associated with an abnormal origin of the LPA from the ascending aorta. Thieme Medical Publishers 2012-07-16 2012-11 /pmc/articles/PMC3653514/ /pubmed/23946907 http://dx.doi.org/10.1055/s-0032-1316464 Text en © Thieme Medical Publishers
spellingShingle Article
Aly, Ashraf M.
Reid, Deborah A.
Jiwani, Amyn K.
Desai, Pooja H.
Absent Pulmonary Valve Syndrome with Left Hemitruncus
title Absent Pulmonary Valve Syndrome with Left Hemitruncus
title_full Absent Pulmonary Valve Syndrome with Left Hemitruncus
title_fullStr Absent Pulmonary Valve Syndrome with Left Hemitruncus
title_full_unstemmed Absent Pulmonary Valve Syndrome with Left Hemitruncus
title_short Absent Pulmonary Valve Syndrome with Left Hemitruncus
title_sort absent pulmonary valve syndrome with left hemitruncus
topic Article
url https://www.ncbi.nlm.nih.gov/pmc/articles/PMC3653514/
https://www.ncbi.nlm.nih.gov/pubmed/23946907
http://dx.doi.org/10.1055/s-0032-1316464
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