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...
| Main Authors: | , , , |
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| Format: | Article |
| Language: | English |
| Published: |
Thieme Medical Publishers, Inc.
2012-11-01
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| Series: | American Journal of Perinatology Reports |
| Subjects: | |
| Online Access: | https://www.thieme-connect.de/products/ejournals/html/10.1055/s-0032-1316464 |
| _version_ | 1818296212164444160 |
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| author | Ashraf M. Aly Deborah A. Reid Amyn K. Jiwani Pooja H. Desai |
| author_facet | Ashraf M. Aly Deborah A. Reid Amyn K. Jiwani Pooja H. Desai |
| author_sort | Ashraf M. Aly |
| collection | DOAJ |
| 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. |
| first_indexed | 2024-12-13T03:59:57Z |
| format | Article |
| id | doaj.art-5b5fe99afa88464489542ea502d7f494 |
| institution | Directory Open Access Journal |
| issn | 2157-6998 2157-7005 |
| language | English |
| last_indexed | 2024-12-13T03:59:57Z |
| publishDate | 2012-11-01 |
| publisher | Thieme Medical Publishers, Inc. |
| record_format | Article |
| series | American Journal of Perinatology Reports |
| spelling | doaj.art-5b5fe99afa88464489542ea502d7f4942022-12-22T00:00:30ZengThieme Medical Publishers, Inc.American Journal of Perinatology Reports2157-69982157-70052012-11-01020105105410.1055/s-0032-1316464Absent Pulmonary Valve Syndrome with Left HemitruncusAshraf M. Aly0Deborah A. Reid1Amyn K. Jiwani2Pooja H. Desai3University of Texas Medical Branch-GalvestonUniversity of Texas Medical Branch-GalvestonUniversity of Texas Medical Branch-GalvestonUniversity of Texas Medical Branch-GalvestonAbsent 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.https://www.thieme-connect.de/products/ejournals/html/10.1055/s-0032-1316464tetralogy of Fallotabsent pulmonary valveleft hemitruncusfetal echocardiography |
| spellingShingle | Ashraf M. Aly Deborah A. Reid Amyn K. Jiwani Pooja H. Desai Absent Pulmonary Valve Syndrome with Left Hemitruncus American Journal of Perinatology Reports tetralogy of Fallot absent pulmonary valve left hemitruncus fetal echocardiography |
| 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 | tetralogy of Fallot absent pulmonary valve left hemitruncus fetal echocardiography |
| url | https://www.thieme-connect.de/products/ejournals/html/10.1055/s-0032-1316464 |
| work_keys_str_mv | AT ashrafmaly absentpulmonaryvalvesyndromewithlefthemitruncus AT deborahareid absentpulmonaryvalvesyndromewithlefthemitruncus AT amynkjiwani absentpulmonaryvalvesyndromewithlefthemitruncus AT poojahdesai absentpulmonaryvalvesyndromewithlefthemitruncus |