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Aortopulmonary window

MedGen UID:
365
Concept ID:
C0003516
Congenital Abnormality
Synonyms: Aortic-pulmonary window; Aorto-pulmonary window; Aortopulmonary septal defect
SNOMED CT: Aortic septal defect (17024001); Aorticopulmonary septal defect (17024001); Aorticopulmonary window (17024001); Aortopulmonary septal defect (17024001); APW - Aortopulmonary window (17024001); Aortopulmonary window (17024001); AP - Aortopulmonary window (17024001)
 
HPO: HP:0011604
Monarch Initiative: MONDO:0021902

Definition

A congenital anomaly with an abnormal connection between the aorta and the main pulmonary artery resulting in an aortopulmonary shunt. [from HPO]

Conditions with this feature

Diaphragmatic hernia 4, with cardiovascular defects
MedGen UID:
1823983
Concept ID:
C5774210
Disease or Syndrome
Diaphragmatic hernia-4 with cardiovascular defects (DIH4) is an autosomal recessive congenital anomaly syndrome characterized by the presence of diaphragmatic hernia or eventration apparent at birth. Affected infants have associated pulmonary hypoplasia and respiratory insufficiency resulting in death in infancy. Most also have variable cardiovascular defects, including aortopulmonary window or conotruncal anomalies. Dysmorphic facial features and mild distal limb anomalies are sometimes observed (Beecroft et al., 2021). For a discussion of genetic heterogeneity of congenital diaphragmatic hernia (CDH), see DIH1 (142340).

Professional guidelines

PubMed

Kaw A, Kaw K, Hostetler EM, Beleza-Meireles A, Smith-Collins A, Armstrong C, Scurr I, Cotts T, Aatre R, Bamshad MJ, Earl D, Groner A, Agre K, Raveh Y, Kwartler CS, Milewicz DM
Am J Med Genet A 2022 Aug;188(8):2389-2396. Epub 2022 May 14 doi: 10.1002/ajmg.a.62775. PMID: 35567597Free PMC Article
Li W, Bin G, Jiang W, Shuang Y
J Ultrasound Med 2019 Mar;38(3):795-803. Epub 2018 Oct 12 doi: 10.1002/jum.14756. PMID: 30312989
Yu S, Han J, Gao S, Liu X, Gu X, Zhang Y, Sun L, He Y
Echocardiography 2018 Nov;35(11):1835-1840. Epub 2018 Sep 7 doi: 10.1111/echo.14131. PMID: 30192407

Recent clinical studies

Etiology

Kumar V, Singh RS, Thingnam SKS, Mishra AK, Jaswal V
J Card Surg 2019 May;34(5):300-304. Epub 2019 Mar 21 doi: 10.1111/jocs.14023. PMID: 30900319
Li W, Bin G, Jiang W, Shuang Y
J Ultrasound Med 2019 Mar;38(3):795-803. Epub 2018 Oct 12 doi: 10.1002/jum.14756. PMID: 30312989
Yu S, Han J, Gao S, Liu X, Gu X, Zhang Y, Sun L, He Y
Echocardiography 2018 Nov;35(11):1835-1840. Epub 2018 Sep 7 doi: 10.1111/echo.14131. PMID: 30192407
Barnes ME, Mitchell ME, Tweddell JS
Semin Thorac Cardiovasc Surg Pediatr Card Surg Annu 2011;14(1):67-74. doi: 10.1053/j.pcsu.2011.01.017. PMID: 21444051
Gangana CS, Malheiros AF, Alves EV, de Azevedo MA, Bernardes RM, Simões LC
Arq Bras Cardiol 2007 Apr;88(4):402-7. doi: 10.1590/s0066-782x2007000400006. PMID: 17546268

Diagnosis

Dev M, Sharma M, Rana N
Curr Cardiol Rev 2020;16(1):73-76. doi: 10.2174/1573403X15666190513105231. PMID: 31092183Free PMC Article
Dhillon GS, Fernandes CJ, Khan A, Heinle JS, Lopez KN
J Clin Ultrasound 2018 Nov;46(9):617-622. Epub 2018 Aug 30 doi: 10.1002/jcu.22615. PMID: 30160304Free PMC Article
Ocazionez D, Shroff GS, Vargas D, Dicks D, Chaturvedi A, Nachiappan AC, Murillo H, Baxi A, Restrepo CS
Semin Ultrasound CT MR 2017 Dec;38(6):584-593. Epub 2017 Aug 4 doi: 10.1053/j.sult.2017.07.004. PMID: 29179898
Varoquaux A, Kebebew E, Sebag F, Wolf K, Henry JF, Pacak K, Taïeb D
Endocr Relat Cancer 2016 Sep;23(9):R371-9. Epub 2016 Jul 12 doi: 10.1530/ERC-16-0241. PMID: 27406876Free PMC Article
Lupinski RW, Shankar S, Agasthian T, Lim CH, Mancer K
Ann Thorac Surg 2004 Sep;78(3):e43-4. doi: 10.1016/j.athoracsur.2004.01.015. PMID: 15337082

Therapy

Kazemi MA, Shamshiri M, Kiani A, Davarpanah AH, Ghanaati H, Moradi B, Chavoshi M
J Voice 2024 Mar;38(2):492-495. Epub 2021 Nov 6 doi: 10.1016/j.jvoice.2021.09.033. PMID: 34753626
Lee KJ, Manepalli A, Tollinche LE, Yeoh CB
A A Pract 2021 May 17;15(5):e01469. doi: 10.1213/XAA.0000000000001469. PMID: 33999865Free PMC Article
Bayramoglu Z, Yılmaz R, Demir AA, Yekeler E, Dursun M, Dindar A, Nisli K, Omeroglu R
J Cardiovasc Comput Tomogr 2017 Sep-Oct;11(5):417-418. Epub 2017 Apr 20 doi: 10.1016/j.jcct.2017.04.008. PMID: 28438441
Daitoku K, Yamauchi S, Suzuki Y, Fukuda I
Congenit Heart Dis 2014 Mar-Apr;9(2):E58-60. Epub 2013 May 7 doi: 10.1111/chd.12074. PMID: 23648073
Nakamura H, Taniguchi Y, Fujioka S, Miwa K, Haruki T, Takagi Y, Yurugi Y, Kubouchi Y
Gen Thorac Cardiovasc Surg 2012 Mar;60(3):183-7. Epub 2012 Mar 15 doi: 10.1007/s11748-011-0817-x. PMID: 22419192

Prognosis

Kumar V, Singh RS, Thingnam SKS, Mishra AK, Jaswal V
J Card Surg 2019 May;34(5):300-304. Epub 2019 Mar 21 doi: 10.1111/jocs.14023. PMID: 30900319
Li W, Bin G, Jiang W, Shuang Y
J Ultrasound Med 2019 Mar;38(3):795-803. Epub 2018 Oct 12 doi: 10.1002/jum.14756. PMID: 30312989
Dhillon GS, Fernandes CJ, Khan A, Heinle JS, Lopez KN
J Clin Ultrasound 2018 Nov;46(9):617-622. Epub 2018 Aug 30 doi: 10.1002/jcu.22615. PMID: 30160304Free PMC Article
Gangana CS, Malheiros AF, Alves EV, de Azevedo MA, Bernardes RM, Simões LC
Arq Bras Cardiol 2007 Apr;88(4):402-7. doi: 10.1590/s0066-782x2007000400006. PMID: 17546268
Lupinski RW, Shankar S, Agasthian T, Lim CH, Mancer K
Ann Thorac Surg 2004 Sep;78(3):e43-4. doi: 10.1016/j.athoracsur.2004.01.015. PMID: 15337082

Clinical prediction guides

Singh R, Ojha V, Nagulakonda S, Malhi AS, Handa A, Kumar S
J Cardiovasc Comput Tomogr 2022 Sep-Oct;16(5):e40-e41. Epub 2022 Apr 4 doi: 10.1016/j.jcct.2022.03.007. PMID: 35422410
Khurana R, Sharma A, Sharma G, Chandrashekhara SH
J Cardiovasc Comput Tomogr 2021 Mar-Apr;15(2):e10-e11. Epub 2020 Sep 16 doi: 10.1016/j.jcct.2020.09.001. PMID: 33004298
Dhillon GS, Fernandes CJ, Khan A, Heinle JS, Lopez KN
J Clin Ultrasound 2018 Nov;46(9):617-622. Epub 2018 Aug 30 doi: 10.1002/jcu.22615. PMID: 30160304Free PMC Article
Bayramoglu Z, Yılmaz R, Demir AA, Yekeler E, Dursun M, Dindar A, Nisli K, Omeroglu R
J Cardiovasc Comput Tomogr 2017 Sep-Oct;11(5):417-418. Epub 2017 Apr 20 doi: 10.1016/j.jcct.2017.04.008. PMID: 28438441
Lopez L, Varela A, Freixinet J, Quevedo S, Lopez Pujol J, Rodriguez de Castro F, Salvatierra A
Ann Thorac Surg 1994 Mar;57(3):555-7; discussion 557-8. doi: 10.1016/0003-4975(94)90544-4. PMID: 8147621

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