U.S. flag

An official website of the United States government

Format

Send to:

Choose Destination

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

Tang H, Wang Y, Sun X, Zhang Y
Echocardiography 2020 May;37(5):732-737. Epub 2020 Apr 29 doi: 10.1111/echo.14666. PMID: 32347569
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

Bouazzi M, Jørgensen DES, Andersen H, Krusenstjerna-Hafstrøm T, Ekelund CK, Jensen AN, Sandager P, Sperling L, Steensberg J, Sundberg K, Vejlstrup NG, Petersen OBB, Vedel C
Ultrasound Obstet Gynecol 2023 Nov;62(5):681-687. doi: 10.1002/uog.26249. PMID: 37191390
Reddy C, Kaskar A, Karthick E, Siddaiah S, Kiran VS, Suresh P
World J Pediatr Congenit Heart Surg 2022 May;13(3):334-340. doi: 10.1177/21501351221077888. PMID: 35446205
Bauser-Heaton H, Ma M, McElhinney DB, Goodyer WR, Zhang Y, Chan FP, Asija R, Shek J, Wise-Faberowski L, Hanley FL
Ann Thorac Surg 2019 Sep;108(3):820-827. Epub 2019 Apr 10 doi: 10.1016/j.athoracsur.2019.03.022. PMID: 30980823
Talwar S, Agarwal P, Choudhary SK, Kothari SS, Juneja R, Saxena A, Airan B
J Card Surg 2017 Feb;32(2):138-144. Epub 2017 Jan 30 doi: 10.1111/jocs.12936. PMID: 28139013
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

Diagnosis

Simsek B, Ozyuksel A, Saygi M
Cardiol Young 2023 Sep;33(9):1787-1789. Epub 2023 Apr 24 doi: 10.1017/S1047951123000914. PMID: 37092647
Tang H, Wang Y, Sun X, Zhang Y
Echocardiography 2020 May;37(5):732-737. Epub 2020 Apr 29 doi: 10.1111/echo.14666. PMID: 32347569
Tongprasert F, Sittiwangkul R, Jatavan P, Tongsong T
J Ultrasound Med 2017 Aug;36(8):1733-1738. Epub 2017 Apr 10 doi: 10.7863/ultra.16.08025. PMID: 28393388
Talwar S, Agarwal P, Choudhary SK, Kothari SS, Juneja R, Saxena A, Airan B
J Card Surg 2017 Feb;32(2):138-144. Epub 2017 Jan 30 doi: 10.1111/jocs.12936. PMID: 28139013
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

Therapy

Nagashima T, Taira M, Hasegawa M, Kugo Y, Watanabe T, Yoshioka D, Shimamura K, Ueno T, Miyagawa S
J Cardiothorac Surg 2023 Jul 1;18(1):201. doi: 10.1186/s13019-023-02305-2. PMID: 37393254Free PMC Article
Jacks A, Schatz B, Li J, Kim P, Adams K, Harn N, Kraft S
J Voice 2023 Mar;37(2):289.e15-289.e21. Epub 2020 Dec 24 doi: 10.1016/j.jvoice.2020.11.027. PMID: 33358294
de Perrot M, McRae K
J Thorac Cardiovasc Surg 2017 Jul;154(1):314-316. Epub 2016 Jan 9 doi: 10.1016/j.jtcvs.2016.01.006. PMID: 26880052
Naik AV, Salvi P, Mhatre A, Soomar SM, Joshi S
Asian Cardiovasc Thorac Ann 2014 Mar;22(3):272-5. Epub 2013 Sep 24 doi: 10.1177/0218492313481222. PMID: 24585901
Murin P, Sinzobahamvya N, Blaschczok HCh, Photiadis J, Haun C, Asfour B, Hraska V
Thorac Cardiovasc Surg 2012 Apr;60(3):215-20. Epub 2012 Jan 17 doi: 10.1055/s-0031-1298061. PMID: 22252330

Prognosis

Reddy C, Kaskar A, Karthick E, Siddaiah S, Kiran VS, Suresh P
World J Pediatr Congenit Heart Surg 2022 May;13(3):334-340. doi: 10.1177/21501351221077888. PMID: 35446205
Bauser-Heaton H, Ma M, McElhinney DB, Goodyer WR, Zhang Y, Chan FP, Asija R, Shek J, Wise-Faberowski L, Hanley FL
Ann Thorac Surg 2019 Sep;108(3):820-827. Epub 2019 Apr 10 doi: 10.1016/j.athoracsur.2019.03.022. PMID: 30980823
Talwar S, Siddharth B, Gupta SK, Choudhary SK, Kothari SS, Juneja R, Saxena A, Airan B
Interact Cardiovasc Thorac Surg 2017 Nov 1;25(5):740-744. doi: 10.1093/icvts/ivx158. PMID: 28633352
Talwar S, Agarwal P, Choudhary SK, Kothari SS, Juneja R, Saxena A, Airan B
J Card Surg 2017 Feb;32(2):138-144. Epub 2017 Jan 30 doi: 10.1111/jocs.12936. PMID: 28139013
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

Clinical prediction guides

Verma M, Pandey NN, Ramakrishnan S, Jagia P
J Card Surg 2022 Dec;37(12):4475-4484. Epub 2022 Nov 2 doi: 10.1111/jocs.17075. PMID: 36321703
Reddy C, Kaskar A, Karthick E, Siddaiah S, Kiran VS, Suresh P
World J Pediatr Congenit Heart Surg 2022 May;13(3):334-340. doi: 10.1177/21501351221077888. PMID: 35446205
Bi WJ, Xiao YJ, Liu YJ, Hou Y, Ren WD
BMC Cardiovasc Disord 2021 Jan 6;21(1):15. doi: 10.1186/s12872-020-01837-y. PMID: 33407161Free 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
de Perrot M, McRae K
J Thorac Cardiovasc Surg 2017 Jul;154(1):314-316. Epub 2016 Jan 9 doi: 10.1016/j.jtcvs.2016.01.006. PMID: 26880052

Supplemental Content

Table of contents

    Clinical resources

    Practice guidelines

    • PubMed
      See practice and clinical guidelines in PubMed. The search results may include broader topics and may not capture all published guidelines. See the FAQ for details.

    Recent activity

    Your browsing activity is empty.

    Activity recording is turned off.

    Turn recording back on

    See more...