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Congenital aortopulmonary window

MedGen UID:
419119
Concept ID:
C2931610
Disease or Syndrome
Synonyms: Aorta-pulmonary artery fistula; Aorto-pulmonary artery fistula; Aortopulmonary fistula
 
Monarch Initiative: MONDO:0016077
Orphanet: ORPHA2037

Definition

A rare congenital non-syndromic heart malformation characterized by a communication between the ascending aorta and the pulmonary trunk in the presence of two normally formed semilunar valves. It may be an isolated finding or occur in association with other anomalies. Severe clinical manifestations, such as congestive heart failure or pulmonary hypertension, typically develop in early life. [from ORDO]

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • CROGVCongenital aortopulmonary window
Follow this link to review classifications for Congenital aortopulmonary window in Orphanet.

Professional guidelines

PubMed

Laux D, Derridj N, Stirnemann J, Lucron H, Stos B, Levy M, Houyel L, Bonnet D
Ultrasound Obstet Gynecol 2022 Aug;60(2):223-233. Epub 2022 Jun 30 doi: 10.1002/uog.24873. PMID: 35118719Free 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

Tkebuchava T, von Segesser LK, Vogt PR, Bauersfeld U, Jenni R, Künzli A, Lachat M, Turina M
Eur J Cardiothorac Surg 1997 Feb;11(2):293-7. doi: 10.1016/s1010-7940(96)01048-2. PMID: 9080158

Diagnosis

Rider OJ, Bissell M, Myerson SG
Heart 2013 Oct;99(20):1546. Epub 2013 Jun 4 doi: 10.1136/heartjnl-2013-303753. PMID: 23735938
Tkebuchava T, von Segesser LK, Vogt PR, Bauersfeld U, Jenni R, Künzli A, Lachat M, Turina M
Eur J Cardiothorac Surg 1997 Feb;11(2):293-7. doi: 10.1016/s1010-7940(96)01048-2. PMID: 9080158

Prognosis

Tkebuchava T, von Segesser LK, Vogt PR, Bauersfeld U, Jenni R, Künzli A, Lachat M, Turina M
Eur J Cardiothorac Surg 1997 Feb;11(2):293-7. doi: 10.1016/s1010-7940(96)01048-2. PMID: 9080158

Supplemental Content

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