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Pulmonary artery sling

MedGen UID:
341071
Concept ID:
C1856123
Finding
HPO: HP:0004961

Definition

An anomalous origin of the left pulmonary artery, such that it arises from the posterior aspect of the right pulmonary artery and passes between the trachea and esophagus to reach the left hilum. [from HPO]

Conditions with this feature

Mowat-Wilson syndrome
MedGen UID:
341067
Concept ID:
C1856113
Disease or Syndrome
Mowat-Wilson syndrome (MWS) is characterized by distinctive facial features (widely spaced eyes, broad eyebrows with a medial flare, low-hanging columella, prominent or pointed chin, open-mouth expression, and uplifted earlobes with a central depression), congenital heart defects with predilection for abnormalities of the pulmonary arteries and/or valves, Hirschsprung disease or chronic constipation, genitourinary anomalies (particularly hypospadias in males), and hypogenesis or agenesis of the corpus callosum. Most affected individuals have moderate-to-severe intellectual disability. Speech is typically limited to a few words or is absent, with relative preservation of receptive language skills. Growth restriction with microcephaly and seizure disorder are also common. Most affected people have a happy demeanor and a wide-based gait that can sometimes be confused with Angelman syndrome.
Alzahrani-Kuwahara syndrome
MedGen UID:
1782127
Concept ID:
C5543274
Disease or Syndrome
Alzahrani-Kuwahara syndrome (ALKUS) is an autosomal recessive neurodevelopmental syndrome characterized by global developmental delay with severely impaired intellectual function and poor or absent speech. Patients have poor overall growth and dysmorphic facial features. More variable findings include early-onset cataracts, hypotonia, congenital heart defects, lower limb spasticity, and hypospadias (summary by Alzahrani et al., 2020).

Professional guidelines

PubMed

Peacock G, Kothari D, D'Orsogna L, Dickinson JE, Andrews D, Yim D
Heart Lung Circ 2023 Jun;32(6):735-744. Epub 2023 Apr 13 doi: 10.1016/j.hlc.2023.03.009. PMID: 37061362
Stephens EH, Eltayeb O, Kennedy C, Rigsby CK, Rastatter JC, Carr MR, Mongé MC, Backer CL
Ann Thorac Surg 2022 Feb;113(2):630-636. Epub 2021 Jan 29 doi: 10.1016/j.athoracsur.2021.01.025. PMID: 33524348
Li X, Mu Z, Li X, Weng Z
Prenat Diagn 2018 Apr;38(5):310-317. Epub 2018 Mar 23 doi: 10.1002/pd.5235. PMID: 29451683

Recent clinical studies

Etiology

K Rahmath MR, Durward A
Pediatr Pulmonol 2023 May;58(5):1299-1309. Epub 2023 Feb 24 doi: 10.1002/ppul.26345. PMID: 36790334
Choi ES, Park CS, Kim DH, Kwon BS, Yun TJ, Kim MJ, Cha S, Baek JS, Yu JJ, Yang DH
J Thorac Cardiovasc Surg 2023 Aug;166(2):317-324.e1. Epub 2022 Nov 21 doi: 10.1016/j.jtcvs.2022.11.017. PMID: 36528435
Binsalamah ZM, Thomason A, Ibarra C, Spigel Z, Adachi I, Barton KE, Edmunds E, Caldarone CA, Imamura M, Heinle JS
Cardiol Young 2021 Jan;31(1):52-59. Epub 2020 Oct 13 doi: 10.1017/S1047951120003212. PMID: 33046146
Muthialu N, Martens T, Kanakis M, Bezuska L, Nakao M, Derrick G, Marek J, Khambadkone S, Kostolny M, Tsang V
Asian Cardiovasc Thorac Ann 2020 Oct;28(8):463-469. Epub 2020 Jul 12 doi: 10.1177/0218492320943342. PMID: 32659103
Chowdhury MM, Chakraborty S
Semin Pediatr Surg 2015 Aug;24(4):168-75. Epub 2015 Feb 27 doi: 10.1053/j.sempedsurg.2015.02.001. PMID: 26051049

Diagnosis

K Rahmath MR, Durward A
Pediatr Pulmonol 2023 May;58(5):1299-1309. Epub 2023 Feb 24 doi: 10.1002/ppul.26345. PMID: 36790334
Worhunsky DJ, Levy BE, Stephens EH, Backer CL
Semin Pediatr Surg 2021 Dec;30(6):151128. Epub 2021 Oct 23 doi: 10.1016/j.sempedsurg.2021.151128. PMID: 34930596
Backer CL, Mongé MC, Popescu AR, Eltayeb OM, Rastatter JC, Rigsby CK
Semin Pediatr Surg 2016 Jun;25(3):165-75. Epub 2016 Feb 22 doi: 10.1053/j.sempedsurg.2016.02.009. PMID: 27301603
Gumbiner CH, Mullins CE, McNamara DG
Am J Cardiol 1980 Feb;45(2):311-5. doi: 10.1016/0002-9149(80)90651-7. PMID: 7355740
Castaneda AR
Ann Thorac Surg 1979 Sep;28(3):210-1. doi: 10.1016/s0003-4975(10)63107-4. PMID: 485623

Therapy

Chen L, Zhu L, Wang H, Lu Z, Xu Z, Du X, Wang S
J Thorac Cardiovasc Surg 2022 Jun;163(6):2218-2228. Epub 2021 Nov 4 doi: 10.1016/j.jtcvs.2021.10.047. PMID: 34872757
Kaneko N, Hasegawa T
Cardiol Young 2022 Apr;32(4):579-583. Epub 2021 Jul 12 doi: 10.1017/S1047951121002663. PMID: 34247683
Muthialu N, Martens T, Kanakis M, Bezuska L, Nakao M, Derrick G, Marek J, Khambadkone S, Kostolny M, Tsang V
Asian Cardiovasc Thorac Ann 2020 Oct;28(8):463-469. Epub 2020 Jul 12 doi: 10.1177/0218492320943342. PMID: 32659103
Li D, Zhou X, Li M
Pediatr Cardiol 2020 Oct;41(7):1376-1385. Epub 2020 Jun 3 doi: 10.1007/s00246-020-02386-z. PMID: 32494877
Li D, Zhou X, Li M, Liu X
Medicine (Baltimore) 2019 Oct;98(40):e17449. doi: 10.1097/MD.0000000000017449. PMID: 31577770Free PMC Article

Prognosis

Choi ES, Park CS, Kim DH, Kwon BS, Yun TJ, Kim MJ, Cha S, Baek JS, Yu JJ, Yang DH
J Thorac Cardiovasc Surg 2023 Aug;166(2):317-324.e1. Epub 2022 Nov 21 doi: 10.1016/j.jtcvs.2022.11.017. PMID: 36528435
Ruiz-Solano E, Mitchell M
Curr Cardiol Rep 2022 Oct;24(10):1495-1503. Epub 2022 Oct 3 doi: 10.1007/s11886-022-01764-8. PMID: 36190599Free PMC Article
Carlson L, Haider M, Liu H, Baird C, Mayer JE Jr, Nathan M
World J Pediatr Congenit Heart Surg 2021 Nov;12(6):715-727. doi: 10.1177/21501351211040741. PMID: 34846972
Muthialu N, Martens T, Kanakis M, Bezuska L, Nakao M, Derrick G, Marek J, Khambadkone S, Kostolny M, Tsang V
Asian Cardiovasc Thorac Ann 2020 Oct;28(8):463-469. Epub 2020 Jul 12 doi: 10.1177/0218492320943342. PMID: 32659103
Fiore AC, Brown JW, Weber TR, Turrentine MW
Ann Thorac Surg 2005 Jan;79(1):38-46; discussion 38-46. doi: 10.1016/j.athoracsur.2004.06.005. PMID: 15620911

Clinical prediction guides

Alkhasov A, Ratnikov S, Lezhnev A, Razumovskiy A, Sergeeva I, Kuzin S, Komina E, Gusev A, Yatzik S, Romanova E, Savelieva M, Fisenko A
J Pediatr Surg 2023 Apr;58(4):624-628. Epub 2022 Dec 22 doi: 10.1016/j.jpedsurg.2022.12.022. PMID: 36653203
Xu L, Wang L, Gai Y, Ma Q, Xiang G, Ouyang C, Shang N
J Clin Ultrasound 2021 Mar;49(3):257-261. Epub 2020 Sep 14 doi: 10.1002/jcu.22913. PMID: 32929784
Muthialu N, Martens T, Kanakis M, Bezuska L, Nakao M, Derrick G, Marek J, Khambadkone S, Kostolny M, Tsang V
Asian Cardiovasc Thorac Ann 2020 Oct;28(8):463-469. Epub 2020 Jul 12 doi: 10.1177/0218492320943342. PMID: 32659103
Leonardi B, Secinaro A, Cutrera R, Albanese S, Trozzi M, Franceschini A, Silvestri V, Tomà P, Carotti A, Pongiglione G
Pediatr Pulmonol 2015 Aug;50(8):781-8. Epub 2014 Jun 30 doi: 10.1002/ppul.23075. PMID: 24979312
Huang SC, Wu ET, Wang CC, Chen SJ, Chen YS, Chang CI, Chiu IS, Wang SS
Pediatr Pulmonol 2012 Sep;47(9):903-8. Epub 2012 Mar 13 doi: 10.1002/ppul.22516. PMID: 22416005

Recent systematic reviews

Li D, Zhou X, Li M
Pediatr Cardiol 2020 Oct;41(7):1376-1385. Epub 2020 Jun 3 doi: 10.1007/s00246-020-02386-z. PMID: 32494877

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