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Valvular pulmonary stenosis

MedGen UID:
348595
Concept ID:
C1860336
Congenital Abnormality
Synonym: Pulmonary valvular stenosis
 
HPO: HP:0034350

Definition

A cardiovascular malformation associated with narrowing of the outflow tract of the right ventricle immediately at the level of the pulmonary valve. [from HPO]

Term Hierarchy

Conditions with this feature

Syndactyly-telecanthus-anogenital and renal malformations syndrome
MedGen UID:
394424
Concept ID:
C2678045
Disease or Syndrome
Syndrome with the association of toe syndactyly, facial dysmorphism including telecanthus and a broad nasal tip, urogenital malformations and anal atresia. Around ten cases have been reported so far. The syndrome is caused by mutations in the FAM58A gene (located on the X chromosome) encoding a protein of unknown function.
Cardiac valvular defect, developmental
MedGen UID:
1823949
Concept ID:
C5774175
Disease or Syndrome
Cardiac valvular dysplasia-1 (CVDP1) is characterized by congenital malformations of the pulmonic, tricuspid, and mitral valves. Structural cardiac defects, including atrial and ventricular septal defects, single left ventricle, and hypoplastic right ventricle have also been observed in affected individuals (Ta-Shma et al., 2017). Genetic Heterogeneity of Cardiac Valvular Dysplasia CVDP2 (620067) is caused by mutation in the ADAMTS19 gene (607513) on chromosome 5q23.

Professional guidelines

PubMed

Atiq M, Khan SA, Tipu FA, Amin Z
Pediatr Cardiol 2008 Sep;29(5):890-3. Epub 2008 May 15 doi: 10.1007/s00246-008-9223-2. PMID: 18481136
Hartyánszky IL, Kádár K, Faller K, Lozsádi K
Acta Paediatr Hung 1991;31(4):443-56. PMID: 1790027
Rocchini AP, Kveselis DA, Crowley D, Dick M, Rosenthal A
J Am Coll Cardiol 1984 Apr;3(4):1005-12. doi: 10.1016/s0735-1097(84)80360-5. PMID: 6707337

Recent clinical studies

Etiology

Iwai S, Miwa K, Nagashima T
Interact Cardiovasc Thorac Surg 2022 May 2;34(5):930-932. doi: 10.1093/icvts/ivac022. PMID: 35137109Free PMC Article
Poupart S, Navarro-Castellanos I, Raboisson MJ, Lapierre C, Dery J, Miró J, Dahdah N
Pediatr Cardiol 2021 Apr;42(4):814-820. Epub 2021 Jan 19 doi: 10.1007/s00246-021-02545-w. PMID: 33464372
El-Saeidi SA, Hamza HS, Agha HM, Soliman MM, Attia WA, El-Kaffas R, Abdel-Aziz F, Abdel-Aziz O, Shaker S, Esmat A, Ammar R, Fattouh A, Mohi-Eldin K, El-Sisi AM
Cardiol Young 2020 Apr;30(4):482-488. Epub 2020 Feb 19 doi: 10.1017/S1047951120000360. PMID: 32070441
Fedderly RT, Beekman RH 3rd
J Interv Cardiol 1995 Oct;8(5):451-61. doi: 10.1111/j.1540-8183.1995.tb00575.x. PMID: 10159512
Radtke W, Lock J
Pediatr Clin North Am 1990 Feb;37(1):193-213. doi: 10.1016/s0031-3955(16)36840-7. PMID: 2408000

Diagnosis

Ku L, Lv H, Yu Z, Ma X
J Card Surg 2022 Sep;37(9):2842-2844. Epub 2022 Jul 3 doi: 10.1111/jocs.16727. PMID: 35785437
Poupart S, Navarro-Castellanos I, Raboisson MJ, Lapierre C, Dery J, Miró J, Dahdah N
Pediatr Cardiol 2021 Apr;42(4):814-820. Epub 2021 Jan 19 doi: 10.1007/s00246-021-02545-w. PMID: 33464372
El-Saeidi SA, Hamza HS, Agha HM, Soliman MM, Attia WA, El-Kaffas R, Abdel-Aziz F, Abdel-Aziz O, Shaker S, Esmat A, Ammar R, Fattouh A, Mohi-Eldin K, El-Sisi AM
Cardiol Young 2020 Apr;30(4):482-488. Epub 2020 Feb 19 doi: 10.1017/S1047951120000360. PMID: 32070441
Miller BS
Pediatr Endocrinol Rev 2019 May;16(Suppl 2):424-427. doi: 10.17458/per.vol16.2019.m.historynoonan. PMID: 31115193
Mahfouz RA, Moustafa TM, Gouda M, Gad M
Int J Cardiovasc Imaging 2017 Apr;33(4):533-538. Epub 2016 Dec 10 doi: 10.1007/s10554-016-1036-4. PMID: 27943062

Therapy

Iwai S, Miwa K, Nagashima T
Interact Cardiovasc Thorac Surg 2022 May 2;34(5):930-932. doi: 10.1093/icvts/ivac022. PMID: 35137109Free PMC Article
Mahfouz RA, Moustafa TM, Gouda M, Gad M
Int J Cardiovasc Imaging 2017 Apr;33(4):533-538. Epub 2016 Dec 10 doi: 10.1007/s10554-016-1036-4. PMID: 27943062
Cuypers JA, Menting ME, Opić P, Utens EM, Helbing WA, Witsenburg M, van den Bosch AE, van Domburg RT, Baart SJ, Boersma E, Meijboom FJ, Bogers AJ, Roos-Hesselink JW
Heart 2017 Feb 15;103(4):273-279. Epub 2016 Aug 11 doi: 10.1136/heartjnl-2015-309159. PMID: 27515953
Fedderly RT, Beekman RH 3rd
J Interv Cardiol 1995 Oct;8(5):451-61. doi: 10.1111/j.1540-8183.1995.tb00575.x. PMID: 10159512
Radtke W, Lock J
Pediatr Clin North Am 1990 Feb;37(1):193-213. doi: 10.1016/s0031-3955(16)36840-7. PMID: 2408000

Prognosis

Poupart S, Navarro-Castellanos I, Raboisson MJ, Lapierre C, Dery J, Miró J, Dahdah N
Pediatr Cardiol 2021 Apr;42(4):814-820. Epub 2021 Jan 19 doi: 10.1007/s00246-021-02545-w. PMID: 33464372
El-Saeidi SA, Hamza HS, Agha HM, Soliman MM, Attia WA, El-Kaffas R, Abdel-Aziz F, Abdel-Aziz O, Shaker S, Esmat A, Ammar R, Fattouh A, Mohi-Eldin K, El-Sisi AM
Cardiol Young 2020 Apr;30(4):482-488. Epub 2020 Feb 19 doi: 10.1017/S1047951120000360. PMID: 32070441
Nakamura Y, Thattaliyath B, Ricci M
Ann Thorac Surg 2019 Jan;107(1):e49-e50. Epub 2018 Jun 8 doi: 10.1016/j.athoracsur.2018.05.029. PMID: 29890149
Fedderly RT, Beekman RH 3rd
J Interv Cardiol 1995 Oct;8(5):451-61. doi: 10.1111/j.1540-8183.1995.tb00575.x. PMID: 10159512
Gersony WM
Cardiol Clin 1989 Nov;7(4):915-23. PMID: 2688889

Clinical prediction guides

Poupart S, Navarro-Castellanos I, Raboisson MJ, Lapierre C, Dery J, Miró J, Dahdah N
Pediatr Cardiol 2021 Apr;42(4):814-820. Epub 2021 Jan 19 doi: 10.1007/s00246-021-02545-w. PMID: 33464372
El-Saeidi SA, Hamza HS, Agha HM, Soliman MM, Attia WA, El-Kaffas R, Abdel-Aziz F, Abdel-Aziz O, Shaker S, Esmat A, Ammar R, Fattouh A, Mohi-Eldin K, El-Sisi AM
Cardiol Young 2020 Apr;30(4):482-488. Epub 2020 Feb 19 doi: 10.1017/S1047951120000360. PMID: 32070441
Mahfouz RA, Moustafa TM, Gouda M, Gad M
Int J Cardiovasc Imaging 2017 Apr;33(4):533-538. Epub 2016 Dec 10 doi: 10.1007/s10554-016-1036-4. PMID: 27943062
Behjati-Ardakani M, Forouzannia SK, Abdollahi MH, Sarebanhassanabadi M
Acta Med Iran 2013 May 30;51(5):324-8. PMID: 23737317
Lueker RD, Vogel JH, Blount SG Jr
Br Heart J 1970 Nov;32(6):779-82. doi: 10.1136/hrt.32.6.779. PMID: 5212352Free PMC Article

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