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Pulmonary subvalvular stenosis

MedGen UID:
11030
•Concept ID:
C0034084
•
Congenital Abnormality
Synonyms: Conus, Obstructive Subaortic; Infundibular Stenoses, Pulmonary; Infundibular Stenosis, Pulmonary; Obstructive Subaortic Conus; Pulmonary Infundibular Stenoses; Pulmonary Infundibular Stenosis; Pulmonary Stenoses, Subvalvular; Pulmonary Stenosis, Subvalvular; Pulmonary Subvalvular Stenoses; Pulmonary Subvalvular Stenosis; Stenoses, Pulmonary Infundibular; Stenoses, Pulmonary Subvalvular; Stenoses, Subvalvular Pulmonary; Stenosis, Pulmonary Infundibular; Stenosis, Pulmonary Subvalvular; Stenosis, Subvalvular Pulmonary; Subaortic Conus, Obstructive; Subvalvular Pulmonary Stenoses; Subvalvular Pulmonary Stenosis; Subvalvular Stenoses, Pulmonary; Subvalvular Stenosis, Pulmonary
SNOMED CT: Infundibular pulmonic stenosis (204370002); Stenosis of infundibulum of right ventricle (204370002); Subvalvular pulmonic stenosis (204370002); Pulmonary infundibular stenosis (204370002)
 
Monarch Initiative: MONDO:0006935

Definition

The obstruction of the right ventricular outflow tract that originates within the body of the right ventricle, that exists at the time of birth; it often occurs in association with other intracardiac anomalies. [from NCI]

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • CROGVPulmonary subvalvular stenosis

Professional guidelines

PubMed

Left ventricular outflow tract obstruction in complete transposition of the great arteries--echocardiography criteria for surgical strategies.
Wu KL, Wang JK, Lin MT, Chen CA, Wu ET, Chiu SN, Wu MH
Circ J 2010 Jun;74(6):1214-8. Epub 2010 Apr 27 doi: 10.1253/circj.cj-09-0081. PMID: 20453388

Recent clinical studies

Etiology

20 years of arterial switch operation for simple TGA.
De Praetere H, Vandesande J, Rega F, Daenen W, Marc G, Eyskens B, Heying R, Cools B, Meyns B
Acta Chir Belg 2014 Mar-Apr;114(2):92-8. PMID: 25073205
Characteristics and management of cleft mitral valve.
Fraisse A, Massih TA, Kreitmann B, Metras D, Vouhé P, Sidi D, Bonnet D
J Am Coll Cardiol 2003 Dec 3;42(11):1988-93. doi: 10.1016/j.jacc.2003.07.019. PMID: 14662264
Shortening patterns of the infundibulum in valvular pulmonary stenosis before and after balloon valvuloplasty.
Akiba T, Nakasato M, Suzuki H, Sato S, Sato T
Pediatr Cardiol 2000 Sep-Oct;21(5):448-51. doi: 10.1007/s002460010105. PMID: 10982703
Early hypercyanotic spells unrelated to ductal constriction in tricuspid atresia with infundibular stenosis.
Holmes G, Epstein ML
Pediatr Cardiol 1993 Jul;14(3):151-4. doi: 10.1007/BF00795643. PMID: 7692429
Congenitally corrected transposition with mirror-image atrial arrangement.
Attie F, Cerda J, Richheimer R, Chávez-Domínguez R, Buendia A, Ovseyevitz J, Muñoz-Castellanos L
Int J Cardiol 1987 Feb;14(2):169-75. doi: 10.1016/0167-5273(87)90006-4. PMID: 3818133

Diagnosis

Double-orifice mitral valve repair.
Gonzalez T, Vera F, Ruiz E
Cardiol Young 2019 Feb;29(2):214-215. Epub 2018 Dec 4 doi: 10.1017/S1047951118001890. PMID: 30511602
20 years of arterial switch operation for simple TGA.
De Praetere H, Vandesande J, Rega F, Daenen W, Marc G, Eyskens B, Heying R, Cools B, Meyns B
Acta Chir Belg 2014 Mar-Apr;114(2):92-8. PMID: 25073205
LEOPARD syndrome.
Massoure PL, Latremouille C, Lamblin G, Leca F
Cardiovasc J Afr 2012 Jul 12;23(6):e19-20. doi: 10.5830/CVJA-2012-011. PMID: 22832481
Imaging congenital heart disease in adults.
Kilner PJ
Br J Radiol 2011 Dec;84 Spec No 3(Spec Iss 3):S258-68. doi: 10.1259/bjr/74240815. PMID: 22723533Free PMC Article
Subvalvular pulmonic stenosis.
Khuddus MA, von Mering GO
Clin Cardiol 2006 Oct;29(10):468. doi: 10.1002/clc.4960291012. PMID: 17063954Free PMC Article

Therapy

Infundibular pulmonary stenosis after radiotherapy for breast cancer.
Hashimoto M, Koshima R, Doi H, Mitsube K
J Card Surg 2017 Mar;32(3):196-198. Epub 2017 Feb 14 doi: 10.1111/jocs.13111. PMID: 28194811
Prosthetic aortic valve endocarditis complicated with annular abscess, sub-aortic obstruction and valve dehiscence.
Hassoulas J, Patrianakos AP, Parthenakis FI, Vardas PE
Hellenic J Cardiol 2009 Jul-Aug;50(4):319-23. PMID: 19622502
Pseudo-Bartter syndrome in a neonate on prostaglandin infusion.
Vanhaesebrouck S, Allegaert K, Vanhole C, Devlieger H, Gewillig M, Proesmans W
Eur J Pediatr 2003 Sep;162(9):569-71. Epub 2003 Jun 17 doi: 10.1007/s00431-003-1201-3. PMID: 12811550
Pulmonary infundibular stenosis, coronary artery disease, and aortic regurgitation caused by mediastinal radiation.
Horimoto M, Igarashi K, Takenaka T, Batra S
Am Heart J 1993 Oct;126(4):1002-5. doi: 10.1016/0002-8703(93)90723-m. PMID: 8213419
Late cardiac effects of mediastinal radiotherapy in patients with Hodgkin's disease.
Pohjola-Sintonen S, Tötterman KJ, Salmo M, Siltanen P
Cancer 1987 Jul 1;60(1):31-7. doi: 10.1002/1097-0142(19870701)60:1<31::aid-cncr2820600107>3.0.co;2-d. PMID: 3581032

Prognosis

20 years of arterial switch operation for simple TGA.
De Praetere H, Vandesande J, Rega F, Daenen W, Marc G, Eyskens B, Heying R, Cools B, Meyns B
Acta Chir Belg 2014 Mar-Apr;114(2):92-8. PMID: 25073205
Dextro-transposition of the great arteries in a neonate with ventricular septal defect and pulmonary stenosis complicated by premature closure of the ductus arteriosus and possible pathophysiology of both defects.
Nakajima J, Kawakami T, Takeuchi K, Tsuchiya K
Pediatr Cardiol 2013;34(8):2009-12. Epub 2012 Nov 2 doi: 10.1007/s00246-012-0557-4. PMID: 23117331
LEOPARD syndrome.
Massoure PL, Latremouille C, Lamblin G, Leca F
Cardiovasc J Afr 2012 Jul 12;23(6):e19-20. doi: 10.5830/CVJA-2012-011. PMID: 22832481
Characteristics and management of cleft mitral valve.
Fraisse A, Massih TA, Kreitmann B, Metras D, Vouhé P, Sidi D, Bonnet D
J Am Coll Cardiol 2003 Dec 3;42(11):1988-93. doi: 10.1016/j.jacc.2003.07.019. PMID: 14662264
Early hypercyanotic spells unrelated to ductal constriction in tricuspid atresia with infundibular stenosis.
Holmes G, Epstein ML
Pediatr Cardiol 1993 Jul;14(3):151-4. doi: 10.1007/BF00795643. PMID: 7692429

Clinical prediction guides

LEOPARD syndrome.
Massoure PL, Latremouille C, Lamblin G, Leca F
Cardiovasc J Afr 2012 Jul 12;23(6):e19-20. doi: 10.5830/CVJA-2012-011. PMID: 22832481
Characteristics and management of cleft mitral valve.
Fraisse A, Massih TA, Kreitmann B, Metras D, Vouhé P, Sidi D, Bonnet D
J Am Coll Cardiol 2003 Dec 3;42(11):1988-93. doi: 10.1016/j.jacc.2003.07.019. PMID: 14662264
Unequal pressures in the central pulmonary arterial branches in patients with pulmonary stenosis. The influence of blood velocity and anatomy.
Muster AJ, van Grondelle A, Paul MH
Pediatr Cardiol 1982;2(1):7-14. doi: 10.1007/BF02265610. PMID: 7199713
Subpulmonary obstruction in congenitally corrected transposition of the great arteries due to ventricular membranous septal aneurysms.
Krongrad E, Ellis K, Steeg CN, Bowman FO Jr, Malm JR, Gersony WM
Circulation 1976 Oct;54(4):679-83. doi: 10.1161/01.cir.54.4.679. PMID: 986879
Echocardiographic differentiation of infundibular from valvular pulmonary stenosis.
Weyman AE, Dillon JC, Feigenbaum H, Chang S
Am J Cardiol 1975 Jul;36(1):21-6. doi: 10.1016/0002-9149(75)90862-0. PMID: 125035

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