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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
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
Left ventricular function in cyanotic congenital heart disease.
Graham TP Jr, Erath HG Jr, Boucek RJ Jr, Boerth RC
Am J Cardiol 1980 Jun;45(6):1231-6. doi: 10.1016/0002-9149(80)90483-x. PMID: 7377122

Diagnosis

Congenital supravalvular and subvalvular pulmonary stenosis with hypoplastic pulmonary annulus associated with congenital rubella syndrome.
Khanra D, Shrivastava Y, Duggal B, Soni S
BMJ Case Rep 2019 Jul 10;12(7) doi: 10.1136/bcr-2019-231008. PMID: 31296628Free PMC Article
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
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
Respiratory distress after surgery of RVOT pathologies: a word of caution on pseudoaneurysm development.
Antal AD, Cikirikcioglu M, Myers PO, Didier D, Kalangos A
Thorac Cardiovasc Surg 2010 Sep;58(6):356-8. Epub 2010 Sep 7 doi: 10.1055/s-0029-1185883. PMID: 20824590
Diagnosis by sheer coincidence.
Wróbel W, Sosnowski M, Wita K, Trusz-Gluza M
Int J Cardiovasc Imaging 2009 Oct;25(7):649-51. Epub 2009 Jul 1 doi: 10.1007/s10554-009-9473-y. PMID: 19568957
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
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

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
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
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
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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