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Subpulmonary stenosis

MedGen UID:
757434
•Concept ID:
C3165028
•
Congenital Abnormality; Disease or Syndrome
Synonyms: Infundibular pulmonary stenosis; Infundibular pulmonic stenosis; subpulmonary stenosis; Subvalvar pulmonary stenosis; Subvalvar pulmonic stenosis; Subvalvular pulmonary stenosis; Subvalvular pulmonic stenosis
SNOMED CT: Subpulmonary stenosis (448476001)
 
HPO: HP:0034348
Monarch Initiative: MONDO:0017866
Orphanet: ORPHA3190

Definition

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

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • CROGVSubpulmonary stenosis
Follow this link to review classifications for Subpulmonary stenosis in Orphanet.

Conditions with this feature

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.
See: Condition Record
Cardiac valvular defect, developmental

Professional guidelines

PubMed

Percutaneous balloon valvuloplasty for the treatment of pulmonary valve stenosis in children - a single centre experience.
Weryński P, Rudziński A, Król-Jawień W, Kuźma J
Kardiol Pol 2009 Apr;67(4):369-75. PMID: 19492249

Recent clinical studies

Etiology

Successful long-term ventricular pacing via the coronary sinus after the Fontan operation.
Rosenthal E, Qureshi SA, Crick JC
Pacing Clin Electrophysiol 1995 Nov;18(11):2103-5. doi: 10.1111/j.1540-8159.1995.tb03874.x. PMID: 8552527
A bicuspid pulmonary valve is not a contraindication for the arterial switch operation.
Uemura H, Yagihara T, Kawashima Y, Yamamoto F, Nishigaki K, Matsuki O, Kamiya T, Ho SY, Anderson RH
Ann Thorac Surg 1995 Feb;59(2):473-6. doi: 10.1016/0003-4975(94)00861-z. PMID: 7847969
Right-sided heart with mirror-image atrial arrangement, complete transposition, ventricular septal defect and subpulmonary stenosis.
Davidson A, Luhmer I, Kallfelz HC
Int J Cardiol 1989 Feb;22(2):264-7. doi: 10.1016/0167-5273(89)90077-6. PMID: 2914748
Morphology of ventricular septal defect in complete transposition of the great arteries.
Moene RJ, Oppenheimer-Dekker A, Wenink AC, Bartelings MM, Gittenberger-de Groot AC
Am J Cardiol 1985 Jun 1;55(13 Pt 1):1566-70. doi: 10.1016/0002-9149(85)90974-9. PMID: 4003300
Surgical treatment of double-outlet left ventricle in 2 patients with D-position and L-position of the aorta.
Stegmann T, Oster H, Bissenden J, Kallfelz HC, Oelert H
Ann Thorac Surg 1979 Feb;27(2):121-9. doi: 10.1016/s0003-4975(10)63251-1. PMID: 453970

Diagnosis

Anesthesia and LEOPARD syndrome: a review of forty-nine anesthetic exposures.
Yeoh TY, Wittwer ED, Weingarten TN, Sprung J
J Cardiothorac Vasc Anesth 2014 Oct;28(5):1243-50. Epub 2014 Jan 23 doi: 10.1053/j.jvca.2013.09.015. PMID: 24461361
Eisenmenger ventricular septal defect: classification, morphology, and indications for surgery.
Chen H, Xu Z, Wang S, Shen J, Zhang Z, Hong H
Pediatr Cardiol 2011 Jan;32(1):17-23. Epub 2010 Nov 17 doi: 10.1007/s00246-010-9797-3. PMID: 21079941
Whole-heart magnetic resonance imaging of isolated subpulmonary stenosis associated with hypertrophic cardiomyopathy.
Matsumoto N, Sato Y, Nagao K
Can J Cardiol 2009 Oct;25(10):e361. doi: 10.1016/s0828-282x(09)70725-1. PMID: 19812812Free PMC Article
Cyanotic congenital heart disease with decreased pulmonary blood flow in children.
Waldman JD, Wernly JA
Pediatr Clin North Am 1999 Apr;46(2):385-404. doi: 10.1016/s0031-3955(05)70125-5. PMID: 10218082
Right-sided heart with mirror-image atrial arrangement, complete transposition, ventricular septal defect and subpulmonary stenosis.
Davidson A, Luhmer I, Kallfelz HC
Int J Cardiol 1989 Feb;22(2):264-7. doi: 10.1016/0167-5273(89)90077-6. PMID: 2914748

Therapy

Anesthesia and LEOPARD syndrome: a review of forty-nine anesthetic exposures.
Yeoh TY, Wittwer ED, Weingarten TN, Sprung J
J Cardiothorac Vasc Anesth 2014 Oct;28(5):1243-50. Epub 2014 Jan 23 doi: 10.1053/j.jvca.2013.09.015. PMID: 24461361
Palliative arterial switch as first-line treatment before the fontan procedure in patients with single-ventricle physiology and subaortic stenosis.
Gil-Jaurena JM, Zabala JI, Albert DC, Castillo R, González M, Miró L
Rev Esp Cardiol (Engl Ed) 2013 Jul;66(7):553-5. Epub 2013 May 15 doi: 10.1016/j.rec.2013.01.020. PMID: 24776204
Percutaneous balloon valvuloplasty for the treatment of pulmonary valve stenosis in children - a single centre experience.
Weryński P, Rudziński A, Król-Jawień W, Kuźma J
Kardiol Pol 2009 Apr;67(4):369-75. PMID: 19492249
Pacing lead adhesions after long-term ventricular pacing via the coronary sinus.
Rosenthal E, Cook A
Pacing Clin Electrophysiol 1999 Dec;22(12):1846-8. doi: 10.1111/j.1540-8159.1999.tb00427.x. PMID: 10642147
Anatomic causes of pulmonary stenosis in complete transposition.
Shrivastava S, Tadavarthy SM, Fukuda T, Edwards JE
Circulation 1976 Jul;54(1):154-9. doi: 10.1161/01.cir.54.1.154. PMID: 776439

Prognosis

Cyanotic congenital heart disease with decreased pulmonary blood flow in children.
Waldman JD, Wernly JA
Pediatr Clin North Am 1999 Apr;46(2):385-404. doi: 10.1016/s0031-3955(05)70125-5. PMID: 10218082
Successful long-term ventricular pacing via the coronary sinus after the Fontan operation.
Rosenthal E, Qureshi SA, Crick JC
Pacing Clin Electrophysiol 1995 Nov;18(11):2103-5. doi: 10.1111/j.1540-8159.1995.tb03874.x. PMID: 8552527
A bicuspid pulmonary valve is not a contraindication for the arterial switch operation.
Uemura H, Yagihara T, Kawashima Y, Yamamoto F, Nishigaki K, Matsuki O, Kamiya T, Ho SY, Anderson RH
Ann Thorac Surg 1995 Feb;59(2):473-6. doi: 10.1016/0003-4975(94)00861-z. PMID: 7847969
Right-sided heart with mirror-image atrial arrangement, complete transposition, ventricular septal defect and subpulmonary stenosis.
Davidson A, Luhmer I, Kallfelz HC
Int J Cardiol 1989 Feb;22(2):264-7. doi: 10.1016/0167-5273(89)90077-6. PMID: 2914748
Surgical treatment of double-outlet left ventricle in 2 patients with D-position and L-position of the aorta.
Stegmann T, Oster H, Bissenden J, Kallfelz HC, Oelert H
Ann Thorac Surg 1979 Feb;27(2):121-9. doi: 10.1016/s0003-4975(10)63251-1. PMID: 453970

Clinical prediction guides

Tetralogy of Fallot: nosological, morphological, and morphogenetic considerations.
Anderson RH, Spicer DE, Giroud JM, Mohun TJ
Cardiol Young 2013 Dec;23(6):858-66. doi: 10.1017/S1047951113001686. PMID: 24401259
Subpulmonary stenosis assessed in midtrimester fetuses with tetralogy of Fallot: a novel method for predicting postnatal clinical outcome.
Kwon EN, Parness IA, Srivastava S, Nielsen JC, Geiger M
Pediatr Cardiol 2013 Aug;34(6):1314-20. Epub 2013 Feb 7 doi: 10.1007/s00246-013-0642-3. PMID: 23389098
Percutaneous balloon valvuloplasty for the treatment of pulmonary valve stenosis in children - a single centre experience.
Weryński P, Rudziński A, Król-Jawień W, Kuźma J
Kardiol Pol 2009 Apr;67(4):369-75. PMID: 19492249
The ventricular septal defect in complete transposition of the great arteries: pathologic anatomy in 57 cases with emphasis on subaortic, subpulmonary, and aortic arch obstruction.
Milanesi O, Ho SY, Thiene G, Frescura C, Anderson RH
Hum Pathol 1987 Apr;18(4):392-6. doi: 10.1016/s0046-8177(87)80171-5. PMID: 3557442

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