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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

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
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
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
Institutional experience with a protocol of early primary repair of double-outlet right ventricle.
Tchervenkov CI, Marelli D, Béland MJ, Gibbons JE, Paquet M, Dobell AR
Ann Thorac Surg 1995 Dec;60(6 Suppl):S610-3. doi: 10.1016/0003-4975(95)00855-1. PMID: 8604947

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
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
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
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
Two-dimensional echocardiographic and angiocardiographic diagnosis of subpulmonary stenosis due to tricuspid valve pouch in complete transposition of the great arteries.
Riggs TW, Muster AJ, Aziz KU, Paul MH, Ilbawi M, Idriss FS
J Am Coll Cardiol 1983 Feb;1(2 Pt 1):484-91. doi: 10.1016/s0735-1097(83)80077-1. PMID: 6826959

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
Impaired myocardial blood flow and coronary flow reserve of the anatomical right systemic ventricle in patients with congenitally corrected transposition of the great arteries.
Hauser M, Bengel FM, Hager A, Kuehn A, Nekolla SG, Kaemmerer H, Schwaiger M, Hess J
Heart 2003 Oct;89(10):1231-5. doi: 10.1136/heart.89.10.1231. PMID: 12975428Free PMC Article
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

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
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
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
Dacron conduit with a stented porcine xenograft valve in the anatomic correction of transposition of the great arteries and subpulmonary stenosis.
Gonzalez-Lavin L, Sparrow AW
Thorax 1975 Dec;30(6):644-9. doi: 10.1136/thx.30.6.644. PMID: 130694Free PMC Article

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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