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Doppler Echocardiography in Infancy and Childhood pp 95–110Cite as

Pulmonary Stenosis

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Abstract

Pulmonary stenosis may occur at valvular, subvalvular and supravalvular level. The morphology and size of the right ventricular outflow tract, the pulmonary valve, the main pulmonary artery and the pulmonary bifurcation can be well displayed by two-dimensional echocardiography. Colour Doppler reveals possible sites of obstruction and direction of the jet in valvular stenoses. It is the method of choice for assessment of pulmonary and tricuspid regurgitation. Pulsed wave Doppler and continuous wave Doppler provide the possibility of noninvasive determination of gradients in the right ventricular outflow tract as well as determination of right ventricular systolic pressure by interrogation of tricuspid regurgitation.

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References

  • Aldousany AW, DiSessa TG et al (1989) Doppler estimation of pressure gradient in pulmonary stenosis: maximal instantaneous vs peak-to-peak, vs mean catheter gradient. Pediatr Cardiol 10(3):145–149

    Article  CAS  PubMed  Google Scholar 

  • Alva C, Ho SY et al (1999) The nature of the obstructive muscular bundles in double-chambered right ventricle. J Thorac Cardiovasc Surg 117(6):1180–1189

    Article  CAS  PubMed  Google Scholar 

  • Bashore TM (2007) Adult congenital heart disease: right ventricular outflow tract lesions. Circulation 115(14):1933–1947

    Article  PubMed  Google Scholar 

  • Baumstark A, Fellows KE et al (1978) Combined double chambered right ventricle and discrete subaortic stenosis. Circulation 57(2):299–303

    Article  CAS  PubMed  Google Scholar 

  • Bove T, Francois K et al (2012) Assessment of a right-ventricular infundibulum-sparing approach in transatrial-transpulmonary repair of tetralogy of Fallot. Eur J Cardiothorac Surg 41(1):126–133

    PubMed  Google Scholar 

  • Brown DW, McElhinney DB et al (2012) Reliability and accuracy of echocardiographic right heart evaluation in the U.S. Melody Valve Investigational Trial. J Am Soc Echocardiogr 25(4):383–392.e4

    Article  PubMed  Google Scholar 

  • Buheitel G, Hofbeck M et al (1999) Incidence and treatment of reactive infundibular obstruction after balloon dilatation of critical pulmonary valve stenoses. Z Kardiol 88(5):347–352

    Article  CAS  PubMed  Google Scholar 

  • Burch M, Sharland M et al (1993) Cardiologic abnormalities in Noonan syndrome: phenotypic diagnosis and echocardiographic assessment of 118 patients. J Am Coll Cardiol 22(4):1189–1192

    Article  CAS  PubMed  Google Scholar 

  • Chaowalit N, Durongpisitkul K et al (2012) Echocardiography as a simple initial tool to assess right ventricular dimensions in patients with repaired tetralogy of fallot before undergoing pulmonary valve replacement: comparison with cardiovascular magnetic resonance imaging. Echocardiography 29(10):1239–1246

    Article  PubMed  Google Scholar 

  • Chatelain P, Oberhansli I et al (1993) Physiological pulmonary branch stenosis in newborns: 2D-echocardiographic and Doppler characteristics and follow up. Eur J Pediatr 152(7):559–563

    Article  CAS  PubMed  Google Scholar 

  • Collins RT 2nd (2013) Cardiovascular disease in Williams syndrome. Circulation 127(21):2125–2134

    Article  PubMed  Google Scholar 

  • Duncan WJ, Fowler RS et al (1981) A comprehensive scoring system for evaluating Noonan syndrome. Am J Med Genet 10(1):37–50

    Article  CAS  PubMed  Google Scholar 

  • Eicken A, Ewert P et al (2011) Percutaneous pulmonary valve implantation: two-centre experience with more than 100 patients. Eur Heart J 32(10):1260–1265

    Article  PubMed  Google Scholar 

  • Elzenga NJ, von Suylen RJ et al (1990) Juxtaductal pulmonary artery coarctation. An underestimated cause of branch pulmonary artery stenosis in patients with pulmonary atresia or stenosis and a ventricular septal defect. J Thorac Cardiovasc Surg 100(3):416–424

    CAS  PubMed  Google Scholar 

  • Feigenbaum H, Armstrong WF et al (2005) Feigenbaum’s echocardiography. Lippincott Williams & Wilkins, Baltimore/New York/London/Philadelphia

    Google Scholar 

  • Freedom RM, Benson L (2004) Congenital pulmonary stenosis and isolated pulmonary insufficiency. In: Freedom RM, Yoo SJ, Mikailian H, Williams WG (eds) The natural and modified history of congenital heart disease. Elmsford, New York, Futura

    Google Scholar 

  • Galal O, Al-Halees Z et al (2000) Double-chambered right ventricle in 73 patients: spectrum of the disease and surgical results of transatrial repair. Can J Cardiol 16(2):167–174

    CAS  PubMed  Google Scholar 

  • Gerlis LM (1999) The prevalence of bifoliate pulmonary valves. Cardiol Young 9(5):499–502

    Article  CAS  PubMed  Google Scholar 

  • Grosse-Wortmann L, Yoo SJ (2010) Magnetic resonance imaging and computed tomography. In: Anderson RH, Baker EJ, Penny DJ et al (eds) Pediatric cardiology. Churchill Livingstone/Elsevier, Philadelphia

    Google Scholar 

  • Koch A, Buheitel G et al (2003) Spectrum of arterial obstructions caused by one elastin gene point mutation. Eur J Pediatr 162(1):53–54

    Article  PubMed  Google Scholar 

  • Lancellotti P, Tribouilloy C et al (2010) European Association of Echocardiography recommendations for the assessment of valvular regurgitation. Part 1: aortic and pulmonary regurgitation (native valve disease). Eur J Echocardiogr 11(3):223–244

    Article  PubMed  Google Scholar 

  • Lancellotti P, Tribouilloy C et al (2013) Recommendations for the echocardiographic assessment of native valvular regurgitation: an executive summary from the European Association of Cardiovascular Imaging. Eur Heart J Cardiovasc Imaging 14(7):611–644

    Article  PubMed  Google Scholar 

  • Lee ST, Lin MH (2010) Color Doppler echocardiographic assessment of valvular regurgitation in normal infants. J Formos Med Assoc 109(1):56–61

    Article  PubMed  Google Scholar 

  • Lemler MS, Ramaciotti C (2009) Anomalies of the right ventricular outflow tract and pulmonary valve. In: Lai WW, Mertens LL, Cohen MS, Geva T (eds) Echocardiography in pediatric and congenital heart disease. Blackwell Publishing Ltd, Chichester

    Google Scholar 

  • Lindinger A, Schwedler G et al (2010) Prevalence of congenital heart defects in newborns in Germany: results of the first registration year of the PAN study (july 2006 to june 2007). Klin Padiatr 222(5):321–326

    Article  CAS  PubMed  Google Scholar 

  • Luhmer I, Ziemer G (1993) Coarctation of the pulmonary artery in neonates. Prevalence, diagnosis, and surgical treatment. J Thorac Cardiovasc Surg 106(5):889–894

    CAS  PubMed  Google Scholar 

  • Martinez RM, Anderson RH (2005) Echocardiographic features of the morphologically right ventriculo-arterial junction. Cardiol Young 15(Suppl 1):17–26

    Article  PubMed  Google Scholar 

  • McAleer E, Kort S et al (2001) Unusual echocardiographic views of bicuspid and tricuspid pulmonic valves. J Am Soc Echocardiogr 14(10):1036–1038

    Article  CAS  PubMed  Google Scholar 

  • Mertens LL, Rigby ML et al (2010) Cross sectional echocardiographic and Doppler imaging. In: Anderson RH, Baker EJ, Penny DJ et al (eds) Pediatric cardiology. Churchill Livingstone/Elsevier, Philadelphia

    Google Scholar 

  • Metcalfe K, Rucka AK et al (2000) Elastin: mutational spectrum in supravalvular aortic stenosis. Eur J Hum Genet 8(12):955–963

    Article  CAS  PubMed  Google Scholar 

  • Momma K, Takao A et al (1986) Juxtaductal left pulmonary artery obstruction in pulmonary atresia. Br Heart J 55(1):39–44

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  • Musewe NN, Robertson MA et al (1987) The dysplastic pulmonary valve: echocardiographic features and results of balloon dilatation. Br Heart J 57(4):364–370

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  • Pankau R, Siebert R et al (2001) Familial Williams-Beuren syndrome showing varying clinical expression. Am J Med Genet 98(4):324–329

    Article  CAS  PubMed  Google Scholar 

  • Puchalski MD, Askovich B et al (2008) Pulmonary regurgitation: determining severity by echocardiography and magnetic resonance imaging. Congenit Heart Dis 3(3):168–175

    Article  PubMed  Google Scholar 

  • Restivo A, Cameron AH et al (1984) Divided right ventricle: a review of its anatomical varieties. Pediatr Cardiol 5(3):197–204

    Article  CAS  PubMed  Google Scholar 

  • Roberts AE, Allanson JE et al (2013) Noonan syndrome. Lancet 381(9863):333–342

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  • Romano AA, Allanson JE et al (2010) Noonan syndrome: clinical features, diagnosis, and management guidelines. Pediatrics 126(4):746–759

    Article  PubMed  Google Scholar 

  • Said SM, Burkhart HM et al (2012) Outcomes of surgical repair of double-chambered right ventricle. Ann Thorac Surg 93(1):197–200

    Article  PubMed  Google Scholar 

  • Schwedler G, Lindinger A et al (2011) Frequency and spectrum of congenital heart defects among live births in Germany : a study of the competence network for congenital heart defects. Clin Res Cardiol Off J German Cardiac Soc 100(12):1111–1117

    Article  Google Scholar 

  • Silversides CK, Veldtman GR et al (2003) Pressure half-time predicts hemodynamically significant pulmonary regurgitation in adult patients with repaired tetralogy of fallot. J Am Soc Echocardiogr 16(10):1057–1062

    Article  PubMed  Google Scholar 

  • Silvilairat S, Cabalka AK et al (2005a) Echocardiographic assessment of isolated pulmonary valve stenosis: which outpatient Doppler gradient has the most clinical validity? J Am Soc Echocardiogr 18(11):1137–1142

    Article  PubMed  Google Scholar 

  • Silvilairat S, Cabalka AK et al (2005b) Outpatient echocardiographic assessment of complex pulmonary outflow stenosis: Doppler mean gradient is superior to the maximum instantaneous gradient. J Am Soc Echocardiogr 18(11):1143–1148

    Article  PubMed  Google Scholar 

  • Snider RA, Serwer GA et al (1997) Echocardiography in pediatric heart disease. Mosby, St. Louis

    Google Scholar 

  • So BH, Watanabe T et al (1996) Doppler assessment of physiological stenosis at the bifurcation of the main pulmonary artery: a cause of functional murmur in neonates. Biol Neonate 69(4):243–248

    Article  CAS  PubMed  Google Scholar 

  • Turnpenny PD, Ellard S (2012) Alagille syndrome: pathogenesis, diagnosis and management. Eur J Hum Genet 20(3):251–257

    Article  CAS  PubMed  Google Scholar 

  • Vogel M, Smallhorn JF et al (1988) An echocardiographic study of the association of ventricular septal defect and right ventricular muscle bundles with a fixed subaortic abnormality. Am J Cardiol 61(10):857–860

    Article  CAS  PubMed  Google Scholar 

  • Wessel A, Pankau R et al (1994) Three decades of follow-up of aortic and pulmonary vascular lesions in the Williams-Beuren syndrome. Am J Med Genet 52(3):297–301

    Article  CAS  PubMed  Google Scholar 

  • Yang H, Pu M et al (2008) Quantitative assessment of pulmonary insufficiency by Doppler echocardiography in patients with adult congenital heart disease. J Am Soc Echocardiogr 21(2):157–164

    Article  PubMed  Google Scholar 

  • Zalzstein E, Moes CA et al (1991) Spectrum of cardiovascular anomalies in Williams-Beuren syndrome. Pediatr Cardiol 12(4):219–223

    Article  CAS  PubMed  Google Scholar 

Download references

Author information

Authors and Affiliations

  1. Universitätskinderklinik Tübingen Abt. Kinderkardiologie, Tübingen, Germany

    Michael Hofbeck

  2. Pediatric Clinic of the Sozialstiftung Bamberg, Bamberg, Germany

    Karl-Heinz Deeg

  3. Klinikum Bayreuth GmbH Klinik für Kinderheilkunde/Jugendmedizin, Bayreuth, Germany

    Thomas Rupprecht

Authors
  1. Michael Hofbeck
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  2. Karl-Heinz Deeg
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  3. Thomas Rupprecht
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7.1 Electronic Supplementary Material

Video 7.1

The apical four-chamber view in a neonate with critical pulmonary stenosis shows significant apical hypertrophy of the right ventricle. The atrial septum bulges in diastole from right to left (AVI 15122 kb)

Video 7.2

Colour Doppler in the apical four-chamber view shows severe tricuspid regurgitation (same patient as in Video 7.2) (AVI 4036 kb)

Video 7.3

Parasternal long-axis view of the right ventricular outflow tract in a neonate with severe pulmonary valve stenosis shows limited separation (doming) of the valve in systole (AVI 22853 kb)

Video 7.4

Colour Doppler in the parasternal long-axis view of the right ventricular outflow tract (same patient as in Video 7.3) shows acceleration of flow in systole and minor pulmonary regurgitation in diastole (AVI 4961 kb)

Video 7.5

The parasternal short-axis view in a neonate with critical pulmonary stenosis displays significantly thickened leaflets of the pulmonary valve resulting in severely restricted opening of the valve (AVI 5014 kb)

Video 7.6

In a patient with Noonan syndrome and dysplastic pulmonary valve, the parasternal short-axis view shows narrowing at the sinotubular junction. Although the valve was dysplastic and thickened (proven by angiocardiography), this finding is not demonstrated by echocardiography (AVI 10028 kb)

Video 7.7

Colour Doppler in the parasternal short-axis view (same patient as in Video 7.6) shows turbulence and significant acceleration of flow starting at valvular level (AVI 3598 kb)

Video 7.8

The high left parasternal short-axis view in a newborn with stenotic bicuspid valve shows thickening and incomplete separation of the valve leaflets (AVI 8674 kb)

Video 7.9

Significant thickening of the valve leaflets in the setting of a tricuspid pulmonary valve is present in this neonate with critical pulmonary stenosis (AVI 18851 kb)

Video 7.10

Colour Doppler in the parasternal short-axis view at the level just below the aortic valve in a child with double-chambered right ventricle shows obstruction due to anomalous muscle bundles well below the pulmonary valve (WMV 1540 kb)

Video 7.11

Obstructing anomalous muscles in an adolescent with double-chambered right ventricle are well displayed in the parasternal short-axis view at the level of the aortic valve (same patient as in Video (WMV 1290 kb)

Video 7.12

Colour Doppler in the subcostal coronal view shows significant obstruction due to anomalous muscle bundles below the outflow tract in this child with double-chambered right ventricle (AVI 3224 kb)

Video 7.13

Colour Doppler in the parasternal short-axis view at the level of the pulmonary bifurcation in a neonate with tetralogy of Fallot shows stenosis at the origin of the left pulmonary artery due to narrowing by ductal tissue (AVI 2470 kb)

Video 7.14

Colour Doppler in the parasternal short-axis view in an infant with Williams-Beuren syndrome shows turbulence and acceleration of flow in the small left due to stenosis of the pulmonary bifurcation (AVI 7391 kb)

Video 7.15

Colour Doppler in the subcostal coronal view of the right ventricular outflow tract in a newborn with critical pulmonary valve stenosis shows significant obstruction and acceleration of flow at valvular level (AVI 3187 kb)

Video 7.16

Colour Doppler in the parasternal long-axis view of the right ventricular outflow tract shows minor amount of physiological pulmonary regurgitation in a 4-year-old child with normal cardiovascular findings (AVI 4301 kb)

Video 7.17

Colour Doppler in the subcostal RAO view of a child with combined pulmonary stenosis and regurgitation shows acceleration of flow during systole due to obstruction at valvular level and significant pulmonary regurgitation directed towards the transducer in diastole. Note the dilated central pulmonary arteries (AVI 15597 kb)

Video 7.18

Colour Doppler in the parasternal short-axis view of the pulmonary bifurcation shows reverse flow in the main and central pulmonary arteries indicating severe pulmonary regurgitation in a 3-year-old child following repair of tetralogy of Fallot. Pulmonary regurgitation in this case can be explained by the requirement of a transannular patch (AVI 2494 kb)

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Hofbeck, M., Deeg, KH., Rupprecht, T. (2017). Pulmonary Stenosis. In: Doppler Echocardiography in Infancy and Childhood. Springer, Cham. https://doi.org/10.1007/978-3-319-42919-9_7

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  • DOI: https://doi.org/10.1007/978-3-319-42919-9_7

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  • Publisher Name: Springer, Cham

  • Print ISBN: 978-3-319-42917-5

  • Online ISBN: 978-3-319-42919-9

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