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Cardiology in the Young Cardiology in the Young

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Results of balloon pulmonary valvoplasty in children with Noonan’s syndrome

Published online by Cambridge University Press:  10 January 2018

Jonathon Holzmann ,
Shane M. Tibby ,
Eric Rosenthal ,
Shakeel Qureshi ,
Gareth Morgan  and
Thomas Krasemann
Jonathon Holzmann
Affiliation:
Department of Paediatric Cardiology, Evelina London Children’s Hospital, Westminster Bridge Road, London, UK
Shane M. Tibby
Affiliation:
Department of Paediatric Intensive Care, Evelina London Children’s Hospital, Westminster Bridge Road, London, UK
Eric Rosenthal
Affiliation:
Department of Paediatric Cardiology, Evelina London Children’s Hospital, Westminster Bridge Road, London, UK
Shakeel Qureshi
Affiliation:
Department of Paediatric Cardiology, Evelina London Children’s Hospital, Westminster Bridge Road, London, UK
Gareth Morgan
Affiliation:
Department of Paediatric Cardiology, Evelina London Children’s Hospital, Westminster Bridge Road, London, UK
Thomas Krasemann*
Affiliation:
Department of Paediatric Cardiology, Evelina London Children’s Hospital, Westminster Bridge Road, London, UK Department of Pediatric Cardiology, Sophia Kinderziekenhuis, Erasmus MC Rotterdam, Wytengaweg 80, Rotterdam, The Netherlands
*
Author for correspondence: T. Krasemann, Department of Pediatric Cardiology, Sophia Kinderziekenhuis, Erasmus MC Rotterdam, Wytengaweg 80, 300CH Rotterdam, The Netherlands. Tel: +31-(0)10 7032188; E-mail: t.krasemann@erasmusmc.nl
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Abstract

Pulmonary valve stenosis is common in patients with Noonan’s syndrome. The response to balloon valvoplasty varies.

We assessed the correlation between re-intervention rate, immediate response, and the progress of the valve gradient over time after intervention.

Methods

This is a retrospective study conducted from 1995 to 2014.

Results

Of 14 patients identified, seven had re-intervention 28±54 months (range 3–149, median 3.3) after valvoplasty. These patients did not have a significant decrease in gradient after intervention. Their gradient subsequently decreased during follow-up and then became static before increasing years after intervention. In contrast, the gradient of patients not requiring further intervention continually reduced over time. Demographics did not differ between these groups.

Conclusion

We could not identify predisposing factors for long-term success of pulmonary valvoplasty in Noonan’s patients, but the trajectory of gradients differs significantly between patients needing re-intervention from those who remain free from re-intervention.

Keywords

Pulmonary stenosisNoonan’s syndromevalvoplasty
Type
Original Articles
Information
Cardiology in the Young , Volume 28 , Issue 5 , May 2018 , pp. 647 - 652
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Copyright
© Cambridge University Press 2018 

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Footnotes

*

Work performed at the Department of Paediatric Cardiology, Evelina London Children’s Hospital, Westminster Bridge Road, London SE1 7EH, United Kingdom.

References

1. Prendiville, TW, Guvreau, K, Tworog-Dube, E, et al. Cardiovascular disease in Noonan syndrome. Arch Dis Child 2014; 99: 629–634.CrossRefGoogle ScholarPubMed
2. Masura, J, Burch, M, Deanfield, J, et al. Five-year follow-up after balloon pulmonary valvulopasty. J Am Coll Cardiol 1993; 21: 132–136.CrossRefGoogle Scholar
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4. Rao, P, Galal, O, Patnana, M, et al. Results of three to 10 year follow up of dilatation of the pulmonary valve. Heart 1998; 80: 591–595.CrossRefGoogle ScholarPubMed
5. McCrindle, B. Independent predictors of long-term results after balloon pulmonary valvuloplasty. Circulation 1994; 89: 1751–1759.CrossRefGoogle ScholarPubMed
6. Shaw, A, Kalidis, K, Crosby, A, et al. The natural history of Noonan syndrome: a long-term follow-up study. Arch Dis Child 2007; 92: 128–132.CrossRefGoogle ScholarPubMed
7. Jarrar, M, Betbout, F, Farhat, M, et al. Long-term invasive and noninvasive results of percutaneous balloon pulmonary valvuloplasty in children, adolescents and adults. Am Heart J 1999; 138: 950–954.CrossRefGoogle Scholar
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