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Congenital heart disease
Pulmonary stenosis: update on diagnosis and therapeutic options
  1. Judith Anne Adriane Ellen Cuypers,
  2. Maarten Witsenburg,
  3. Denise van der Linde,
  4. Jolien W Roos-Hesselink
  1. Department of Cardiology, Erasmus Medical Center, Rotterdam, The Netherlands
  1. Correspondence to Professor Jolien W Roos-Hesselink, Department of Cardiology, Ba-583a, Erasmus MC, ‘s-Gravendijkwal 230, Rotterdam 3015 CE, The Netherlands; j.roos{at}

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Pulmonary stenosis (PS) accounts for approximately 8% of all congenital heart defects.1 Valvular PS is usually an isolated defect, but it can be associated with other congenital heart defects, such as atrial septal defect (ASD), ventricular septal defect (VSD), and persistent ductus arteriosus. Combined valvular and infundibular PS can be part of tetralogy of Fallot (ToF).

The clinical presentation of PS may vary from critical stenosis in the newborn, to asymptomatic mild stenosis without need for therapy throughout life. The need for treatment of critical PS in the newborn is obvious, but the optimal timing, type of treatment, and follow-up strategy for the asymptomatic patient is less well defined.


PS occurs in about 1 per 2000 live births worldwide.1 The prevalence seems to be steadily increasing over time (figure 1A). There is a slightly higher birth prevalence in Asia as compared to Europe and the USA1 (figure 1B). The underlying cause is not well known, but should be sought in genetic, environmental, and dietary factors. There is increasing evidence that epigenetic modifications play an important role in certain diseases apart from genetics, and this might also be true for PS.w1 w2 Since an increasing number of patients had surgical or interventional repair during childhood, more and more adults will be seen with long term residual lesions like pulmonary regurgitation (PR) and restenosis.

Figure 1

Birth prevalence of pulmonary stenosis. (A) Time course of birth prevalence until 2010. (B) Reported birth prevalence per continent. Adapted with permission from van der Linde et al.1

Morphologic classification and aetiology

PS can be valvular, subvalvular (infundibular) or supravalvular (figure 2A). Valvular stenosis is by far the most common form.

Figure 2

Morphology of pulmonary stenosis (PS). (A) Schematic representation of different types of PS. RA, right atrium; RV, right ventricle. (B) Doming pulmonary …

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  • Contributors All authors have made significant contributions to the design, literature review and writing of this manuscript.

  • Competing interests In compliance with EBAC/EACCME guidelines, all authors participating in Education in Heart have disclosed potential conflicts of interest that might cause a bias in the article. The authors have no competing interests.

  • Provenance and peer review Commissioned; internally peer reviewed.