Department of Cardiothoracic Surgery, The First Hospital of Putian, Teaching Hospital, Fujian Medical University, Putian, Fujian Province, People’s Republic of China


Introduction: Congenital supravalvular pulmonary stenosis (SVPS) is rare, whereas acquired SVPS is increasingly reported due to surgical repair of complex congenital heart defects specially developed for pediatric patients and prolonged follow-up. The clinical features and the genetic basis of this entity are scantily described.

Materials and methods: Comprehensive literature retrieval for collecting articles published in English from 2000 to 2016 was the research material of this article.

Results: A total of 34 articles were collected with 333 patients involved: 20 (6.0%) of the SVPS cases were congenital and 313 (94.0%) were acquired. Totally 12 (3.6%) patients were in association with syndromes. Postoperative SVPS occured at a mean of 82.7 months following the surgical operation. Management strategies for postoperative SVPS were medical in 1.6%, interventional in 55.6%, surgical in 41.1% and hybrid in 0.8% of the patients. The reintervention rate was 15.9% in the interventional group. The ove- rall mortality was 0.6%.

Conclusion: The incidence of postoperative SVPS depends on the follow-up period after surgical operation and the diagnostic criteria of SVPS. For avoiding postoperative SVPS, careful preoperative assessment of the potential surgical outcomes in relation to clinical, hemodynamic, morphological and genetic investigations is mandatory for the decision-making of choices of optimal operati- ve techniques.


congenital heart defects, complication, pulmonary stenosis