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Congenital heart disease
Original article
The unnatural history of pulmonary stenosis up to 40 years after surgical repair
  1. Judith A AE Cuypers1,
  2. Myrthe E Menting1,
  3. Petra Opić1,
  4. Elisabeth M WJ Utens2,
  5. Willem A Helbing3,
  6. Maarten Witsenburg1,
  7. Annemien E van den Bosch1,
  8. Ron T van Domburg1,
  9. Sara J Baart1,
  10. Eric Boersma1,
  11. Folkert J Meijboom4,
  12. Ad J JC Bogers5,
  13. Jolien W Roos-Hesselink1
  1. 1Department of Cardiology, Erasmus MC, Rotterdam, The Netherlands
  2. 2Department of Child and Adolescent Psychiatry/Psychology, Erasmus MC-Sophia Children's Hospital, Rotterdam, The Netherlands
  3. 3Department of Pediatrics, Division of Cardiology, Erasmus MC-Sophia Children's Hospital, Rotterdam, The Netherlands
  4. 4Department of Cardiology, University Medical Center Utrecht, Utrecht, The Netherlands
  5. 5Department of Cardio-thoracic Surgery, Erasmus MC, Rotterdam, The Netherlands
  1. Correspondence to J A A E Cuypers, Department of Cardiology, Erasmus Medical Center, PO Box 2040, Rotterdam 3015 CA, The Netherlands; j.cuypers{at}erasmusmc.nl

Abstract

Objective To provide prospective information on long-term outcome after surgical correction of valvular pulmonary stenosis (PS).

Methods Fifty-three consecutive patients operated for PS during childhood between 1968 and 1980 in one centre are followed longitudinally for 37±3.4 years, including extensive in-hospital examination every 10 years.

Results Survival information was available in 100% of the original 53 patients. Cumulative survival was 94% at 20 years and 91% at 40 years. Excluding perioperative mortality (<30 days), survival was 94% at 40 years. Of 46 eligible survivors, 29 participated in the in-hospital examination and 15 gave permission to use their hospital records (96% participation). Cumulative event-free survival was 68% after 40 years: 25% needed a reintervention, 12% underwent pacemaker implantation and 9% had supraventricular arrhythmias. Early reinterventions were mainly for residual PS, late reinterventions for pulmonary regurgitation. Subjective health status was good. Exercise capacity was normal in 74% (median 96 (82–107)% of expected workload). Right ventricular and left ventricular (LV) dysfunction was found in 13% and 41%, respectively. The use of a transannular patch and younger age at surgery were predictive for late events (HR 3.02 (95% CI 1.09 to 8.37) and HR 0.81/year (95% CI 0.66 to 0.98), respectively). Use of inflow occlusion compared with cardiopulmonary bypass showed a trend towards more reinterventions (HR 3.19 (95% CI 0.97 to 10.47)).

Conclusions Survival up to 40 years after successful PS repair is nearly normal. Subjective health status is good and there is a low incidence of arrhythmias. Reinterventions, however, are necessary in one-quarter and 40 years postoperatively several patients show LV dysfunction.

https://doi.org/10.1136/heartjnl-2015-309159

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    Footnotes

    • Contributors JAAEC: executive investigator, design and execution of the study, data-gathering, data entry, statistical analyses, interpretation of results, drafting the article. Additional statistical analyses and adaptation of the manuscript as requested by the editors and reviewer. MEM: data-gathering, data entry, statistical analyses, interpretation of results, co-drafting and revising the article. Additional statistical analyses and adaptation of the manuscript as requested by the editors and reviewer. PO: data-gathering, data entry, statistical analyses, interpretation of results, revising the article. EMWJU: concept and design, interpreting the results, revising the article. WAH: scientific input, data-gathering, revising the article. MW: scientific input, data-gathering, revising the article. AEvdB: scientific input, data-gathering, revising the article. RTvD: supervision of statistical analyses, revising the article. SJB: statistical analyses, interpretation of statistical results, revising the article. EB: statistical analyses, interpretation of statistical results, revising the article. FJM: concept and design, scientific input, data-gathering, revising the article. AJJCB: concept and design, scientific input, revising the article. JWR-H: principal investigator, concept and design, data gathering interpreting the results, revising the article.

    • Funding This work was supported by a grant from the Dutch Heart Foundation (grant number 2009-B-073).

    • Competing interests None declared.

    • Ethics approval Erasmus MC Medical Ethics Committee (2010-15).

    • Provenance and peer review Not commissioned; externally peer reviewed.

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