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Valve-sparing root replacement in children with connective tissue disease: Long-term risk of aortic events

Ilkun Park1, Ji-Hyuk Yang1, Kiick Sung1

  • 1Department of Thoracic and Cardiovascular Surgery, Samsung Medical Center, Sungkyunkwan University School of Medicine, Seoul, Republic of Korea.

The Journal of Thoracic and Cardiovascular Surgery
|October 27, 2023
PubMed

Insights

Valve-sparing root replacement (VSRR) in pediatric patients with connective tissue disease (CTD) shows good long-term survival. Careful surveillance is needed for later aortic aneurysm or dissection, especially in younger patients receiving VSRR.

Area of Science:

  • Cardiovascular Surgery
  • Pediatric Cardiology
  • Connective Tissue Diseases

Background:

  • Cardiovascular involvement, particularly aortic root dilatation, is a life-threatening complication in pediatric connective tissue diseases (CTD).
  • Valve-sparing root replacement (VSRR) is a surgical option for aortic root dilatation in this population.

Purpose of the Study:

  • To evaluate the long-term outcomes of VSRR in pediatric patients with CTD.
  • To assess reoperation rates for aortic aneurysm and dissection following VSRR.

Main Methods:

  • Retrospective analysis of pediatric CTD patients undergoing VSRR (2002-2021).
  • Primary endpoint: composite of all-cause death and cardiovascular reoperations.
  • Median follow-up of 8.3 years.

Main Results:

  • 15-year survival rate was 91.7% in 24 pediatric patients (median age 14.4 years).
  • 10-year cumulative incidence of reoperation for aortic regurgitation was 15.6%; for aneurysm/dissection, it was 29.1%.
  • Younger age at surgery and VSRR before 13 years were independent predictors of adverse events.

Conclusions:

  • VSRR offers good long-term survival and low reoperation for aortic regurgitation in pediatric CTD.
  • Later aortic aneurysm or dissection can occur, necessitating vigilant surveillance.
  • Younger patients undergoing VSRR require particular attention during follow-up.
Abstract

Keywords:
Loeys–Dietz syndromeMarfan syndromevalve-sparing root replacement

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