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Outcomes of Subaortic Obstruction Resection in Children

Julia S Donald1, Phillip S Naimo2, Yves d'Udekem2

  • 1Royal Children's Hospital, Melbourne, Vic., Australia; University of Melbourne, Melbourne, Vic., Australia.

Heart, Lung & Circulation
|August 15, 2016
PubMed

Insights

Surgical resection of discrete subaortic stenosis in children offers low mortality but has high rates of recurrence and aortic insufficiency. Long-term follow-up is crucial for managing these outcomes.

Area of Science:

  • Pediatric Cardiology
  • Cardiovascular Surgery
  • Congenital Heart Disease

Background:

  • Discrete subaortic stenosis (DSS) is a congenital heart defect requiring surgical intervention.
  • Long-term outcomes following surgical correction of DSS are not well-documented.
  • Fibromuscular resection is a common surgical approach for DSS in pediatric patients.

Purpose of the Study:

  • To evaluate the long-term outcomes of fibromuscular resection for discrete subaortic stenosis in children.
  • To identify risk factors for recurrence and reoperation after DSS surgery.
  • To assess the progression of aortic insufficiency following subaortic obstruction resection.

Main Methods:

  • Retrospective review of 72 children undergoing subaortic obstruction resection for DSS between 1989 and 2015.
  • Analysis of operative mortality, late mortality, and overall survival using Kaplan-Meier estimates.
  • Assessment of left ventricular outflow tract (LVOT) Doppler gradients pre- and post-operatively.
  • Evaluation of recurrence, reoperation rates, and progression of aortic insufficiency.

Main Results:

  • No operative deaths; 3 late deaths (4.2%). 10-year survival was 93.0%.
  • Significant reduction in LVOT Doppler gradients post-surgery (peak instantaneous and mean).
  • High rates of recurrence (29.0%) and reoperation (18.8%) observed, with freedom from reoperation at 10 years at 71.1%.
  • Younger age (<5 years) and extension to the aortic valve were risk factors for reoperation.
  • Aortic insufficiency present in 54.2% preoperatively, with progression in 38.9%; aortic valve involvement predicted need for further aortic valve procedures.

Conclusions:

  • Fibromuscular resection for pediatric DSS is associated with low operative mortality.
  • High rates of recurrence and reoperation necessitate vigilant long-term monitoring.
  • Progression of aortic insufficiency is common, requiring careful follow-up and potential intervention.
  • Patients with DSS require lifelong surveillance into adulthood due to potential late complications.
Abstract

Keywords:
Congenital heart diseaseSubaortic stenosisSurgery

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