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Children with corrected or palliated congenital heart disease on home mechanical ventilation

Jeffrey D Edwards1, Sheila S Kun, Thomas G Keens

  • 1Department of Anesthesiology Critical Care Medicine, Childrens Hospital of Los Angeles, Los Angeles, CA, USA. edwardsj@peds.ucsf.edu

Pediatric Pulmonology
|June 25, 2010
PubMed

Insights

Children with congenital heart disease (CHD) needing home mechanical ventilation (HMV) face high mortality, especially those with complex conditions like single ventricle anatomy or high RACHS scores. Success in weaning off HMV is limited in this vulnerable pediatric population.

Area of Science:

  • Pediatric Cardiology
  • Respiratory Medicine
  • Critical Care

Background:

  • Infants and children with congenital heart disease (CHD) often require interventions for chronic respiratory failure.
  • Home mechanical ventilation (HMV) via tracheostomy is a common management strategy for these patients.
  • Limited data exists on the outcomes of children with CHD requiring HMV.

Purpose of the Study:

  • To retrospectively review outcomes of children with CHD on HMV.
  • To identify factors influencing mortality and success in weaning from HMV.
  • To inform clinical decision-making for families considering HMV for children with CHD.

Main Methods:

  • Retrospective chart review of children with CHD in a hospital's HMV program (1994-2009).
  • Data collected included heart lesion type, surgeries, extubation failures, tracheostomy timing, mortality, HMV duration, weaning status, comorbidities, and Risk Adjusted classification for Congenital Heart Surgery (RACHS-1) category.
  • Analysis focused on survival rates, weaning success, and correlation with lesion complexity and RACHS-1 scores.

Main Results:

  • Thirty-five children with CHD on HMV were identified; 66% (23) were alive, and 23% (8) were weaned off HMV.
  • Mortality was higher in patients with single ventricle anatomy (50%) and those with RACHS-1 scores >= 4 (89%).
  • Five-year survival was 68% overall, significantly lower for patients with RACHS-1 scores >= 4 (12%) compared to < 3 (90%).

Conclusions:

  • Children with CHD requiring HMV have significant mortality risks, particularly those with complex heart defects.
  • Single ventricle physiology and higher RACHS-1 scores are associated with increased mortality and reduced success in weaning from HMV.
  • Caregivers must carefully consider the complexity of the heart defect when advising families about HMV for children with CHD.

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