CHD associated with syndromic diagnoses: peri-operative risk factors and early outcomes

Benjamin J Landis1, David S Cooper1, Robert B Hinton1

  • 1Heart Institute,Cincinnati Children's Hospital Medical Center,Cincinnati,Ohio,United States of America.

Cardiology in the Young
|September 9, 2015
PubMed

Insights

Congenital heart disease (CHD) linked to genetic syndromes poses significant peri-operative risks. Recognizing these risks and developing syndrome-specific protocols can improve patient outcomes and reduce mortality.

Area of Science:

  • Cardiology
  • Genetics
  • Pediatric Surgery

Background:

  • Congenital heart disease (CHD) is often associated with genetic syndromes.
  • These syndromes present complex cardiovascular and non-cardiovascular co-morbidities.
  • These co-morbidities significantly increase peri-operative risks and can lead to poor outcomes.

Purpose of the Study:

  • To highlight the importance of recognizing co-morbidities in syndromic CHD.
  • To emphasize the impact of these co-morbidities on peri-operative mortality and morbidity.
  • To advocate for the development of syndrome-specific management protocols.

Main Methods:

  • Review of existing literature on genetic syndromes associated with CHD.
  • Analysis of peri-operative risks and outcomes in patients with syndromic CHD.
  • Case study examples, such as 22q11 deletion syndrome, to illustrate key points.

Main Results:

  • Syndromic CHD patients face substantial peri-operative risks due to co-morbidities.
  • These risks contribute significantly to mortality and morbidity, with long-term effects on neurodevelopment.
  • Improved outcomes are achievable through tailored peri-operative evaluation and management.

Conclusions:

  • Early recognition of syndrome-specific risks is crucial for managing patients with CHD.
  • Developing syndrome-based protocols can optimize peri-operative care and improve patient outcomes.
  • Proactive management strategies are essential for mitigating risks in frequently encountered syndromes like 22q11 deletion syndrome.

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