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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.
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.
Abstract:
CHD is frequently associated with a genetic syndrome. These syndromes often present specific cardiovascular and non-cardiovascular co-morbidities that confer significant peri-operative risks affecting multiple organ systems. Although surgical outcomes have improved over time, these co-morbidities continue to contribute substantially to poor peri-operative mortality and morbidity outcomes. Peri-operative morbidity may have long-standing ramifications on neurodevelopment and overall health. Recognising the cardiovascular and non-cardiovascular risks associated with specific syndromic diagnoses will facilitate expectant management, early detection of clinical problems, and improved outcomes--for example, the development of syndrome-based protocols for peri-operative evaluation and prophylactic actions may improve outcomes for the more frequently encountered syndromes such as 22q11 deletion syndrome.
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