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Aortic Root Replacement for Children With Loeys-Dietz Syndrome
Nishant D Patel1, Diane Alejo1, Todd Crawford1
1Division of Cardiac Surgery, Department of Surgery, The Johns Hopkins Medical Institutions, Baltimore, Maryland.
The Annals of Thoracic Surgery
|April 4, 2017
Summary
Loeys-Dietz syndrome (LDS) is a severe aortic disease. Prophylactic aortic root replacement (ARR) in children with LDS showed no operative mortality, but late interventions are common, emphasizing lifelong surveillance.
Area of Science:
- Cardiovascular Surgery
- Genetics
- Pediatric Cardiology
Background:
- Loeys-Dietz syndrome (LDS) is characterized by aggressive aortopathy, increasing risks of aortic aneurysm rupture and dissection.
- Early surgical intervention is crucial for managing aortic root dilation in pediatric patients with LDS.
Purpose of the Study:
- To evaluate the surgical outcomes of prophylactic aortic root replacement (ARR) in children diagnosed with Loeys-Dietz syndrome.
- To validate institutional guidelines for timing and technique of ARR in pediatric LDS patients.
Main Methods:
- A retrospective review of pediatric patients (<18 years) with LDS who underwent ARR.
- Analysis of primary endpoint (mortality) and secondary endpoints (complications, reinterventions).
Main Results:
- Thirty-four children underwent ARR, with a mean age of 10 years. No operative mortality was observed.
- Valve-sparing ARR was performed in 91% of cases. Late interventions were required in 6-15% for ascending aorta, arch replacement, or valve issues.
- Two late deaths (6%) occurred. Complications included coronary button aneurysms and need for redo valve-sparing ARR.
Conclusions:
- Pediatric Loeys-Dietz syndrome presents an aggressive aortopathy requiring vigilant management.
- Valve-sparing ARR is preferred when feasible to mitigate risks associated with mechanical prostheses.
- Lifelong serial imaging of the entire arterial tree is essential due to the high rate of subsequent interventions.