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Mitral valve replacement and subsequent composite graft replacement of the aortic root for infantile Marfan syndrome
Y Kamikubo1, T Murashita, K Yasuda
1Department of Cardiovascular Surgery, Hokkaido University School of Medicine, Sapporo, Japan.
Abstract:
The cardiovascular lesions commonly seen in Marfan syndrome can frequently be the primary cause of premature death. Cardiac lesions involving both the mitral valve and the aortic root are commonly observed among patients diagnosed during early infancy, as so-called infantile Marfan syndrome. Since the lesions tend to progress rapidly with the end results of high morbidity and mortality, the majority of patients require surgical intervention at a young age. However, patients who undergo surgical intervention for both lesions during the first decade of life have been rarely reported in literature. In this report, we present a case of a 9-year-old boy who underwent aortic root replacement with a composite graft at 3.5 years after a prior prosthetic valve replacement of the mitral valve. Although the immediate result was satisfactory, the long-term result remains to be seen.
Insights
Marfan syndrome can cause fatal cardiovascular lesions, often requiring early surgery. This report details a rare case of a 9-year-old undergoing aortic root replacement after mitral valve surgery.
Area of Science:
- Cardiovascular Surgery
- Pediatric Cardiology
- Genetic Disorders
Background:
- Marfan syndrome frequently causes severe cardiovascular complications, primarily affecting the aortic root and mitral valve.
- These lesions are a leading cause of premature death, necessitating early surgical intervention in many cases.
- Infantile Marfan syndrome presents with rapid progression, high morbidity, and mortality.
Observation:
- This report focuses on a 9-year-old boy diagnosed with Marfan syndrome.
- The patient underwent mitral valve replacement with a prosthetic valve.
- Subsequently, he underwent aortic root replacement with a composite graft at 3.5 years post-mitral valve surgery.
Findings:
- Surgical intervention for both mitral and aortic root lesions in the first decade of life is rarely documented.
- The case highlights a successful aortic root replacement following prior mitral valve surgery in a pediatric patient.
- Immediate surgical outcomes were satisfactory, though long-term results require continued observation.
Implications:
- This case contributes to the limited literature on early, combined surgical management of cardiovascular lesions in pediatric Marfan syndrome.
- It underscores the feasibility of staged surgical repair for complex cardiac issues in young patients with Marfan syndrome.
- Further long-term follow-up is crucial to evaluate the durability and efficacy of these interventions in this high-risk population.