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Surgical management of Marfan syndrome in children
Insights
Surgery for cardiovascular complications in children with Marfan syndrome, including infants, shows favorable medium-term outcomes. Echocardiography aids in determining the optimal timing for surgical intervention in these young patients.
Area of Science:
- Cardiology
- Pediatric Surgery
- Genetics
Background:
- Marfan syndrome is a genetic disorder affecting connective tissue, often leading to severe cardiovascular complications.
- Cardiovascular manifestations, including aortic aneurysms and valve regurgitation, are a major cause of morbidity and mortality in Marfan syndrome.
- Early diagnosis and intervention are crucial for managing cardiovascular risks in affected children.
Purpose of the Study:
- To evaluate the surgical outcomes for severe cardiovascular complications in pediatric patients with Marfan syndrome.
- To assess the medium-term efficacy of surgical interventions in infants and older children diagnosed with Marfan syndrome.
- To determine the impact of surgical timing, guided by echocardiography, on patient outlook.
Main Methods:
- A retrospective review of seven pediatric patients with Marfan syndrome who underwent cardiovascular surgery between 1983 and 1991.
- Patients were divided into an infant group (n=3) and an older children group (n=4).
- Surgical procedures included composite valved conduits, aortic homografts, and mechanical prostheses for aortic and mitral valve issues.
Main Results:
- All seven patients survived the primary surgical procedure.
- In the infant group, two patients required and underwent further successful surgeries for valve replacement and aortic root repair.
- The study demonstrated a favorable change in major cardiovascular risk factors with an encouraging medium-term outlook.
Conclusions:
- Surgical intervention for severe cardiovascular complications in pediatric Marfan syndrome, even in infancy, can lead to positive medium-term outcomes.
- Echocardiography plays a vital role in guiding the appropriate timing for surgical intervention.
- These findings suggest that timely surgical management can significantly improve the cardiovascular health of young patients with Marfan syndrome.
Abstract:
Between August 1983 and January 1991, seven patients with Marfan syndrome underwent surgery for severe cardiovascular complications. The mean age at presentation was 5.7 months (range 4 to 9 months) in the infant group (n = 3), and 13.3 years (range 10 to 16 years) in a group of older children (n = 4). The primary indications for surgery in the infant group (performed at a mean of 3 years after diagnosis) were ascending aortic aneurysm with valvar regurgitation in one patient, and severe mitral valve prolapse with regurgitation in two. In the older group, surgical indications (performed at a mean of 2.8 years after diagnosis) were ascending aortic aneurysm with valvar regurgitation in three patients and acute aortic dissection in one. For aortic surgery, a composite valved conduit was used in four patients, and an aortic homograft in one. For mitral valve surgery, mechanical prostheses were used. All patients survived the primary operation. Over a mean follow-up of 17.5 patient-years (range 1 to 9 years), two patients in the infant Marfan group went on to further successful surgery (prosthetic mitral valve replacement and aortic root repair with aortic homograft) at a mean interval of 4.3 years after the initial surgery. Our results suggest that the major cardiovascular risk factors of Marfan syndrome in the young, even in those diagnosed during infancy, have been favorably changed by surgery with an encouraging medium-term outlook. The correct timing of surgery is aided by echocardiography.