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Marfan syndrome: the variability and outcome of operative management
J S Coselli1, S A LeMaire, S Büket
1Department of Surgery, Baylor College of Medicine, Houston, TX.
Purpose:
This study reviews the contemporary surgical outcome of 69 patients with Marfan syndrome who underwent one or more aortic operations between July 18, 1989, and April 14, 1994.
Methods:
During this 5-year period, 79 operations were performed including composite valve graft replacement (n = 28) and graft replacement of the thoracoabdominal aorta (n = 29). Fifty-seven additional operations had been performed before referral, for a total of 136 aortic operations in this group. Forty-two patients (60.9%) required multiple operations; 16 patients (23.2%) underwent three or more procedures.
Results:
Follow-up was complete on all patients. The 30-day and long-term survival rates were 98.7% and 96.2%, respectively. Distal aortic replacement resulted in paraparesis in only one (2.6%) of 38 patients; no patient had development of paraplegia. No patient had a stroke after the use of profound hypothermic circulatory arrest.
Conclusion:
Aortic surgery prolongs survival in patients with Marfan syndrome and currently has low associated morbidity and mortality rates. Treatment often requires multiple staged operations. The variety and multiplicity of aortic manifestations in these patients demonstrate the necessity for lifelong cardiovascular surveillance to detect new or recurrent disease.
Insights
Aortic surgery significantly improves survival for Marfan syndrome patients, with low mortality and morbidity. Many patients require multiple staged operations for aortic repair, highlighting the need for lifelong monitoring.
Area of Science:
- Cardiovascular Surgery
- Genetics
- Thoracic Surgery
Background:
- Marfan syndrome is a genetic disorder affecting connective tissue, often leading to aortic complications.
- Aortic dissections and aneurysms are life-threatening manifestations of Marfan syndrome.
Purpose of the Study:
- To review the surgical outcomes of Marfan syndrome patients undergoing aortic operations.
- To assess the contemporary morbidity and mortality associated with aortic surgery in this population.
Main Methods:
- Retrospective review of 69 Marfan syndrome patients undergoing 136 aortic operations over a 5-year period.
- Analysis of operative procedures including composite valve graft replacement and thoracoabdominal aortic replacement.
- Evaluation of patient outcomes, including survival rates and complications.
Main Results:
- High 30-day (98.7%) and long-term (96.2%) survival rates were observed.
- Low incidence of paraparesis (2.6%) and no paraplegia after distal aortic replacement.
- No strokes occurred following profound hypothermic circulatory arrest.
Conclusions:
- Aortic surgery is effective in prolonging survival for Marfan syndrome patients.
- Current surgical techniques offer low morbidity and mortality rates.
- Lifelong cardiovascular surveillance is essential due to the potential for multiple, recurrent aortic issues.