초록접수 현황
| 16F-285 | 구연 발표 |
Surgery on Dilated Aorta Associated with Connective Tissue Disease or Inflammatory Vasculitis in Children and Adolescent Patients.
Jae Gun Kwak, Yong Jin Kim, Jeong Ryul Lee, Woong-Han Kim
Department of Thoracic and Cardiovascular Surgery, Seoul National University Children’s Hospital, Seoul National University College of Medicine, Seoul, Korea
Background : We reviewed the patients who underwent surgery for dilated aorta, especially which was associated with connective tissue diseases or inflammatory vasculitis in children and adolescent.
Methods : Medical records of the patients who underwent aortic surgery for dilatation due to connective tissue diseases or inflammatory vasculitis between 2000 and 2015 were retrospectively reviewed. We excluded the cases of post-stenotic dilatation, acquired dilative aortic diseases, or secondary dilative change due to other congenital heart diseases or procedures. The connective tissue diseases which induce stenotic lesion rather than dilative lesion were also excluded.
Results : We have 11 patients who underwent surgery for dilated aorta. The median age was 10.4 years (5.3 months~20.1 years), and the median body weight was 28.6 kg (6.8kg~91.5kg).
There were 4 Marfan syndrome patients, 3 Loeys-Dietz syndrome patients, 1 Kawasaki patient, 1 Takayasu’s arteritis patient, and 1 PHACE syndrome patient. One patient was suspicious of having a connective tissue disease, however, not confirmed. Most commonly involved area were aortic root and ascending aorta, but distal aortic arch was involved in 1 patient. Various surgical techniques were used to reduce dimension of dilated aorta and aortic root or aortic valve regurgitation, from simple aneurysmorrhaphy to David operation.
For 79.2±29.4 months of follow-up duration, there were 2 cases of follow-up loss in Marfan syndrome patients, and 5 reoperations in 3 patients. Except 1 patient, functional class and ventricular function were well maintained in all followed-up patients, however, the dimension of aortic root and involved aorta showed progressive dilation by time.
Conclusion : The cases of surgical treatment for the dilated aortopathy associated with connective tissue diseases and inflammatory vasculitis were very rare in children and adolescent. Most of the patients showed tolerable postoperative course, however, they showed progressive dilation of involved aorta by time even after surgical treatment. Close and regular follow-up is mandatory.
Methods : Medical records of the patients who underwent aortic surgery for dilatation due to connective tissue diseases or inflammatory vasculitis between 2000 and 2015 were retrospectively reviewed. We excluded the cases of post-stenotic dilatation, acquired dilative aortic diseases, or secondary dilative change due to other congenital heart diseases or procedures. The connective tissue diseases which induce stenotic lesion rather than dilative lesion were also excluded.
Results : We have 11 patients who underwent surgery for dilated aorta. The median age was 10.4 years (5.3 months~20.1 years), and the median body weight was 28.6 kg (6.8kg~91.5kg).
There were 4 Marfan syndrome patients, 3 Loeys-Dietz syndrome patients, 1 Kawasaki patient, 1 Takayasu’s arteritis patient, and 1 PHACE syndrome patient. One patient was suspicious of having a connective tissue disease, however, not confirmed. Most commonly involved area were aortic root and ascending aorta, but distal aortic arch was involved in 1 patient. Various surgical techniques were used to reduce dimension of dilated aorta and aortic root or aortic valve regurgitation, from simple aneurysmorrhaphy to David operation.
For 79.2±29.4 months of follow-up duration, there were 2 cases of follow-up loss in Marfan syndrome patients, and 5 reoperations in 3 patients. Except 1 patient, functional class and ventricular function were well maintained in all followed-up patients, however, the dimension of aortic root and involved aorta showed progressive dilation by time.
Conclusion : The cases of surgical treatment for the dilated aortopathy associated with connective tissue diseases and inflammatory vasculitis were very rare in children and adolescent. Most of the patients showed tolerable postoperative course, however, they showed progressive dilation of involved aorta by time even after surgical treatment. Close and regular follow-up is mandatory.
책임저자: Woong-Han Kim
Department of Thoracic and Cardiovascular Surgery, Seoul National University Children’s Hospital, Seoul National University College of Medicine, Seoul, Korea
발표자: Jae Gun Kwak, E-mail : switch.surgeon@gmail.com