CRP4.Cardiac involvement of Behcet's disease in a school-age boy
Presented During: AATS Cardiothoracic Resident Case Report-Poster Display
Shanghai Children's Medical Center
Shanghai, Shanghai
China - Contact Me
Resident at Department of Cardiothoracic Surgery, Shanghai Children's Medical Center, Shanghai, China
Graduated from Shanghai Jiaotong University School of Medicine, Shanghai, China
Fellowship training at Boston Children's Hospital
Saturday, May 6, 2023: 5:00 PM - Tuesday, May 9, 2023: 5:00 PM
Los Angeles Convention Center
Room: ePoster Area, Exhibit Hall
Description
Behcet's disease is a recurrent inflammatory multi-organ illness characterized by ulcers and skin lesions. Cardiac involvement is estimated to range from 7% to 46%, but its onset before adulthood is extremely rare. We present a case of a school-age boy with aortic valve insufficiency with later prosthetic aortic valve dehiscence caused by Behcet's disease.
This patient presented with fever and an incidental finding of bicuspid aortic valve with severe insufficiency at the age of 7 at the outside hospital. Infective endocarditis was suspected, but the blood culture was negative. Perforation of the anterior cusp was seen intraoperatively, and single-leaflet aortic valve reconstruction utilizing the Ozaki technique was performed. Severe aortic valve insufficiency occurred shortly after the procedure with deteriorating left ventricular function, and the patient was transferred to our hospital three months later. The pericardial patch used for leaflet reconstruction was found detached from the aortic annulus during reoperation, and mechanical aortic valve replacement was performed. Follow-up echocardiograms revealed a normally functioning prosthetic valve with reduced left ventricular systolic function.
One year later, at 8 years old, he presented with a fever of unknown origin, with ineffective antibiotic therapy. Laboratory tests showed elevated leucocyte count, C-responsive protein of 73 mg/L, and erythrocyte sedimentation rate (ESR) of 83 mm/h. Blood culture tested negative for microorganisms. Echocardiogram revealed a surprising rocking motion of the prosthetic aortic valve indicating prosthetic dehiscence (Figure A). Cardiac CT demonstrated a large paravalvular pseudo-aneurysm (Figure B). With no evidence of infection, the patient was highly suspected of cardiac involvement of a systematic inflammatory disease. Although the patient had recurrent oral ulcers, there was no history of skin lesions or genital ulcers, with a negative pathergy test. Human leukocyte antigen-B*15 was positive. Both clinical presentation and imaging findings strongly suggested cardiac involvement of Behcet's disease despite failure to meet the diagnostic criteria. Pediatric rheumatologists initiated the immunosuppressant with methylprednisolone. The patient was afebrile two days later with gradually decreasing ESR, which returned to normal after two weeks.
Another two weeks later, modified Bentall procedure was performed. Consistent with the preoperative imaging findings, the prosthetic aortic valve was partially detached from the aortic annulus (Figure C) with no signs of infective endocarditis. After removing the prosthesis, the pseudo-aneurysm was closed using the bovine pericardial patch. The valved conduit was sutured to the left ventricular outflow tract using interrupted mattress stitches with imbrication suture from the outside in and was fixed with felt. The postoperative course was uneventful. Histological analysis of the thickened aortic wall showed a disrupted myofibroblast arrangement with no significant inflammatory cell infiltration (Figure D). The patient was on oral prednisone therapy with Adalimumab after discharge. He has done well with no postoperative complications at three months of follow-up.
To our knowledge, it is the youngest patient with Behcet's disease requiring aortic valve surgery. Timely diagnosis is essential, and immunosuppressive therapy with modified Bentall procedure may be effective. Long-term follow-up is needed.
This patient presented with fever and an incidental finding of bicuspid aortic valve with severe insufficiency at the age of 7 at the outside hospital. Infective endocarditis was suspected, but the blood culture was negative. Perforation of the anterior cusp was seen intraoperatively, and single-leaflet aortic valve reconstruction utilizing the Ozaki technique was performed. Severe aortic valve insufficiency occurred shortly after the procedure with deteriorating left ventricular function, and the patient was transferred to our hospital three months later. The pericardial patch used for leaflet reconstruction was found detached from the aortic annulus during reoperation, and mechanical aortic valve replacement was performed. Follow-up echocardiograms revealed a normally functioning prosthetic valve with reduced left ventricular systolic function.
One year later, at 8 years old, he presented with a fever of unknown origin, with ineffective antibiotic therapy. Laboratory tests showed elevated leucocyte count, C-responsive protein of 73 mg/L, and erythrocyte sedimentation rate (ESR) of 83 mm/h. Blood culture tested negative for microorganisms. Echocardiogram revealed a surprising rocking motion of the prosthetic aortic valve indicating prosthetic dehiscence (Figure A). Cardiac CT demonstrated a large paravalvular pseudo-aneurysm (Figure B). With no evidence of infection, the patient was highly suspected of cardiac involvement of a systematic inflammatory disease. Although the patient had recurrent oral ulcers, there was no history of skin lesions or genital ulcers, with a negative pathergy test. Human leukocyte antigen-B*15 was positive. Both clinical presentation and imaging findings strongly suggested cardiac involvement of Behcet's disease despite failure to meet the diagnostic criteria. Pediatric rheumatologists initiated the immunosuppressant with methylprednisolone. The patient was afebrile two days later with gradually decreasing ESR, which returned to normal after two weeks.
Another two weeks later, modified Bentall procedure was performed. Consistent with the preoperative imaging findings, the prosthetic aortic valve was partially detached from the aortic annulus (Figure C) with no signs of infective endocarditis. After removing the prosthesis, the pseudo-aneurysm was closed using the bovine pericardial patch. The valved conduit was sutured to the left ventricular outflow tract using interrupted mattress stitches with imbrication suture from the outside in and was fixed with felt. The postoperative course was uneventful. Histological analysis of the thickened aortic wall showed a disrupted myofibroblast arrangement with no significant inflammatory cell infiltration (Figure D). The patient was on oral prednisone therapy with Adalimumab after discharge. He has done well with no postoperative complications at three months of follow-up.
To our knowledge, it is the youngest patient with Behcet's disease requiring aortic valve surgery. Timely diagnosis is essential, and immunosuppressive therapy with modified Bentall procedure may be effective. Long-term follow-up is needed.