Successful surgical treatment of intramural aortoatrial fistula, severe aortic regurgitation, mitral prolapse, and tricuspid insufficiency in a patient with Ehlers-Danlos syndrome type IV.
Patients with Ehlers-Danlos syndrome (EDS) type IV, an inherited connective tissue disorder, are predisposed to vascular and digestive ruptures, and arterial ruptures account for the majority of deaths. A 31-year-old man with EDS presented with an intramural aortoatrial fistula, severe aortic regurgitation, mitral valve prolapse, and severe tricuspid valve insufficiency combined with a severely dilated left ventricle. Determining the best surgical option for the patient was not easy, especially regarding the course of action for the aortic root with a tear in the sinus of Valsalva. The fistula tract was closed at the aorta with suture and with a patch in the right atrium, the mitral valve was repaired with edge-to-edge suture and then annuloplasty with a Cosgrove ring, the aortic valve was replaced with a mechanical prosthesis, and a modified De Vega technique was used for the tricuspid valvuloplasty. The postoperative course was uncomplicated, and the patient was discharged 2 weeks later. The considerations made to arrive at the chosen surgical course of action in this complex case are reviewed.
Department of Cardiovascular Surgery of Chinese PLA General Hospital, Institute of Cardiac Surgery of Chinese PLA, Beijing, P. R. China.
Shengli
S
Jiang
Changqing
C
Gao
Chonglei
C
Ren
Tao
T
Zhang
D000328:Adult; D001011:Aorta / Q000002:abnormalities* / Q000601:surgery; D001022:Aortic Valve Insufficiency / Q000150:complications / Q000601:surgery*; D001159:Arterio-Arterial Fistula / Q000150:complications / Q000601:surgery*; D003131:Combined Modality Therapy; D004535:Ehlers-Danlos Syndrome; D006325:Heart Atria / Q000002:abnormalities* / Q000601:surgery; D019918:Heart Valve Prosthesis Implantation / Q000379:methods*; D006801:Humans; D008297:Male; D008945:Mitral Valve Prolapse / Q000150:complications / Q000601:surgery*; D016896:Treatment Outcome; D014262:Tricuspid Valve Insufficiency / Q000150:complications / Q000601:surgery*