Successful Management for Aortic Valve Insufficiency in a Childbearing Age Patient with Type II Ehlers-Danlos Syndrome / 日本心臓血管外科学会雑誌

ABSTRACT

A 16-year-old schoolgirl had aortic valve insufficiency, detected incidentally on electrocardiogram taken for a physical checkup. She was noted to have hyperextensibility of joints, hyperelasticity of the skin and an atrophic scar over the knees on physical examination and type II Ehlers-Danlos syndrome (EDS) was diagnosed by skin biopsy after admission. A Carpentier-Edwards pericardial bioprosthesis was chosen for aortic valve replacement because she was of childbearing age and had EDS. Seven years after the replacement she became pregnant, resulting in spontaneous vaginal delivery of a healthy male infant at 39 weeks. However, a second aortic valve replacement was staged for structural failure of the bioprosthesis which was accelerated during the gestation. Aortic valve re-replacement was carried out using a 21-mm Sorin Bicarbon mechanical prosthesis 4 months after the delivery. She was discharged on the 10th postoperative day without any complications.