Successful surgical repair for Emery-Dreifuss muscular dystrophy valvular disease with long-term follow-up.
Emery-Dreifuss muscular dystrophy (EDMD) is an X-linked muscular dystrophy in which cardiac involvement can be serious. The disease progresses rapidly and the prognosis is strongly associated with cardiac involvement. We present the case of a 49-year-old man who was admitted with shortness of breath. Echocardiography revealed a huge right atrium and severe tricuspid regurgitation caused by annular dilatation and destruction of leaflets and chordae by pacemaker leads. Tricuspid valve replacement, right atrial plication and implantation of epicardial pacemaker leads were performed. The patient is in a good condition seven years after surgery. Cardiac transplantation is the desired therapy for valvular disease in EDMD cardiomyopathy, but this case indicates that valve replacement is also acceptable if performed with the appropriate timing.
Takai
Hideaki
H
Department of Cardiovascular Surgery, Oita Prefectural Hospital, Oita-city, Oita Prefecture, Japan. cvstakai@yahoo.co.jp
Yamada
Takafumi
T
Tada
Seiichi
S
Matsumaru
Ichiro
I
D009202:Cardiomyopathies / Q000209:etiology / Q000503:physiopathology / Q000628:therapy; D002315:Cardiopulmonary Bypass / Q000379:methods; D003131:Combined Modality Therapy; D015150:Echocardiography, Doppler; D005500:Follow-Up Studies; D006325:Heart Atria / Q000473:pathology / Q000601:surgery; D006350:Heart Valve Prosthesis; D019918:Heart Valve Prosthesis Implantation / Q000379:methods*; D006801:Humans; D008297:Male; D008875:Middle Aged; D020389:Muscular Dystrophy, Emery-Dreifuss / Q000150:complications* / Q000175:diagnosis; D010138:Pacemaker, Artificial; D011300:Preoperative Care / Q000379:methods; D018570:Risk Assessment; D012720:Severity of Illness Index; D013995:Time; D014057:Tomography, X-Ray Computed / Q000379:methods; D016896:Treatment Outcome; D014262:Tricuspid Valve Insufficiency / Q000175:diagnosis / Q000209:etiology* / Q000601:surgery*