Complete atrioventricular block in Duchenne muscular dystrophy

A. Fayssoil, D. Orlikowski, O. Nardi, D. Annane
2008 Europace  
Duchenne muscular dystrophy (DMD) is an inherited myogenic disorder due to mutations in the dystrophin gene on chromosome Xp21.1. It is characterized by progressive muscle wasting and weakness of variable distribution and severity. Heart is involved leading to heart failure. Conduction abnormalities are unusual. We report a case of complete atrio-ventricular block in a DMD patient. Case report A 33-year-old man with Duchenne muscular dystrophy (DMD), on wheelchair, was admitted in hospital for
more » ... ed in hospital for dyspnea, bradycardia, and asthenia. Diagnosis was made in the childhood by genetic analysis, which found deletion exon 50-52 (dystrophin gene). His past medical history was pertinent for scoliosis, tracheotomy, and home ventilation for respiratory insufficiency. He had no cardiovascular risks factors. He had taken perindopril 2 mg and furosemide 20 mg daily for cardiomyopathy known for 13 years. On admission, body surface area was 1.5 cm 2 , body temperature was 368C, blood pressure was 126/59 mmHg, and heart rate was 40 bpm. Oxygen saturation was 100% with mechanical ventilation. Electrocardiogram (ECG) showed complete atrioventricular (AV) block ( Figure 1 ). Echocardiography showed a global left ventricular dysfunction with estimated ejection fraction of 45%. Blood count and electrolytes were normal. A temporary transvenous pacing lead was inserted because of complete AV block and 24 h later, replaced by a dualchamber permanent pacemaker. Patient was discharged home 5 days later without any complications.
doi:10.1093/europace/eun264 pmid:18815187 fatcat:vq6cnejnzrgx7pi36mdnc7wmo4