A 42 year-old female carrier of Duchenne muscular dystrophy (DMD) was referred with suspected subacute myocarditis and non-sustained ventricular tachycardia. Echochardiography and cardiac ...catheterization revealed severely reduced left ventricular function (LVF). Coronary artery disease was excluded. Cardiac magnetic resonance imaging showed transmural, intramural and subepicardial late gadolinium enhancement. Myocardial biopsy excluded viral infection and showed severe myopathic changes with abnormal expression of dystrophin and utrophin. Moleculargenetic analysis of the DMD gene revealed frameshift duplication of exon 2. The patient received conventional heart failure therapy, implantable cardioverter/defibrillator-implantation and prednisolone to attenuate cardiac degradation. 6 months later she had improved clinically though LVF was still severely reduced.
For potential application in ablating left free-wall accessory AV pathways with direct current shocks, a new epicardial-endocardial electrode configuration, designed to focus the current field across ...the mitral annulus, was tested in dogs. A catheter electrode in the coronary sinus (epicardial electrode) was used as the cathode, and a catheter electrode in the left ventricle (endocardial electrode) placed beneath the mitral valve, high against the mitral annulus and directly across from the epicardial electrode formed the anode. Two shocks, each of 30, 40, or 50 joules (J) were delivered in nine, three, and four dogs, respectively. The first shock was applied to the anterior or lateral wall and the second shock to the posterior wall, except in one dog which received one anterior and one lateral shock. Two dogs receiving 50 J shocks died acutely, one due to rupture of the coronary sinus and cardiac tamponade and the other had unexplained electromechanical dissociation. The remaining 14 dogs tolerated the two shocks well and were sacrificed 3-5 days later for pathological examination of the heart. Shocks in the anterior and lateral regions produced atrial necrosis (height 1.5-11 mm, width 1.5-12 mm and depth 1-3 mm) in 10 of 14 dogs and ventricular necrosis (height 4-27 mm, width 4-33 mm, and depth 5-14 mm) in all 14 dogs. Ideal lesions with atrial necrosis extending down to the annulus and ventricular necrosis extending to the epicardial aspect of the ventricular crest occurred in five dogs in which the endocardial electrode was positioned high against the annulus. In the other nine dogs, the endocardial electrode was located 6-18 mm below the annulus, as estimated by the center of ventricular necrosis. In these dogs, the ventricular lesions did not extend to the epicardial aspect of the crest and, in four dogs, no atrial necrosis was found. Shocks delivered to the posterior wall produced no atrial or ventricular necrosis except in one dog receiving a 50 J shock. It is concluded that, using the epicardial-endocardial electrode configuration with the LV catheter positioned high against the annulus, shocks of less than 50 J in dogs safely produce atrial and ventricular necrosis adjacent to the mitral annulus in the anterior and lateral regions but not in the posterior regions. Similar lesions in man may be capable of interrupting left anterior and lateral accessory AV pathways.
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