A family is presented in which 7 members over 3 generations were affected by cardioskeletal myopathy. A vacuolar myopathy with excessive free and intralysosomal glycogen storage in skeletal and cardiac striated muscle was identified in biopsy studies. Post-mortem studies in several patients revealed changes of a congestive cardiomyopathy with myocardial fibrosis. Acid maltase, phosphorylase, debrancher and lysosomal enzyme screens, and glycolytic enzyme levels in skeletal muscle, were normal in 1 case. This is the third report of non-acid maltase deficient lysosomal glycogen storage disease and adds to previous reports with the presentation of detailed family studies, examined of ante- and post-mortem cardiac histology and reports of detailed glycolytic and lysosomal enzyme analysis. This syndrome is unusual among glycogenoses in having a dominant inheritance pattern.
Three patients with ischaemic heart disease developed profound cardiac failure, hypotension and bradycardia during combined therapy with verapamil and beta-adrenergic blocking drugs. This clinical picture resolved completely with cessation of the combined therapy. Baseline left ventricular function, assessed by cardiac catheterisation or nuclear angiography, was normal in two patients and only mildly reduced in the other. Simultaneously administration of beta-adrenergic blocking drugs and verapamil may result in profound adverse interactions and should only be administered with great caution.
Echoes from a right atrial mass in a patient with pulmonary embolism simulated a typical echocardiographic appearance of a right atrial myxoma. The patient was admitted with recurrent pulmonary emboli and had evidence of deep venous thrombosis on venography. M-mode echocardiography showed the appearance of a right atrial mass and right atrial angiography confirmed the presence of a mass prolapsing from right atrium into right ventricle. Subsequent 2-dimensional echocardiography and careful repeat M-mode echocardiography failed to demonstrate the mass suggesting embolisation to the lungs or lysis of the thrombus.
Two hundred and five patients who received a total of 247 electrode systems-129 transvenous, 68 transmediastinal and 50 transthoracic-are compared after complete uniform two-year follow-up. The transvenous patients had low hospital morbidity (19%) and short hospital stays (75% ≤ 8 days) but a high incidence of electrode failure by 24 months (38%). The transmediastinal and transthoracic patients had more hospital complications (35% and 34%) and longer periods of hospitalization (57% and 70% > 8 days) but fewer instances of failure by 24 months (16% and 11%). Most transvenous electrode failures were secondary to dislodgement. Transmediastinal right epicardial electrodes had the unique problem of threshold elevation and failure between six and 12 months after implantation as well as a high incidence of sudden death in this same period. Although the high incidence of endocardial electrode instability dictates the need for an alternative approach to permanent pacing, the failure of the transmediastinal approach to significantly alter postoperative morbidity (as compared with transthoracic electrodes) and the incidence of threshold elevation remote from right ventricular implantation suggest that limitation of thoracotomy (via the transmediastinal approach) should not take precedence over left ventricular implantation. Development of electrodes which would provide more permanent low resistance fixation to right ventricular endocardium or epicardium may be necessary before the transthoracic approach can be abandoned.
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