Aims: To avoid frequent discharges of implantable cardioverter defibrillators, antiarrhythmic drugs may be needed in some patients with ventricular tachyarrhythmias. For ventricular tachyarrhythmias refractory to conventional antiarrhythmic drugs, we evaluated the efficacy of bepridil, a multiple ion-channel blocker. Methods and results: Sixteen patients with structural heart disease and ventricular tachyarrhythmias refractory to multiple antiarrhythmic drugs (4.1±1.6 drugs including class III drugs) were enrolled. Bepridil was prescribed at a mean dose of 156±40 mg/day. Bepridil prolonged the QT/QTc interval without affecting heart rate or the QRS duration. During a mean follow-up of 52±44 months, bepridil completely suppressed ventricular tachyarrhythmias in 6 of the 16 patients (38%) and the drug decreased the frequency of ventricular tachyarrhythmia recurrences by>75% in 3 of the other 10 patients. The markers of complete suppression of ventricular tachyarrhythmias during bepridil treatment included a smaller number of VT morphologies, a better NYHA functional class, and a greater drug-induced prolongation of the QT/QTc interval. The result of electrophysiologic study-guided evaluation of bepridil was closely associated with the clinical efficacy of bepridil in 7 of 8 patients. Conclusion: Bepridil appears to be useful to suppress drug-refractory ventricular tachyarrhythmia recurrence.
We report a case of angiosarcoma complicated by bilateral massive bloody pleurisy (hematocrit of 7.2%) in an 83-year-old woman. An autopsy revealed hemorrhagic tumors infiltrating both the diaphragm and serosal surface of the peritoneum. Histological examination confirmed an anastomosing vascular channel pattern of the tumor cells with characteristic immunohistochemical findings for angiosarcoma, such as positive staining for vimentin, CD31, CD34, D2-40, and factor VIII-related antigen. The tumor was thought to have originated from a small vessel on the serosal surface of the duodenum. We also reviewed cases of hemothorax associated with angiosarcoma, which suggested that primary or metastatic pleural angiosarcoma should be considered a cause of spontaneous hemothorax, especially in patients with bilateral and/or intractable hemothorax.
We report 2 cases of Gasperini syndrome and consider them with the 11 previously reported cases to describe the clinical characteristics of this rare syndrome: Core neurological signs are peripheral facial nerve palsy and abducens nerve palsy of the affected side: Among all cases, imaging demonstrated a small lesion in the mediolateral tegmental pons (10/13 cases of microinfarction; 2/13 cases of microbleeding). We found that the responsible artery in ischemic Gasperini syndrome is mainly the long circumferential branch of the anterior inferior cerebellar artery; Case 1 is the first case thought to be caused by infarction of the basilar artery's paramedian branch.
POEMS syndrome (POEMS) is a rare multi-system disease which is typically associated with polyneuropathy, organomegaly, endocrinopathy, monoclonal gammopathy and skin changes. We report herein a case of POEMS with progressive polyneuropathy resistant to high-dose intravenous immunoglobulin therapy who experienced improvement in polyneuropathy after the administration of high-dose chemotherapy followed by autologous peripheral blood stem cell transplantation. Improvements in polyneuropathy remain despite recognition of monoclonal gammopathy by immunofixation as of 1 year after treatment. We demonstrated that autologous peripheral blood stem cell transplantation can improve clinical symptoms and signs due to polyneuropathy in POEMS even without achieving complete remission.