ABSTRACT
Hereditary hemorrhagic telangiectasia (Osler-Weber-Rendu) disease is characterized by cutaneous, mucosal, and visceral vascular anomalies. Two patients were previously described with coronary artery aneurysms (ectasia) associated with this disease. This report describes a patient with Osler-Weber-Rendu disease in whom multiple coronary arteriovenous malformations were identified during coronary angiography. The patient presented with anginal chest pain resulting from severe anemia. Upper gastrointestinal endoscopy revealed multiple angiodysplastic lesions throughout the esophagus and stomach.
Subject(s)
Arteriovenous Malformations/complications , Coronary Vessel Anomalies/complications , Telangiectasia, Hereditary Hemorrhagic/complications , Anemia, Iron-Deficiency/complications , Anemia, Iron-Deficiency/therapy , Arteriovenous Malformations/diagnostic imaging , Blood Transfusion , Coronary Angiography , Coronary Vessel Anomalies/diagnostic imaging , Endoscopy, Digestive System , Esophagus/blood supply , Follow-Up Studies , Gastrointestinal Hemorrhage/complications , Gastrointestinal Hemorrhage/diagnosis , Humans , Male , Middle Aged , Stomach/blood supply , Telangiectasia, Hereditary Hemorrhagic/diagnosisABSTRACT
Morbidity and mortality following Automatic Implantable Cardioverter Defibrillation (AICD) is mostly related to thoracotomy performed during placement of epicardial leads. From July 1991 to January 1994, a transvenous lead system (TLS) (Endotak C; CPI) was implanted in 28 patients (24 males, 4 females, age 15-76 years) who suffered from life threatening ventricular tachyarrhythmias. Twenty four patients had a previous MI, mean LVEF was 26% (14-65%), two had mitral valve prolapse (one with prolonged QT), one had hypertrophic and another had dilated cardiomyopathy. The implantation procedure was performed in the operating room (n = 26) or in the Cath lab. (n = 2). Nineteen (out of the 28) patients were treated with Amiodarome before implantation (5 out of 7 had LVEF < 30%). A satisfactory defibrillation threshold (DFT) was achieved using Endotak lead alone in 22 (79%) patients. These patients had LVEF between 10% to 65% (less than 30% in three patients). In six patients the DFT was measured as lower than 20 J, and a subcutaneous patch was added (LVEF 12-38%, 4 patients with Amiodarome therapy). The implanted devices were all from CPI: 1600 (n = 5), PRX (n = 7), P2 (n = 15), PRX II (n = 1). During a 1-32 months of follow-up one patient died from severe CHF. The implanted device operated in 9/28 patients. The only complication related to implantation was infection at the pocket site (n = 2), which responded to antibiotic therapy in one patient, but required device explantation in another patient. The device was reimplanted in that patient three month later, with no further complications.(ABSTRACT TRUNCATED AT 250 WORDS)
