Percutaneous left atrial appendage closure: in a patient with hereditary hemorrhagic telangiectasia and atrial fibrillation.

A 73-year-old man presented emergently with severe abdominal pain and hematochezia. He had a history of idiopathic hypertension and hereditary hemorrhagic telangiectasia with chronic nosebleed (Fig. 1). Physical examination showed abdominal distention and new atrial fibrillation with high ventricular response. Laboratory tests revealed leukocytosis with metabolic acidosis, and a bedside ultrasonogram showed an edematous bowel with decreased peristalsis and free fluid in the abdominal cavity. The patient underwent urgent laparotomy, which revealed a 50-cm small-bowel infarction. The next day, transesophageal echocardiography (TEE) revealed spontaneous contrast and a thrombus-like image in the left atrial appendage (LAA). Intravenous heparin and then warfarin were administered. The patient was discharged from the hospital after 11 days. Weeks later, the nosebleed progressively worsened, shortness of breath occurred, and dark stool appeared. The anticoagulation was discontinued due to severe anemia (hemoglobin, 6.7 g/dL). Fig. 1 Photographs show dark-red vascular papules the size of match heads, limited to A) the mucous membrane (tongue) and B) the skin (fingers). In view of the patient's condition, percutaneous exclusion of the LAA was undertaken. An AMPLATZER Cardiac Plug® (AGA Medical, part of St. Jude Medical, Inc.; St. Paul, Minn) was successfully implanted via transseptal puncture (Figs. 2 and ​and3).3). The patient took clopidogrel for a month, and TEE then showed the device in situ without leaks or thrombus formation (Fig. 4). Nine months later, the patient had no complications. Fig. 2 Angiogram (right anterior oblique view, via transseptal puncture) shows the mouth, body, and 3 distal lobes of the left atrial appendage. Contrast medium renders the left atrium partially visible. Fig. 3 Intraprocedural transesophageal echocardiogram shows the AMPLATZER device in situ, still attached to the delivery cable, with distal lobe and proximal retention disc excluding the left atrial appendage. Fig. 4 Transesophageal echocardiogram performed 1 month postprocedurally shows the final position of the AMPLATZER device and complete exclusion of the left atrial appendage.