Risk of stroke in idiopathic hypertrophic subaortic stenosis.

and the rest of the family were still in the Marquesas Islands and could not be examined. Heparin treatment (Calciparine, Du Pont) associated with AT-III was administered on April 27. Despite anticoagulant therapy, pulmonary embolism occurred, resulting in an abortion and inferior vena cava occlusion. Currently, the patient is taking oral anticoagulants and has speech and gait defects. One of two etiologies may account for our patient's manifestations. Sneddon's syndrome is a rare clinical entity prevalent in younger women. Approximately 60 cases have been reported to date. The neurological clinical picture includes recurrent transient or permanent ischemic cerebrovascular attacks, fits, and deterioration of intellect. Livedo reticularis may precede neurological manifestations for many years. Many cases have included the presence of phospholipid antibodies, leading to the hypothesis of Sneddon's syndrome as a phospholipid antibody disease. The association of Sneddon's syndrome and mitral heart disease, as observed in our case, was also recently reported, but because the pathophysiology of this entity remains unclear, no specific treatment strategy has been developed. A second possible etiology is familial AT-III deficiency, an autosomal dominant disorder that causes a predisposition to recurrent venous thromboembolic disease and arterial occlusion. Thromboembolic disease is frequently encountered during pregnancy in association with Sneddon's syndrome or AT-III deficiency. To our knowledge, the association of Sneddon's syndrome and a familial deficiency of AT-III has never been reported. The combination of livedo reticularis, mitral valve prolapse, and AT-III deficiency observed in our patient probably accounts for the multiple arterial and venous thrombi and the failure of anticoagulant therapy.