Stenotic aortic and mitral valves in three adult brothers with arthrogryposis multiplex congenita.

Three brothers with arthrogryposis multiplex congenita survived into adulthood. In their fourth decade, the clinical course for each brother became complicated by severe stenoses of their aortic and mitral valves. One brother died suddenly, and the remaining 2 developed heart failure.

A phase III, double-blind, placebo-controlled, multicenter study on the efficacy of recombinant human antithrombin in heparin-resistant patients scheduled to undergo cardiac surgery necessitating cardiopulmonary bypass.

Background: The study evaluated the efficacy of recombinant human antithrombin (rhAT) for restoring heparin responsiveness in heparin resistant patients undergoing cardiac surgery.

Methods: This was a multicenter, randomized, double-blind, placebo-controlled study in heparin-resistant patients undergoing cardiac surgery with cardiopulmonary bypass. Heparin resistance was diagnosed when the activated clotting time was less than 480 s after 400 U/kg heparin.