Immediately from the ACCP Pulmonary medication Board evaluation 2009 direction, this article covers each subject in a concise, easy-to-use layout. Use as a self-study source to organize for the pulmonary medication subspecialty board exam.
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Congenital or hereditary hypercoagulable states include the following: activated protein C resistance (factor V Leiden mutation); antithrombin III deficiency (can also be acquired with the nephrotic syndrome); protein C deficiency; protein S deficiency; dysfibrinogenemia; disorders of plasminogen and plasminogen activation; the presence of antiphospholipid antibodies and lupus anticoagulant; and hyperhomocysteinemia. Many patients may present with multiple risk factors for VTE that can further increase their likelihood of this complication developing.
A V/Q scan should be performed to evaluate for potential CTEPH because patients who qualify for thromboendarterectomy can have a dramatic improvement with this procedure. Right-heart catheterization is the “gold standard” for diagnosis and should be performed in all patients with suspected PH, especially if specific treatment is planned. Cardiac catheterization is indicated to establish severity and prognosis, measure wedge pressure, exclude congenital heart disease, and test the response to selective pulmonary vasodilators (eg, inhaled NO or IV adenosine or epoprostenol).
Their overall recommended approach is shown in Figure 4. If pharmacologic treatment fails, surgical (the sixth line) treatment (lung transplantation or atrial septostomy) should be considered. 8 years. Since that time, survival has improved. Patients without evidence of right ventricular failure may survive Ͼ 10 years. Responders to calcium-channel blockers have a 95% 5-year survival rate. Patients in NYHA classes II and IV who have been treated with epoprostenol have a 5-year survival rate that is twice that of matched control patients.