Chapter 2. Pulmonary Vascular Diseases

COL Lisa K Moores, MC, USA, FCCP
DOI: 10.1378/pulm.26.2
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  • Describe the risk factors and epidemiology of VTE.

  • Provide approaches to the diagnosis and treatment of VTE.

  • List recommendations for the prevention of VTE.

  • Describe the epidemiology and pathophysiology of pulmonary hypertension (PH).

  • Describe the steps in the diagnosis of pulmonary arterial hypertension (PAH).

  • List the recommended treatment regimens for PAH.


VTE is a common and potentially fatal disease. Prevention is paramount, yet remains underutilized. Diagnostic algorithms that use clinical prediction scores, D-dimer testing, and noninvasive imaging have been developed and validated. Treatment is centered on prevention of clot extension and embolization in the acute phase and prevention of recurrence and chronic complications long-term. The mainstay of therapy is heparin and the vitamin K antagonists, although newer agents are now in the final phases of testing and will soon be available for both prevention and treatment of VTE. Pulmonary arterial hypertension is a syndrome characterized by increased pulmonary artery pressures and resistance. There are multiple underlying causes and associated diseases. Clinical features include dyspnea and signs of right heart failure. Diagnosis is reliant on clinical suspicion, echocardiography, and right heart catheterization. We have learned much in the past 20 years about the underlying pathophysiology, which involves several pathways involving vasoconstrictors, mitogens, and procoagulants. Treatment is based on functional limitations and degree of right ventricular dysfunction, and often involves a combination of medications.


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