Cardiology>>>>>Peripheral Vascular Disease
Question 10#

A 49-year-old man presents to the clinic with complaints of progressive exertional dyspnea for several weeks. His speech is mildly breathless. Neck veins are distended bilaterally and there is moderate lower extremity edema. He denies chest pain. Electrocardiogram (ECG) shows sinus tachycardia without ST-segment abnormality. Physical examination reveals a parasternal heave and systolic ejection murmur. Past medical history is significant for splenectomy after a car accident several years ago.

Which of the following statements is most accurate concerning this patient’s underlying diagnosis?

A. Inflammatory mechanisms have not been implicated in the pathogenesis
B. Patients should be anticoagulated with a vitamin K antagonist and target INR of 2.5 to 3.5
C. IV epoprostenol is an effective therapy in patients with advanced disease
D. Inhaled iloprost has been demonstrated to improve exercise capacity
E. Bosentan has been shown to improve exercise capacity in patients with mild-to-moderate liver disease

Correct Answer is C

Comment:

IV epoprostenol is an effective therapy in patients with advanced disease. Patients with CTEPH may be bridged to pulmonary endarterectomy with IV epoprostenol. The other answers are incorrect. Anticoagulation with a vitamin K antagonist is indicated; however, the INR target of 2.0 to 3.0 is recommended. The Aerosolized Iloprost Randomization (AIR) study did not demonstrate improved exercise capacity with inhaled iloprost. Bosentan does improve exercise capacity and decreases pulmonary vascular resistance, but is not advocated for use in patients with moderate-to-severe hepatic dysfunction.

Reference:

Hoeper MM, Mayer E, Simonneau G, et al. Chronic thromboembolic pulmonary hypertension. Circulation. 2006;113:2011–2020.