CME Self-Assessment Examination

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1. Which of the following statements is NOT part of changes implemented to the Dana Point Classification of Pulmonary Hypertension?
   
   1. The term Heritable PH replaced the term Familial PH
   2. Schistosomiasis is now part of Group I PAH
   3. Hemolytic anemias are reclassified as their own subgroup under Group I PAH
   4. PAH associated with congenital heart disease has been reclassified to Group II PH (left heart disease)
   5. Group IV CTEPH has been simplified with proximal and distal descriptions removed   
2. All of the following reflect current understanding of genetics in PAH except:
   1. BMPR2 is a cell surface receptor of the TGF-ß
   2. Heritable PAH includes only those individuals whose family members have been identified to carry the mutation in the BMPR2 gene
   3. Other cell surface receptors whose mutations have been implicated in PAH include ALK-1 and endoglin
   4. BMPR2 mutations have been identified in about 20% of IPAH patients   
3. Which of the following statements is true about heritable PAH (HPAH)?
   1. HPAH is more common among older individuals presenting with newly diagnosed PAH
   2. Patients with HPAH are more likely to respond to acute vasodilator testing and hence increased likelihood of responding to calcium channel blockers
   3. Patients with HPAH are likely to have more severe hemodynamic impairment at the time of diagnosis
   4. Genetic mutations involved in HPAH affect every generation with complete penetrance   
4. With regard to diagnosing and assessing prognosis in PAH, which of the statement(s) is/are most correct?
   1. Tricuspid jet velocity >2.8 m/s and tricuspid insufficiency pressure gradient >31 mm Hg at rest are elevated and diagnostic of PAH
   2. Patients who remain FC III or IV after 3 months of therapy portend poor prognosis
   3. The current definition of PAH has excluded exercise hemodynamics due to inconclusive evidence
   4. B and C
   5. All of the above   
5. All of the statements are correct with regard to current state of PAH therapies except:
   1. There are currently 8 PAH-specific therapies approved in the US
   2. Oral anticoagulation is recommended for idiopathic PAH and all subgroups of PAH patients in the absence of contraindication
   3. High doses of calcium channel blockers are indicated only in those patients who are responders to acute vasoreactivity testing
   4. Treatment of choice for functional class IV patients remains continuous administration of intravenous epoprostenol
   5. Combination therapy is recommended for patients treated with either PDE5 inhibitors or endothelin receptor antagonist who remain FC III   
6. In reference to PH associated with chronic obstructive pulmonary disease (COPD) and interstitial lung disease:
   1. Even mild PH is an independent risk factor of mortality in patients with COPD and PH
   2. Echocardiogram is an effective screening test but has limitations with significant false positive and negative findings in patients with lung disease and PH
   3. No PAH-specific therapies have demonstrated benefit in patients with "out-of-proportion" PH and COPD
   4. A and B
   5. All are correct   
7. All of the following statements regarding chronic thromboembolic pulmonary hypertension (CTEPH) are true except:
   1. CTEPH differs from PAH by its major vessel involvement of the vascular remodeling process
   2. CT angiography is the test of choice for ruling out CTEPH
   3. Pulmonary endarterectomy is the treatment of choice for CTEPH for surgically appropriate patients
   4. BENEFIT study is the only randomized, controlled study conducted among patients with inoperable CTEPH   
8. In PH associated with left heart disease:
   1. Elevated PA pressures in left heart disease can be due to combination of passive chronic pulmonary venous hypertension and superimposed active component due to pulmonary arterial remodeling
   2. Risk factors for diastolic dysfunction include older age, hypertension, obesity, and diabetes
   3. Invasive measurement of pulmonary capillary wedge pressure or left ventricular end diastolic pressure is necessary to distinguish PH associated with diastolic dysfunction from PAH
   4. B and C
   5. All of the above