Question 1

What is the most common pulmonary function abnormality found in patients with idiopathic pulmonary artery hypertension (IPAH)?

Accepted Answer

Pulmonary function tests are useful to rule out the presence of significant restrictive or obstructive airway disease. The most common abnormality at pulmonary function testing of patients with IPAH is a low carbon monoxide diffusing capacity (DLCO).

Question 2

Why are calcium-channel blockers not used in all patients with idiopathic pulmonary artery hypertension?

Accepted Answer

Patients with IPAH who respond to vasodilators in the short term have improved survival with long-term use of calcium-channel blockers. Thus, these agents should be considered in all patients who have significant and definite response to a short-acting vasodilator. Unfortunately, only a small fraction of IPAH patients qualify for and benefit from long-term therapy with oral calcium-channel blockers.

Question 3

Which of the following physical examination findings are found in patients with primary pulmonary hypertension? 1) Cyanosis 2) Diastolic heart murmur 3) Digital clubbing 4) Loud second heart sound (P₂)

Accepted Answer

The answer of Which of the following physical examination findings...

Question 4

In what type of IPAH patient is lung transplantation indicated?

Accepted Answer

Single or double lung transplantation has been used successfully in the treatment of patients with IPAH. Lung transplantation is indicated in the care of patients who do not respond to vasodilators and have significant cardiac impairment.

Question 5

What is the most important noninvasive diagnostic test for evaluation of a patient with idiopathic pulmonary artery hypertension (IPAH)?

Accepted Answer

The most important noninvasive test for IPAH is the 11ea8d2d_21d0_fad2_b445_f9890d4bd84c_TB4333_11 lung scan, which helps to rule out the possibility of chronic thromboembolic pulmonary hypertension (a mimic of IPAH).

Question 6

What ECG finding is most often associated with right-sided heart failure and pulmonary hypertension?

Accepted Answer

ECG findings usually include right-axis deviation, right ventricular hypertrophy, and strain. The echocardiogram may show dilatation of the right ventricle and right atrium and tricuspid regurgitation.

Question 7

What is the 5-year survival rate for patients with untreated idiopathic pulmonary artery hypertension (IPAH)?

Accepted Answer

IPAH can be life threatening and carries a poor prognosis. Without therapy, only 33% of patients are alive 5 years after the onset of the disorder.

Question 8

What type of drug is Bosentan?

Accepted Answer

Endothelin antagonists represent another class of medications that are newly available for treating pulmonary hypertension. Bosentan, an orally administered endothelin receptor antagonist, improves walking distance, hemodynamic variables, and functional class in patients with PAH.

Question 9

What ECG findings are typically seen in patients with idiopathic pulmonary artery hypertension?
1) Frequent premature ventricular complexes
2) Right-axis deviation
3) Right ventricular hypertrophy
4) Left bundle-branch block

Accepted Answer

Patients with idiopathic pulmonary artery hypertension typically have right ventricular hypertrophy on ECG, which is represented by an increased R wave amplitude in leads V1-V4 and a rightward shift or prominence of the QRS axis. Left bundle-branch block is not commonly seen in this population. Premature ventricular complexes may occur but are not a consistent finding. Therefore, the best choices are options 2 and 3.

Question 10

Most hospitalized patients who are immobile need prophylaxis for venous thromboembolism.

Accepted Answer

Most hospitalized patients who are immobile need prophylaxis for venous thromboembolism.

Question 11

Which of the following radiographic findings is not typical for patients with primary pulmonary hypertension?

Accepted Answer

The chest radiographic findings include enlargement of the main and hilar pulmonary arteries, "pruning" (or narrowing) of the peripheral arteries, and enlargement of the right ventricle and atrium though the chest radiograph may remain normal in 6% of patients. Pleural effusions are not present.

Question 12

The presence of previous chronic obstructive pulmonary disease (COPD) reduces the diagnostic usefulness of scans in acute pulmonary embolism.

Accepted Answer

The presence of concomitant cardiopulmonary disease even if severe (e.g., hypoxemia with or without ventilatory support, a condition that necessitates intensive care), or COPD does not diminish the diagnostic usefulness of scans in the diagnosis of acute pulmonary embolism.

Question 13

What is the most important factor contributing to the onset of pulmonary hypertension in the patient with COPD?

Accepted Answer

Alveolar hypoxia, because of its potent pulmonary vasoconstrictive effect, is probably the most important factor contributing to pulmonary hypertension in patients with COPD. Sustained alveolar hypoxia causes pulmonary vasoconstriction and eventually medial hypertrophy, fibrosis of the intima, and narrowing of the lumen of the pulmonary blood vessels.

Question 14

What therapy has been proven to improve survival in patients with COPD and pulmonary hypertension?

Accepted Answer

Oxygen therapy is the main treatment that improves survival among patients with COPD and pulmonary hypertension, though smoking cessation and lung volume reduction (in selected individuals) may also confer survival benefits in patients with COPD.

Question 15

Why is the use of oxygen to maintain oxygen saturations greater than 90% particularly important in the management of idiopathic pulmonary artery hypertension?

Accepted Answer

Supplemental oxygen should be used to maintain oxygen saturation greater than 90%, especially because hypoxemia is a major cause of pulmonary vasoconstriction.

Question 16

What IV infusion has been found to improve exercise capacity, hemodynamic variables, and survival in patients with severe IPAH?

Accepted Answer

Epoprostenol delivered by continuous intravenous infusion, improves exercise capacity, hemodynamic variables, and survival in IPAH patients and is the treatment of choice for severely ill patients.

Question 17

Unless there are contraindications, what treatment is given universally to all patients with idiopathic pulmonary artery hypertension (IPAH)?

Accepted Answer

Oral anticoagulation improves survival in IPAH and is recommended in all these patients unless there is a contraindication to anticoagulation.

Quiz 28: Pulmonary Vascular Disease

What is the most common pulmonary function abnormality found in patients with idiopathic pulmonary artery hypertension (IPAH) ?

Why are calcium-channel blockers not used in all patients with idiopathic pulmonary artery hypertension?

Which of the following physical examination findings are found in patients with primary pulmonary hypertension? 1) Cyanosis 2) Diastolic heart murmur 3) Digital clubbing 4) Loud second heart sound (P₂)

In what type of IPAH patient is lung transplantation indicated?

What is the most important noninvasive diagnostic test for evaluation of a patient with idiopathic pulmonary artery hypertension (IPAH) ?

What ECG finding is most often associated with right-sided heart failure and pulmonary hypertension?

What is the 5-year survival rate for patients with untreated idiopathic pulmonary artery hypertension (IPAH) ?

What type of drug is Bosentan?

What ECG findings are typically seen in patients with idiopathic pulmonary artery hypertension? 1) Frequent premature ventricular complexes 2) Right-axis deviation 3) Right ventricular hypertrophy 4) Left bundle-branch block

Most hospitalized patients who are immobile need prophylaxis for venous thromboembolism.

Which of the following radiographic findings is not typical for patients with primary pulmonary hypertension?

The presence of previous chronic obstructive pulmonary disease (COPD) reduces the diagnostic usefulness of scans in acute pulmonary embolism.

What is the most important factor contributing to the onset of pulmonary hypertension in the patient with COPD?

What therapy has been proven to improve survival in patients with COPD and pulmonary hypertension?

Why is the use of oxygen to maintain oxygen saturations greater than 90% particularly important in the management of idiopathic pulmonary artery hypertension?

What IV infusion has been found to improve exercise capacity, hemodynamic variables, and survival in patients with severe IPAH?

Unless there are contraindications, what treatment is given universally to all patients with idiopathic pulmonary artery hypertension (IPAH) ?

Quiz 28: Pulmonary Vascular Disease

What is the most common pulmonary function abnormality found in patients with idiopathic pulmonary artery hypertension (IPAH) ?

Why are calcium-channel blockers not used in all patients with idiopathic pulmonary artery hypertension?

Which of the following physical examination findings are found in patients with primary pulmonary hypertension? 1) Cyanosis 2) Diastolic heart murmur 3) Digital clubbing 4) Loud second heart sound (P2)

In what type of IPAH patient is lung transplantation indicated?

What is the most important noninvasive diagnostic test for evaluation of a patient with idiopathic pulmonary artery hypertension (IPAH) ?

What ECG finding is most often associated with right-sided heart failure and pulmonary hypertension?

What is the 5-year survival rate for patients with untreated idiopathic pulmonary artery hypertension (IPAH) ?

What type of drug is Bosentan?

What ECG findings are typically seen in patients with idiopathic pulmonary artery hypertension? 1) Frequent premature ventricular complexes 2) Right-axis deviation 3) Right ventricular hypertrophy 4) Left bundle-branch block

Most hospitalized patients who are immobile need prophylaxis for venous thromboembolism.

Which of the following radiographic findings is not typical for patients with primary pulmonary hypertension?

The presence of previous chronic obstructive pulmonary disease (COPD) reduces the diagnostic usefulness of scans in acute pulmonary embolism.

What is the most important factor contributing to the onset of pulmonary hypertension in the patient with COPD?

What therapy has been proven to improve survival in patients with COPD and pulmonary hypertension?

Why is the use of oxygen to maintain oxygen saturations greater than 90% particularly important in the management of idiopathic pulmonary artery hypertension?

What IV infusion has been found to improve exercise capacity, hemodynamic variables, and survival in patients with severe IPAH?

Unless there are contraindications, what treatment is given universally to all patients with idiopathic pulmonary artery hypertension (IPAH) ?

Which of the following physical examination findings are found in patients with primary pulmonary hypertension? 1) Cyanosis 2) Diastolic heart murmur 3) Digital clubbing 4) Loud second heart sound (P₂)