Pulmonary Arterial Hypertension Flashcards
You have been assessing a patient with pulmonary symptoms for 2 years. On angiogram, you apprehend fibrosis and thickening of the pulmonary vessels. Which diagnosis do you suspect?
A. Pulmonary edema
B. Pulmonary arterial hypertension
C. Acute respiratory distress syndrome
D. Pneumonia
Correct Answer: B. Pulmonary arterial hypertension
Rationale:
Fibrosis and thickening of the pulmonary vessels observed on an angiogram are characteristic of chronic changes seen in pulmonary arterial hypertension. This condition leads to remodeling of the pulmonary vasculature, which is not typically seen in pulmonary edema, ARDS, or pneumonia.
PAH / Will Kill)
Which complication of untreated pulmonary arterial hypertension leads to poor prognosis?
A. Mild chronic dyspnea
B. Right ventricular failure
C. Left atrial enlargement
D. Pneumothorax
Correct Answer: B. Right ventricular failure
Rationale:
Untreated pulmonary arterial hypertension leads to increased pressure in the pulmonary arteries, which places a significant workload on the right ventricle. Over time, this results in right ventricular hypertrophy and ultimately right ventricular failure, a complication that is closely linked to a poor prognosis. Mild chronic dyspnea, left atrial enlargement, or pneumothorax are not the primary fatal complications associated with PAH
- (PAH / Management)
In managing PAH, which nonpharmacologic step is commonly recommended?
A. Complete bed rest with no activity
B. Supervised exercise training and supplemental oxygen
C. Aggressive fluid loading
D. High-salt diet
Correct Answer: B. Supervised exercise training and supplemental oxygen
Rationale:
In pulmonary arterial hypertension (PAH), nonpharmacologic management includes lifestyle modifications that can improve functional capacity and quality of life. Supervised exercise training is recommended as it safely improves endurance and cardiovascular health, while supplemental oxygen is used to maintain adequate oxygenation. In contrast, complete bed rest, aggressive fluid loading, or a high-salt diet are not advised as they can worsen symptoms and overall prognosis in PAH
- Pulmonary arterial hypertension is defined by a mean pulmonary artery pressure greater than:
A. 15 mm Hg
B. 20 mm Hg
C. 25 mm Hg at rest
D. 40 mm Hg during exercise
o Answer: C
o Rationale: PAH is diagnosed with a mean pulmonary artery pressure >25 mm Hg at rest via right heart catheterization.
- Which pathophysiologic process underlies pulmonary arterial hypertension?
A. Pulmonary vein occlusion
B. Endothelial dysfunction and vascular remodeling in the pulmonary arteries
C. Left ventricular dysfunction only
D. Alveolar collapse
o Answer: B
o Rationale: PAH is characterized by endothelial dysfunction, vasoconstriction, and remodeling of the pulmonary arterial tree.
- What is a “life‐threatening” complication of PAH if not managed appropriately?
A. Improved exercise tolerance
B. Right ventricular failure
C. Increased oxygen saturation
D. Mild fatigue
o Answer: B
o Rationale: PAH leads to increased right ventricular afterload, eventually resulting in right heart failure, which can be fatal.
- Which nonpharmacologic measure is “common” in the management of PAH?
A. Strict bed rest
B. Supplemental oxygen and supervised exercise programs
C. High-intensity anaerobic exercise
D. Complete avoidance of physical activity
o Answer: B
o Rationale: Oxygen supplementation and tailored exercise improve symptoms and functional capacity in PAH patients.
- In the pharmacologic management of PAH, which drug class is most commonly used?
A. Beta-blockers
B. Endothelin receptor antagonists
C. ACE inhibitors
D. Diuretics exclusively
o Answer: B
o Rationale: Endothelin receptor antagonists help counteract vasoconstriction and vascular remodeling in PAH.
A 45-year-old woman with systemic sclerosis presents with progressive dyspnea and fatigue. Echocardiogram suggests elevated right ventricular pressure. What is the gold standard diagnostic test for pulmonary hypertension?
A) Echocardiogram
B) Pulmonary function tests
C) Right heart catheterization
D) Ventilation-perfusion (V/Q) scan
Answer: C
Rationale: Right heart catheterization is the gold standard for diagnosing pulmonary hypertension and assessing severity.
Which of the following findings is most suggestive of pulmonary hypertension on echocardiogram?
A) Left atrial enlargement
B) Right ventricular hypertrophy and flattening of the interventricular septum
C) Normal right ventricle with increased left ventricular thickness
D) Reduced left ventricular ejection fraction
Answer: B
Rationale: Pulmonary hypertension leads to right ventricular hypertrophy, right atrial enlargement, and interventricular septal flattening due to pressure overload.
A patient with pulmonary arterial hypertension (PAH) undergoes vasoreactivity testing with inhaled nitric oxide. The test is positive. What is the best initial treatment?
A) Phosphodiesterase-5 inhibitors (sildenafil)
B) Calcium channel blockers
C) Endothelin receptor antagonists
D) Prostacyclin analogs
Answer: B
Rationale: Calcium channel blockers (e.g., amlodipine, nifedipine) are used in vasoreactive patients with PAH.
Which of the following conditions is most commonly associated with Group 1 pulmonary hypertension (pulmonary arterial hypertension)?
A) Chronic thromboembolic disease
B) COPD
C) Left-sided heart failure
D) Connective tissue diseases
Answer: D
Rationale: Connective tissue diseases (e.g., systemic sclerosis) are strongly associated with Group 1 PAH.
A 58-year-old man with COPD presents with worsening exertional dyspnea and peripheral edema. Which of the following is the most likely cause of his symptoms?
A) Pulmonary embolism
B) Pulmonary hypertension due to chronic lung disease (Group 3)
C) Acute heart failure exacerbation
D) Primary pulmonary arterial hypertension
Answer: B
Rationale: COPD and other chronic lung diseases (Group 3) commonly lead to pulmonary hypertension.
Which of the following medications should be avoided in pulmonary hypertension with left heart disease (Group 2)?
A) Diuretics
B) Endothelin receptor antagonists
C) Beta-blockers
D) Vasodilators
Answer: B
Rationale: Endothelin receptor antagonists (bosentan) and pulmonary vasodilators can worsen left heart failure by increasing pulmonary congestion.
Which World Health Organization (WHO) classification of pulmonary hypertension is caused by chronic thromboembolic disease?
A) Group 1
B) Group 2
C) Group 3
D) Group 4
Answer: D
Rationale: Group 4 pulmonary hypertension results from chronic thromboembolic disease, often after recurrent pulmonary emboli.
A patient with newly diagnosed pulmonary arterial hypertension (PAH) is started on first-line therapy. Which class of medication is commonly used as initial treatment?
A) Phosphodiesterase-5 inhibitors (e.g., sildenafil)
B) Beta-blockers
C) Inhaled corticosteroids
D) Anticoagulants
Answer: A
Rationale: Phosphodiesterase-5 inhibitors (sildenafil, tadalafil) are first-line treatments for PAH as they promote vasodilation.
Which clinical finding suggests right-sided heart failure due to pulmonary hypertension?
A) Bibasilar crackles
B) Orthopnea
C) Jugular venous distension and peripheral edema
D) S3 gallop at the apex
Answer: C
Rationale: Signs of right heart failure include jugular venous distension, hepatomegaly, and peripheral edema.
Which diagnostic test is most useful in differentiating pulmonary arterial hypertension (PAH) from pulmonary hypertension due to left heart disease?
A) Chest X-ray
B) Echocardiogram
C) Right heart catheterization
D) Pulmonary function test
Answer: C
Rationale: Right heart catheterization differentiates pre-capillary (PAH) vs. post-capillary (left heart disease) pulmonary hypertension.
A 42-year-old woman with systemic sclerosis presents with worsening dyspnea on exertion. Echocardiography shows an estimated pulmonary artery systolic pressure of 55 mmHg. What is the next best diagnostic step?
A) Start sildenafil and repeat echocardiography in 3 months
B) Perform right heart catheterization
C) Order a pulmonary function test
D) Obtain a high-resolution chest CT
Answer: B
Rationale: Right heart catheterization is required to confirm pulmonary hypertension and assess disease severity.
A 55-year-old man with chronic thromboembolic pulmonary hypertension (CTEPH) is being evaluated for definitive treatment. Which intervention has the potential to cure his condition?
A) Oral anticoagulation
B) Riociguat therapy
C) Pulmonary endarterectomy
D) Lung transplant
Answer: C
Rationale: Pulmonary endarterectomy is a surgical procedure that can potentially cure CTEPH by removing organized thrombi from pulmonary arteries.
A patient with pulmonary arterial hypertension (PAH) is being treated with a phosphodiesterase-5 inhibitor. What is the mechanism of action of this class of drugs?
A) Inhibits endothelin-1, reducing vasoconstriction
B) Blocks PDE-5, leading to increased nitric oxide and vasodilation
C) Increases prostacyclin levels, improving pulmonary vascular relaxation
D) Inhibits calcium influx into vascular smooth muscle
Answer: B
Rationale: Phosphodiesterase-5 inhibitors (sildenafil, tadalafil) increase cyclic GMP levels, enhancing nitric oxide–mediated vasodilation.
A 60-year-old woman with COPD presents with worsening dyspnea, peripheral edema, and an accentuated S2 heart sound. Which type of pulmonary hypertension is most likely?
A) Group 1: Pulmonary arterial hypertension
B) Group 2: Pulmonary hypertension due to left heart disease
C) Group 3: Pulmonary hypertension due to lung disease
D) Group 4: Chronic thromboembolic pulmonary hypertension
Answer: C
Rationale: COPD is the most common cause of Group 3 pulmonary hypertension, resulting from chronic hypoxia-induced pulmonary vasoconstriction.
A 70-year-old man with heart failure with preserved ejection fraction (HFpEF) is diagnosed with pulmonary hypertension. What is the most appropriate management strategy?
A) Pulmonary vasodilators such as sildenafil
B) Diuretics and blood pressure control
C) Immediate lung transplant evaluation
D) Long-term anticoagulation
Answer: B
Rationale: Group 2 pulmonary hypertension (due to left heart disease) is best managed by treating the underlying heart condition with diuretics and blood pressure control.
Which of the following pulmonary hypertension groups is most commonly treated with pulmonary vasodilators?
A) Group 1 (Pulmonary arterial hypertension)
B) Group 2 (Left heart disease)
C) Group 3 (Lung disease/hypoxia)
D) Group 4 (CTEPH)
Answer: A
Rationale: Pulmonary vasodilators (e.g., sildenafil, bosentan) are primarily used in Group 1 PAH. They can worsen heart failure in Group 2 patients.
A 52-year-old woman with severe PAH is started on dual therapy with a prostacyclin analog and an endothelin receptor antagonist. Which of the following is the most concerning side effect of prostacyclin therapy?
A) Hepatic toxicity
B) Systemic hypotension
C) Renal dysfunction
D) Severe bradycardia
Answer: B
Rationale: Prostacyclin analogs (e.g., epoprostenol) cause vasodilation, which can lead to systemic hypotension.
Which medication is the first-line therapy for patients with CTEPH who are not surgical candidates?
A) Endothelin receptor antagonists
B) Phosphodiesterase-5 inhibitors
C) Riociguat
D) Calcium channel blockers
Answer: C
Rationale: Riociguat is a soluble guanylate cyclase stimulator and is the only FDA-approved treatment for CTEPH in patients who are not surgical candidates.
A patient with pulmonary hypertension presents with acute decompensation, including hypotension and syncope. Which of the following is the most likely cause?
A) Right ventricular failure
B) Acute pulmonary embolism
C) Sepsis
D) Aortic dissection
Answer: A
Rationale: Right ventricular failure is a major cause of decompensation in pulmonary hypertension and can lead to syncope and hemodynamic collapse.
Which of the following is the most common cause of death in patients with pulmonary hypertension?
A) Acute myocardial infarction
B) Progressive right heart failure
C) Pulmonary embolism
D) Sudden cardiac arrest
Answer: B
Rationale: Progressive right heart failure is the leading cause of death in pulmonary hypertension due to the increased afterload on the right ventricle.
Which of the following clinical findings is highly suggestive of severe pulmonary hypertension?
A) Loud P2 on cardiac auscultation
B) Systolic murmur over the aortic valve
C) Diminished breath sounds bilaterally
D) Fine inspiratory crackles
Answer: A
Rationale: A loud P2 (accentuated pulmonic component of S2) is a hallmark of pulmonary hypertension due to increased pulmonary artery pressure.
A patient with PAH develops worsening symptoms despite monotherapy. What is the next best step in management?
A) Add a second vasodilator therapy
B) Increase diuretic dose
C) Perform balloon pulmonary angioplasty
D) Immediate lung transplant referral
Answer: A
Rationale: Combination therapy (e.g., phosphodiesterase-5 inhibitor + endothelin receptor antagonist) is recommended in patients worsening on monotherapy.
A 65-year-old man with chronic lung disease develops pulmonary hypertension. What is the primary mechanism leading to his condition?
A) Endothelial dysfunction
B) Hypoxia-induced vasoconstriction
C) Pulmonary artery thrombosis
D) Left ventricular dysfunction
Answer: B
Rationale: In Group 3 pulmonary hypertension (due to lung disease), chronic hypoxia leads to pulmonary vasoconstriction.
Which of the following is a contraindication to lung transplant in a patient with pulmonary hypertension?
A) Right ventricular dysfunction
B) Severe deconditioning
C) Use of endothelin receptor antagonists
D) Age over 40
Answer: B
Rationale: Severe deconditioning (poor physical reserve) is a contraindication to lung transplant.
Which of the following is a key lifestyle modification in all patients with pulmonary hypertension?
A) High-sodium diet to maintain blood pressure
B) Avoidance of high-altitude exposure
C) Routine high-intensity exercise
D) Increased diuretic use to prevent fluid overload
Answer: B
Rationale: High-altitude environments exacerbate pulmonary hypertension by increasing pulmonary vascular resistance.
A 55-year-old woman with a history of pulmonary hypertension presents with exertional syncope. Her blood pressure is 85/55 mmHg, and she has a loud P2 on auscultation. What is the most likely cause of her symptoms?
A) Orthostatic hypotension
B) Pulmonary embolism
C) Right ventricular failure with low cardiac output
D) Aortic stenosis
Answer: C
Rationale: Exertional syncope in pulmonary hypertension is often due to right ventricular failure and low cardiac output, indicating worsening disease progression.
Which of the following findings is most consistent with cor pulmonale due to pulmonary hypertension?
A) Systolic murmur at the apex
B) Left ventricular hypertrophy on ECG
C) Right axis deviation and right atrial enlargement on ECG
D) Fine inspiratory crackles and wheezing
Answer: C
Rationale: Right ventricular hypertrophy, right axis deviation, and right atrial enlargement are classic ECG findings in cor pulmonale due to pulmonary hypertension.
A 50-year-old man with idiopathic pulmonary arterial hypertension (PAH) presents with worsening dyspnea despite monotherapy with sildenafil. What is the next best step in management?
A) Add an endothelin receptor antagonist (e.g., bosentan)
B) Increase sildenafil dosage
C) Switch to warfarin therapy
D) Perform balloon pulmonary angioplasty
Answer: A
Rationale: Combination therapy (e.g., phosphodiesterase-5 inhibitor + endothelin receptor antagonist) is recommended when monotherapy fails.
Which of the following physical exam findings is most specific for pulmonary hypertension?
A) Bibasilar inspiratory crackles
B) Loud, palpable second heart sound (P2)
C) Systolic murmur at the apex
D) Diminished breath sounds bilaterally
Answer: B
Rationale: A loud and accentuated P2 indicates elevated pulmonary artery pressure, making it a hallmark sign of pulmonary hypertension.
Which of the following is a major contraindication to the use of phosphodiesterase-5 inhibitors in pulmonary arterial hypertension?
A) Concomitant use of nitrates
B) Pulmonary hypertension due to left heart failure
C) Chronic thromboembolic pulmonary hypertension
D) Use of diuretics
Answer: A
Rationale: Phosphodiesterase-5 inhibitors (e.g., sildenafil) can cause severe hypotension when used with nitrates and are contraindicated in such cases.
A 65-year-old woman with pulmonary hypertension presents with worsening dyspnea and ascites. Which of the following laboratory findings would be expected?
A) Decreased brain natriuretic peptide (BNP)
B) Elevated central venous pressure (CVP)
C) Increased left ventricular ejection fraction
D) Normal pulmonary artery pressure
Answer: B
Rationale: Right heart failure in pulmonary hypertension leads to elevated CVP, systemic congestion, and signs of volume overload.
Which of the following is an appropriate treatment for a patient with chronic thromboembolic pulmonary hypertension (CTEPH) who is not a surgical candidate?
A) Endothelin receptor antagonist (e.g., bosentan)
B) Riociguat (soluble guanylate cyclase stimulator)
C) Beta-blockers
D) Inhaled prostacyclin therapy
Answer: B
Rationale: Riociguat is FDA-approved for treating CTEPH in patients who are not candidates for pulmonary endarterectomy.
A 55-year-old man with Group 3 pulmonary hypertension due to interstitial lung disease is evaluated for therapy. Which treatment is most appropriate?
A) High-dose sildenafil
B) Supplemental oxygen therapy
C) Endothelin receptor antagonist
D) Pulmonary artery thrombolysis
Answer: B
Rationale: In Group 3 (lung disease–associated pulmonary hypertension), oxygen therapy is the most effective treatment to reduce hypoxia-induced vasoconstriction.
A 68-year-old woman with pulmonary hypertension presents with new onset of hemoptysis and pleuritic chest pain. What is the most concerning diagnosis?
A) Pulmonary embolism
B) Right heart failure
C) Acute respiratory distress syndrome (ARDS)
D) Myocardial infarction
Answer: A
Rationale: Hemoptysis and pleuritic chest pain in pulmonary hypertension raise concern for acute pulmonary embolism, which can cause rapid deterioration.
A patient with advanced pulmonary hypertension presents with severe fatigue, dyspnea, and profound hypotension. Which of the following is the most likely cause?
A) Septic shock
B) Acute right ventricular failure
C) Aortic dissection
D) Pulmonary infarction
Answer: B
Rationale: Acute right ventricular failure in severe pulmonary hypertension can lead to hemodynamic collapse, presenting with hypotension and severe dyspnea.
gold standard for diagnosing and classifying pulmonary hypertension
Right heart catheterization
Exertional syncope in pulmonary hypertension
suggests right ventricular failure and low cardiac output
key physical and ECG findings in PAH
Loud P2, right ventricular hypertrophy, and right axis
is treated with vasodilators
Group 1 PAH
is managed by treating heart failure
Group 2 (left heart disease)
Group 3 (chronic lung disease)
responds best to oxygen therapy
Phosphodiesterase-5 inhibitors (sildenafil)
should be avoided in patients taking nitrates
Riociguat is the first-line therapy for CTEPH
in nonsurgical candidates
Acute decompensation in pulmonary hypertension
is most often due to right ventricular failure
