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.
