PH Flashcards
What is the updated mean pulmonary artery pressure (mPAP) threshold for diagnosing PH?
A) ≥25 mmHg
B) >25 mmHg
C) >20 mmHg
D) ≥20 mmHg
Answer: C
Rationale: The diagnostic threshold for PH has been revised from ≥25 mmHg to >20 mmHg, recognizing that significant disease may be present even at lower pressures.
Which of the following is the gold standard for diagnosing PH?
A) Echocardiography
B) Pulmonary function test
C) Right heart catheterization (RHC)
D) 6-minute walk distance test
Answer: C
Rationale: Right heart catheterization (RHC) remains the gold standard for PH diagnosis, providing direct hemodynamic measurements of pulmonary artery pressure and vascular resistance.
What is the most common cause of pulmonary hypertension?
A) Chronic thromboembolism
B) Primary lung disease
C) Left heart disease
D) Sarcoidosis
Answer: C
Rationale: Left heart disease is the most common cause of PH, often resulting from left ventricular dysfunction, valvular disease, or heart failure.
Which imaging modality is most useful for evaluating chronic thromboembolic pulmonary hypertension (CTEPH)?
A) High-resolution computed tomography (HRCT)
B) Ventilation-perfusion (V/Q) scan
C) Chest X-ray
D) Pulmonary function test
Answer: B
Rationale: A ventilation-perfusion (V/Q) scan is highly sensitive for detecting CTEPH, which results from unresolved pulmonary emboli leading to vascular obstruction and fibrosis.
Which PH subtype is commonly associated with chronic obstructive pulmonary disease (COPD)?
A) PH Type 1 (PAH)
B) PH Type 2 (Left heart disease)
C) PH Type 3 (Lung disease)
D) PH Type 4 (Chronic thromboembolic disease)
Answer: C
Rationale: PH Type 3 is associated with lung diseases like COPD and interstitial lung disease.
PH Type 3: Pulmonary Hypertension Associated with Lung Disease
* Intrinsic lung disease is the second most common cause of PH and has been observed in both chronic obstructive pulmonary disease (COPD) and interstitial lung disease.
* When associated with chronic lung disease, PH is usually modest.
* Sleep-disordered syndromes generally result in mild PH.
What is the hallmark feature of precapillary PH?
A) PAWP >15 mmHg
B) PVR <3.0 WU
C) PAWP ≤15 mmHg
D) Left ventricular hypertrophy
Answer: C
Rationale: Precapillary PH is characterized by a pulmonary artery wedge pressure (PAWP) of ≤15 mmHg and increased pulmonary vascular resistance (PVR ≥3.0 WU).
- Precapillary PH
- PAWP or LVEDP- ≤15 mmHg
- PVR ≥3.0 WU
- isolated precapillary PH is most often due to primary lung disease, PAH, or CTEPH
- Postcapillary PH
- PAWP >15 mmHg
- PVR <3.0 WU
- Isolated postcapillary PH occurs in patients with mitral valvular disease, left ventricular systolic dysfunction, or heart failure with preserved ejection faction
- Combined pre- and postcapillary PH
- mPAP >20 mmHg
- PVR ≥3.0 WU
- PAWP >15 mmHg
Which pharmacologic pathway is targeted by sildenafil and tadalafil in PH treatment?
A) Endothelin receptor pathway
B) Nitric oxide (NO) pathway
C) Prostacyclin pathway
D) Renin-angiotensin pathway
Answer: B
Rationale: Sildenafil and tadalafil are PDE-5 inhibitors that enhance the nitric oxide pathway by preventing cGMP degradation, leading to vasodilation.
- The two PDE-5 inhibitors used for the treatment of PAH are sildenafil and tadalafil
- Both agents have been shown to improve hemodynamics and 6-MWD.
Which condition is the leading cause of PH globally?
A) Chronic thromboembolism
B) Sickle cell disease
C) Schistosomiasis
D) Sarcoidosis
Answer: C
Rationale: Schistosomiasis is a leading cause of PH worldwide due to its association with hepatosplenic disease and portal hypertension.
Which class of drugs includes bosentan and macitentan?
A) Endothelin receptor antagonists
B) PDE-5 inhibitors
C) Prostacyclin analogs
D) Beta-blockers
Answer: A
Rationale: Bosentan and macitentan are endothelin receptor antagonists that inhibit endothelin-1, a potent vasoconstrictor.
- nonselective ETA/B receptor antagonists bosentan and macitentan
- The randomized, placebo-controlled, phase 3 Bosentan Randomized Trial of Endothelin Antagonist Therapy (BREATHE)-1
- The Endothelin Antagonist Trial in Mildly Symptomatic Pulmonary Arterial Hypertension Patients (EARLY) study
- selective ETA antagonist ambrisentan
- phase 3, placebo-controlled Ambrisentan in Pulmonary Arterial Hypertension, (ARIES)-1 trial
What is the purpose of vasoreactivity testing in PH?
A) To assess lung function in patients with PH
B) To determine if a patient will respond to calcium channel blockers
C) To diagnose PH Type 2
D) To evaluate exercise capacity
Answer: B
Rationale: Vasoreactivity testing assesses responsiveness to vasodilators like nitric oxide and helps determine if patients may benefit from calcium channel blockers.
* should be reserved mainly for patients with idiopathic or hereditary PAH * inhaled nitric oxide (NO) or inhaled epoprostenol * decrease in mPAP by ≥10 mmHg to an absolute level ≤40 mmHg without a decrease in CO is defined as a positive pulmonary vasodilator response, and such responders are considered for long-term treatment with calcium channel blockers
Which PH subtype is most common in patients with mitral valvular disease?
A) PH Type 1
B) PH Type 2
C) PH Type 3
D) PH Type 4
Answer: B
Rationale: PH Type 2 is linked to left heart disease, including mitral valve disease and heart failure.
What was the primary outcome of the AMBITION trial for PH treatment?
A) Triple therapy was superior to dual therapy
B) Ambrisentan and tadalafil reduced clinical worsening by 50%
C) Bosentan was superior to sildenafil
D) PDE-5 inhibitors improved mortality rates
Answer: B
Rationale: The AMBITION trial showed that combination therapy with ambrisentan and tadalafil reduced clinical worsening risk by 50%
What is the primary mechanism of action of riociguat?
A) PDE-5 inhibition
B) Endothelin receptor antagonism
C) Soluble guanylate cyclase (sGC) stimulation
D) Calcium channel blockade
Answer: C
Rationale: Riociguat stimulates soluble guanylate cyclase (sGC), enhancing nitric oxide signaling and vasodilation.
- Riociguat - increases bioactive cGMP by (1) stabilizing the molecular interaction between NO* and sGC, and (2) directly stimulating sGC independent of NO* bioavailability
- sole approved pharmacotherapy for CTEPH patients for whom surgical pulmonary endarterectomy is ineffective or contraindicated.
What is the target 6-minute walk distance (6-MWD) in low-risk PAH patients?
A) >250 meters
B) >300 meters
C) >440 meters
D) >500 meters
Answer: C
Rationale: A 6-MWD >440 meters indicates a low-risk clinical profile in PAH patients.
