Question 1

Which of the following is a defining hemodynamic criterion for diagnosing pulmonary arterial hypertension (PAH) via right heart catheterization?

Accepted Answer

Pulmonary artery mean pressure $$\u003e 25$$ mmHg and pulmonary capillary wedge pressure $$\u003c 15$$ mmHg

Question 2

A patient with idiopathic PAH is found to have a mutation in the BMPR2 gene. What is the normal function of the protein encoded by this gene?

Accepted Answer

Inhibits arterial smooth muscle cell and endothelial cell proliferation

Question 3

Which of the following best explains why chronic hypoxemia leads to pulmonary hypertension in Group 3 PAH?

Accepted Answer

Hypoxemia stimulates pulmonary vasoconstriction, increasing pulmonary artery pressure and resistance

Question 4

Which pathway is primarily responsible for vasodilation and inhibition of smooth muscle cell (SMC) proliferation in the pulmonary circulation?

Accepted Answer

Nitric oxide (NO) pathway

Question 5

A patient with PAH is treated with a prostacyclin analog. What is the expected effect of this medication on the pulmonary vasculature?

Accepted Answer

Promote vasodilation and inhibit SMC proliferation and platelet aggregation

Question 6

Which of the following drugs is associated with drug-induced pulmonary arterial hypertension (PAH)?

Accepted Answer

Fenfluramine

Question 7

In the pathophysiology of PAH, which change in the prostacyclin/thromboxane pathway contributes to disease progression?

Accepted Answer

Shift toward increased thromboxane A2 and decreased prostacyclin (PGI2)

Question 8

Which of the following is a common feature of remodeled pulmonary vessels in PAH?

Accepted Answer

Vascular lumen narrows, leading to increased resistance

Question 9

A patient with chronic thromboembolic pulmonary hypertension (CTEPH) is most likely to have which underlying pathophysiological process?

Accepted Answer

Chronic microthrombi causing persistent obstruction of pulmonary arteries

Question 10

Which receptor subtype is responsible for vasoconstriction, smooth muscle cell proliferation, hypertrophy, and fibrosis in the endothelin-1 pathway?

Accepted Answer

ETA receptor on smooth muscle cells

Question 11

A patient with PAH presents with jugular venous distension (JVD), lower extremity edema, and exertional chest pain. These symptoms are most consistent with which complication?

Accepted Answer

Right-sided heart failure

Question 12

Which of the following mechanisms is LEAST likely to contribute to the development of pulmonary arterial hypertension?

Accepted Answer

Decreased inflammation in pulmonary vessels

Question 13

Which equation best represents the relationship between pulmonary vascular resistance (PVR), mean pulmonary artery pressure (PAP), pulmonary capillary wedge pressure (PCWP), and cardiac output (CO)?

Accepted Answer

$$PVR = \frac{PAP - PCWP}{CO}$$

Question 14

Which of the following is a direct effect of nitric oxide (NO) on pulmonary vascular smooth muscle cells?

Accepted Answer

Inhibits smooth muscle cell proliferation

Question 15

A patient with PAH is prescribed a phosphodiesterase-5 inhibitor. What is the mechanism of action of this drug in the pulmonary vasculature?

Accepted Answer

Inhibits breakdown of cGMP, promoting vasodilation and reduced SMC proliferation

Question 16

Which of the following is NOT a common etiology of Group 2 pulmonary hypertension?

Accepted Answer

COPD

Question 17

Which of the following clinical findings is most specific for right-sided heart failure secondary to PAH?

Accepted Answer

Jugular venous distension (JVD)

Question 18

Which of the following statements about the endothelin-1 (ET-1) pathway in PAH is correct?

Accepted Answer

ETB receptor on endothelial cells promotes NO and PGI2 production

Question 19

A patient with PAH secondary to connective tissue disease is classified under which group of pulmonary hypertension?

Accepted Answer

Group I (Pulmonary Arterial Hypertension)

Disorders of Pulmonary Circulation: Pulmonary Hypertension

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