Pulmonary arterial hypertension

Question 1

What is pulmonary arterial hypertension (PAH)?

Answer 1

PAH is defined as a resting mean pulmonary artery pressure of >= 20 mmHg.

Question 2

What role does endothelin play in PAH?

Answer 2

Endothelin is thought to play a key role in the pathogenesis of PAH.

Question 3

Who is more commonly affected by PAH?

Answer 3

PAH is more common in females and typically presents between the ages of 30-50 years.

Question 4

What can cause pulmonary hypertension secondary to PAH?

Answer 4

Chronic lung diseases such as COPD can cause pulmonary hypertension.

Question 5

What factors increase the risk of developing PAH?

Answer 5

Risk factors include HIV, cocaine use, and anorexigens (e.g. fenfluramine).

Question 6

What percentage of PAH cases are inherited?

Answer 6

Around 10% of cases are inherited in an autosomal dominant fashion.

Question 7

What is the classical presentation of PAH?

Answer 7

Progressive exertional dyspnoea is the classical presentation.

Question 8

What are other possible features of PAH?

Answer 8

Other features include exertional syncope, exertional chest pain, and peripheral oedema.

Question 9

What clinical signs indicate PAH?

Answer 9

Signs include right ventricular heave, loud P2, raised JVP with prominent ‘a’ waves, and tricuspid regurgitation.

Question 10

What does a loud P2 indicate in PAH?

Answer 10

A loud P2 reflects increased pulmonary artery pressure and may be accompanied by a palpable P2 in severe cases.

Question 11

What is the first step in managing PAH?

Answer 11

Management should first involve treating any underlying conditions, such as with anticoagulants or oxygen.

Question 12

What is the purpose of acute vasodilator testing in PAH?

Answer 12

Acute vasodilator testing aims to determine which patients show a significant fall in pulmonary arterial pressure after vasodilator administration.

Question 13

What is the treatment for patients with a positive response to acute vasodilator testing?

Answer 13

Oral calcium channel blockers are used for patients with a positive response.

Question 14

What treatments are available for patients with a negative response to acute vasodilator testing?

Answer 14

Treatments include prostacyclin analogues, endothelin receptor antagonists, and phosphodiesterase inhibitors.

Question 15

What are examples of prostacyclin analogues?

Answer 15

Examples include treprostinil and iloprost.

Question 16

What are examples of endothelin receptor antagonists?

Answer 16

Examples include bosentan (non-selective) and ambrisentan (selective antagonist of endothelin receptor A).

Question 17

What should be considered for patients with progressive symptoms of PAH?

Answer 17

Patients with progressive symptoms should be considered for a heart-lung transplant.

Question 18

What is pulmonary hypertension?

Answer 18

Pulmonary hypertension is defined as a sustained elevation in mean pulmonary arterial pressure of greater than 20 mmHg at rest.

Question 19

How has pulmonary hypertension been reclassified by the WHO?

Answer 19

Pulmonary hypertension has been reclassified into five groups.

Question 20

What are the subcategories of Group 1 pulmonary hypertension?

Answer 20

Group 1 includes pulmonary arterial hypertension (PAH), which can be idiopathic, familial, or associated with conditions such as collagen vascular disease, congenital heart disease with systemic to pulmonary shunts, HIV, drugs and toxins, and sickle cell disease, as well as persistent pulmonary hypertension of the newborn.

Question 21

What is included in Group 2 pulmonary hypertension?

Answer 21

Group 2 includes pulmonary hypertension with left heart disease, such as left-sided atrial, ventricular or valvular disease, including left ventricular systolic and diastolic dysfunction, mitral stenosis, and mitral regurgitation.

Question 22

What conditions are classified under Group 3 pulmonary hypertension?

Answer 22

Group 3 includes pulmonary hypertension secondary to lung disease or hypoxia, such as COPD, interstitial lung disease, sleep apnoea, and high altitude.

Question 23

What is Group 4 pulmonary hypertension?

Answer 23

Group 4 is pulmonary hypertension due to thromboembolic disease.

Question 24

What does Group 5 pulmonary hypertension encompass?

Answer 24

Group 5 includes miscellaneous conditions, such as lymphangiomatosis, e.g., secondary to carcinomatosis or sarcoidosis.

Question 25

What was the previous term for idiopathic pulmonary arterial hypertension?

Answer 25

Idiopathic pulmonary arterial hypertension was previously termed primary pulmonary hypertension.

Question 26

What is known about the mechanism of HIV-related pulmonary hypertension?

Answer 26

The mechanism by which HIV infection produces pulmonary hypertension remains unknown.