Pulmunory HTN

Question 1

What is the definition of pulmonary arterial hypertension (PAH)?

Answer 1

Resting mean pulmonary artery pressure of >= 20 mmHg

PAH is a specific type of pulmonary hypertension that occurs in the absence of other chronic lung diseases.

Question 2

What role does endothelin play in pulmonary arterial hypertension?

Answer 2

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

Endothelin is a peptide that causes vasoconstriction and is linked to increased blood pressure.

Question 3

In which demographic is pulmonary arterial hypertension more common?

Answer 3

Females

The typical age range for presentation is between 30-50 years.

Question 4

List some factors that increase the risk of developing PAH.

Answer 4

• HIV
• Cocaine
• Anorexigens (e.g. fenfluramine)

These factors can contribute to the development of PAH in susceptible individuals.

Question 5

What percentage of PAH cases are inherited in an autosomal dominant fashion?

Answer 5

Around 10%

This genetic aspect highlights the heritable nature of certain cases of PAH.

Question 6

What is the classical presentation of pulmonary arterial hypertension?

Answer 6

Progressive exertional dyspnoea

Dyspnoea refers to difficulty or discomfort in breathing.

Question 7

Name other possible features of pulmonary arterial hypertension.

Answer 7

• Exertional syncope
• Exertional chest pain
• Peripheral oedema
• Cyanosis

These symptoms can vary in severity and may not be present in all patients.

Question 8

What clinical sign indicates right ventricular hypertrophy or dilatation?

Answer 8

Right ventricular heave

This physical examination finding suggests increased workload on the right ventricle.

Question 9

What does a loud P2 indicate in the context of PAH?

Answer 9

Increased pulmonary artery pressure

A palpable P2 may occur in severe cases of PAH.

Question 10

What does raised JVP with prominent ‘a’ waves reflect in PAH?

Answer 10

Increased resistance to right atrial emptying due to elevated right ventricular end-diastolic pressure

This is indicative of right heart strain.

Question 11

What is the first step in managing pulmonary arterial hypertension?

Answer 11

Treating any underlying conditions

This may include the use of anticoagulants or oxygen.

Question 12

What is the purpose of acute vasodilator testing in PAH management?

Answer 12

To determine which patients show a significant fall in pulmonary arterial pressure following vasodilator administration.

This testing is central to deciding the management strategy.

Question 13

What should be done if there is a positive response to acute vasodilator testing?

Answer 13

Administer oral calcium channel blockers

nifidipine

This treatment is targeted towards patients who respond positively to vasodilators.

Question 14

What are the treatment options for patients with a negative response to acute vasodilator testing?

Answer 14

• Prostacyclin analogues (e.g., treprostinil, iloprost)
• Endothelin receptor antagonists
  
  • Non-selective: bosentan
  • Selective antagonist of endothelin receptor A: ambrisentan
• Phosphodiesterase inhibitors: sildenafil

These medications help to manage symptoms and improve quality of life.

Question 15

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

Answer 15

Heart-lung transplant

This is a surgical option for patients with advanced disease who do not respond to medical therapy.

Question 16

what heart sound can be hear on PHTN due to PE

Answer 16

loud s2