Pulmonary arterial hypertension Flashcards
What is pulmonary arterial hypertension (PAH)?
PAH is defined as a resting mean pulmonary artery pressure of >= 20 mmHg.
What role does endothelin play in PAH?
Endothelin is thought to play a key role in the pathogenesis of PAH.
Who is more commonly affected by PAH?
PAH is more common in females and typically presents between the ages of 30-50 years.
What can cause pulmonary hypertension secondary to PAH?
Chronic lung diseases such as COPD can cause pulmonary hypertension, but PAH is diagnosed in their absence.
What factors increase the risk of PAH?
Factors include HIV, cocaine use, and anorexigens (e.g. fenfluramine).
What percentage of PAH cases are inherited?
Around 10% of cases are inherited in an autosomal dominant fashion.
What is the classical presentation of PAH?
Progressive exertional dyspnoea is the classical presentation.
What are other possible features of PAH?
Other features include exertional syncope, exertional chest pain, and peripheral oedema.
What clinical signs indicate PAH?
Signs include right ventricular heave, loud P2, raised JVP with prominent ‘a’ waves, and tricuspid regurgitation.
What does a loud P2 indicate in PAH?
A loud P2 reflects increased pulmonary artery pressure and may be accompanied by a palpable P2 in severe cases.
What is the first step in managing PAH?
Management should first involve treating any underlying conditions, for example with anticoagulants or oxygen.
What is the purpose of acute vasodilator testing in PAH?
Acute vasodilator testing aims to decide which patients show a significant fall in pulmonary arterial pressure following vasodilator administration.
What is the management for patients with a positive response to acute vasodilator testing?
Oral calcium channel blockers are used for patients with a positive response.
What is the management for patients with a negative response to acute vasodilator testing?
Management includes prostacyclin analogues, endothelin receptor antagonists, and phosphodiesterase inhibitors.
What are examples of prostacyclin analogues?
Examples include treprostinil and iloprost.
What are examples of endothelin receptor antagonists?
Non-selective: bosentan; selective antagonist of endothelin receptor A: ambrisentan.
What should be considered for patients with progressive symptoms of PAH?
Patients with progressive symptoms should be considered for a heart-lung transplant.
What is pulmonary hypertension?
Pulmonary hypertension is defined as a sustained elevation in mean pulmonary arterial pressure of greater than 20 mmHg at rest.
How is pulmonary hypertension classified by the WHO?
Pulmonary hypertension is classified into five groups.
What are the subcategories of Group 1 pulmonary hypertension?
Group 1 includes pulmonary arterial hypertension (PAH), which can be idiopathic, familial, or associated with conditions such as collagen vascular disease, congenital heart disease, HIV, drugs and toxins, and sickle cell disease, as well as persistent pulmonary hypertension of the newborn.
What conditions are included in Group 2 pulmonary hypertension?
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.
What causes Group 3 pulmonary hypertension?
Group 3 is pulmonary hypertension secondary to lung disease or hypoxia, including COPD, interstitial lung disease, sleep apnoea, and high altitude.
What is Group 4 pulmonary hypertension associated with?
Group 4 is pulmonary hypertension due to thromboembolic disease.
What conditions are included in Group 5 pulmonary hypertension?
Group 5 includes miscellaneous conditions such as lymphangiomatosis, e.g., secondary to carcinomatosis or sarcoidosis.
What was the previous term for idiopathic pulmonary arterial hypertension?
Idiopathic pulmonary arterial hypertension was previously termed primary pulmonary hypertension.
What is known about HIV’s role in pulmonary hypertension?
The mechanism by which HIV infection produces pulmonary hypertension remains unknown.
