Pulmonary hypertension Flashcards
At the most basic level, what does pulmonary hypertension mean?
pressure in the pulmonary arteries
What is the most common cause of pulmonary hypertension and what group does this belong to?
Group 2 - pulmonary hypertension due to LEFT heart disease
> mechanism: elevated left atrial pressure goes to post capillary venues > alveolar bed > pulmonary vasculature = elevated pressure
What is the second most common cause of pulmonary HTN and what group does this belong to?
Group 3
- underlying lung disease & hypoxia
> COPD
> Intertistial lung diseases (related to cor pulmonale that is alteration in R heart structure + function due to a respiratory system disorder)
> obesity hypoventilation syndrome
What is group 1 pulmonary hypertension?
isolated pre-capillary PH (treated with vasodilators)
What is group 4 pulmonary hypertension?
pulmonary artery obstruction (most commonly due to thromboembolic disease)
In one sentence, summarise the 5 types of pulmonary HTN
What is the least common cause of pulmonary HTN?
- haematological conditions
- systemic conditions
(e.g. sarcoidosis)
List 3 causes of Type 1 pulmonary arterial hypertension
What pulmonary hypertension classification does sleep apnoea belong?
Thought to be group 5 - related to a metabolic type picture
Briefly describe Eisenmenger syndrome
Eisenmenger syndrome (ES) is a constellation of symptoms that arise from a congenital heart defect and result in large anatomic shunts. Due to anatomic variations present at birth, hemodynamic forces initially result in a left-right shunt, which develops into severe pulmonary arterial hypertension (PAH) and elevated vascular resistance. Ultimately, due to increased pulmonary vascular resistance, the left-to-right shunt will become a right-to-left shunt, resulting in significant hypoxemia and cyanosis
What is a common cause of idopathic pulmonary hypertension (group 1)
- genetic causes (e.g. BMPR2 mutation)
- note may be heriditary*
What are the key symptoms of pulmonary arterial hypertension?
- insidious onset of dyspnoea - months - years
- chest pain/fatigue
- syncope/pre-syncope in later disease stages
- evidence of right heart failure in advanced disease (peripheral oedema, liver congestion)
List the 3 main conditions associated with Group 2 pulmonary hypertension
- Heart failure
- HfpEF, HFref - Valvular heart disease
- congenitial/acquired cardiovascular conditions leading to post capillary HTN
Outline the pre-capillary vs. post-capillary conditions when classifying pulmonary HTN
Pre-capillary
- Group 1 - isolated pre-capillary PAH
- Group 4 - thromboelmbolic diseases
Post-capillary
- Group 1*
- Group 2 - secondary to LEFT heart disease most commonly
Group 3 sort of sits in the middle…
List 3 conditions associated with group 3 PH
List 4 types of conditions associated with ISOLATED pulmonary HTN
List 3 systemic conditions group 1 pulmonary HTN is associated with
- connective tissue disease (esp scleroderma)
- HIV infection
- porti-pulmonary HTN
- congenitial heart disease > resulting in Einsenmonger syndrome
- Schistomiasis
What group in the pulmonary HTN classification does Eisenmenger syndrome fall under?
- Group 1
- Eisenmenger syndrome is a condition that results from high blood pressure in the lungs (pulmonary hypertension) due to congenital heart disease (due to a shunt in most cases)
- Eventually, due to increased resistance and decreased compliance of the pulmonary vessels, elevated pulmonary pressures cause the myocardium of the right heart to hypertrophy (RVH). - The onset of Eisenmenger syndrome begins when right ventricular hypertrophy causes right heart pressures to exceed that of the left heart, leading to reversal of blood flow through the shunt (i.e., blood moves from the right side of the heart to the left side).
> As a consequence, deoxygenated blood returning from the body bypasses the lungs through the reversed shunt and proceeds directly to systemic circulation, leading to cyanosis and resultant organ damage
What key features of a clinical history would point to a diagnosis of pulmonary aterial hypertension (group 1)?
- consider this in patients with
> undifferentiated dyspnoea
> exercise limitation - onsent of dypnoea insidiously over months > years
- consider non-specific symptoms of fatigue/chest pain/pre-syncope/syncope
What clinical signs would point to advanced right heart failure?
- hepatic congestion
- raised JVP
- peripheral oedema
What is the key investigation that will aid/favour in a diagnosis of hypertension?
Echocardiogram!
When looking at an Echocardiogram to aid in the diagnosis of pulmonary HTN, what is the first parameter that would assist?
- peak tricuspid regurg velocity (probability of severe PH), not this is not diagnostic as it does not provide other data such as PAWP (which helps diagnose pulmonary venous hypertension)
- if this number is >2.8, which corresponds to a pulmonary artery systolic pressure of >35 this is suggestive of pulmonary HTN
*note estimate can be inacurate in patients with obesity or obstructive lung disease