Pulmonary Artery Hypertension Flashcards
What is the definition of pulmonary artery hypertension
defined as a sustained elevation in mean pulmonary arterial pressure of greater than 25 mmHg at rest
Classifications of pulmonary artery hypertension
Group 1: Pulmonary arterial hypertension (PAH)
Group 2: Pulmonary hypertension with left heart disease
Group 3: Pulmonary hypertension secondary to lung disease/hypoxia
Group 4: Pulmonary hypertension due to thromboembolic disease
Group 5: Miscellaneous conditions
What causes group 1 PAH
idiopathic
- familial - 10 percent AD fashion
- associated conditions:
collagen vascular disease,
congenital heart disease with systemic to pulmonary shunts,
HIV**,
drugs and toxins,
sickle cell disease - persistent pulmonary hypertension of the newborn
What causes Group 2: Pulmonary hypertension
with left heart disease
- left-sided atrial, ventricular or valvular disease such as left ventricular systolic and diastolic dysfunction, mitral stenosis and mitral regurgitation
What causes Group 3: Pulmonary hypertension
secondary to lung disease/hypoxia
- COPD
- interstitial lung disease
- sleep apnoea
- high altitude
What causes group 4 PAH
PE
What causes group 5 PAH
Miscellaneous conditions
- lymphangiomatosis e.g. secondary to carcinomatosis or sarcoidosis
Features of PAH ?
exertional dyspnoea
exertional syncope, exertional chest pain and peripheral oedema
cyanosis
aised JVP
Auscultory signs of PAH ?
loud P2,
JVP wave gram sign ?
Prominent A wave
Management of PAH depends on
acute vasodilator testing
If there is a positive response to acute vasodilator testing (a minority of patients)
oral calcium channel blockers
If there is a negative response to acute vasodilator testing (the vast majority of patients)
prostacyclin analogues: treprostinil, iloprost
endothelin receptor antagonists: bosentan, ambrisentan
phosphodiesterase inhibitors: sildenafil
Patients with progressive symptoms should be considered for a
heart-lung transplant.
