- Pulmonary artery hypertension
- Defined as MPAP of >25 mmHg at rest or 30mmHg on exercise
- Difficult to diagnose and to treat. Can take 3 yrs from presentation to diagnosis
- In the absence of disease, the healthy right ventricle can only generate a pressure of 50mmHg so in an acute situation it should raise the possibility of chronic TED
- Pathophysiology
- The hallmark is increased PVR
- There is increased workload of the right ventricle and CO subsequently falls
- Treatments
- Standard therapy
- Digoxin
- Warfarin
- Diuretics
- Oxygen therapy
- Calcium channel blockers
- Targeted therapies
- Prostacyclin
- Iloprost
- Via Hickman line
- Nebulised
- Bosentan
- Sildenafil
- Pulmonary endarterectomy in TED
- Transplantation
- Clinical features
- SOB is common
- Chest pain and syncope can be seen
- Can be due to enlarged R sided chambers encroaching on the left side
- Tachycardia, elevated JVP, AO, TR, hepatomegaly, RV heave and loud P2 are late
- Investigations
- ECG
- RVH with strain (dominant R in V1, RAD, RBBB and TWI may be seen)
- CXR
- May be normal
- Prominent pulmonary arteries
- Plethoric lung fields
- PFTs
- Often normal
- Reduced CO transfer may be present
- Mild restrictive or obstructive defect
- SOB is disproportional
- Echo
- Can suggest PAH
- Dilated R heart chambers, LV dysfunction may be seen
- Estimated from the velocity of the TR jet (v) and estimate of RA pressure (10mmHg)
- SPAP = 4v2 + RA
- ABGs
- Cardiac catheterisation
- A vasodilator challenge is carried out and pressures measured
- Poor prognosis with high RA pressures, cardiac index <2.1, and mixed venous SpO₂ < 63%, poor response to vasodilator challenge
- Causes
- Idiopathic/primary pulmonary hypertension
- Secondary
- Collagen vascular disease
- Portal hypertension
- Congenital heart disease
- HIV
- Drugs/toxins
- Chronic thromboembolic
- CEACCP
Link:ceaccp.oxfordjournals.org/content/6/1/17.full.pdf