SAQ: iPAH Pathophysiology and treatment Flashcards
1
Q
What characterises PH
Diagnosis
A
- Increase in mean pulmonary arterial pressure
> 25mmHg at rest
30 mmHg upon exercise
- Determined by right heart catheterisation
2
Q
PAH
5 causes/ subtypes
A
- characterised pre-capillary PH
Causes/subtypes
- 1 = Idiopathic
- 2 = Heritable
- 3 = Drug/toxin induced
- 4 = Associated diseases (connective tissue disorders, HIV, chronic HA)
- 5 = Persistant PH of the newborn (PPHN)
3
Q
Pathology
A
Severe arteriopathy:
- Thickened intima/media/adventitia of peripheral arteries
- Muscularisation of pre-capillary arterioles and capillaries
Vascular lesions:
- Plexiform lesions (~unique to iPAH)
- Neo-intimal proliferation
- Obstruction of arterioles/arteries
4
Q
Plexiform lesions
Cause
A
- Monoclonal proliferation of endothelial cells
- Migration/proliferation of VSMCs
- Accumulation of circulating inflammatory and progenitor cells
5
Q
PAH and cancer
A
- Resistance to apoptosis
- Expression of malignant cell biomarkers
- Proliferation and angiogenesis
- Risk of thrombosis
6
Q
Progression
A
- Increased PVR causes increase RC strain
- RV hypertrophies to compensate
- Eventually, RV fails and dilates
7
Q
Medical treatment
A
- Prostaglandins
- ET-R antagonists
- Phosphodiesterase-5 inhibitors
- Sildafinal
- CCBs
8
Q
Surgical Treatment
A
- Pulmonary endarterectomy
- Balloon pulmonary angioplasty
- Atrial septostomy
- Lung/heart transplant
