26 Pulmonary Vascular disease

Question 0

PE Long term consequences

Answer 0

Obstruction of proximal and distal pulmonary arteries

Question 1

PE testing

Answer 1

VQ scan- ventilation and perfusion don’t line up

Question 2

Pulmonary hypertension classification

Answer 2

1. Pulmonary arteral hyppertension- precapillary
2. Pulmonary venous htn- post capillary- LHF *Most
3. Respiratory PHTN- i.e. fibrosis
4. Thromboembolic disorders
5. Other disorders

Question 3

PAH 1

Answer 3

Idiopathic, Familial (BMPR2), Scleroderma/CollagenVD
Mean pulmonary artery pressure >25 mmHg at rest
Normal PCWP

Histo-
Arterial thickening
Plexiform lesions

Question 4

PH2

Answer 4

LHF- PAP>25, CWP elevated

Question 5

PAH1 pathogenesis

Answer 5

Increased endothlin pathway- constriction and smc proliferation
Decreased NO pathway
Decreased prostacyclin

Treat with NO pathway treatments like sildenafil

Question 6

Goodpastures syndrome

Answer 6

Anti Basement membrane of capillaries

Question 7

Granulomatosis with polyangiitis

Answer 7

c-ANCA associatted granulomatosis vasculitis
Necrotizing granulomas hemorrhage

Treatent
Prednisone

Question 8

Idiopathic hemosidarosis

Answer 8

Increased hemosideran laden macrophages on bronchoscopy lavage.