Pulmonary Arterial Hypertension

Question 1

PAH
-pathophysiology
-risk factors

Answer 1

Collective term for conditions with mean pulmonary arterial pressure above 25mmHg at rest

Impaired PA VD and overactive endothelin pathway => high VC
Increased cardiac contractility => thrombus formation
Leads to increased pulmonary vascular resistance

Rare

Question 2

PAH
-risk factors
-causes of other causes of pulmonary hypertension

Answer 2

FHx
Scleroderma
HIV

LHF
COPD
ILD
CAD, valvular disease
PE

Question 3

PAH
-presentation

Answer 3

Symptoms relate to right ventricular dysfunction => reduced organ perfusion
Worse on exertion

SOB
Tired, weak
Angina
Syncope
Cough
Low BP

Question 4

PAH
-investigations

Answer 4

Lung function tests

CXR
ECG - signs visible if severe
-RAD, RBBB, RVH, QTc prolongation
Echo -visible changes?

Gold standard - Right heart catherisation
-determine pressure within pulmonary artery, RV, RA

Question 4

PAH
-investigations

Answer 4

Lung function tests

CXR
ECG - signs visible if severe
-RAD, RBBB, RVH, QTc prolongation
Echo -visible changes?

Gold standard - Right heart catherisation
-determine pressure within pulmonary artery, RV, RA

Question 5

PAH
-management

Answer 5

Supportive
-AC (due to increased risk of thrombotic events

Symptomatic
-diuretics if fluid retaining
-O2 - if consistently hypoxic

Prognostic
-CCB/Endothelin ant/PDE5 inh/PCI