Pulmonary HPTN

Question 1

Signs of pulmonary hypertension

Answer 1

Peripheral oedema (ascites when severe)
Cyanosis
Palpitations
Raised JVP.
RV heave
Systolic murmur (tricuspid regurg)
Hepatomegaly

Question 2

Symptoms of pulmonary HPTN

Answer 2

SOB gradually deteriorating
Fatigue
Dizziness or fainting spells when severe
Chest pressure or pain

Question 3

What conditions to examine for that can cause PHPTN

Answer 3

Liver disease - portal HPTN
Connective tissue disease - systemic sclerosis
HIC
Lung disease
Heart - congential eisenmongers
Schistosomiasis

Question 4

Initial screening test for Pulmonary hypertension

Answer 4

Transthroacic ECHO
If negative and strong clinica suspicion still do a transoesophageal ECHO/ right heart catheter

Question 5

What investigation can diagnose and judge severity of PH?

Answer 5

Right heart catheter

Question 6

What is a right heart catheter

Answer 6

Small tube inserted into jugular vein passed into righ theart and pulmonary arterial tree
Pressure measurements taken at RA, RV, mainpulmonary artery and wedeg
Smaples of arterial blood can also be taken

Question 7

Why is capillary wedge pressure important

Answer 7

Helps with classifying PAH - pre or post capillary

Question 8

Pre-capillary vs post capillary pulmonary hypertension wedge pressure

Answer 8

Pre = low wedge pressure
Post - high wedge pressure

Question 9

What is post capillary PH?

Answer 9

Pulmonary hypertension due to problems after the capillary bed eg
Left heart disease, unclear or multifactorial mechanisms
Pre is all other causes

Question 10

Pressures in Pre vs post capillary pulmonary hypertension

Answer 10

Both >25mmHg PAPm
<15 PAWP in pre
>15 PAWP in post

Question 11

What test can give useful prognostic, treatment response and deterioration info after PH confirmed?

Answer 11

6 minute walk test

Question 12

What is it important to investigate with PH

Answer 12

Lung disease - spirometry, CXR, CT thorax, KCO, TLCO
Cardiac disease
PE/CTEPH
Connective tissue disease - autoantibodies for dsDNS, ENAs, ANA, Rf
HIV
LFTs
US liver

Question 13

Treatment for PH

Answer 13

Diuretics
Oxygen (if resp failure)
Drugs - anticoagualtion, vasodilators, digoxin
Surgery

Question 14

What is ulmonary HPTN defined as

Answer 14

Increase in MAP
P>20mmHg at rest
nOMRAL = 14+/-3

Question 15

How assess pulmonary HPTN

Answer 15

R sided heart catheterisation

Question 16

Conditions underpulmonary HTPN

Answer 16

pulmonary venoocclusive disease/capillary haemoangiomatosis

Persistent pulmonary HPTN of the newborn

Question 17

Pulmonary HPTN cause - Left heart orifin

Answer 17

Congestive HF
LVSDD, LVDD
Vavlular disease
Congeital or acquired L heart inflow/outlflow tract obstruction and congenital CMs
Congenital or acquired pumonary vein stenosis

Question 18

Lung diseases causing pulmonary HPTN

Answer 18

Cor pulmonaly -
COPD
ILD
Restritive or obstucitife sidease
OSA
Chronic high altitude exposure
Developmental lung diseases

Question 19

What is chronic TE PH

Answer 19

Acute PE fails to clear
Severe -> RH strain

Question 20

Why do an ECHO 6 months after severe PE

Answer 20

Check for RH strain -> CTEPH

Question 21

Drugs treating
pulmonary HPTN

Answer 21

CCBs
Prostacyclines eg epoprosenol
Endothelial receptor antagonists eg bosentan
PDE5 inhibitors eg suldenafil

Anticoagulate + digoxin + diuretics

Question 22

Surgical amangement for CEPTH

Answer 22

Pulmonary enartectomy - proxial disease
Balloon dilatation - distal ]Only done in cambridge
Lung or heart transplant

Question 23

What is idiopathic pulmonary arterial HPTN

Answer 23

Rare pre capillary cause of pulmonary HPTN in young women, genetic or drug unduces

Question 24

Causes of drug induced IPAH

Answer 24

• SSRIs
• St Johns Wort
• Pergolide #
• Amphetamines
• Cocaine
• Appetite suppressives - fenfluramine, aminorex