pulmonary hypertension

Question 1

what is the definition of pulmonary hypertension?

Answer 1

mean pulmonary arterial pressure 25 or more mmHg as assessed by right heart catheterisation (RHC).

Question 2

what is the normal values for mean pulmonary arterial pressure?

Answer 2

144 plus or minus 3 mmHg

Question 6

what are the 5 internationally agreed categories for patients with pulmonary hypertension?

Answer 6

1) pulmonary arterial hypertension
2) PH due to left heart disease
3) PH due to lung disease/hypoxia
4) chronic thromboembolic pulmonary hypertension (CTEPH) and other pulmonary artery obstructions
5) pulmonary hypertension with unclear/multifactorial mechanisms

Question 6

what are the subcauses of type 1-pulmonary arterial hypertension?

Answer 6

-idiopathic pulmonary arterial hypertension (IPAH_-most important cause
-familial PAH
-drug and toxin induced PAH
-associated with:
connective tissue disease
HIV
portal hypertension
CHD
schistosomiasis

Question 6

what are the subcauses of type 3-pulmonary hypertension due to lung disease/hypoxia?

Answer 6

COPD
ILD
other lung diseases with mixed obstructive/restrictive
sleep disordered breathing eg OSA
chronic exposure to high altitudes

Question 7

what percent of people with systemic sclerosis get pulmonary hypertension?

Answer 7

15%

Question 8

what percent of people with portal hypertension get pulmonary hypertension?

Answer 8

5%

Question 9

what is an example of CHD causing pulmonary hypertension and what happens during it?

Answer 9

eisenmonger’s -chronic L–>R chunt that causes RVH and eventual reversal of the shunt.

Question 10

what happens in idiopathic pulmonary arterial hypertension (IPAH)?

Answer 10

no specific cause (but genetic links) damage to capillary endothelium regrowth and modelling causes idiopathic pulmonary arterial hypertension

Question 11

who does IPAH usually affect and which drugs can induce it?

Answer 11

young women
SSRIs, st john’s wort, pergolide (parkinson’s drug), amphetamines, cocaine, appeitite suppressives (fenfluramine, aminorex)

Question 12

what is the prognosis for IPAH?

Answer 12

untreated survival =2.8 years
drug treatments used to extend survival -under specialist

Question 13

what are the risk factors for PH?

Answer 13

• Pre-existing diseases: lung disease, left heart disease, congenital heart disease, connective tissue disorders, obesity and sleep apnoea, liver disease
  -prolonged exposure to high altitude

Question 14

what are the symptoms of pulmonary hypertension?

Answer 14

SOB, initially while exercising and eventually while at rest
fatigue
dizziness/syncope
chest pressure or pain
haemopysis if CTEPH
cyanosis
palpitations
oedema in ankles, legs, and eventually ascites
sx of associated conditions

Question 15

what percent of acute PEs don’t clear and how can we identify these?

Answer 15

3%
post severe PE, echo needs to be done at 6 months to ensure CTEPH isn’t occuring

Question 16

what are the signs of PH?

Answer 16

raised JVP
right ventricular heave
tricuspid regurg-systolic murmur
hepatomegaly
ascites, oedema
cyanosis
signs of associated conditions

Question 17

what are the most useful investigations in ?PH and ?PAH?

Answer 17

?PH -TTE
?PAH -right heart catheterisation

Question 18

bedside tests for ?PH?

Answer 18

ECG -looking for evidence of RV strain -RVH, RBBB, RAD, arrhythmias eg AF, aflutter.
normal ECG doesn’t exclude PH
ABG -for evidence of resp disease

Question 19

blood tests for pulmonary hypertension?

Answer 19

looking for causes:
cardiac-nt-pro-BNP
connective tissue disease -autoantibody screen (dsDNA, ENAs, ANA, RF)
HIV test
LFTs

Question 20

beyond tests for pulmonary hypertension?

Answer 20

CXR -signs of resp disease, LV dysfunction, PAH
TTE -first initial inv. TOE -if needed more detail
CTPA/VQ -for ?CTEPH
PFTs -for ?resp disease
right heart catheter (RHC) -actual diagnostic test
six minute walk test -info on severity, prognosis, improvement
CT thorax -more info
USS liver -more info on liver disease

Question 21

what happens in RHC?

Answer 21

right heart catheter inserted into jugular vein and passed into right heart and pulmonary arterial tree.
pressure measurements taken at RA, RV, main pulmonary, and wedge pressure (in capillaries -wedge it into capillaries till it doesn’t go any further)

Question 22

what is the significance of wedge pressure results?

Answer 22

high pre-capillary wedge pressure -pathology before capillaries-eg most causes eg resp disease, PAH
high post capillary wedge pressure -pathology (high BP) coming from after capillaries -ie originating from left heart eg LV failure, valve disease

Question 23

when in PH do you refer for specialist services?

Answer 23

PAH severe PH

Question 24

what is the conservative management for PH?

Answer 24

exercise within symptom limits pulmonary rehab flu and pneumococcal vaccines contraceptive advice -pregnancy is associated with 30-50% mortality in PAH patients psychosocial support

Question 25

what is the medical management for PAH?

Answer 25

all under specialist, consider: -specific drugs which vasodilate and lower pulmonary pressure -CCBs, bosentan, PDE inhibitors eg sildenafil, prostanoids, prostacyclin analogues eg iloprost. -diuretics for sx benefits in RHF -LTOT if resp failure -digoxin -improves cardiac output acutely -oral anticoagulation

Question 26

what is the management for PH caused by lung disease?

Answer 26

treat lung disease specialist drugs for PH actually harmful when due to lung disease

Question 27

what is a potential treatment for some cases of PH to consider after medical management?

Answer 27

cardiopulmonary transplantation

Question 28

what is the treatment for CTEPH?

Answer 28

lifelong anticoagulation some specific PH drugs rule out thrombophilia consider surgery -pulmonary endarterectomy or balloon dilation. v rare and difficult-only done in Cambridge.

Question 29

what are the complications of PH?

Answer 29

relating to worsening right heart failure -peripheral oedema, severe exertional dyspnoea pneumonia sudden cardiac death, inc during childbirth.

Question 32

what is the basic treatment strategy of PH?

Answer 32

treat underlying cause! treat each specific cause differently refer to specialist if needed