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MBBS I CPRS > L47 – Pulmonary hypertension > Flashcards

L47 – Pulmonary hypertension Flashcards

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1
Q

What is the criteria for pulmonary hypertension?

A

mean pulmonary
artery pressure (MPAP) >25 mmHg at rest (measured by
right heart catheterization)

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2
Q

What is normal Pulmonary arterial systolic, diastolic pressure?

A 
Systolic = 15-30 mmHg
Diastolic = 4-12 mmHg
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3
Q

What is normal MPAP?

A

mPAP ≤20 mmHg

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4
Q

Sub-classification of pulmonary hypertension is divided into?

A

according to pulmonary artery wedge pressure (PAWP)

 Pre-capillary (PAWP ≤15 mm Hg)
 Post-capillary (PAWP >15 mm Hg)

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5
Q

Cause of Type 1 pulmonary hypertension?

A

Pulmonary arterial hypertension (PAH)

  1. Heritable (aka familial PAH)
  2. Idiopathic (“primary pulmonary hypertension”) (IPAH)
  3. Secondary to systemic disorders (secondary pulmonary artery hypertension)
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6
Q

Cause of type 2 pulmonary hypertension?

A

due to left-sided heart disease (pulmonary venous hypertension)

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7
Q

Cause of Type 3 pulmonary hypertension?

A

due to respiratory / lung diseases and/or hypoxia

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8
Q

Cause of Type 4 pulmonary hypertension?

A

Chronic thromboembolic / embolic pulmonary hypertension (CTEPH) and other pulmonary artery
obstructions

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9
Q

Cause of Type 5 pulmonary hypertension?

A

unclear multifactorial mechanisms

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10
Q

Difference between type 1 vs type 2,3,4,5 pulmonary classification?

A

2,3,4,5 are PH, POST-capillary problems

1 is PAH, Pre-capillary with no problems with L-Heart or Lungs

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11
Q

3 steps in pathogenesis of PAH?

A

Risk factors and associated conditions > Vascular injury & endothelial injury > Disease progression

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12
Q

How does pulmonary artery change in PAH?

A

Smooth muscle hypertrophy

Adventitial and Intimal proliferation

In-situ thrombosis and plexiform lesion formation

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13
Q

3 causes of Group 1 PAH?

A

Idiopathic
Heritable
Diseases localize to pulmonary arterioles

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14
Q

Can you distinguish patients with idiopathic PAH and heritable PAH?

A

No

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15
Q

How to diagnose Heritable PAH vs idiopathic PAH?

A

When heritable genetic defects known to cause PAH can be identified

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16
Q

What is a very common genetic defect that causes hereditary PAH?

A

mutation of BMPR2

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17
Q

What are some causes of secondary pulmonary artery hypertension?

A 
Connective Tissue disease
Liver disease 
Congenital heart disease 
Drugs/Toxins 
HIV
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18
Q

What drugs/ toxins can cause secondary pulmonary hypertension?

A

Definite risk factors : appetite suppressants (e.g. aminorex)

Possible risk factor: e.g. cocaine

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19
Q

What connective tissue diseases causes secondary PAH?

A

Systemic sclerosis (SSc) - obliterate alveolar capillaries

Rheumatoid arthritis, Systemic lupus erythematosus (SLE) - fibrous obliteration of pulmonary vascular bed

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20
Q

Which gender is at higher risk of connective tissue diseases?

A

Female

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21
Q

What pressures change in type 2 PH?

A

Left heart disease

Associated with elevated:

1) Left atrial pressure (e.g. mean pressure >14 mmHg)
2) Pulmonary venous pressure

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22
Q

What are some main causes of type 2 PH?

A

Left atrial hypertension caused by:
-LV systolic or diastolic dysfunction/ Left heart failure

-Mitral and aortic valve disease

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23
Q

Explain how pulmonary venous pressure increases in type 2 PH?

A

Left heart failure > left heart pressure increase due to regurgitation > Increase LA pressure causes increase pulmonary vein pressure

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24
Q

What are some leading causes of Type 3 PH?

A

Lung diseases include :

1) COPD
2) Interstitial lung disease
3) Sleep apnea
4) Combined pulmonary fibrosis and emphysema (CPFE)

+ Other hypoxemia conditions

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25
Pulmonary embolus is a risk for which types of PH?
All types of PH are at risk of embolus
26
What is the full name of Type 4 PH? CTEPH
Chronic thromboembolic / embolic pulmonary hypertension
27
Name some leading causes of Type 4 PH?
deep vein thrombosis | angiosarcoma
28
Name some leading causes of Type 5 PH?
Multifactorial, unknown causes, including: 1) Chronic hemolytic anemia (e.g. sickle cell disease) 2) Systemic and metabloic disorders (e.g. sarcoidosis) 3) Chronic kidney disease + Miscellaneous causes
29
Is PH common? Is it deadly?
Rare but high risk of mortality and morbidity
30
Which cause of PH is most common?
Most common = Left heart disease = Type 2 PH Second= sleep apnea = Type 3 PH Third = Scleroderma = Type 1 PAH
31
Why is delay in diagnosis of PH common?
Symptoms and signs are usually not specific
32
What is early symptom of PH?
Dyspnea with activity (shortness of breath, chest tightness, wheezing...)
33
What are 4 late symptoms of PH? CASE
Chest pain/ pressure Ankle edema (RH failure) Syncope Elevated JVP (congestive HF, RH failure)
34
What tests can be done to confirm PH?
CXR, ECG
35
Upon CVS physical examination, what traits would a PH patient present?
1) Parasternal heave 2) Loud 2nd heart sound 3) Cyanosis (respiratory disease) 4) Elevated JVP (congestive HF) 5) Ankle oedema 6) Hepatic congestion
36
What is used to determine prognosis of PAH ?
Several clinical variables including functional class and comorbidities
37
How to assess functional capacity of CVS?
Ask for exercise capacity to assess
38
What is used to determine prognosis of PH (type 2 to 5) ?
1) Prognosis of underlying disease 2) Severity of PH 3) Response to therapy
39
What is the main cause of death from PH?
Type 2 PH left heart failure > cause Right heart failure = leading cause of death
40
How does pulmonary artery pressure change with severity of PH against cardiac output?
Normal = gradual increase in MPAP with CO Severe = steep increase in MPAP with CO
41
What are the main features of PH on a CXR?
Elevated cardiac apex due to RV hypertrophy Enlarged RA Enlarged pulmonary artery Clear lung fields
42
What are the main features of PH on an ECG?
V1-V3:  ST depression  T wave inversion RH hypertrophy > V1 positive with big R wave (V1 normally negative, small R wave) Lead II > enlarged P wave
43
What does trans-thoracic echocardiogram estimate?
Uses Doppler ultrasound to estimate pulmonary artery systolic pressure
44
What is the method to directly measure right heart systolic + diastolic pressure + MPAP?
Right heart catheterization
45
When is right heart catherization unnecessary?
When problem has been identified in heart e.g. sufficient evidence showing left heart failure
46
What can Chest CT scan for?
Lung diseases > type 3 PH
47
What can V/Q scan scan for?
Chronic thromboembolism
48
How to identify sleep apnea in type 3 PH?
Overnight oximetry testing
49
What therapy is used in all PH patients?
Oxygen therapy
50
What therapy is used for group 4 PH with CTEPH?
Anticoagulation
51
What therapy is used to relieve oedema in type 2 PH patients?
Diuretics
52
What drug is used in group 3 PH due to COPD/ biventricular (both sides of heart) failure?
Digoxin
53
What surgery is done in patients with very severe type 4 PH?
Surgical thromboendarterectomy For patients with thromboembolic obstruction of proximal pulmonary arteries
54
Is vasodilator therapy recommended for type 2-5 PH?
No
55
What approach should be taken when treating group 2-5 PH?
Look for underlying cause and treat that specific cause
56
What test is done after confirmation of PAH?
Acute Vasoreactivity test
57
What therapy is given to PAH patient with vasoreactive result?
CCB therapy (nifedipine, diltiazem, amlopidine)
58
What therapy is given to PAH patient with Non-vasoreactive result?
Classify into risk catgeories > give according treatments
59
What classifies as vasoreactive?
Right heart catherization vasodilator challenge > 20% acute drop in MPAP and PVR = vasoreactive
60
What 3 tests are done to monitor therapy on PAH patient ?
Exercise capacity Brain Naturetic Peptide test Echocardiography
61
What are the 5 drugs for type 1 PH?
``` Endothelin receptor antagonist (ERA) Phosphodiesterase-5 inhibitor (PDEi) Soluble guanylate cyclase stimulator (sGC) Prostacyclin analogues Prostacyclin receptor agonists ```
62
What are the effects of all of 5 drugs for type 1 PH?
Vasodilate pulmonary artery
63
Name one ERA drug for type 1 PH? Main major side effect?
Macitentan ERA reduce action of ET-1 > vasodilate pulmonary artery Elevated hepatic aminotransferase
64
What are some common side effects of ERA?
peripheral edema Headache Flushing
65
How is NO produced endogenously?
NO made by inhibiting Phosphodiesterase 5 (PDE5) thus inhibit degradation cyclic guanosine monophosphate NO is natural vasodilator
66
Name 2 PDE inhibitors?
Sildenafil, Tadalafil
67
Name some side effects of PDEi?
Headache Acid reflux Back pain Myalgia
68
Which type of PH is treated with soluble guanylate cyclase stimulator ?
Chronic thromboembolic pulmonary hypertension | Type 4
69
Which drug cannot be used with soluble guanylate cyclase stimulator?
PDE inhibitor | Risk of hypotension
70
How does lack of prostacyclin synthase cause vasoconstriction and platelet aggregation ?
Lack of synthase = lack of prostacyclin > lack of antiplatelet and anti-proliferative effect
71
What prostacyclin analogue drug is given to improve cardiac output ?
Epoprostenol/ Treprostinil (IV admin) Direct vasodilate pulmonary and systemic arterial Reduce ventricular afterload, resistance > improve CO
72
Name one drug given as a prostacyclin receptor agonist and type of PH treated?
Selexipag PAH
73
Name some side effects of protacyclin type drugs?
Headache Nausea Jaw pain
74
What screens for PH? What gives definitive diagnosis?
Screen = Transthoracic Doppler Echo Definitive = Right heart catherization

MBBS I CPRS (78 decks)

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