L47 – Pulmonary hypertension Flashcards Preview

MBBS I CPRS > L47 – Pulmonary hypertension > Flashcards

Flashcards in L47 – Pulmonary hypertension Deck (74):
1

What is the criteria for pulmonary hypertension?

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

2

What is normal Pulmonary arterial systolic, diastolic pressure?

Systolic = 15-30 mmHg
Diastolic = 4-12 mmHg

3

What is normal MPAP?

mPAP ≤20 mmHg

4

Sub-classification of pulmonary hypertension is divided into?

according to pulmonary artery wedge pressure (PAWP)

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

5

Cause of Type 1 pulmonary hypertension?

Pulmonary arterial hypertension (PAH)

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

6

Cause of type 2 pulmonary hypertension?

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

7

Cause of Type 3 pulmonary hypertension?

due to respiratory / lung diseases and/or hypoxia

8

Cause of Type 4 pulmonary hypertension?

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

9

Cause of Type 5 pulmonary hypertension?

unclear multifactorial mechanisms

10

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

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

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

11

3 steps in pathogenesis of PAH?

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

12

How does pulmonary artery change in PAH?

Smooth muscle hypertrophy

Adventitial and Intimal proliferation

In-situ thrombosis and plexiform lesion formation

13

3 causes of Group 1 PAH?

Idiopathic
Heritable
Diseases localize to pulmonary arterioles

14

Can you distinguish patients with idiopathic PAH and heritable PAH?

No

15

How to diagnose Heritable PAH vs idiopathic PAH?

When heritable genetic defects known to cause PAH can be identified

16

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

mutation of BMPR2

17

What are some causes of secondary pulmonary artery hypertension?

Connective Tissue disease
Liver disease
Congenital heart disease
Drugs/Toxins
HIV

18

What drugs/ toxins can cause secondary pulmonary hypertension?

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

Possible risk factor: e.g. cocaine

19

What connective tissue diseases causes secondary PAH?

Systemic sclerosis (SSc) - obliterate alveolar capillaries

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

20

Which gender is at higher risk of connective tissue diseases?

Female

21

What pressures change in type 2 PH?

Left heart disease

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

22

What are some main causes of type 2 PH?

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

-Mitral and aortic valve disease

23

Explain how pulmonary venous pressure increases in type 2 PH?

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

24

What are some leading causes of Type 3 PH?

Lung diseases include :

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

+ Other hypoxemia conditions

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

Decks in MBBS I CPRS Class (78):