Flashcards in L47 – Pulmonary hypertension Deck (74):
What is the criteria for pulmonary hypertension?
artery pressure (MPAP) >25 mmHg at rest (measured by
right heart catheterization)
What is normal Pulmonary arterial systolic, diastolic pressure?
Systolic = 15-30 mmHg
Diastolic = 4-12 mmHg
What is normal MPAP?
mPAP ≤20 mmHg
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)
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)
Cause of type 2 pulmonary hypertension?
due to left-sided heart disease (pulmonary venous hypertension)
Cause of Type 3 pulmonary hypertension?
due to respiratory / lung diseases and/or hypoxia
Cause of Type 4 pulmonary hypertension?
Chronic thromboembolic / embolic pulmonary hypertension (CTEPH) and other pulmonary artery
Cause of Type 5 pulmonary hypertension?
unclear multifactorial mechanisms
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
3 steps in pathogenesis of PAH?
Risk factors and associated conditions > Vascular injury & endothelial injury > Disease progression
How does pulmonary artery change in PAH?
Smooth muscle hypertrophy
Adventitial and Intimal proliferation
In-situ thrombosis and plexiform lesion formation
3 causes of Group 1 PAH?
Diseases localize to pulmonary arterioles
Can you distinguish patients with idiopathic PAH and heritable PAH?
How to diagnose Heritable PAH vs idiopathic PAH?
When heritable genetic defects known to cause PAH can be identified
What is a very common genetic defect that causes hereditary PAH?
mutation of BMPR2
What are some causes of secondary pulmonary artery hypertension?
Connective Tissue disease
Congenital heart disease
What drugs/ toxins can cause secondary pulmonary hypertension?
Definite risk factors : appetite suppressants (e.g. aminorex)
Possible risk factor: e.g. cocaine
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
Which gender is at higher risk of connective tissue diseases?
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
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
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
What are some leading causes of Type 3 PH?
Lung diseases include :
2)Interstitial lung disease
4)Combined pulmonary fibrosis and emphysema (CPFE)
+ Other hypoxemia conditions
Pulmonary embolus is a risk for which types of PH?
All types of PH are at risk of embolus
What is the full name of Type 4 PH? CTEPH
Chronic thromboembolic / embolic pulmonary hypertension
Name some leading causes of Type 4 PH?
deep vein thrombosis
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
Is PH common? Is it deadly?
Rare but high risk of mortality and morbidity
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
Why is delay in diagnosis of PH common?
Symptoms and signs are usually not specific
What is early symptom of PH?
Dyspnea with activity (shortness of breath, chest tightness, wheezing...)
What are 4 late symptoms of PH? CASE
Chest pain/ pressure
Ankle edema (RH failure)
Elevated JVP (congestive HF, RH failure)
What tests can be done to confirm PH?
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
What is used to determine prognosis of PAH ?
Several clinical variables including functional class and comorbidities
How to assess functional capacity of CVS?
Ask for exercise capacity to assess
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
What is the main cause of death from PH?
Type 2 PH left heart failure > cause Right heart failure = leading cause of death
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
What are the main features of PH on a CXR?
Elevated cardiac apex due to RV hypertrophy
Enlarged pulmonary artery
Clear lung fields
What are the main features of PH on an ECG?
T wave inversion
RH hypertrophy >
V1 positive with big R wave
(V1 normally negative, small R wave)
Lead II > enlarged P wave
What does trans-thoracic echocardiogram estimate?
Uses Doppler ultrasound to estimate pulmonary artery systolic pressure
What is the method to directly measure right heart systolic + diastolic pressure + MPAP?
Right heart catheterization
When is right heart catherization unnecessary?
When problem has been identified in heart e.g. sufficient evidence showing left heart failure
What can Chest CT scan for?
Lung diseases > type 3 PH
What can V/Q scan scan for?
How to identify sleep apnea in type 3 PH?
Overnight oximetry testing
What therapy is used in all PH patients?
What therapy is used for group 4 PH with CTEPH?
What therapy is used to relieve oedema in type 2 PH patients?
What drug is used in group 3 PH due to COPD/ biventricular (both sides of heart) failure?
What surgery is done in patients with very severe type 4 PH?
For patients with thromboembolic obstruction of proximal pulmonary arteries
Is vasodilator therapy recommended for type 2-5 PH?
What approach should be taken when treating group 2-5 PH?
Look for underlying cause and treat that specific cause
What test is done after confirmation of PAH?
Acute Vasoreactivity test
What therapy is given to PAH patient with vasoreactive result?
CCB therapy (nifedipine, diltiazem, amlopidine)
What therapy is given to PAH patient with Non-vasoreactive result?
Classify into risk catgeories > give according treatments
What classifies as vasoreactive?
Right heart catherization vasodilator challenge > 20% acute drop in MPAP and PVR = vasoreactive
What 3 tests are done to monitor therapy on PAH patient ?
Brain Naturetic Peptide test
What are the 5 drugs for type 1 PH?
Endothelin receptor antagonist (ERA)
Phosphodiesterase-5 inhibitor (PDEi)
Soluble guanylate cyclase stimulator (sGC)
Prostacyclin receptor agonists
What are the effects of all of 5 drugs for type 1 PH?
Vasodilate pulmonary artery
Name one ERA drug for type 1 PH? Main major side effect?
ERA reduce action of ET-1 > vasodilate pulmonary artery
Elevated hepatic aminotransferase
What are some common side effects of ERA?
How is NO produced endogenously?
NO made by inhibiting Phosphodiesterase 5 (PDE5) thus inhibit degradation cyclic guanosine monophosphate
NO is natural vasodilator
Name 2 PDE inhibitors?
Name some side effects of PDEi?
Which type of PH is treated with soluble guanylate cyclase stimulator ?
Chronic thromboembolic pulmonary hypertension
Which drug cannot be used with soluble guanylate cyclase stimulator?
Risk of hypotension
How does lack of prostacyclin synthase cause vasoconstriction and platelet aggregation ?
Lack of synthase = lack of prostacyclin > lack of antiplatelet and anti-proliferative effect
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
Name one drug given as a prostacyclin receptor agonist and type of PH treated?
Name some side effects of protacyclin type drugs?