Understanding PH: The Basics

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Understanding PHThe Basics
John Swiston MD FRCPC University of British
Columbia
September 2009
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PH Basics - Overview

• 1. What is pulmonary hypertension (PH) -
  Physiology
• 2. How do we determine who has PH and what type
  Diagnosis
• 3. How severe is the PH - Evaluation
• 4. How do we treat PH Therapy
• 5. What happens after treatment is started
  Re-evaluation

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Blood flow plumbing

• Blood flows through the body the way water flows
  through pipes in your house
• Pipes
• Pump

blood vessels
heart
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Normal Blood Circulation
Lungs (pulmonary circulations)
Left heart
Right heart
Body (systemic circulation)
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Blood flow in the lungs
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Pulmonary Hypertension high blood pressure in
the lungs
What is Pulmonary Hypertension?
Pressure is needed to maintain flow but. Too
much pressure strains the system
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PH Basics - Overview

• 1. What is pulmonary hypertension (PH) -
  Physiology
• 2. How do we determine who has PH and what type
  Diagnosis
• 3. How severe is the PH - Evaluation
• 4. How do we treat PH Therapy
• 5. What happens after treatment is started
  Re-evaluation

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Right Heart Catheterization
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Right heart catheterization

• How high is the pressure in the arteries of your
  lungs
• How well is your heart working
• What factors are affecting the pressures in your
  lungs

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Diagnostic Classification of Pulmonary
Hypertension4th World Symposium on PH Dana
Point 2009

• 1. Pulmonary Arterial Hypertension
• 2. Left Heart Disease - PH
• 3. Lung Disease/Hypoxia - PH
• 4. Chronic Thromboembolic PH
• 5. Unclear/multifactorial mechanisms - PH

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Pathogenesis of PAH
Pulmonary arteriole in PAH
Normal pulmonary arteriole
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Pulmonary Arterial Hypertension
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Normal Blood Circulation
Lungs (pulmonary circulations)
Left heart
Right heart
Body (systemic circulation)
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What causes Pulmonary Arterial Hypertension?

• Connective tissue diseases
• HIV infection
• Chronic liver disease
• Heart defects
• Drugs and toxins
• Genetic abnormalities
• Unknown - Idiopathic

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Pulmonary arterial hypertension
Lungs (pulmonary circulations)
Left heart
Right heart
Body (systemic circulation)
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2. PH secondary to Left Heart Disease
Lungs (pulmonary circulations)
Left heart
Right heart
Body (systemic circulation)
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2. PH secondary to Left Heart Disease

• Left heart does not pump blood effectively
• Blood backs up into the lungs

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3. PH secondary to Lung disease/hypoxia
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Normal Blood Circulation
Lungs (pulmonary circulations)
Left heart
Right heart
Body (systemic circulation)
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4. Chronic Thromboembolic PH

• Blood flow through the lungs is blocked by clots
  of blood
• The same amount of blood must flow through fewer
  vessels
• So the pressure goes up

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Chronic thromboembolic PH
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5. PH secondary to multifactorial mechanisms
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PH Basics - Overview

• 1. What is pulmonary hypertension (PH) -
  Physiology
• 2. How do we determine who has PH and what type
  Diagnosis
• 3. How severe is the PH - Evaluation
• 4. How do we treat PH Therapy
• 5. What happens after treatment is started
  Re-evaluation

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WHO/NYHA Functional Class
Symptoms Dyspnea, fatigue, chest pain or
pre-syncope
European Society of Cardiology Guidelines.
European Heart Journal. 2004252243-78 Barst R,
et al. J Am Coll Cardiol. 200443S40-7 Hoeper
M, et al. Am Coll Cardiol. 200443S48-55
McLauglin V, et al. Circulation. 2002 106
1477-82.
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6 minute walk test

• How far can you walk in 6 minutes
• Functional ability

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Physical examination

• How well is your heart functioning
• Is your heart coping with the increased pressure
  in the arteries of your lungs

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Echocardiography

• How well is your heart functioning
• How high is the pressure in the arteries of your
  lungs (estimate)

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Demonstration of tests

• www.livingwithph.ca

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PH Basics - Overview

• 1. What is pulmonary hypertension (PH) -
  Physiology
• 2. How do we determine who has PH and what type
  Diagnosis
• 3. How severe is the PH - Evaluation
• 4. How do we treat PH Therapy
• 5. What happens after treatment is started
  Re-evaluation

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Options for treatment in PH

• Supportive care
• Calcium channel blockers (oral)
• Prostocyclins (not oral - intravenous or
  subcutaneous)
• Epoprostenol (Flolan)
• Treprostinil (Remodulin)
• Endothelin Receptor Antagonists (oral)
• Bosentan (Tracleer)
• Sitaxsentan (Thelin)
• Ambrisentan (Volibris)
• Phosphodiesterase inhibitors (oral)
• Sildenafil (Viagra or Revatio)
• Tadalafil (Cialis)
• Experimental drugs
• Transplantation

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Humbert M, et al. N Engl J Med. 20043511425-36.
The New England Journal of Medicine (c) 2004.
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PAH Treatments - A Historical Overview
CCB, anticoagulation, digitalis, diuretics
IV treprostinil
Sildenafil
Sitaxsentan
SC treprostinil
Ambrisentan
Epoprostenol
Tadalafil
Bosentan
2001
lt1995
1995
1996
1997
1998
1999
2000
2002
2003
2004
2005
2006
2007
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Flolan therapy
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Pump Options
Intravenous Remodulin
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How do we decide which treatments to use?

• Therapy depends on the type of PH and the
  severity of disease
• Functional class II
• oral monotherapy
• Functional class III
• Oral monotherapy
• Oral combination therapy (two drugs)
• IV/SC prostacyclin therapy
• Functional class IV
• IV/SC prostacyclin therapy

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PH Basics - Overview

• 1. What is pulmonary hypertension (PH) -
  Physiology
• 2. How do we determine who has PH and what type
  Diagnosis
• 3. How severe is the PH - Evaluation
• 4. How do we treat PH Therapy
• 5. What happens after treatment is started
  Re-evaluation

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What are the Goals of Therapy?

• What is the plan after starting treatment for PH?
• What are the goals of therapy?
• When should we re-evaluate?
• When should we change therapy?
• When should we add therapy?
• When should we stay the course?

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Options for treatment in PH

• We now have more than one treatment option
• How do we make the most of these options?

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Clinical response Tools for assessment

• Re-evaluation at 1-3 months
• Functional class
• 6 minute walk test
• Echocardiography
• Physical exam
• /- repeat right heart catherization

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Goals of therapy

• Functional class II stability
• Functional class III stability and improvement
• Functional class IV improvement

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Combination Therapies
Transplantation
European Society of Cardiology Guidelines.
European Heart Journal. 2004252243-78 Canadian
Cardiovascular Society and Canadian Thoracic
Society PAH Position Statement. Can J Cardiol.
200521909-14 Badesch DB, et al. Chest.
20071311917-28 Rubin LJ. Proc Am Thor Soc.
20063111-5 Galie N, et al. J Am Coll Cardiol.
20044381-8S.
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PH Basics - Overview

• 1. What is pulmonary hypertension (PH) -
  Physiology
• 2. How do we determine who has PH and what type
  Diagnosis
• 3. How severe is the PH - Evaluation
• 4. How do we treat PH Therapy
• 5. What happens after treatment is started
  Re-evaluation

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Day to day management of PH

• Medication compliance
• Exercise
• Fluid balance
• Self monitoring

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Steps in Diagnosis of PAH
1
2
3
4
Canadian Cardiovascular Society and Canadian
Thoracic Society position statement on PAH. Can J
Cardiol. 200521909-14.
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Treatment algorithm
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Clinical Classification of Pulmonary Hypertension
(Venice 2003)
Simonneau G, et al. J Am Coll Cardiol.
200443S5-12.
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Pathogenesis of PAH
Pulmonary arteriole in PAH
Normal pulmonary arteriole
Barst et al. J Am Coll Cardiol. 20044340S-47S.