Transcript PHA Thanks the Medical Education Fund Sponsors!

PHA Thanks the Medical
Education Fund Sponsors!
PHA Thanks the Washington, DC Planning
Committee and Presenters!
•
Christopher Barnett, M.D., MPH; Co-chair
•
Grant Farr, D.O.
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Oksana A. Shlobin, M.D., FCCP; Co-chair
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Mardi Gomberg-Maitland, M.D., MSc
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Janet Arp
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Hunter Groninger, M.D., FACP, FAAHPM
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Daniel C. Grinnan, M.D.
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Shilpa Johri, M.D.
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Denise Lewis, RN, BSN
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Todd M. Kolb, M.D., Ph.D.
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Stephen Mathai, M.D., MHS
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Katherine Kroner
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Betsie Miklos
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Tunay Kuru, M.D.
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Gerilynn Connors, B.S., RRT,MAACVPR, FAARC
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Janet Pinson, MSN, ACNP
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Rachel Damico, M.D., Ph.D.
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Gautam Ramani, M.D.
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Jason M. Elinoff, M.D.
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Virginia Steen, M.D.
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Karen Fagan, M.D.
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Nargues Weir, M.D.
PH Patients and Families Education Forum
A Program of the Pulmonary Hypertension Association Medical Education Fund
Washington, D.C.
October 1, 2016
What’s Your Type?
Types of Pulmonary Hypertension and
Associated Means of Diagnosis
Dan Grinnan, MD
October 1, 2016
Understanding
Problem
Available
Solutions
Outcome
Shared
Decisions
Objectives
• Understand how the pulmonary circulation interacts with the
heart and lungs
• Understand the different problems that can lead to pulmonary
hypertension (PH)
• Understand how pathophysiology shaped the current
classification system
• Understand why diagnostic tests are ordered and important
• Understand how management can be different for different
types of PH
RV
RV = Right
Ventricle
RV
LV
RV
LV = Left
Ventricle
120/7
0
LV
RV
120/7
0
LV
20/1
0
RV
Right
Left
Right
Left
Right
Left
120/7
0
LV
20/10
100/40
RV
120/7
0
LV
100/40
RV
8-12
120/7
0
Left heart
failure
LV
60/30
RV
25
120/7
0
Severe Lung
Disease
LV
100/40
70/30
RV
8-12
How Does Lung Disease Cause Pulmonary
Hypertension?
Normal Lung
Tissue
Severe Emphysema
120/7
0
LV
100/40
8-12
Chronic
Thromboembolic
Disease
Permanent Blood
Clot
RV
Current Classification System
• Pulmonary Arterial Hypertension
• Pulmonary Hypertension due to Left Heart
Disease
• Pulmonary Hypertension due to Lung
Disease and/or Hypoxia
• Chronic Thromboembolic Pulmonary
Hypertension
• Pulmonary Hypertension with Unclear
Multifactorial Mechanisms
Types, Groups, and Classes
• Type = Group (see Classification)
• Functional Class is different
o Class I: no symptoms
o Class II: symptoms occur with significant
activity
o Class III: symptoms occur with mild physical
activity
o Class IV: symptoms occur with minimal activity
or at rest
• There is no stage!
Pulmonary Arterial Hypertension
Types of Pulmonary Arterial
Hypertension
Vasodilator &
Vasoconstricto
r Imbalance
Genetic
Familial, Idiopathic
Autoimmune
Scleroderma
Serotonin
Anorexigen
Infection
PAH
HIV,
Schistosomiasis
Hormonal
Cirrhosis
Shear Stress
Congenital Heart
Disease
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Pulmonary Hypertension Due to Left Heart
Disease
The problem starts on the left side of the heart
 Bad valve
 Bad squeeze
 Bad relaxation
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Less blood is moved forward from the faulty heart
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More blood can back up behind the heart
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Pulmonary hypertension occurs “secondary to” the heart problem
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Over time, the pulmonary vessels change
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Eventually, there are 2 problems:
 Bad heart
 Bad pulmonary arteries
Example: The Mitral Valve
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The mitral valve separates the left ventricle from the left atrium
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3 ‘wedge shaped’ leaflets attach to a ring, or annulus
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Mitral valve regurgitation, or leak, is a common problem
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The backflow of blood may reach the pulmonary arteries and cause
pulmonary hypertension
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Repairing or replacing the mitral valve is a common surgery
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Following mitral valve repair or replacement, pulmonary hypertension can:
 Resolve
 Improve
 Persist
Another Example: Diastolic Dysfunction
• Systole = left ventricle squeezing (ejection
fraction)
• Diastole = left ventricle relaxing
• Thick left ventricle = impaired relaxation
 Diastolic dysfunction
 Heart failure with preserved ejection fraction (HFpEF)
• Blood backs up behind the stiff left ventricle
• Pulmonary hypertension often results
• Risks for HFpEF:
 Hypertension, diabetes, sleep apnea, obesity
Pulmonary Hypertension due to
Lung Disease and/or Hypoxia
Pulmonary Hypertension due to
Lung Disease and/or Hypoxia
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Sleep apnea occurs each night in millions of homes
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People are often unaware that they stop breathing in their sleep
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Part of the definition of sleep apnea is that oxygen levels drop during the pause in breathing
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When oxygen levels drop, the pulmonary circulation is under stress, and pulmonary pressure
is transiently raised
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Over time, the increase in pulmonary pressures occurs 24/7…pulmonary hypertension
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Without medication or surgery, this process can be reversed
Chronic Thromboembolic
Pulmonary Hypertension (CTEPH)
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Deep Vein Thrombus is a blood clot that typically forms in the leg
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If the clot travels, it goes through the right heart and lodges in the lungs…a
pulmonary embolism
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If the pulmonary embolism does not dissolve over time, then it becomes scar
tissue within the pulmonary arteries, occluding the flow of blood and leading
to CTEPH
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CTEPH is treated differently than other ‘types’ of pulmonary hypertension
Surgical Removal of Chronic
Thromboembolism
Group V: Many people,
unanswered questions
• Advanced kidney disease
• Sickle cell disease
• Sarcoidosis
• Others
Understanding diagnostic
tests: Chest X-ray
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Quick (less than 5 minutes)
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Widely available
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Relatively inexpensive
 Is there lung disease that was not
expected?
 Is the heart enlarged?
 Are the pulmonary arteries enlarged?
 Should we have a baseline ‘film’ in
someone who has difficulty breathing?
Understanding diagnostic
tests: EKG
• Quick (less than 5 minutes)
• Widely available
• Relatively inexpensive
Is there heart disease that was not
expected?
What is the rhythm of the heart?
Is there evidence of pulmonary
hypertension?
Understanding diagnostic tests:
6 Minute Walk
• Quick (about 6 minutes)
• Widely available
• Very inexpensive
• How is your heart tolerating
pulmonary hypertension?
• Has the distance walked
changed over time?
Understanding diagnostic tests:
Blood tests
• Less than 5 minutes
• Safe
• Widely available
• Variable expense, typically
covered
Is the right heart under strain
(BNP)?
Are the organs tolerating
treatment?
Many other questions!
Understanding diagnostic tests:
Echocardiogram (Echo)
• 45 minutes
• ‘Noninvasive’
• Somewhat available
• Expensive, typically covered
Can an estimate of the lung’s blood
pressure be obtained?
How does the right side of the heart
look?
How does the left side of the heart
look?
Understanding diagnostic tests:
Overnight oximetry
• Overnight Study
• Done at home
• Somewhat available
• Moderate expense, typically
covered
 Do oxygen levels decrease during
sleep?
 Is sleep apnea likely?
 Should oxygen be used at night?
Understanding diagnostic tests:
Overnight sleep study
(polysomnography)
• Overnight study
• Done at a sleep center
• Variable availability
• Expensive, typically covered
 Do you have sleep apnea?
 What level of CPAP treats the
sleep apnea?
Understanding diagnostic tests: CTA of
lungs
• 30 minutes
• ‘Noninvasive’ but requires IV
• Somewhat available
• Expensive, typically covered
• Is there a blood clot in the
lungs?
• Is there any evidence of lung
disease?
Understanding diagnostic tests: VQ
scan of lungs
• 60-90 minutes
• ‘Noninvasive’ but requires IV
• To be done at experienced facility
• Expensive, typically covered
 Is there a blood clot in the
lungs?
Understanding diagnostic tests:
Pulmonary Function Tests
• 60-90 minutes
• ‘Noninvasive’ but requires IV
• To be done at experienced facility
• Expensive, typically covered
 Is there a blood clot in the
lungs?
Understanding diagnostic tests:
Right Heart Catheterization
• Outpatient procedure
• Invasive
• To be done at an experienced facility
• Expensive, and typically covered
 Do I have pulmonary hypertension?
 Is this PAH or due to left heart
disease?
 Does the pulmonary hypertension
respond to a vasodilator challenge?
Understanding diagnostic tests:
Right Heart Catheterization
• Outpatient procedure
• Invasive
• To be done at an experienced facility
• Expensive, and typically covered
 Do I have pulmonary hypertension?
 Is this PAH or due to left heart
disease?
 Does the pulmonary hypertension
respond to a vasodilator challenge?
Prognosis: Your type matters
• CTEPH  surgical cure
• Sleep apnea  resolution after treatment
of sleep apnea
• Heart Failure with Preserved Ejection
Fraction  Risk Factor Modification
• Congenital Heart Disease  Favorable
• Cirrhosis  Poor if liver disease advanced
Prognosis in Pulmonary Arterial
Hypertension
Benza et al, Chest, 2011.
Prognosis can Change as your Score
Changes
Benza et al, Chest, 2011.
Prognosis in Pulmonary
Hypertension
• Improved survival over past 25 years
• Moving target
• Often depends on the health of the
right heart
Thanks!
 PHA
 Lori Lopez
 PHA on the Road Steering Committee
 Patients and Caretakers
Questions?
Dan Grinnan, M.D.
Christopher Barnett, M.D.
Grant Farr, D.O.