suspecting pulmonary hypertension in the dyspneic patient: who, … · 2018-09-25 · osteopathic...
TRANSCRIPT
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Osteopathic Physicians and Surgeons of Oregon
Annual Primary Care ConferenceSunday, September 16, 2018
Suspecting Pulmonary Hypertension
in the Dyspneic Patient:
Who, When, and How
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Catherine J. Markin, MDClinical Vice President, Medical Subspecialties
Legacy Medical Group
Medical Director, Pulmonary Hypertension Program
Legacy Health
Portland, Oregon
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Faculty Disclosures
• Has no financial conflicts to disclose.
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The Pulmonary Hypertension Association (PHA)
is the leading non-profit organization for PH research,
public awareness, and services. The organization
has over 16,000 members, including patients, family
members, and medical professionals.
www.PHAssociation.org
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Let’s get started…
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PH Lessons
1. Pulmonary Hypertension Is Common
2. WHO Group I PAH Is Rare but Deadly—
Make the Diagnosis Early
3. Know the PH Clinical Clues in the Dyspneic
Patient
4. Look Beyond the PA Pressure on
Echocardiography
5. Definitive Diagnosis of PAH Requires Invasive
Hemodynamic Testing
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PH Lessons (cont’d)
6. Always Look for the Underlying Cause of PH
7. Treatment of PH—Get the Diagnosis Correct
and Determine Functional Status
8. Lack of Response to Acute Vasodilator
Challenge in PAH ≠ Untreatable
9. First Do No Harm—Learn to Differentiate WHO
Group I PAH From Other Forms of PH
10. Appropriate, Timely, and Collaborative Care:
Key to Early and Effective Treatment of PH in
the Dyspneic Patient
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Case Presentations
2 Women With Dyspnea
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2 Women With Dyspnea
Patient 2Patient 1 Patient 2
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Age: 57 years
Patient 1
Comorbidities:
• HTN
• Diabetes
• CKD
• Atrial fibrillation
2 Women With Dyspnea
Patient 2
Age: 48 years
Comorbidities:
• HTN
• CKD
• Systemic sclerosis
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Patient 2
NYHA Class III
• BP: 120/84 mm Hg
• JVP elevated
• Regular rate, rhythm
• Loud 2
• 3/6 murmur
(holosystolic,
left sternal border)
• 2+ leg edema
NYHA Class III
Patient 1
• BP: 172/65 mm Hg
• JVP elevated
• Irregularly irregular
• Loud P2
• 2/6 murmur
(holosystolic,
left sternal border)
• 2+ leg edema
2 Women With Dyspnea
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What do we do?
Does this patient have pulmonary hypertension (PH)?
If PH, does this patient have pulmonary arterial hypertension, PAH?
PAH versus PH, why should I care?
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Pulmonary Arterial Hypertension (PAH): Key Points• Average 14-mo delay from initial presentation to
diagnosis: need to diagnose early
• Evaluation must be methodical and include
echocardiography and right heart catheterization
• To treat effectively and avoid harm, PAH must be
differentiated from pulmonary venous hypertension
• Prognosis improves with therapy, but PAH remains a
progressive fatal disease
• Therapies and management strategies continue to
evolve
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RVRA LVLAPA PVVC A
Pulmonary Hypertension = abnormally
high pressure in the pulmonary arteries
Mitral/Aortic valvular
disease
Systolic dysfunction
Diastolic dysfunction
Veno-occlusive disease
Pericarditis
Lung disease
Sleep apnea
Thromboembolic diseasePre-
capillary
disease
Pulmonary Arterial
(PAH)
Cor PulmonalePost-capillary (PVH)
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RVRA LVLAPA PVVC A
Pulmonary Hypertension = abnormally
high pressure in the pulmonary arteries
Mitral/Aortic valvular
disease
Systolic dysfunction
Diastolic dysfunction
Veno-occlusive disease
Pericarditis
Lung disease
Sleep apnea
Thromboembolic diseasePre-
capillary
disease
Pulmonary Arterial
(PAH)
Cor PulmonalePost-capillary (PVH)
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5th World Symposium on PH: Hemodynamic Definition of PH/PAH
PH
PAHMean PAP ≥25 mm Hg plus
PAWP ≤15 mm Hg plus
PVR >3 Wood units
Mean PAP ≥25 mm Hg
at rest during RHC
Hoeper MM et al. J Am Coll Cardiol. 2013;62:D42-D50.
Look for 6th World Symposium Update
End of 2018
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5th World Symposium on PH:Classification1. Pulmonary arterial hypertension
1.1 Idiopathic PAH
1.2 Heritable PAH
1.2.1 BMPR2
1.2.2 ALK1, ENG, Smad 9, CAV1, KCNK3
1.2.3 Unknown
1.3 Drug- and toxin-induced
1.4 Associated with
1.4.1 Connective tissue disease
1.4.2 HIV infection
1.4.3 Portal hypertension
1.4.4 Congenital heart diseases
1.4.5 Schistosomiasis
1’. Pulmonary veno-occlusive disease and/or
pulmonary capillary hemangiomatosis
1’’. Persistent PH of the newborn
2. PH due to left heart disease
2.1 LV systolic dysfunction
2.2 LV diastolic dysfunction
2.3 Valvular disease
2.4 Congenital/acquired left heart inflow/outflow tract
obstruction and congenital cardiomyopathies
3. PH due to lung diseases and/or hypoxia
3.1 Chronic obstructive pulmonary disease
3.2 Interstitial lung disease
3.3 Other pulmonary diseases with mixed restrictive
and obstructive pattern
3.4 Sleep-disordered breathing
3.5 Alveolar hypoventilation disorders
3.6 Chronic exposure to high altitude
3.7 Developmental lung diseases
4. Chronic thromboembolic PH
5. PH with unclear multifactorial mechanisms
5.1 Hematological disorders: chronic hemolytic anemia,
myeloproliferative disorders, splenectomy
5.2 Systemic disorders: sarcoidosis, pulmonary
histiocytosis, lymphangioleiomyomatosis,
5.3 Metabolic disorders: glycogen storage disease,
Gaucher disease, thyroid disorders
5.4 Others: tumoral obstruction, fibrosing mediastinitis,
chronic renal failure, segmental PH
Simonneau G et al. J Am Coll Cardiol. 2013;62:D34-D41. Look for 6th World Symposium Update End of 2018
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Lesson 1
Pulmonary Hypertension
Is Common
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PH in the Community: PASP and Survival
All Participants
(N=1413)Overall Log Rank p<0.001
No Cardiopulmonary Disease
(N=778)Overall Log Rank p=0.002
*Bonferroni-adjusted p<0.05 in pairwise comparison with lowest tertile.
Lam CSP et al. Circulation. 2009;119:2663-2670.
86420
Time (yr)
Cu
mu
lati
ve
su
rviv
al
1.00
0.95
0.90
0.85
PASP quintile
1: 15-23 mm Hg
2: 24-25 mm Hg
3: 26-29 mm Hg
4: 30-32 mm Hg*
5: 34-66 mm Hg*
86420
Time (yr)C
um
ula
tive
su
rviv
al
PASP tertile
1: 15-24 mm Hg
2: 24-28 mm Hg
3: 28-43 mm Hg*
1.000
0.975
0.950
0.925
0.900
Up to 20% of the US population has echo
evidence of PH
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100
75
50
25
0
PH Is Common in Elderly Patients With Heart Failure With Preserved EF
• PH by echo in a community-
based sample:
– heart failure with
preserved EF: 83% with
PH
– HTN but no CHF (control):
8% with PH
• Patients with PH:
– older
– higher systolic BP
– larger LA size
– higher E/e’ ratio
Lam CS. J Am Coll Cardiol. 2009;53:1119.
Cu
mu
lative
(%
)
10 30 50 70 90 110
PASP (mm Hg)
HTN
HFpEF
p<0.001
100
75
50
25
0
PH
pre
va
len
ce
(%
)
HTN
8%
HFpEF
83%
p<0.001
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Epidemiology of PH by Echo
• Single echo lab / Australian community of 165,450
• Etiology of PH noted on echocardiogram
N=936 of 10,314 patients with echo PASP >40 mm Hg.
Strange G et al. Heart. 2012;98:1805-1811.
Miscellaneous, 2.7%
Lung disease,
Sleep-related
hypoventilation,
9.3%
CTEPH, 2.0%
PAH, 2.7%
Unknown,
15.4%
Left heart
disease, 67.9%
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Lesson 2
WHO Group I PAH Is Rare
but Deadly—
Make the Diagnosis Early
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Adapted from: Sitbon O et al. J Am Coll Cardiol. 2002;40:780-788. D’Alonzo GE et al. Ann Intern Med.
1991;115:343-349. McLaughlin VV et al. Chest. 2004;126:78S-91S.
Idiopathic PAH: Survival If Untreated
0 0.5 1 1.5 2 2.5 3 3.5 4 4.5 5
0
20
40
60
80
100
Years of follow-up
Perc
enta
ge s
urv
ivin
g
NIH registry
Sitbon historical control
ACCP estimate
• Incidence: 2-6 cases per
million in US
• Poor prognosis in an era
lacking therapy
• Therapeutic options and
research efforts now offer
more hope
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Schematic Progression of PAH
Time
PAP
PVR
Presymptomatic/
Compensated
Symptomatic/
Decompensating
Symptom Threshold
Right Heart
Dysfunction
Declining/
Decompensated
CO=TPG
PVR
TPG=transpulmonary gradient.
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Advanced Functional Class at Diagnosis Common and Indicates Delayed Recognition
Perc
ent
of
patients
FC
III/IV
at
dia
gnosis
REVEAL Registry(N=1831)
NIH Registry(N=187)
French Registry(N=674)
100
80
60
40
20
0
• Approximate prevalence: 15 cases/million
• More common in women
• Spans broad age range
• Delay in diagnosis persists
• Most patients diagnosed with late symptoms
• Poor prognosis if untreated (median survival <3 yr)
Badesch DB et al. Circulation. 2010;137:376-387. D’Alonzo GE. Ann Intern Med. 1991;115:343-349.
Humbert M et al. Am J Respir Crit Care Med. 2006;173:1023-1030.
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Lesson 3
Know the PH Clinical Clues
in the Dyspneic Patient
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Patient Presentation: Nonspecific Symptoms
0 10 20 30 40 50 60 70
Edema
Palpitations
Chest pain
Near syncope/syncope
Fatigue
Dyspnea
Patients (%)
Rich A et al. Ann Intern Med. 1987;107:216-223.
Badesch DB et al. Chest. 2010;137:376-387.
NIH Registry (1981 to 1985) 1.3 years
REVEAL Registry (2006 to 2007) 1.1 years
Multiple
educational
efforts
Median Time From Symptom Onset to Diagnosis
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Is There a Reason to Suspect PAH?Clinical Presentation
History Exam (PH) Exam (RV Failure)
• Dyspnea (86%)
• Fatigue (27%)
• Chest pain (22%)
• Edema (22%)
• Syncope (17%)
• Dizziness (15%)
• Cough (14%)
• Palpitations
(13%)
• Loud P2 (listen at apex)
• RV lift (left parasternal –
fingertips)
• RV S3, S4
• Systolic murmur (TR;
inspiratory augmentation)
• Early systolic click
• Midsystolic ejection
murmur
• Diastolic murmur (PR)
• JVD; increased A
wave, V wave;
hepatojugular reflex
• Pulsatile liver
• Hepatomegaly
• Edema
• Ascites
• Low BP, low PP, cool
extremities
REVEAL. Brown LM et al. Chest. 2011;140:19-26.
Adapted from McLaughlin VV et al. J Am Coll Cardiol. 2009;53:1573-1619.
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Risk Factors
Is There a Reason to Suspect PAH?
• Family history
• Connective tissue disease
• Congenital heart disease
• Portal hypertension—orthotopic liver transplant
candidate
• Environmental/drug factors
• HIV
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Right Axis RVH
Right
Atrial
Enlarge-
ment
RV Strain
Is There a Reason to Suspect PAH?ECG
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Normal
Abnormal
RV enlargement
into retrosternal
clear space
Peripheral hypo-
vascularity (pruning)
Prominent central
pulmonary artery
Adapted from McLaughlin VV et al. J Am Coll Cardiol. 2009;53:1573-1619.
Is There a Reason to Suspect PAH?Chest X-ray
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Is There a Reason to Suspect PAH? Echo• RV enlargement
• RA enlargement
• Septal straightening
• Loss of IVC inspiratory collapse
• Tricuspid regurgitation
• Pericardial effusion
• Decreased RV systolic dysfunction
– TAPSE (tricuspid annular plane systolic
excursion)
TAPSE 1.5 cmTAPSE 2.3 cm
Relatively preserved
RV function
RV dysfunction
McLaughlin VV et al.
J Am Coll Cardiol.
2009;53:1573-1619.
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Is There a Reason to Suspect PAH?VQ Scan
Idiopathic
Pulmonary
Arterial
Hypertension
Chronic
Pulmonary
Embolism
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Chronic Thromboembolic PH (CTEPH)
Not a PAH subgroup, but:
• Should never be missed
• Is potentially curable with thromboendarterectomy (PEA)
• 3% to 4% of acute PE do not entirely resolve
• One half of those with CTEPH do not have an apparent
history of acute PE
• Normal VQ scan excludes chronic PE
• CT angiogram can detect chronic clot (experienced
radiologist is required)
McLaughlin VV et al. J Am Coll Cardiol. 2009;53:1573-1619.
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retraction with
partial obstruction
retraction with
total obstruction
CTEPH: CT Angiogram
Disturbed resolution of thrombus*
*Castañer E et al. Radiographics. 2009;29:31-53.
Less
subtle
thrombus
PEA
web or bands
recanalizationstenosis
fresh
thrombus
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Is There a Reason to Suspect PAH?Pulmonary Function
• Underlying lung disease (diagnostic group III)
• Abnormalities consistent with PH
• 20% have an isolated reduction
in DLCO
• DLCO mildly reduced
(60%-80% predicted NIH registry)
• PVR correlates with reduction in
DLCO
IPAH and CTEPH
• 20% have an isolated reduction
in DLCO
• Severity predicts future PAH
• DLCO correlates inversely with
PASP
Systemic Sclerosis
DLCO=diffusing capacity of the lungs for carbon monoxide
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Is There a Reason to Suspect PAH? Overnight Oximetry
• Hypoxia may signal underlying sleep apnea
• In patients with obstructive sleep apnea (OSA),
pulmonary artery pressures (PAP) are reported to
decrease in response to CPAP therapy
• Untreated—response to other treatment likely to be
less effective
Somers VK et al. J Am Coll Cardiol. 2008;52:686-717.
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Substrate Further Assessment Rationale
Known BMPR2
mutation
Echo yearly; RHC if echo shows
evidence of PAH
Early PAH detection; 20%
chance of developing PAH
Systemic
sclerosis*
Echo yearly; RHC if echo shows
evidence of PAH8% prevalence of PAH
HIVEcho if symptomatic; RHC if echo
shows evidence of PAH0.5% prevalence of PAH
Portal
hypertension
Echo if OLT considered; RHC if
echo shows evidence of PAH
4% prevalence of PAH;
predictive of poor outcome
Congenital
heart disease
Echo and RHC at diagnosis;
consider repair of L-R shunt defect
High PAH probability if
unrepaired (Eisenmenger)
Screening Guidelines: Patients With Known PAH Risk
McLaughlin VV et al. J Am Coll Cardiol. 2009;53:1573-1619.
*Systemic sclerosis: consider echocardiogram if or unexplained declining DLCO.% DLCO
% FVC>1.6
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Diagnostic Algorithm for PH
• Identical for local practitioners and PH specialists
• Requirements:
– thorough evaluation
– high quality studies and interpretation
• Establish a suspicion of PAH
• Confirm the diagnosis (right heart catheterization)
• Classify the type of PH (Group I-V)
• Determine the disease severity
• Select the appropriate treatment for patients with PAH
McLaughlin VV et al. J Am Coll Cardiol. 2009;53:1573-1619.
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Strengths Limitations
Echocardiography in PH
• Best screening tool for PH
• Inexpensive, portable, readily
available, non-invasive, no
radiation
• Allows for serial assessment
• Provides clues to other
diagnoses (eg, LHD, CHD)
• Experienced techs/MDs
essential
• Imaging quality suboptimal in
patients with poor windows
(eg, lung disease, obesity)
• Right ventricle not imaged
adequately in some labs
• TR jet inadequate to
determine RVSP in
10%–25% of patients
McLaughlin VV et al. J Am Coll Cardiol. 2009;53:1573-1619.
Bossone E et al. Chest. 2005;127:1836-1843.
Brecker SJ. Br Heart J. 1994;72:384-389.
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Echocardiogram
PFT’s
Polysomnography
VQ Scan
• Sleep Disorder
• Chronic PE
Functional Test
(6MWT, CPET)
Overnight
Oximetry
History
Exam
CXR
ECG
HIV
ANA
LFT’s
RH Cath
TEE
Exercise Echo
Pulmonary Angiography
Chest CT Angiogram
Coagulopathy Profile
Vasodilator Test
Exercise RH Cath
Volume Loading
ABG’s
• Index of Suspicion of PH
• RVE, RAE, RVSP, RV
Function
• Left Heart Disease
• VHD, CHD
• Ventilatory Function
• Gas Exchange
Other CTD Serologies
• HIV Infection
• Scleroderma, SLE, RA
• Portopulmonary Htn
• Establish Baseline
• Prognosis
• Confirmation of PH
• Hemodynamic Profile
• Vasodilator Response
Pivotal Tests Contingent Tests Contribute to
Assessment of:
Left Heart CathMcLaughlin VV et al. J Am Coll Cardiol.
2009;53:1573-1619.
AC
CF
/AH
A D
iagnostic A
lgorith
m
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Lesson 4
Look Beyond the PA Pressure
on Echocardiography
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PAH: RV Changes
Normal PH
RA
LA
RV
LV
TTE apical
4-chamber view
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Key Features of PAH on Echo
• Enlarged RV with normal or small LV
• RA, RV, and PA enlargement
• RV dysfunction
• Increased RVSP
• Interventricular septal flattening during systole
± diastole
• Mitral E/A wave ratio <1.0
Bossone E et al. Chest. 2005;127:1836-1843.
McLaughlin VV et al. J Am Coll Cardiol. 2009;53:1573-1619.
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Essential Components of the Echocardiogram in PH
• Doppler estimate of RVSP
• Assess biventricular size and systolic function
• Look for interventricular septal shift
• Discriminate between pulmonary arterial and pulmonary
venous causes of PH (if possible)
• Assess for congenital heart shunt lesions
• Document pericardial effusion
Bossone E et al. Chest. 2005;127:1836-1843.
McLaughlin VV et al. J Am Coll Cardiol. 2009;53:1573-1619.
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Estimation of RV Systolic Pressure (RVSP)
RVSP = 4(velocity of TR)2 + RA
pressure
= 4(4)2 + 20
= ~84 mm Hg
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Lesson 5
Definitive Diagnosis of PAH
Requires Invasive
Hemodynamic Testing
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Catheterization is required when pulmonary
arterial hypertension is suspected
Diagnostic Cardiac Catheterization
• Establish the presence of PH
• Make the diagnosis of PAH
– measure wedge pressure and/or LVEDP
• Determine severity and prognosis of disease
• Exclude congenital heart disease
• Perform acute vasodilator test
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Post-capillary PH
Pre-capillary PH
RA RV PA PVPC
LA LV Ao
PCWP >15 mm Hg
PVR <3 Wood units
PCWP <15 mm Hg
PVR ≥3 Wood units
Other:
High COPCWP <15 mm Hg
PVR <3 Wood units
PH: Define the Lesion(mean PAP ≥25 mm Hg)
Mixed PHPCWP >15 mm Hg
PVR ≥3 Wood units
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Lesson 6
Always Look for the
Underlying Cause of PH
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Echocardiogram
PFT’s
Polysomnography
VQ Scan
• Sleep Disorder
• Chronic PE
Functional Test
(6MWT, CPET)
Overnight
Oximetry
History
Exam
CXR
ECG
HIV
ANA
LFT’s
RH Cath
TEE
Exercise Echo
Pulmonary Angiography
Chest CT Angiogram
Coagulopathy Profile
Vasodilator Test
Exercise RH Cath
Volume Loading
ABG’s
• Index of Suspicion of PH
• RVE, RAE, RVSP, RV
Function
• Left Heart Disease
• VHD, CHD
• Ventilatory Function
• Gas Exchange
Other CTD Serologies
• HIV Infection
• Scleroderma, SLE, RA
• Portopulmonary Htn
• Establish Baseline
• Prognosis
• Confirmation of PH
• Hemodynamic Profile
• Vasodilator Response
Pivotal Tests Contingent Tests Contribute to
Assessment of:
Left Heart CathMcLaughlin VV et al. J Am Coll Cardiol.
2009;53:1573-1619.
AC
CF
/AH
A D
iagnostic A
lgorith
m
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Lesson 7
Treatment of PH—Get the
Diagnosis Correct and
Determine Functional Status
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PAH Treatment Goals
• Improve survival
• Improve quality of life
• Improve exercise
capacity
– 6MWD
– WHO functional
classification
• Improve hemodynamics
• Fewer/less severe
symptoms
• Prevent clinical
worsening
– escalation of therapy
– hospitalization
– lung transplantation
– death
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Chronic Adjuvant Treatment
Digoxin
• Use to prevent hypoxic vasoconstriction
• Consider exercise, sleep, altitude
• Aim for target saturation >90%
• May not correct hypoxia with shunt
Oxygen
McLaughlin VV et al. J Am Coll Cardiol. 2009;53:1573-1619.
Badesch DB et al. Chest. 2007;131:1917-1928.
• Variable inotropic effect and use
• No long-term data; need to balance unproven benefits
with known risks
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0 1 2 3
Chronic Adjuvant Treatment
• Recommended in IPAH and
CTEPH
• Observational data only
• Need to balance unproven
benefits with known risks
• INR goal 1.5 – 2.5
Poor CO
and hypotension
Diuretics
Improved CO
and BP
Observed Survival in IPAH Patients
With and Without Anticoagulation
(60)
(49)
(36)(21)
(14)(7)
Years
Percent
surviving
No Anticoagulation
Anticoagulation
Fuster V et al. Circulation. 1984;70:580-587. Badesch DB et al.
Chest. 2004;126:35S-62S. Olsson KM et al. Circulation. 2014; 29:57–65.
Diuretics
Anticoagulation
• Most patients need
• Hypotension not a contraindication
• Renal function and electrolytes
must be monitored closely
20
40
60
80
100
0
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(+) Vasodilator Response—ONLY IF RESPONDER
(−) Vasodilator Response or Non-sustained
Vasodilator Response
PAH Therapy
• Calcium channel blockers
• Endothelin receptor antagonists
• Phosphodiesterase-5 inhibitors
• sGC stimulator
• Prostanoids
McLaughlin VV et al. J Am Coll Cardiol. 2009;53:1573-1619.
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Mechanisms of Action of Approved Therapies for PAH
Adapted from Humbert M et al. N Engl J Med. 2004;351:1425-1436.
cGMP
cAMP
Vasoconstriction
and proliferation
Endothelinreceptor A
Endothelin-
receptor
antagonists
Endothelinreceptor B
Phosphodiesterase
type 5 inhibitor
Vasodilation
and antiproliferation
Phosphodiesterase type 5
Vasodilation
and antiproliferation
Prostacyclin
derivatives
Nitric Oxide
Endothelin-1
Pre-proendothelin
L-arginine
Prostaglandin I2
L-citrulline
Nitric OxidePathway
EndothelinPathway
ProstacyclinPathway
Endothelial cells
Proendothelin
Endothelial cells
Arachidonic acid
Smooth muscle cells
Prostacyclin (prostaglandin I2)
Smooth muscle cells
Exogenou
s nitric
oxide
sGC
stimulator
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Local Practitioners
Choice of Therapy
• Knowledge and management of comorbid illnesses
• Geographically close to patients
• Established therapeutic relationship
PH Centers
• Experience with therapy escalation
• Knowledge of drug-drug interactions; eg, PDE-5 inhibitors
and antiretroviral therapy
• Nursing support for management of parenteral medications
• Relationships with other subspecialists; eg, lung transplant
centers
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Initial Therapy: Making the Right Decision
• Make sure the patient truly has PAH and not
another type of PH (especially pulmonary venous
hypertension)
• Severity of disease
• Patient preference
• Trying to weigh the data (evidence-based)
• When “comparing” trials, examine objective
baseline characteristics (6MWD, hemodynamics)
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PAH Determinants of Risk
McLaughlin VV et al. J Am Coll Cardiol. 2009;53:1573-1619.
LOWER RISK DETERMINANTS OF RISK HIGHER RISK
NoClinical evidence of
RV failureYes
Gradual Progression of symptoms Rapid
II, III WHO class IV
Longer (>400 m) 6MWD Shorter (<300 m)
Peak VO2 >10.4 mL/kg/min CPET Peak VO2 <10.4 mL/kg/min
Minimal RV dysfunction Echocardiography
Pericardial effusion,
significant RV
enlargement/dysfunction;
RA enlargement
RAP <10 mm Hg;
CI >2.5 L/min/m2 HemodynamicsRAP >20 mm Hg;
CI <2.0 L/min/m2
Minimally elevated BNP Significantly elevated
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• Supervised exercise training (I-A)
• Psycho-social support (I-C)
• Avoid strenuous physical activity
(I-C)
• Avoid pregnancy (I-C)
• Influenza and pneumococcal
immunization (I-C)
• Oral anticoagulants:
– IPAH, heritable PAH, and PAH
due to anorexigens (IIa-C)
– APAH (Ilb-C)
• Diuretics (I-C)
• Oxygen (I-C)
• Digoxin (IIb-C)
Continue CCB
WHO FC I-III
CCB (I-C)
Sustained response
(WHO FC I-II)
VASOREACTIVE NON-VASOREACTIVE
5th World Symposium on PH:PAH Treatment Algorithm
Galiè N et al. J Am Coll Cardiol. 2013;62:D60-D72.
INITIAL THERAPY WITH
PAH-APPROVED DRUGSYES
NO
Acute vasoreactivity test
(I-C for IPAH) (IIb-C for APAH)
Expert Referral (I-C)
General measures and supportive therapy
Look for 6th World Symposium Update End of 2018
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Approved Therapeutic Targets
Humbert M et al. N Engl J Med. 2004;351:1425-1436.
cGMP
cAMP
Vasoconstriction
and proliferation
Endothelinreceptor A
Endothelin-
receptor
antagonists
Endothelinreceptor B
Phosphodiesterase
type 5 inhibitor
Vasodilation
and antiproliferation
Phosphodiesterase type 5
Vasodilation
and antiproliferation
Prostacyclin
derivatives
Nitric Oxide
Endothelin-1
Pre-proendothelin
L-arginine
Prostaglandin I2
L-citrulline
Nitric OxidePathway
EndothelinPathway
ProstacyclinPathway
Endothelial cells
Proendothelin
Endothelial cells
Arachidonic acid
Smooth muscle cells
Prostacyclin (prostaglandin I2)
Smooth muscle cells
Exogenous
nitric oxide
sGC
stimulator
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PDE-5 Inhibitor Pivotal Trials
Study Name
Drug
N
Etiol
Class
Design Positive Results
SUPER-1
Oral sildenafil*
vs placebo
278
PAH
I-IV
Double-blind
12-week
• 6MWD
• Symptoms
• Hemodynamics
PHIRST-1
Oral tadalafil§
vs placebo
405
PAH
I-IV
Double-blind
16-week
• 6MWD
• Delay clinical
worsening
• Hemodynamics
• HRQoL
Galiè N et al. N Engl J Med. 2005:353:2148-2157.
Galiè N et al. Circulation. 2009;119;2894-2903.
*Sildenafil = Revatio®. Approved for FC II-III. 20 mg po tid.§Tadalafil = Adcirca®. Approved for FC I-IV. 40 mg po qd.
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PDE-5 Side Effects
• Nose bleed
• Headache
• Dyspepsia
• Flushing
• Diarrhea
• Visual changes
Contraindicated with use of nitrate
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sGC Stimulator Pivotal Trials
Study Name
Drug
N
Etiol
Class
Design Positive Results
PATENT-1
Oral riociguat*
vs placebo
443
PAH
I-IV
Double-blind
12-week
• 6MWD
• Symptoms
• Hemodynamics
• Delay clinical worsening
CHEST-1
Oral riociguat
vs placebo
261
CTEPH
I-IV
Double-blind
16-week
• 6MWD
• Symptoms
• Hemodynamics
*Riociguat = Adempas®. Approved for WHO Group 1; persistent CTEPH (WHO Group 4)
after surgical treatment, or inoperable CTEPH; titrated to maximum 2.5 mg po tid.
Ghofrani HA et al. N Engl J Med. 2013;369:319-329.
Ghofrani HA et al. N Engl J Med. 2013;369:330-340.
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sGC Stimulator Side Effects
• Headache
• Dizziness
• Dyspepsia/gastritis
• Nausea
• Diarrhea
• Hypotension
• Vomiting
• Anemia
• Gastroesophageal reflux
• Constipation
Contraindicated in pregnancy, with use of nitrates or
NO donors in any form, or with use of PDE inhibitors
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Endothelin Receptor Antagonists: Pivotal Trials
Study Name
Drug
N
Etiology
Class Design
Positive
Results
BREATHE-1
Oral bosentan*
vs placebo
213
PAH
III, IV
Double-blind
16-week
• 6MWD
• Delay clinical worsening
• Symptoms
EARLY
Oral bosentan
vs placebo
185
PAH
II
Double-blind
6-month
• Delay clinical worsening
• Hemodynamics
ARIES-1&2
Oral ambrisentan§
vs placebo
394
PAH
II, III
Double-blind
12-week
• 6MWD
• Delay clinical worsening
SERAPHIN
Oral macitentan†
vs placebo
742
PAH
II,III
Double-blind
Event-driven
morbidity/mortality
• Delay disease progression
• 6MWD
• Symptoms
Rubin L et al. N Engl J Med. 2002;346:896-903. Channick RN et al. Lancet. 2001;358:1119-1123. Galiè N et al. Lancet.
2008;371:2093-2100. Galiè N et al. Circulation. 2008;117:3010-3019. Pulido T et al. N Engl J Med. 2013;369:809-818.
*Bosentan = Tracleer®. Approved for FC II-IV. 62.5-125 mg po bid.§Ambrisentan = Letairis®. Approved for FC II-III. 5-10 mg po qd†Macitentan = Opsumit®. Approved for FC II-III. 10 mg po qd.
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Endothelin Receptor Antagonists: Side Effects
• Nasal congestion
• Abnormal hepatic function*
– monthly LFTs required
for bosentan
• Anemia
– monitor CBC quarterly
• Edema
– lower extremity edema
may require diuretic
adjustment
• Teratogenic
– use requires dual
contraceptive methods
(hormonal plus barrier)
*PHA Scientific Leadership Council recommends LFT testing at onset of
all treatments for PAH and periodically thereafter, at prescriber’s
discretion.
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Iloprost (Ventavis®)
Treprostinil (Tyvaso®)
Prostacyclin Analogues: Intravenous, Implanted, Subcutaneous, Inhaled, or Oral
WG
Treprostinil (Remodulin®)
Treprostinil (Orenitram®)
Selexipag (Uptravi®)
Epoprostenol (Flolan®
or Veletri®)
Treprostinil (Remodulin®)
Epoprostenol IV: FC III-IV, 2 ng/kg/min titrated to desired clinical response in 1-2 ng/kg/min increments.
Treprostinil IV / SC: FC II-IV, 1.25-2.5 ng/kg/min/wk. IV=diluted. Inhaled: FC III, to 54 mcg, 4 inh/d. Oral: FC II-III, starting
at 0.25 mg bid and titrated in 0.25 mg increments as tolerated. Selexipag: FC II-III, starting at 200 mcg bid, and titrated as
tolerated up to 1600 mcg bid . Iloprost Inhaled: FC III-IV, 2.5-5 mcg, 6-9 inh/d. Treprostinil Implanted: FC II-IV, same as
patient’s current IV dose
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Prostacyclin Analogues: Pivotal Trials for IV and SC Formulations
Study Name / DrugN / Etiol /
ClassDesign Positive Results
IV epoprostenol
vs conventional Rx
81
IPAH/FPAH
III,IV
Open-label
12-week
• 6MWD
• Symptoms
• Hemodynamics
• Survival
IV epoprostenol
vs conventional Rx
111
APAH SSc
III,IV
Open-label
12-week
• 6MWD
• Hemodynamics
• Symptoms
TRUST
IV treprostinil vs
placebo
44
PAH
III
Double-blind,
placebo-
controlled
12-week
• 6MWD
• Symptoms
SC treprostinil
vs SC placebo
470
PAH
II-IV
Double-blind
12-week
• 6MWD
• Symptoms
• Hemodynamics
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Prostacyclin Analogues: Pivotal Trials for Inhaled and Oral Formulations
Study Name / Drug
N / Etiol /
Class Design Positive Results
AIR
Inhaled iloprost vs placebo
203
PH
III-IV
Double-blind
12-week
• Composite end point
• 6MWD
• Symptoms
• Hemodynamics
TRIUMPH 1
Inhaled treprostinil vs
placebo§
235
PAH
III-IV*
Double-blind
12-week on
background oral Rx
• 6MWD
FREEDOM-M
Oral treprostinil vs placebo
228
PAH
II-III
Double-blind,
placebo-controlled
12-week
• 6MWD
GRIPHON
Oral selexipag vs placebo
1156
PAH
II-III
Double-blind, naïve
or on background
ERA and/or PDE5I,
event-driven
morbidity/mortality
• Time to first morbid or
mortality event
* Approved for class III only. §Included background therapy with ERA or PDE5-I.
Olschewski H et al. N Engl J Med. 2002;347:322-329. McLaughlin VV et al. J Am Coll Cardiol. 2010;55:1915-1922
Hiremath J et al. J Heart Lung Transplant. 2010;29:137-149. Jing Z-C et al. Circulation. 2013;127:624-633.
Sitbon O et al. NEJM. 2015;373:2522-33.
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Prostanoid Side Effects
• Flushing
• Headache
• Diarrhea, nausea,
vomiting
• Jaw pain
• Leg pain
• Hypotension
• Dizziness
• Syncope
• Rebound PH if interruption
of epoprostenol delivery
(due to short half-life)
• Delivery site complications
(pain, infection, cough,
thrombosis, infusion)
Vary according to drug and route of delivery
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5th World Symposium on PH:2013 Treatment Algorithm
Galiè N et al. J Am Coll Cardiol. 2013;62:D60-D72.
Sequential Combination
Therapy (I-A)
Referral for LungTransplantation (I-C)
Consider Eligibility for
Lung Transplantation
Inadequate Clinical
Response
on Maximal Therapy
INITIAL THERAPY WITH PAH-APPROVED DRUGS
PDE-5 I orsGCs
ERAs
Prostanoids
++
+
Balloon AtrialSeptostomy (IIa-C)
Inadequate Clinical
Response
Look for 6th World Symposium Update End of 2018
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Combination Therapy
sGC
StimulatorsProstanoids
Endothelin
Receptor
Antagonists
Phospho-
diesterase
Inhibitors
TRIUMPH
STEP
SERAPHIN†
GRIPHONγ
TRIUMPH
PACES
GRIPHON
PATENT-1*
PATENT-1*
?
?
???
PHIRST*
SERAPHIN†
AMBITION
*53% on background ERA for PHIRST, 50% on background ERA or prostanoid for PATENT-1 †64% on background PDE-5I or prostanoid in SERAPHIN. γ84% on background ERA and/or PDE-5I in GRIPHON
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Lesson 8
Lack of Response to Acute
Vasodilator Challenge in
PAH ≠ Untreatable
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Acute Vasodilator Trial
• Purpose:
– identify vasodilator “responders” who are candidates
for CCB therapy
• Short-acting vasodilators
– inhaled nitric oxide is preferred
• Definition of response
– decrease in mPAP by ≥10 mm Hg down to
mPAP of ≤40 mm Hg
– with improvement or maintenance of
cardiac output
Rubin LJ. Chest. 2004;126:4S-6S.
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Lesson 9
First Do No Harm—Learn to
Differentiate WHO Group I
PAH From Other Forms
of PH
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Is It Left Heart Disease?
Symptoms
– paroxysmal nocturnal
dyspnea
– orthopnea
History
– diabetes
– hypertension
– obesity
– coronary artery disease
– metabolic syndrome
ECG
– atrial fibrillation
– absence of right axis
deviation
Echo
– left atrial enlargement
– left ventricular
hypertrophy
– normal RA, RV
– abnormal diastolic filling
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Problems With Incorrect Treatment
• WHO Group 1 PAH: True PAH
– incorrect treatment with systemic vasodilators could lead to
profound hypotension, death
• WHO Group 2 PH: PH due to LHD
– incorrect treatment with pulmonary vasodilators could lead to
pulmonary edema, CHF exacerbation
• WHO Group 3 PH: PH due to hypoxemia/lung disease
– incorrect treatment with pulmonary vasodilators could lead to
increased V/Q mismatch, worsening hypoxemia
• WHO Group 4 PH: CTEPH
– treating with pulmonary vasodilators likely not harmful, but
don’t delay referral for potentially curative surgical
thromboendarterectomy (if indicated)
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Lesson 10
Appropriate, Timely, and
Collaborative Care:
Key to Early and Effective
Treatment of PH in the
Dyspneic Patient
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Goals of Collaborative Care
Clinical Practice Guidelines
Clinical Trial Evidence
Best PracticeLocal Practitioners
PH Specialists
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Local Care PH Center
• Diagnostic dilemmas
• Diagnostic cath/
vasodilator trial
• Fluid management
• Acute issues
• PAH-specific therapies
• Side effects
• Hospitalizations
• Transplant
• Clinical trials
Collaborative Care With PH Centers:Initial Steps
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Collaborative Care With PH Centers:Ongoing Care
Local Care
• Symptom evaluation
• Titrate diuretics
• Monitor Rx
• Need to change Rx
• Manage SEs
• ? Transplant
• Evaluate acute issues
• Acute hospital care
• Emotional support
PH Center
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Timing of Referral to a PH Center
• Dependent on a local physician’s level of comfort
• Referral can occur at multiple junctures
Abnormal symptoms, exam, and initial screening (echo)
Pivotal tests (without RHC)
Diagnosis (RHC with vasodilator challenge)
Treatment escalation
PH Center
PH Center
PH Center
PH Center
PH Center
Treatment
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Case Resolutions
2 Women With Dyspnea
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Patient 2
Echo
• LV: EF 58%
• Grade 1 diastolic
dysfunction
• RV: size
• 3+ RV dysfunction
• PASP: 76 mm Hg
• RA: 10 mm Hg
• 2+ TR
2 Women With Dyspnea
Echo
Patient 1
• LV: EF 65%
• Grade 3 diastolic
dysfunction
• RV: size
• Normal RV function
• PASP: 60 mm Hg
• RA: 10 mm Hg
• 1+ TR
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Patient 2
Invasive
Hemodynamics
• RA: 12 mm Hg
• mPAP: 41 mm Hg
• PCWP: 10 mm Hg
• CO: 2.6 L/min
• PVR: 11.9 Wood units
• BP: 93/69 mm Hg
• Vasodilator challenge with iNO:
non-responder
2 Women With Dyspnea
Invasive
Hemodynamics
Patient 1
• RA: 15 mm Hg
• mPAP: 42 mm Hg
• PCWP: 29 mm Hg
• CO: 6.7 L/min
• PVR: 1.8 Wood units
• BP: 172/65 mm Hg
• Vasodilator challenge with iNO:
not indicated (high PCWP)
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Patient 2
Final Diagnosis
• WHO Group I—
pulmonary arterial
hypertension (PAH)
• PAH due to connective
tissue disease
2 Women With Dyspnea
Final Diagnosis
Patient 1
• WHO Group II—
pulmonary venous
hypertension (PVH)
• Heart failure with
preserved EF (HFpEF)
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Patient 2: PAH
Clinical Course
• PDE-5 inhibitor:
no improvement
• ERA added
• symptoms over time
• Now on parenteral
prostanoid with
improved symptoms
• NYHA II
2 Women With Dyspnea
Clinical Course
Patient 1: PVH
• Carvedilol
• Furosemide
• Co-managed with
nephrology
• Improved symptoms
• NYHA II
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Summary: PH Lessons
1. PH is common, but most often due to LHD or
chronic lung disease: selective pulmonary
vasodilators are not proven in these patients
2. PAH is rare but deadly: outcomes have
improved but not as much as we would like;
diagnosis must be made earlier
3. Know the PH clinical clues in the dyspneic
patient
4. Know the limitations of echo, and look beyond
PA pressure to the RV to evaluate size/function
5. Definitive diagnosis of PH requires heart cath
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Summary: PH Lessons (cont’d)
6. Identify underlying cause of PH: etiology
important = prognostic and Rx implications
7. Treatment of PH is based on correct diagnosis
and functional status
8. Lack of response to acute vasodilator challenge
in PAH does not mean the patient is untreatable
9. Learn to differentiate Group I PAH from other
forms of PH: when in doubt, ask for help
10. Collaborative care: key to early and effective
treatment of PH in the dyspneic patient
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Final Housekeeping
• Hand in CME evaluation
form and be sure you
signed in at the
registration desk
• Bring your PHA information
home
• Expect an email from WUSM
with a link to access your
CME certificate
CME Course Evaluation:
Screening, Diagnosis, and Treatment of Pulmonary Arterial Hypertension: An Overview
Friday, August 7, 2015 – Lincoln, Nebraska
Deborah J. Levine, MD
Your Professional Degree/Certification (check one):
MD/DO NP PA PharmD RPh RN/LPN RRT Other (please specify)
Check&here&also&if&you&are&in&training&as&a: Fellow Resident Medical Student
Your Medical Specialty or Focus of Practice (check one):
☐ Pulmonary/Critical Care/Respiratory/Sleep ☐ Family Practice/General Medicine
☐ Cardiology/Cardiovascular/CCU ☐ Pediatrics
☐ Rheumatology ☐ Pediatric Specialist*
☐ Hospitalist ☐ Emergency/Urgent/Acute Care
☐ Internal Medicine/Primary Care ☐ Surgery*
☐ Other Internal Medicine* ☐ Other* *Please indicate specialty
PRESENTATION
Considering pertinence, presentation, and audiovisual, what is your overall evaluation of the speaker?
(Circle one) Poor Fair Good Very Good Excellent
OVERALL COURSE
Your OVERALL EVALUATION of the course (circle one): Poor Fair Good Very Good Excellent
Target Audience
This activity has been designed for pulmonologists, cardiologists, rheumatologists, internists, and primary care physicians, as well as
nurses, physician assistants, and other allied health professionals who help care for patients with PAH.
Educational Objectives
This CME program is designed to improve competence, performance, and patient care practices by instructing clinicians in the highest
quality of care for patients with PAH. At the conclusion of the program, participants should be able to:
· Accurately diagnose patients through comprehensive screening and early recognition of symptoms
· Evaluate the patient’s condition and prescribe long-term optimal management, including knowing when and how to treat and
when to consult with colleagues at an established PAH center
1. Did the course fulfill the objectives and was the intended result of those objectives achieved? Yes No
If not, please explain:
2. Was the presentation free from commercial bias? Yes No
If not, please explain:
Continued on reverse side
Please complete both sides of this evaluation form and return it to the Registration Desk
before you leave.
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Thank you for your participation!
For more information on upcoming PHA
Medical Education Programs, please visit:
www.PHAssociation.org