Strategies and Best Practices for Improving Outcomes in Pulmonary Arterial Hypertension.

Today’s Faculty

Martha Kingman, FNP-C, DNPFamily Nurse Practitioner

Pulmonary Hypertension Clinic

University of Texas Southwestern Medical Center

Dallas, TX

Objectives

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PAH: Definition, WHO Group

Classification, Risk Assessment,

Diagnosis, and Functional

Capacity Classification

Sandra Lombardi, RN

Clinical Care Coordinator

Pulmonary Vascular Program

University of California, San Diego

San Diego, CA

How is PAH Defined?

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McLaughlin VV et al. Circulation, 2009: 119:2250-2294. Hoeper MM et al. J AM Coll Cardiol, 2013; 62 (25 Suppl):D42-D50.Simonneau G, et al. Eur Respir J. 2019;53(1):1801913.

Historic Hemodynamics Definition of PAH: ◼mPAP ≥ 25 mmHg◼PAWP ≤15 mmHg◼PVR > 3 Wood units

New Proposed Hemodynamic Definition of PAH: ◼mPAP ≥ 20 mmHg◼PAWP ≤15 mmHg◼PVR > 3 Wood units

1. Pulmonary Arterial Hypertension (PAH)

1.1 Idiopathic PAH (IPAH)

1.2 Heritable PAH1.2.1 BMPR21.2.2 Unknown

1.3 Drug and toxin induced PAH

1.4 Associated PAH1.4.1 Connective tissue disease1.4.2 HIV infection1.4.3 Portal hypertension1.4.4 Congenital heart disease1.4.5 Schistosomiasis

1.5 PAH long-term responders to calcium channel blockers

1.6 Pulmonary veno-occlusive disease (PVOD) and or pulmonary capillary haemangiomatosis (PCH)

1.7 Persistent pulmonary hypertension of the newborn (PPHN)

Clinical Classification of Pulmonary Hypertension (Nice, 2018)

•Simonneau G, et al. Eur Respir J. 2019;53(1):1801913.

2. PH due to left heart disease

2.1 PH due to heart failure with preserved LVEF

2.2 PH due to heart failure with reduced LVEF

2.3 Valvular disease

2.4 Congenital/acquired cardiovascular conditions leading to post-capillary

3. PH due to lung disease and/or hypoxia

3.1 Obstructive lung disease

3.2 Restrictive lung disease

3.3 Other lung disease with mixed restrictive/obstructive pattern

3.4 Hypoxia without lung disease

3.5 Developmental lung disorders

4. Chronic thromboembolic pulmonary hypertension (CTEPH)

4.1 Chronic thromboembolic pulmonary hypertension

4.2 Other pulmonary artery obstructions

5. PH with unclear multifactorial mechanism

5.1 Hematologic disorders

5.2 Systemic and metabolic disorders

5.3 Others

5.4 Complex congenital heart disease

5.5. Sarcoidosis

Exercise PH is not defined due to lack of evidence to make a recommendation.

PAH: pulmonary arterial hypertension; PVOD: pulmonary veno-occlusive disease; PCH: pulmonary capillary hemangiomatous; LVEF: left ventricular ejection fraction.

Functional Assessment of PAH

Adapted from Rubin LJ. Chest, 2004; 126 (1 Suppl): 7S-10S.

NYHA Functional ClassificationClass I No symptoms with ordinary physical activity

Class II Symptoms with ordinary activity. Slight limitation of activity

Class III Symptoms with less than ordinary activity. Marked limitation of activity

Class IV Symptoms with any activity or even at rest

Adapted from:1. Trembath RC, Harrison R. Pediatr Res, 2003; 53:883-888. 2. Minai OA, Budev MM. Cleve Clin J Med, 2007; 74:737-747. *These observations are based on reports from in vitro, animal, and human trials. The clinical significance is unknown.

Progression of PAH*

ASYMPTOMATIC1 SYMPTOMATIC/DECOMPENSATING1

DECLINING/DECOMPENSATED2

TIMERAPPVR

PAP

CO

RIGHT HEART DYSFUNCTION RIGHT HEART FAILURE

Risk Factors for the Development of PAH

Drugs and Toxins Known to Induce PAH

Galiè N et al. Eur Respir J. 2019 Jan 24;53(1).

Definite◼ Aminorex◼ Fenfluramine◼ Dexfenfluramine◼ Toxic rapeseed oil◼ Benfluorex◼ Selective serotonin reuptake inhibitorsa

◼ Amphetamine/methamphetamine ◼ Dastinib

Possible◼ Cocaine◼ Phenylpropanolamine◼ St. John’s Wort◼ Interferon 𝛼 and 𝛽◼ Some chemotherapeutic agents such as

alkylating agents (mytomycine C, cyclophosphamide)b

◼ L-tryptophan

a Increased risk of persistent pulmonary hypertension in the newborns of mothers with intake of selective serotonin reuptake inhibitors.bAlkylating agents are possible causes of pulmonary veno-occlusive disease.

Other Risk Factors for PAH

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Sitbon O et al. Am J Respir Crit Care Med, 2008; 177:108-113. Simonneau G et al. J Am Coll Cardiol, 2013; 62 (25 Suppl): D34-D41. Opravil M, Serini D. AIDS, 2008; 22(suppl 3): S35-S40. Lapa M et al. Circulation, 2009; 119: 1518-1523. World Health Organization. Schistosomiasis fact sheet. Available: www.who.int/mediacentre/factsheets/fs115/en/. Accessed: February 23, 2019.

Other Risk Factors for PAH

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Frost A, et al. Eur Respir J. 2019 Jan 24;53(1). Hadengue A et al. Gastroenterololgy, 1991; 100:520-528. Colle IO et al. Hepatology, 2003; 37:401-409. Budhiraja R, Hassoun PM. Chest, 2003; 23:562-576.

Common Symptoms of PAH

Rich S et al. Ann Intern Med, 1987; 107:216-223. Brown LM et al. Chest, 2011; 140:19-26.

REVEAL = Registry to Evaluate Early And Long-term PAH disease management. US-based observational registry involving 54 academic and community-based treatment centers. 2967 patients enrolled between March 2006 and September 2007; 2525 patients met the hemodynamic Criterial for PAH. 32 of these patients were excluded from this analysis due to a missing date of PAH symptom onset, leaving final study population of this cohort analysis of 2493 patients

98

73

4741

37 36 33

86

2722 22

1713

Diagnose rare causes of PH

Frost A, et al. Eur Respir J. 2019 Jan 24;53(1).

History, symptoms, signs and/or

laboratory tests suggestive of PH

Echocardiographic probability of PH

Low

Consider other causes and/or follow-up

No clinically significant left heart disease or

lung disease

High

PAH: Diagnostic Algorithm

Fast-track referral of selected patient

Consider V/Q scan to screen

for CTEPH

Consider left heart diseases (assess

pre-test probability) and lung disease

Refer to PH expert center

Intermediate

V/Q scan abnormal?

Assess probability of PH

Identify high-risk patients

Diagnose common causes of PH

Yes

No

Frost A, et al. Eur Respir J. 2019 Jan 24;53(1).

Management at PH expert center

Review or perform V/Q scan

Normal perfusion

Multimodality diagnostic assessment

Any mismatched perfusion defect

PAH: Diagnostic Algorithm

CTEPH diagnostic algorithm

Consider other causes

PH not confirmed

RCH for PH diagnosis and hemodynamic characterization

Review by multidisciplinary PH team

PH confirmedPH not confirmed

CTEPH not confirmed

CTEPH confirmed

PH ClassifiedPH Classification

not certain

Appropriate treatment

Consider trial of treatment

Monitor and reassess

Diagnosis of PAH: Signs of PAH on Chest X-ray

Image courtesy of Vallerie McLaughlin, MD.

Echocardiographic Features of PVH vs. PAH

PVH PAH

2-D Echo ◼Dilated LA◼Variable ejection fraction◼ Normal RV size◼LV remains round in short axis

◼Normal LA, LV size ◼Normal-to-high ejection fraction◼RV /RA enlargement◼Septal bowing (systolic>diastole)◼Pericardial effusion

Doppler ◼Variable PASP◼ + MR◼Evidence of LV diastolic

dysfunction

◼Variable PASP◼No MR◼

Forfia PR. Echocardiography in pulmonary vascular disease. In: Yuan JX-J, Garcia JGN, Hales CA, Rish S, Archer SL, West JB, eds. Textbook of Pulmonary Vascular Disease. 1st ed. New York, NY: Springer; 2011:1425-1446.

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Echocardiographic Characteristics of PAH

Apical 4-chamber view

Importance of Pulmonary Function Testing in PAH

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McGoon M et al. Chest, 2004; 126:14S-34S. The Task Force for the Diagnosis and Treatment of Pulmonary

Hypertension of the European Society of Cardiology (ESC) and the European Respiratory Society (ERS), endorsed

by the International Society of heart and Lung Transplantation (ISHLT). Eur heart J, 2009; 30:2493-2537.

Hoeper MM et al. J Am Coll Cardiol, 2013; 62 (25 Suppl):D42-D52.

Blood Tests to Detect Potential Underlying Disease in PAH

Barst RJ et al. J Am Coll Cardiol. 2004; 43(1Suppl):40S-47S. ESC/ERS Task Force. Eur Heart J, 2009; 30:2493-2537.

Blood Test◼Antinuclear antibody (ANA)◼Antiphospholipid antibodies-Lupus anticoagulant, anticardiolipin antibodies

◼HIV serology◼CBC with platelets◼Liver function test◼Thyroid function test◼Hemoglobin electrophoresis, if indicated

V/Q Scan to Exclude Chronic Thromboembolic Pulmonary Hypertension (CTEPH)

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Image courtesy of Richard N. Channick, MD.

McGoon M et al. Chest, 2004; 126(1 Suppl): 14S-34S.

Functional Capacity: 6- Minute Walk Test

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McGoon M et al. Chest, 2004; 126(1 Suppl):14S-34S. ATS Committee on Proficiency on Proficiency Standards for Clinical Pulmonary Function Laboratories. Am J Respir Crit Care Med, 2002;166:111-117.

Diagnosis of PAH Requires Right Heart Catheterization

McGoon M et al. Chest, 2004; 126:14S-34S. McLaughlin VV et al. J Am Coll Cardiol, 2009; 53:1573-1619.Galiè N et al. Eur Respir J. 2019 Jan 24;53(1).

Right Heart Catheterization◼Required to confirm diagnosis,

calculate resistance, guide therapy

◼Excludes other etiologies of PH- Intracardiac or extracardiac shunts- Left-heart-disease

◼Measures degree of right-heart dysfunction- RAP- CO

Essential Components of Invasive Hemodynamic Assessment◼O2 Saturations (SVC, IVC, RV, PA, SA)◼RAP◼PAP, Systolic, diastolic, mean◼PAWP, or LVEDP◼CO/CI◼PVR

◼Vasodilator challenge should be performed in patients with idiopathic, heritable, and PAH associated with drugs and toxins.

Risk Assessment and Treatment

Martha Kingman, FNP-C, DNP

Family Nurse Practitioner

Pulmonary Hypertension Clinic

University of Texas Southwestern Medical Center

Dallas, TX

Risk Assessment in PAH

Galiè N, et al. European heart journal. 2016;37(1):67-119.

Determinants of Prognosis(estimated 1-year mortality) Low risk <5% Intermediate risk 5 – 10% High risk >10%

Clinical signs of right heart failure Absent Absent Present

Progression of symptoms No Slow Rapid

Syncope No Occasional syncopeb Repeated syncopec

WHO functional class I, II III IV

6MWD >440 m 165-440 m <165 m

Cardiopulmonary exercise testingPeak VO2 >15 ml/min/kg

(>65% pred.)VE/VCO2 slope <36

Peak VO211-15 ml/min/kg (35 – 65% pred.)

VE/VCO2 slope 36-44.9

Peak VO2 <11 ml/min/kg(<35% pred.)VE/VCO ≥45

NT-proBNP plasma levelsBNP <50 ng/l

NT-proBNP <300 ng/mlBNP 50-300 ng/l

NT-proBNP 300-1400 ng/lBNP >300 ng/l

NT-proBNP >1400 ng/l

Imaging (echocardiography, CMR imaging)RA area <18 cm2

No pericardial effusionRA area 18-26 cm2

No or minimal, pericardial effusionRA area >26 cm2

Pericardial effusion

HemodynamicsRAP <8 mmHg

CI ≥2.5 l/min/m2

SvO2 >65%

RAP 8-14 mmHgCI 2.0-2.4 l/min/m2

SvO2 6—65%

RAP >14 mmHgCI<2.0 l/min/m2

SvO2 <60%

2015 ERS Risk Assessment

Risk Assessment in PAH : REVEAL Calculator

METRIC SCORE

WHO Group I Subgroup APAH-CDT +1 APAH-PoPH +2 APAH-PoPH +2

Demographics & Comorbidities Renal Insufficiency +1 Male age > 60 yrs +2

NYHA/WHO Functional Class I -2 III +1 IV +2

Vital Signs SBP <110 mm Hg +1 HR >92 BPM +1

6-minute Walk Test ≥440 m -1 <165 m +1

BNP <50 pg/ml -2 >180 pg/mL +1

Echocardiogram Pericardial effusion +1

Pulmonary Function Test % prod. Dlco >80 -1 % prod. Dlco <32 +1

Right-heart Catheterization mRAP >20 mm Hg within 1 yr +1 PVR >32 Wood units +2

SUM OF ABOVE:

(STARTING SCORE) +6

= RISK SCORE

Risk scores range from 0 (lowest risk) to 22 (highest risk) LOW AVERAGE MODERATE HIGH VERY HIGH

RISK SCORE 1-7 8 9 10-11 ≥12

PREDICTED 1-YEAR SURVIVAL 95%-100% 90%-<95% 85%-<90% 70%-<85% <70%

APAH=associated PAH; BNP=brain natriuretic peptide; BPM=beats per minute; CTD=connective tissue disease; DLco=carbon monoxide diffusing capacity; FPAH=familial PAH; HR=heart rate; mRAP=mean right atrial pressure; NYHA=New York Heart Association; PAH=pulmonary arterial hypertension; PoPH=portopulmonary hypertension; PVR=pulmonary vascular resistance; SBP=systolic blood pressure; WHO=World Health Organization

PAH – General Care

»Patients should be referred to expert PAH centers, ideally prior to starting any PH medications.

»Oxygen- Hypoxia is a potent vasoconstrictor and can elevate PA pressure.

» Fluid/ Volume control – Diuretics, fluid restriction, low salt diet.

» Anticoagulation - Anticoagulation is not recommended in associated forms of PH while in idiopathic and heritable the dates is conflicting and thus decision is on a case by case basis.

» LOW RISK STATUS is GOAL for all patients: Therapy escalation is recommended in the event that low risk status has not been obtained at follow up.

» Pulmonary rehabilitation should be considered in the treatment plan to improve exercise capacity and quality of life.

Klinger R, et al. Chest. January 2019 ( In Press) Available: https://journal.chestnet.org/article/S0012-3692(19)30002-9/fulltext.

Simonneau G, et al. Eur Respir J. 2019;53(1):1801913.

Galiè N et al. Eur Respir J. 2019 Jan 24;53(1).

PAH Treatment Goals

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Barst RJ et al. J Am Cll Cardiol, 2009; 54(1 Suppl):S78-S84. McLaughlin VV et al. J Am Coll Cardiol, 2013; 62 (25 Suppl); D73-D91.

Variables That Influence Clinical Management Decisions for PAH

PAH: pulmonary arterial hypertension.

McLaughlin VV et al. J Am Coll Cardiol. 2009;53:1573-1619.

PAH

Disease severity

Comorbidillness

Treatmentgoals

Sideeffects

Route of administration

Clinicianpreference

Galiè N et al. Eur Respir J. 2019 Jan 24;53(1).

Treatment-naïve patient

PAH confirmed by expert center

Acute vasoreactivity test (IPAH/HPAH/DPAH only)

Treatment Algorithm 2018 Nice

General measuresSupportive therapy

Triple sequential combination

High risk

Vasoreactivity

CCB therapy

Non-vasoreactivity

Low or intermediate risk

Residual role for initial

monotherapy

Initial oral combination

Initial Combination

including i.v. PCA

After 3-6 months of treatment

Intermediate or high risk

Low risk

Structured follow-up

After 3-6 months of treatment

Intermediate or high risk

Maximal medical therapy and listing for lung transplantation

Consider referral for lung transplantation

cAMP: cyclic adenosine monophosphate; cGMP: cyclic guanosine monophosphate; GTP: guanosine triphosphate; ERA: endothelin receptor antagonist; NO: nitric oxide; PDE-5i: phosphodiesterase-5 inhibitor; PGI2: prostacyclin; sGC: soluble guanylate cyclase

PAH-specific therapies target the three signalling pathways involved in PAH

Adapted from Humbert M, et al. Circulation 2014; 130:2189-2208.

Pro-endothelin-1

ET-1(Vasoconstriction & proliferation)

L-arginine

(Vasodilation & anti-proliferation)

cAMP

PGI2NO

Endothelin

pathway

Prostacyclin

pathway

Nitric oxide

pathway

Arachidonic acid

IP receptor

(Vasodilation & anti-proliferation)

ETAETB

Dual ERASingle ERAcGMPGTP GMP

PDE5sGC

PDE-5 inhibitors

Exogenous NO

sGCstimulator

- +

+

---

Non-prostanoidIP receptor

agonist

PGI2

analogues

+ +

Pathogenesis of PAH: Vasoconstriction

McLaughlin VV, McGoon MD. Circulation. 2006; 114:1417-1431.

VASODILATIONNitric oxideProstacyclin

VASOCONSTRICTIONEndothelinSerotonin

Thromboxane

PAH

Galiè N, et al. European heart journal. 2016;37(1):67-119.

Endothelin Receptor Antagonists (ERAs)

Mechanism of Action »

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WARNINGS»

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PROLIFERATION

HYPERTROPHY

FIBROSIS

VASOCONSTRICTION

INFLAMMATION

ET

Endothelin Receptor Antagonists (ERAs)

Drug Route of administration Comment

Bosentan Oral, BID Monitor LFTs before and monthly during therapy, monthly pregnancy testing for all females of child bearing status; REMS; needs specialty pharmacy

Macitentan Oral, QD Monitor before and monthly pregnancy testing for all females of child bearing status; REMS; needsspecialty pharmacy

Ambrisentan Oral, QD Monthly pregnancy testing for all females of child bearing status as clinically indicated; REMS; needs specialty pharmacy

PAH Is Associated With Alterations in Nitric Oxide (NO) Production*

Mason NA et al. J Pathol, 1998; 185:313-318. Giaid A, Saleh D. N Engl J Med .1995 Jul 27;333(4):214-21. McLaughlin VV et al. J Am Coll Cardiol, 2009; 53:1573-1619.Farber HW, Loscalzo J. N Engl J Med, 2004;351:1655-1665.

* These observations are based on reports from in vitro, animal, and human trials. The clinical significance is unknown.

Levels of eNOS in vascular tissues

NOVasoconstriction

Phosphodiesterase-5 (PDE-5) Inhibitors and Guanylate Cyclase Stimulators (sGC)

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Barst RJ. Vasc Health Risk Manag. 2007;3:11-22.

SP = Requires specialty pharmacy (SP) where medication is delivered to home monthly

DrugRoute of administration Comment

Sildenafil Oral, TID Not be used with nitrates or guanylate cyclase stimulator

Tadalafil Oral, QD Not be used with nitrates or guanylate cyclase stimulator

Riociguat Oral, TID Not be used with nitrates and/or Phosphodiesterase 5 inhibitors (PDE-5Is); REMS; SP

Soluble Guanylate Cyclase (sGC) Stimulator

Mechanism of Action

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WARNINGS:

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FDA approved sGc stimulator:

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Riociguat package insert

Risk Evaluation and Mitigation Strategy Program (REMS)

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Humbert M, N Engl J Med 2004;351:1425-36.

Prostacyclins

Mechanism of Action (a prostaglandin lipid molecule)»

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WARNINGS: »»»

CELL PROLIFERATION

THROMBOSIS

VASOCONSTRICTION

INFLAMMATION

PGI2

Prostacyclin Pathway Meds

NS, normal saline (0.9%); SWFI, sterile water for injection.

NOTE: All IV Prostanoids require a dedicated line; no blood draws; no flushing

Drug Route of administration

Epoprostenol Continuous IV infusion

Continuous IV infusion

Treprostinil Continuous IV infusion

Continuous SC infusion

Inhalation

Oral, BID

Iloprost Inhalation

IP receptor Agonist Oral, BID

Oral Treprostinil (Orenitram)

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Orenitram prescribing information. Available: https://www.orenitram.com/pdf/Orenitram_Full_Prescribing_Information.pdf. Accessed: February 23, 2019.

Selexipag (Uptravi)

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Flolan prescribing information. GlaxoSmithKline.

Epoprostenol (Flolan® or Veletri ®)

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Pain

Erythema

Induration

Treprostinil (Remodulin®) SQ

Infusion Site Pain & Site Reaction

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Subcutaneous Intravenous

CADD-MS 3

CADD-MS 3

CADD-Legacy

CADD-MS is a trademark and CADD-Legacy is a registered trademark of Smiths Medical System. Cane Crono Five is manufactured by Canè Medical Technology.

Treprostinil (Remodulin®) Pump Options: Relative Size

Implantable Pump for Remodulin

• Newly approved 2018 , currently only available at select PH centers

• Eliminates the risk for central line sepsis

• Allows patients to swim /bathe

• Requires pump refills

https://www.remodulin.com/delivery-options/implantable-pump?gclid=CjwKCAiA8OjjBRB4EiwAMZe6yyewK6NoG8m5DnDpJohe2tb6ScEILQQ5OgdXma5qO02Tmo2WXRSU2hoCcckQAvD_BwE

Possible Dose Adjustments Over Time

Goal of Continuous Infusion Dosing

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Balloon pulmonary angioplasty (BPA)

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Ogawa A, et al. Front Cardiovasc Med. 2015;2:4.

Evolving Paradigm

PAH: Evolving Treatment Paradigm

Sequential Combination

Drug 1

Drugs1 + 2

Drugs1 + 2 + 3

+

+

Upfront Combination

2 or 3 Drugs(especially in patients who

present with high risk features)

Humbert M, Lau EMT, Montani D, et al. Circulation. 2014;130:2189-2208.

The Past Current

Lung Transplantation for PAH

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» Patients should be referred for transplant evaluation when they remain intermediate or high risk.

Klinger R, et al. Chest. January 2019 ( In Press) Available: https://journal.chestnet.org/article/S0012-3692(19)30002-9/fulltext.Frost A, et al. Eur Respir J. 2019 Jan 24;53(1).

Long-term Survival of PAH Patients: REVEAL Registry

Farber H, et al. Chest. 2015;148:1043-54.

0

20

40

60

80

100

I II II IV

5-Year Survival by Functional Class I-IV

Previously Diagnosed Newly Diagnosed

Su

rviv

al (

%)

88%

0

20

40

60

80

100

Year 1 Year 5

1- and 5-Year Survival: REVEAL

Previously Diagnosed Newly Diagnosed

90% 86%

65%61%

Su

rviv

al (

%) 72%

76%72%

60%57%

27%

44%

Managing PH Medication SideEffects and Optimizing Therapy

Endothelin Receptor Antagonists: Side Effects

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*PHA Scientific Leadership Council recommends LFT testing at onset of all treatments for PAH and periodically thereafter, at prescriber’s discretion.

PDE-5: Side Effects

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Most side effects can be managed with acetaminophen and anti-diarrhea meds

Contraindicated with use of nitrate

sGC Stimulator: Side Effects

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Contraindicated in pregnancy and with of nitrates in any form, or with use of PDE inhibitors

Prostacyclins: Adverse Events & Side Effects

Side Effects:

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Complications of the Delivery System:

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»Varies according to drug and

route of delivery

Nursing Implications for IV Prostacyclins

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Compliance Challenges

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Stewart S. Pulmonary Hypertension Association. Available at: http://www.phaonlineuniv.org/Journal/Article.cfm?ItemNumber=786. Accessed: February 23, 2019.

Improving Compliance in PAH

» Assessment of medication adherence with every clinic visit.

» Asking patients to bring pill bottles to reconcile meds initiated or discontinued between visits.

» Utilization of pillboxes, timers, cell phone alarms, or notes for reminders

» Discussion of cultural concepts and social support.

» Get the family involved: review perceived burden on family dynamics.

» Consult with social services to address social or financial factors.

Stewart S. Pulmonary Hypertension Association. Available at: http://www.phaonlineuniv.org/Journal/Article.cfm?ItemNumber=786. Accessed: February 23, 2019.

Conclusions

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McLaughlin VV et al. Circulation, 2009;119:2250-2294. Badesh DB et al. Chest, 2010;137:376-387. Hoeper MM et al. J Am Coll Cardiol, 2013;62 (25 Suppl): D42-D50. Galiè N et al. J Am Coll Cardiol, 2013; 62 (25 Suppl):D60-D72.Simonneau G, et al. Eur Respir J. 2019;53(1):1801913.Frost A, et al. Eur Respir J. 2019 Jan 24;53(1).

Thank You! Questions/Answers