1/14/2019 1 Pulmonary Hypertension for the Primary Care Provider Kenneth W. Presberg, MD Professor of Medicine Director, PHA Pulmonary Hypertension Comprehensive Care Center, Division of Pulmonary and Critical Care Medicine Froedtert & Medical College of Wisconsin Milwaukee, Wisconsin Disclosures: (no direct honorariums; research meetings support) Research PI: Actelion, United Therapeutics. Review the DEFINITION of pulmonary hypertension (PH) Discuss the different GROUPS of PH Patients Examine the PROGNOSIS of PH patients in the different GROUPS Differentiate the BENEFITS and SIDE EFFECTS of different treatments for PH patients. Review new guidelines for assessment of ADEQUATE TREATMENT RESPONSE in PAH. Objectives: PH with Left Heart Disease PH with Lung Disease and/or Low Oxygen Levels “PAH” Miscellaneous Miscellaneous Chronic Thrombosis (clot) PH Chronic Thrombosis (clot) PH Pulmonary Hypertension Comes in Several Varieties Right Heart Catheterization: The Definitive Diagnosis: • Normal Pulmonary Artery (PA) Pressure: 30/15, mean 20 mmHg. • Pulmonary Hypertension (PH) when mean > 25 mmHg (40/20 mmHg )and Elevated Pulmonary Vascular Resistance (PVR) Introductory Questions: Is Pulmonary HTN common? If so, Which Group? Is Pulmonary HTN rare, an “orphan” disease If yes, which Group? Can Pulmonary HTN be cured? Are there effective treatments? Yes, Many patients with left sided heart failure have Group II Pulmonary HTN. Yes, Group I PAH Pulmonary HTN remains an uncommon disease. Chronic PE patients who have a successful surgery return to near normal: OSA patients on CPAP. Yes, particularly for Group I PAH patients now. PH with Left Heart Disease PH with Lung Disease and/or Low Oxygen Levels “PAH” Miscellaneous Miscellaneous Chronic Thrombosis (clot) PH Chronic Thrombosis (clot) PH Pulmonary Hypertension Comes in Several Varieties
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1/14/2019
1
Pulmonary Hypertension for the Primary Care ProviderKenneth W. Presberg, MD
Professor of Medicine
Director, PHA Pulmonary Hypertension Comprehensive Care Center,
Division of Pulmonary and Critical Care Medicine
Froedtert & Medical College of Wisconsin Milwaukee, Wisconsin
Disclosures: (no direct honorariums; research meetings support) Research PI: Actelion, United Therapeutics.
Review the DEFINITION of pulmonary hypertension (PH)
Discuss the different GROUPS of PH Patients
Examine the PROGNOSIS of PH patients in the different GROUPS
Differentiate the BENEFITS and SIDE EFFECTS of different treatments for PH patients.
Review new guidelines for assessment of ADEQUATE TREATMENT RESPONSE in PAH.
Objectives:
PH with Left HeartDisease
PH with LungDisease and/or
Low Oxygen Levels
“PAH”
MiscellaneousMiscellaneousChronic Thrombosis
(clot) PHChronic Thrombosis
(clot) PH
Pulmonary HypertensionComes in Several Varieties
Right Heart Catheterization: The Definitive Diagnosis:
• Normal Pulmonary Artery (PA) Pressure: 30/15, mean 20 mmHg.
• Pulmonary Hypertension (PH) when mean > 25 mmHg (40/20 mmHg )and Elevated Pulmonary Vascular Resistance (PVR)
Introductory Questions:
Is Pulmonary HTN common?
If so, Which Group?
Is Pulmonary HTN rare, an “orphan” disease
If yes, which Group?
Can Pulmonary HTN be cured?
Are there effective treatments?
Yes, Many patients with left sided heart failure have Group II Pulmonary HTN.
Yes, Group I PAH Pulmonary HTN remains an uncommon disease.
Chronic PE patients who have a successful surgery return to near normal: OSA patients on CPAP.
Yes, particularly for Group I PAH patients now.
PH with Left HeartDisease
PH with LungDisease and/or
Low Oxygen Levels
“PAH”
MiscellaneousMiscellaneousChronic Thrombosis
(clot) PHChronic Thrombosis
(clot) PH
Pulmonary HypertensionComes in Several Varieties
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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 heartdisease, 67.9%
Right Heart Catheterization: The Definitive Diagnosis:
• Normal Pulmonary Artery (PA) Pressure: 30/15, mean 20 mmHg.
• Pulmonary Hypertension (PH) when mean > 25 mmHg (40/20 mmHg )and Elevated Pulmonary Vascular Resistance (PVR)
Pulmonary Hemodynamics:Pre‐capillary and Post‐capillary Patterns
Pulmonary ArterialPre-Capillary
Pulmonary VenousPost-CapillaryLeft Heart Disease
5th World Symposium on PH:Classification /GROUPS1. Pulmonary arterial hypertension
Right Heart Catheterization done. RA pressure 4, PA pressure mean 26, PAWP 9 (all mmHg). CI 2.8 L/min m2.
Mild PAH , Idiopathic with preserved RV function.
Recommended to complete surgery and then return promptly for treatment. LOW RISK Status to start.
Surgery Successful with no complications.
Lost to fu for period of time.
PH Case -DS
60 year old manager
DOE for months: Stairs at work and Lawn work now more difficult. WHO functional class?
PMH: Viral CM; ? HTN on ARB
FH: Estranged sister with lung condition; on pump medication, oxygen and now pills
SH: Manager, Layoffs coming, Insurance?
Exam: JVP 4 cm, Loud P2, TR Murmur, RV impulse,noedema.
Walk test: 440 m
VQ scan: Normal
ECG: NSR, R axis, RVH
Case DS
What Diagnoses need to be considered?
What additional tests do you want?
Outside Echo suggests pulmonary hypertension with RV dysfunction.
Case DS
PH Echocardiogram on 03/06/07: Left ventricular EF 40-45%. Mild diastolic dysfunction
was identified. Left atrium appears visually normal (adjusted for BSA 17ml/M2).
Right ventricle is severely enlarged and has severe systolic dysfunction.
Estimated PASP: at least 65 mm Hg. IVC compatible with RAP of 10 mm HG. Agitated saline contrast at rest with Valsalva reveals no shunt.
No pericardial effusion is seen.
Case DS Right heart catheterization on 04/16/07 at FMLH:
RA Press 7; RV Press 93/13; PA Press 94/40/mean 62; PWP Wedge Pressure 12, LV EDP: 10 Cardiac Output CO/ Index Cl: 3.9 lpm/1.95 lpm/m2;
HR:65/min ; PVR: 12.2 WU; Mixed Venous Sat% 62%.
*Vasodilator trial with Nitric Oxide (10 ppm) revealed: No significant change.
Severe Precapillary Pulmonary HTN. Normal LV filling pressures.
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Case DS
What Diagnoses to be considered?
Group I PAH. Heritable PAH?, IdiopathicPAH
Are there any barriers to treatment? Insurance.
Lesson 2
WHO Group I PAH Is Rarebut Deadly—
Make the Diagnosis Early
Group I PAH Distributions in the US: REVEAL Registry
Based on Venice Clinical Classification (2003); 2967 patients.Adapted from Badesch DB et al. Chest. 2010;137:376‐387.
Overall Associated
Associated(50.7%)
Idiopathic(46.2%) Connective tissue/
collagen vascular(49.9%)
Heritable (2.7%)
Pulmonaryveno‐occlusive
(0.4%)
Congenitalheart disease(19.5%)
HIV (4.0%)
Other (5.5%)
Drugs/toxins (10.5%)
Portopulmonary (10.6%)
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. Benza RL et al, Chest 2012; 142: 448‐456.
Simonneau G et al. J Am Coll Cardiol. 2013;62:D34-D41.
Incidence of CTEPH
• Approximately 3% to 4% 1-2 yr after acute PE (large, Recurrent higher risk)
• USA: 600,000 cases of acute PE each year
• Only 40% to 50% of CTEPH patients have a history of previous episodes of acute PE
• VQ scan identifies old PE better than CTA
McLaughlin VV et al. J Am Coll Cardiol. 2009;53:1573-1619.Pengo V et al. N Engl J Med. 2004;350:2257-2264.Tapson VF, Humbert M. Proc Am Thorac Soc. 2006;3:564-567.
Years
Cu
mul
ativ
e in
cide
nce
of C
TE
PH
0 1 2 3 4 7 8 9 10 115 60.00
0.01
0.02
0.03
0.04
Pulmonary Thromboendarterectomy*
*This surgery now offered at Froedtert and MCW
PH Treatment Goals
Fewer/less severe symptomsImproved exercise capacityImproved “hemodynamics” Prevention of clinical worsening (heart failure, admissions, increased SOB)
Improved quality of life (benefits versus side effects)
Improved survival ? Achieve “Low Risk” Status
Chronic Adjuvant Therapies in PAH
Oxygen
• Use to prevent hypoxic vasoconstriction
• Consider exercise, sleep, altitude
• Aim for target saturation >90%
• May not correct hypoxia with shunt
Adapted from: Badesch DB et al. Chest. 2004;126:35S-62S. Badesch DB et al. Chest. 2007;131:1917-1928.McLaughlin VV et al. J Am Coll Cardiol. 2009;53:1573-1619.
Chronic Adjuvant Therapies in PAH
Diuretics
• Needed by most patients; hypotension not a contraindication in RV failure ( may need BP support)
Anticoagulation
• Recommended in IPAH
• Observational studies only (2 retrospective, 2 prospective); need to balance unproven benefits with known risks
• INR 1.5 – 2.5
Adapted from: Olsson KM et al. Circulation. 2014; 29:57–65. Fuster V et al. Circulation. 1984;70:580-587. Badesch DB et al. Chest. 2004;126:35S-62S. Badesch DB et al. Chest. 2007;131:1917-1928. McLaughlin VV et al. J Am Coll Cardiol. 2009;53:1573-1619.
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Selection of Appropriate Therapy:
Group I PAH Patients:Chronology1980s: Calcium Channel Blockers, Diuretics, Oxygen. 1996: IV Epoprostenol (Flolan) ‐ “PPH” only; then 1998 All Group I PAH. 2001: Bosentan (Endothelin Receptor Antag.), first oral drug. Group I PAH2005: Sildenafil (PDE5 Inhibitors), Group I PAH. 2013: Riociguat (sGuanylate Cyclase, GMP) Group I PAH, Group IV
2019: 12 Drugs: Group I PAH, ‐‐‐‐‐‐‐‐‐‐‐‐‐‐‐‐‐‐‐‐‐‐‐‐‐‐‐‐‐‐‐‐‐‐‐‐‐‐‐‐‐‐‐‐‐‐‐‐‐‐‐‐‐‐‐‐‐‐‐‐‐‐‐‐‐‐‐‐‐‐‐‐‐‐‐‐‐‐‐‐‐‐‐‐‐‐‐‐‐‐‐‐Group IV CTEPH. 1 Drug. Riociguat.
(No Approved Drugs: Group II PH ‐ Heart Disease; Group III PH ‐ Lung Disease)
Evolution From Exercise Capacity to Morbidity and Mortality Randomized Controlled Trials
*Estimated mean study drug exposure. †Estimated median study drug exposure. ‡Estimated target enrollment.PAH=pulmonary arterial hypertension; RCT=randomized controlled trial. Channick RN et al. Lancet. 2001;358:1119-1123. Rubin LJ et al. N Engl J Med. 2002;346:896-903. Galiè N et al. Lancet. 2008;371:2093-2100. Galiè N et al. Circulation. 2008;117:3010-3019. Galiè N et al. N Engl J Med. 2005;353:2148-2157. Simonneau G et al. Am J Respir Crit Care Med. 2002;165:800-804. McLaughlin VV et al. Am J Respir Crit Care Med. 2006;174:1257-1263. Galiè N et al. Circulation. 2009;119:2894-2903. Simonneau G et al. Ann Intern Med. 2008;149:521-530. Olschewski H et al.N Engl J Med. 2002;347:322-329. Pulido T et al. N Engl J Med. 2013;369:809-818. Sitbon O et al. N Engl J Med. 2015;373:2522-33. Galiè N et al. N Engl J Med. 2015;373:834-44.McLaughlin VV et al. Eur Respir J. 2015;46:405–413
Weeks0 20 40 60 80 100 120 140 160
N=32N=213
N=185N=202N=192N=277N=470N=67
N=405N=267
N=203AIRPACES
PHIRSTSTEP
Simonneau et al (2002)SUPER-1ARIES-2ARIES-1
EARLYBREATHE-1
Study 351
6MWD Trials
n=235TRIUMPHCHEST n=443
FREEDOM-EV n=858
N=742*N=1150*N=545‡†AMBITION
GRIPHONSERAPHIN
COMPASS-2
Morbidity and Mortality Trials
n=344
Mechanisms of Action of Approved Therapies for GROUP I PAH (not other groups)
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
Vasodilationand antiproliferation
Phosphodiesterase type 5
Vasodilationand 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
PAH Determinants of Risk
McLaughlin VV et al. J Am Coll Cardiol. 2009;53:1573-1619. RAP = Right Atrial Press. CI = Cardiac Index
Galiè N et al. J Am Coll Cardiol. 2013;62:D60-D72.AMBITION study, N Engl J Med, 2015*
Sequential, Initial* CombinationTherapy (I-A)
Referral for Lung Transplantation (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 Atrial Septostomy (IIa-C)
Inadequate Clinical Response
ERS Risk Assessment 2015
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Case DS
What Diagnoses to be considered?
Group I PAH. Heritable PAH?, IdiopathicPAH
Is this patient HIGH risk or LOW risk at present?
“ A tweener ”
High because: Low normal Cardiac Ouput. High BNP
Low because: normal RA pressure, Functional class II symptoms, no heart failure.
“INTERMEDIATE RISK” Are there any barriers to treatment? Insurance.
PH Case DS Treated with Sildenafil alone with improvement;
Escalated dose to 80 mg TID
Walk Test 660 m, no desat
WHO functional Class I most days. II on some chore days.
BNP 12
Echo: Severe RV enlargement; Low normal TAPSE. Right Heart Cath 11/08 on Revatio 80 TID. RA 7, PA 91/33 mean 55, PWP 8, CO5.4 CI 2.7 PVR
8.6, MVO2 sat 71, AVO2 diff 5.0 New Medication to discuss?
cGMP
cAMP
Vasoconstriction and proliferation
Endothelinreceptor A
Endothelin-receptor
antagonists
Endothelinreceptor B
Phosphodiesterase type 5 inhibitor
Vasodilationand antiproliferation
Phosphodiesterase type 5
Vasodilationand 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
Approved Therapeutic Targets
Humbert M et al. N Engl J Med. 2004;351:1425-1436.
Exogenousnitric oxide
sGC stimulator
Evolution From Exercise Capacity to Morbidity and Mortality Randomized Controlled Trials
*Estimated mean study drug exposure. †Estimated median study drug exposure. ‡Estimated target enrollment.PAH=pulmonary arterial hypertension; RCT=randomized controlled trial. Channick RN et al. Lancet. 2001;358:1119-1123. Rubin LJ et al. N Engl J Med. 2002;346:896-903. Galiè N et al. Lancet. 2008;371:2093-2100. Galiè N et al. Circulation. 2008;117:3010-3019. Galiè N et al. N Engl J Med. 2005;353:2148-2157. Simonneau G et al. Am J Respir Crit Care Med. 2002;165:800-804. McLaughlin VV et al. Am J Respir Crit Care Med. 2006;174:1257-1263. Galiè N et al. Circulation. 2009;119:2894-2903. Simonneau G et al. Ann Intern Med. 2008;149:521-530. Olschewski H et al.N Engl J Med. 2002;347:322-329. Pulido T et al. N Engl J Med. 2013;369:809-818. Sitbon O et al. N Engl J Med. 2015;373:2522-33. Galiè N et al. N Engl J Med. 2015;373:834-44.McLaughlin VV et al. Eur Respir J. 2015;46:405–413
• Contraindicated in pregnancy, with use of nitrates or NO donors in any form, or with use of PDE inhibitors
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Mechanisms of Action of Approved Therapies for GROUP I PAH (not other groups)
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
Vasodilationand antiproliferation
Phosphodiesterase type 5
Vasodilationand 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
*PHA Scientific Leadership Council recommends LFT testing at onset of all treatments for PAH and periodically thereafter, at prescriber’s discretion.
Endothelin Receptor Antagonists: (Bosentan, Ambrisentan, Macitentan) 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
– Avoid pregnancy.
Weeks0 1921444824 72 96 168120
Hazard ratio, 0.50 (95% Cl, 0.35-0.72)p<0.001
0
60
100
80
40
20
Combination therapy
Pooled monotherapy
AMBITION: Effect of Ambrisentan Plus Tadalafil Versus Monotherapy on Clinical Worsening*
* Death, hospitalization for worsening PAH, disease progression, unsatisfactory long-term clinical response. Galiè N et al. N Engl J Med. 2015;373:834-44.
Pa
rtic
ipa
nts
wit
h N
o E
ven
t (
%)
No. at risk:Combination therapyPooled monotherapy
229
209
186
155
145
108
106
77
71
49
36
25
4
5
253
247
SERAPHIN: Effect of Macitentan on Disease Progression
* Worsening of PAH, initiation of treatment with IV or SC prostanoids, lung transplantation or atrial septostomyPulido T et al. N Engl J Med. 2013;369:809-818.
0
40
80
100
60
20
Months
Pa
tien
ts w
itho
ut
an
eve
nt
rela
ted
to P
AH
or
de
ath
fro
m
fro
m a
ny
cau
se*
(%)
0 6 12 363018 24
No. at risk:PlaceboMacitentan 3 mgMacitentan 10 mg
250250242
188213208
160188187
233241
648091
135166171
122147155
Macitentan 10 mg qdMacitentan 3 mg qdPlacebo
64% on background therapy:- 62% PDE5I- 5% Prostanoid
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
Vasodilationand antiproliferation
Phosphodiesterase type 5
Vasodilationand 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
Iloprost (Ventavis®)Treprostinil (Tyvaso®)
Prostacyclin Analogues: Intravenous, 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.Iloprost Inhaled: FC III‐IV, 2.5‐5 mcg, 6‐9 inh/d.
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Oral Prostacylin Therapy: Time to First Morbidity or Mortality Event—GRIPHON
Delivery site complications (pain, infection, cough, thrombosis,)
Vary according to drug and route of delivery (Po, Inhaled, SQ, IV)
PH Case DS Treated with Sildenafil alone with improvement; Escalated dose
to 80 mg TID
Walk Test 660 m, no desat
WHO functional Class I most days.
BNP 12
Echo:Severe RV enlargement; Low normal TAPSE. Right Heart Cath 11/08 on Revatio 80 TID. RA 7, PA 91/33 mean 55, PWP 8, CO5.4 CI 2.7 PVR
8.6, MVO2 sat 71, AVO2 diff 5.0 New Medication to discuss? Macitentan added to Sildenafil in 2016. Doing Great 12 years later - 2019.
Case TN (3) Returned : Primary Care MD
Sildenafil TID monotherapy for PAH in 2014.
Erratic FU but compensated initially on therapy and better exercise tolerance. ETOH use.
Returned one year later 2015: Increased edema, DOE, consistent with early RV failure.
Unable to get in for repeat Right Heart Catheterization.
Diuretics added to Sildenafil.
Echo shows worsening RV size and Worsening function. Estimated PASPIs > 60 mmHg. Estimated RA pressure is higher
Candidate for additional combinationTherapy? Compliance, Cost? ‐Oral Selexipag added 2016
‐NOT candidate for infusion therapy: Social Barriers
‐ Palliative care: 2018.
Summary PH should be in the differential diagnoses for the dyspneic patient.
Usual diagnostic studies can determine who has a higher likelihood of PH and help to determine the possible cause and Group.
Therapy follows according to the Group.
Group I PAH‐specific therapies promote vasodilation and remodeling, leading to improved RV function and exercise.
Selection of initial therapy largely depends upon severity of disease at diagnosis low‐risk patients can be treated with oral agents Initial or early combination therapy of benefit high‐risk patients require parenteral prostacyclins
Summary—Cont’dLongitudinal assessment of PAH patients includes monitoring of:clinical parameters functional parametershemodynamic parameters laboratory parameters imaging parameters
Current strategy is to achieve “Low Risk” Status in all patients. Directed combination therapy and potent therapies should be used in a timely fashion to achieve this status.
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Mary Furbee: Spiral. (Used with permission)
Pulmonary Hypertension
Correct Diagnosis, Best Plan, Partner before you jump in……