teresa de marco, md professor of clinical medicine director, heart failure and pulmonary...

TERESA DE MARCO, MDTERESA DE MARCO, MD

Professor of Clinical MedicineDirector, Heart Failure and Pulmonary Hypertension Program

Medical Director, Heart TransplantationUniversity of California, San Francisco Medical Center

San Francisco, California

Screening, Referral and Diagnosis of Pulmonary Arterial Hypertension

2

Learning Objectives (CME, CE, CPE)

● At the completion of this educational activity, participants should be able to:

- Identify patients who are at higher risk for PAH

- Identify the common presenting symptoms for PAH

- Discuss the diagnostic workup for symptoms suggestive of PAH, and the appropriate use of various tests

- Identify when a patient with suspected PAH should be referred to a PAH-specific specialty center

Clinical Classification ofPulmonary Arterial Hypertension (PAH)

4

PAH:Definition on Right Heart Catheterization

Gaine SP, et al. Lancet. 1998;352:719-725.

Increased mean pulmonary arterial pressure (mPAP)

>25 mm Hg at restor

>30 mm Hg during exercise

Normal pulmonary artery wedge pressure (PAWP)

<15 mm Hg

Increased pulmonary vascular resistance (PVR)

>3 Wood units

5

Revised Clinical Classification of Pulmonary Hypertension: 2003 Venice

● PAH

- Idiopathic (IPAH)

- Familial (FPAH)

- Associated with (APAH)

• Connective tissue disease

• Congenital systemic-to-pulmonary shunts

• Portal hypertension

• HIV infection

• Drugs and toxins

- Other

• Thyroid disorders, glycogen storage disease, Gaucher disease, hereditary hemorrhagic telangiectasia, hemoglobinopathies, myeloproliferative disorders, splenectomy

- Associated with significant venous or capillary involvement

• Pulmonary veno-occlusive disease (PVOD)

• Pulmonary capillary hemangiomatosis (PCH)

- Persistent pulmonary hypertension of the newborn (PPHN)

Simonneau G, et al. J Am Coll Cardiol. 2004;43:5S-12S.

6


● Pulmonary hypertension with left heart disease

- Left-sided atrial or ventricular heart disease

- Left-sided valvular heart disease

● Pulmonary hypertension associated with lung diseases and/or hypoxemia

- Chronic obstructive pulmonary disease

- Interstitial lung disease

- Sleep-disordered breathing

- Alveolar hypoventilation disorders

- Chronic exposure to high altitude

- Developmental abnormalities


7


● Pulmonary hypertension due to chronic thrombotic and/or embolic disease

- Thromboembolic obstruction of proximal pulmonary arteries

- Thromboembolic obstruction of distal pulmonary arteries

- Non-thrombotic pulmonary embolism

• Tumor, parasites, foreign material

● Miscellaneous

- Sarcoidosis, histiocytosis X, lymphangiomatosis, compression of pulmonary vessels (adenopathy, tumor, fibrosing mediastinitis)


8

Pulmonary Hypertension Connection Registry: Etiology of PAH

Connective Tissue, 30.0%

Idiopathic/familial, 48.0%

Congenital Heart, 11.0%

Portal Hypertension,

7.0%

Anorexigens, 3.0%

HIV, 1.0%

Thenappan T. Eur Respir J. 2007;30:1103-1110.

n=578; female-male ratio: 77% - 33%Calcium channel blocker use at referral: 80%

9

REVEAL Database: Most Frequent Symptoms at Diagnosis

Elliott CG, et al. Chest. 2007;132(4 suppl):631S.

Dyspnea at rest

Cough

Dizzy/lightheaded

Presyncope/syncope

Edema

Chest pain/discomfort

Other

Fatigue

Dyspnea on exertion84.0%

26.0%

24.0%

23%

21.0%

23.0%

16.0%

13.0%

11%

83%

29%

27%

20%

20%

20%

14%

13%

11%

0 25 50 75 100 Incidence (%)

IPAHAPAH

n=1479.

10

Survival in PAH by Etiology

IPAH, idiopathic pulmonary arterial hypertension.McLaughlin VV, et al. Chest. 2004;126:78S-92S.

0 1 2 3 4 5

Years

1.0

0.9

0.8

0.7

0.6

0.5

0.4

0.3

0.2

0.1

0

Per

cen

t S

urv

ival

CongenitalHeart Disease

Portopulmonary

IPAH

Connective Tissue Disease

HIV

Screening for PAH in At-Risk Populations

12

Screening for PAH RequiresHigh Index of Suspicion for Clinician

● Diagnosis is complex

- IPAH and FPAH remain diagnoses of exclusion

● Early symptoms likely to be attributed to variety of more-common conditions

● Echocardiography is most commonly used screening tool

● Right heart cardiac catheterization required for diagnosis

- Best limited to centers of excellence

McGoon M, et al. Chest. 2004;126:14S-34S.

13

Alternative Diagnoses of Patients Referred to PAH Specialty Clinic

Moghbelli MH, et al. Am J Respir Crit Care Med. 2008;177:A923.

ILD

VTE

Other

Structural Heart Disease

OSA

LV Dysfunction

Obstructive Lung Disease

All Alternative Diagnoses 85.0%

24.0%

22.0%

19%

13.0%

12.0%

5.0%

5.0%

n=268, all patients referred to PAH specialty center.

14

FPAH Genetic Screening

and Counseling● BMPR mutations found in 70% of FPAH patients

● Penetrance is low: Only 20% of BMPR germline mutation carriers will develop PAH

● “Second hit” theory implicates other pathways, including serotonin transport

● At present, no guidelines exist regarding routine genetic testing and counseling for patients with FPAH

Humbert M. Am J Respir Crit Care Med. 2008;177:574-579.

15

Screening for PAH Associated WithConnective Tissue Disorders

● Doppler echo recommended for patients at high risk of PAH

● DLCO recommended every 6–12 months to improve detection of pulmonary vascular or interstitial disease

Coghlan JG, et al. Lupus. 2006;15:138-142.

16

PAH and HIV Infection

● Sex-standardized incidence is between 68 to 138 cases per 100,000 HIV seropositive patients PY (versus 0.16 cases per 100,000 PY in the general population)

- Females have 1.7x risk of PAH than males

- Higher prevalence among injection drug users

● AIDS diagnosis and low CD4 cell counts associated with higher incidence of IPAH

- No apparent impact of antiretroviral therapy on IPAH occurrence

● Approximately 2/3 of patients with HIV who develop PAH succumb to complications of PAH

Mary-Krause M. J Intern AIDS Soc. 2008;11(suppl 1):Abstract P299.Limsukon A, et al. Mt Sinai J Med. 2006;73:1037-1044.

Diagnosis of PAH

18

PAH Diagnostic Guidelines:Decision Analysis


Unexplained Symptoms of Dyspnea onExertion, Syncope/Near Syncope, Fatigue

Clinical History, Examination,Chest X-Ray, ECG

19

Physical Findings Consistent With PAH

● Accentuated pulmonary component of second heart sound (P2) at apex

- Noted in 90% of patients with IPAH

● Early systolic ejection click

● Midsystolic ejection murmur

● Left parasternal lift

● Right ventricle S4 gallop

● Prominent jugular “a” wave


20

Physical Findings Consistent With PAH

● Diastolic murmur of pulmonary regurgitation

● Holosystolic murmur of tricuspid valve regurgitation

● Signs of right ventricular failure

- Right-sided third heart sound

- Jugular venous distention

- Peripheral edema, ascites

● Cool extremities

- Indicative of reduced cardiac output and peripheral vasoconstriction


21

Other Physical Findings:Differential Diagnosis/PAH Etiology


Finding Differential Diagnosis/PAH Etiology

Cyanosis Right-to-left shunt

Clubbing Rare in IPAHCongenital heart or pulmonary veno-occlusive disease

Rales, fine rales, abnormal breath sounds

Pulmonary congestion, parenchymal airway disease, PVOD, PCH, etc.

Obesity, kyphoscoliosis, enlarged tonsils

Hypoventilatory disorders

Sclerodermal skin changes, rashes, nail-fold capillary abnormalities

Associated connective tissue disorder

Peripheral venous insufficiency

Venous thrombosis, pulmonary thromboembolic disease

22

Screening Tests for PAH: Electrocardiogram

● Insufficiently sensitive as a screening tool

● May indicate right-heart disease

● May provide prognostic information


23

Electrocardiogram Associated With Right Ventricular Hypertrophy (RVH)

Image courtesy of Vallerie McLaughlin, MD

24

Electrocardiogram Associated With Right Bundle Branch Block Plus RVH


25

Screening Tests for PAH:Chest X-Ray Findings Consistent With PAH

● Enlarged main and hilar pulmonary artery shadows

● “Pruning” of peripheral pulmonary vasculature

● Right ventricular enlargement

● Symptomatic patients may have normal chest x-ray

● Chest x-ray may reveal underlying causes of PH


26

Chest X-Ray Consistent With PH


27

MRI of Pulmonary Artery Distensibility:Preliminary Findings

0

10

20

30

40

mP

AD

(%

)

Nonresponders Responders

Potential Noninvasive Surrogate Markerof Acute Vasodilator Challenge

N = 19. P=0.01. 10% pulmonary artery distensibility predicted response to acute vasodilator challenge with 100% sensitivity and 56% specificity.

Jardim C, et al. Eur Respir J. 2007;29:476-481.

28



Clinical History, Examination,Chest X-Ray, ECG

Is There a Reason to Suspect PH?

Yes No

Echocardiography Work-Upfor Other

Conditions

29



Echocardiography for Suspected PH

RH Analysis CHD Analysis LH Analysis

TRV to EstimateRVSP, RVE, RAE, RV

Dysfunction

Abnormal Morphology, Shunt

LV SystolicDiastolic DysfunctionValvular Dysfunction

30

Echocardiograph: Parasternal Long Axis


31

Echocardiograph: Parasternal Short Axis


32

Echocardiograph: Apical Four Chamber


33

Echocardiograph: Tricuspid Regurgitation

Modified Bernoulli’s Equation:4 x (V)² + RAP = RVSP (PASP)

V=tricuspid jet velocity (m/s); RAP= right atrial pressure; RVSP=right ventricular systolic pressure; PASP=pulmonary artery systolic pressure.


34

Calculations of Estimated PulmonaryArtery Pressures (PAP) by Doppler Echo

Measurement Calculation

sPAP 4 x TR peak velocity2 + “RAP”

mPAP 79 – 0.45 (RVOT AT)

mPAP 4 x peak pulmonary regurgitation velocity2

Pulmonary end diastolic pressure

4 x (pulmonary regurgitation end-diastolic velocity)2 + “RAP”

Bossone E, et al. Chest. 2005;127:1836-1843.

TR=tricuspid regurgitant jet velocity m/sec.“RAP”=estimated right atrial pressure.RVOT AT=right ventricular outflow tract acceleration time.

35

Limitations of Echocardiographyin Diagnosing PH

● 15% of patients will not display TR jets

- Saline contrast can enhance TR jet

● Not all congenital heart lesions will be obvious

● Poor method to measure LH filling pressure or cardiac output (CO)

● Small errors in TRV tracing can significantly alter results

● TRV can underestimate RVSP or overestimate RVSP

Stephen B, et al. Chest. 1999;116:73-77.

36

Accuracy of PH Diagnosis by Echocardiography in Advanced Lung Disease

● Cohort study of lung transplant patients (n=374)

● All patients

- Doppler echo 24 to 48 hours prior to RHC

● Prevalence of PH: 25%

● Echo frequently inaccurate leading to over diagnosis of pulmonary hypertension in patients with advanced lung disease

Arcasoy SM, et al. Am J Respir Crit Care Med. 2003;167:735-740.

0

10

20

30

40

50

60

70

Diagnosis of PH

Stu

die

s (%

)

OverestimationAccurateUnderestimation

NoPulmonary

Hypertension

PulmonaryHypertension

37

Doppler Echo Overestimates PAH in PatientsWith Scleroderma-Related Lung Disease

60

0

4038

25 25

0

20

40

60

80

100

Overestimate PASP Underestimate PASP Accurate PASP

No PH PH

Per

cen

t (%

)

N = 13.

Chan KM. CHEST 2008; October 25-30, 2008, Philadelphia, PA. Session AP2217.

38

PAH Diagnostic Guidelines:Further Evaluation of Patients


Echocardiography Indicates PH

Evaluate for Associated Causes

V/Q scan PFTsArterial Saturation

HIV Infection, Scleroderma, SLE, Other CTD, Liver Disease, CHD, Drug-

Associated

Parenchymal Lung Disease, Hypoxemia,

or Sleep Disorder

SuspectedChronic Pulmonary

Emboli

39

Association Between Stimulant Use and IPAH

Chin KM, et al. Chest. 2006;130:1657-1663.

0

10

20

30

40

Pat

ien

ts (

%)

Idiopathic

Patients Reporting Use (n=340)

28.9%

3.8% 4.3%

PAH with KnownRisk Factors

Thromboembolic PH

Retrospective analysis at single PH center of adults with PH.

40

V/Q Scan for Chronic Thromboembolic Pulmonary Hypertension (CTEPH)

● Normal V/Q scan makes CTEPH unlikely

- Sensitivity: 90% to 100%

- Specificity: 94% to 100%

● >1 segmental-sized or larger mismatched perfusion defects seen with CTEPH

● Spiral CT may underestimate degree of obstruction in chronic CTEPH

- ~7% false negative


41

Chronic Thromboembolic Pulmonary Hypertension (CTEPH) Diagnosis

● Pulmonary angiography remains gold standard

● Signs of CTEPH

- Stenoses, complete obstructions, partial recanalization, and intraluminal webs

● Pulmonary fiberoptic angioscopy can help define operability in selected patients

Klepetko W, et al. J Am Coll Cardiol. 2004;43:73S-80S.

42

Pulmonary Function Testing forPAH Suspected by Doppler Echo

● Lung volumes 60% to 80% of predicted

● Nocturnal hypoxemia occurs in >75% of patients with IPAH

● Desaturation may increase during exercise

● DLCO <55% of predicted associated with future development of PAH in limited systemic sclerosis

McGoon M, et al. Chest. 2004;126:14S-34S. Barst RJ, et al. J Am Coll Cardiol. 2004;43:40S-47S.

43

PH in Patients WithObstructive Sleep Apnea

● Tends to be milder than PH from other causes

● Prevalence range: 17% to 53%

● Spirometric abnormalities strongly associated with PH

● PH is strongly associated with other risk factors

- Left-sided heart disease

- Parenchymal lung disease

- Nocturnal desaturation

- Obesity

Atwood CW Jr, et al. Chest. 2004;126:72S-77S.

44

PAH Diagnostic Guidelines:Confirmation of PAH

Adapted from McGoon M, et al. Chest. 2004;126:14S-34S.

Echocardiography Indicates PH

Refer to PAH Specialty Center forRight Heart Catheterization

45

Right Heart Catheterization

● Required to confirm diagnosis, calculate resistance, and guide therapy for PAH

● Excludes other etiologies for PH

- Intracardiac or extracardiac shunts

- Left-heart disease

● Measures degree of right-heart dysfunction

- Right atrial pressure

- Cardiac output


46

Pulmonary Artery Wedge Pressure

● Measurement is critical in PAH diagnosis

- PAH therapies increase cardiac output, therefore risk pulmonary edema and hypoxemia in patients with left ventricular diastolic dysfunction

● Interobserver variability in interpreting a pulmonary artery pressure waveform is extremely large

● Physicians consistently fail to make the determination of pulmonary wedge pressure only at end-expiration

● Direct measurement of left ventricular end-diastolic pressure may be necessary

Ghofrani HA, et al. Circulation. 2008;118:1195-1201.

47

MixedMixedPHPH

Pre-capillary PHPre-capillary PHHigh-Flow PHHigh-Flow PH(O(O2 2 sat run)sat run)

Hemodynamic Classification of PH(mean PAP >25 mmHg)

VCVC RARA RVRV PAPA PVPVPCPC

LALA LVLV AoAo

Post-Capillary PH Post-Capillary PH

48


VCVC RARA RVRV PAPA PVPV PVPPVPPCPC

LALALAPLAP

LVLV AoAoLVEDPLVEDP

Post-Capillary PH Post-Capillary PH PAWP>15 mmHg; PVR nlPAWP>15 mmHg; PVR nl

49


LALALAPLAP

LVLV AoAoLVEDPLVEDP

Systemic HTNAoV Disease

Myocardial DiseaseMyocardial DiseaseDilated CMP-ischemic/non-ischemicDilated CMP-ischemic/non-ischemicHypertrophic CMPHypertrophic CMPRestrictive/infiltrative CMPRestrictive/infiltrative CMPPericardial diseasePericardial disease

MR



50


LALALAPLAP

LVLV AoAo


Atrial MyxomaCor Triatriatum

MV DiseaseMV Disease


51

PV compression



LALA LVLV AoAo


52


VCVC RARA RVRV PAPA PVPVPCPC

LALA LVLV AoAo

Pre-capillary PHPre-capillary PHPAWP PAWP << 15 mmHg; 15 mmHg;

PVR > 3 WuPVR > 3 Wu

{

{

PAHPAHLung Diseases +/- HypoxemiaLung Diseases +/- Hypoxemia

CTEPHCTEPH

53


LALALAPLAP

LVLV AoAoLVEDPLVEDP

Mixed PHMixed PH(“Reversible” vs. “Fixed”)(“Reversible” vs. “Fixed”)

PAWP >15 mmHgPAWP >15 mmHgPVR > 3 WuPVR > 3 Wu


54

Measuring Pulmonary Artery Wedge Pressure

160

140

120

100

80

60

40

20

012

Time (seconds)

Pre

ssu

re (

mm

Hg

)

BalloonOcclusion

0 2 4 6 8 10

ARDSIPAH

Time Steady State Is Longer in IPAH Than In ARDS

Pulmonary Artery Pressure Decay Curve

ARDS: acute respiratory distress syndrome.

Souza R, et al. Crit Care. 2005;9:R132-R138.

55

Correct and IncorrectReadings of PAWP

PA and RV Recordingsin Patient With PAH

PA Pressure Tracing Erroneously Labeled As PAWP

Oudiz RJ, et al. Advances Pulm Hypertens. 2005;4:15-25.

56

Misclassification of PAH and PVH Through Use of PAWP Versus LVEDP

Halpern SD, et al. Am J Respir Crit Care Med. 2008;177:A259.

37.4

50.2

0

10

20

30

40

50

60

70

80

90

100

n=4,666, all patients undergoing LHC and RHC over 10 years at single center.

Per

cen

t (

%)

Misclassification ofPAH by PAWP

Misclassification ofPVH by PAWP

57

PAH and the Right Ventricle

Neurohormonaland other

mediator activation

RV remodeling

Pulmonary hypertension

Pressure overload

Adaptive RV hypertrophyDecreased wall stress

Maladaptive RV hypertrophy &

fibrosisDiastolic dysfunction

RV dilation & systolic failure

RV ischemia: Wall stress & heart rate

Coronary perfusion gradientTricuspid regurgitation

Preload-afterload mismatchDecreased LV compliance/preload:

Inter-ventricular septal shift Intrapericardial pressure

]

]

Compensated PhaseNormal CO, normal RAP

Decompensating PhaseHigher RAP to maintain

adequate CO

Decompensated Phase CO, RAP

AV-DO2

Hypoxemia, acidosis, life-threatening dysrhythmias

DeMarco T, et al. Adv Pulm Hypertens. 2005;4:16-26.

58

Measuring Diastolic Dysfunction

Bursi F, et al. JAMA. 2006;296:2209-2216.

E: early peak mitral inflow velocity.A: late peak mitral inflow velocity.DT: deceleration time of the E-wave. e’: velocity of annulus early diastolic motion.

Mitral InflowMitral Inflow

NormalNormalDiastolicDiastolicFunctionFunction

MildMildDiastolicDiastolic

DysfunctionDysfunction

ModerateModerateDiastolicDiastolic


SevereSevereDiastolicDiastolic


Ve

loc

ity

(m

/s)

Ve

loc

ity

(m

/s)

DT>140 msDT>140 ms0.75<E/A<20.75<E/A<2

2.02.0

00

EE

AA

00

1.51.5

Ve

loc

ity

(m

/s)

Ve

loc

ity

(m

/s)

Doppler TissueDoppler TissueImaging of MitralImaging of MitralAnnular MotionAnnular Motion

E/eE/e’’<10<10

aa11

E/eE/e’’≥10≥10E/eE/e’’<10<10

ee11

DT>140 msDT>140 ms0.75<E/A<20.75<E/A<2

DT<140 msDT<140 msE/A>2E/A>2

E/A≤0.75E/A≤0.75

E/eE/e’’≥10≥10

59

PAH Diagnostic Workup


Right Heart Catheterization Confirms PAH

6-Minute WalkBorg Score

FunctionalClass

Establish Baseline, Prognosis, and DocumentProgression/Response to Treatment With Serial Re-Assessment

60

Blood Tests for Evaluation of PAH

● Antinuclear antibody (ANA)

- Up to 40% of patients with IPAH have positive but low (>1:80 dilutions) ANA titers

● Antiphospholipid antibodies

- Lupus anticoagulant, anticardiolipin antibodies

● HIV serology

● CBC with platelets

● Liver function

● Thyroid function

● Hemoglobin electrophoresis, if indicated

Barst RJ, et al. J Am Coll Cardiol. 2004;43:40S-47S.

61

NT-proBNP Elevations Correlate WithRight Ventricular Dysfunction in PH

Blyth KG, et al. Eur Respir J. 2007;29:737-744.

0

1000

2000

3000

4000

5000

NT

-pro

BN

P (

ng

/L)

With RVSD

4127

354

Without RVSD

N = 25. Threshold value RVD detection: 1,685 ng/L.Sensitivity for RVD 100%; specificity 94%.

62

BNP Predictive Value For Adverse Outcomes

Death

Cardiogenic shock

Inpatient heart failure

Outpatient heart failure

Ventricular dysfunction

WHO Class IV

WHO Class III

WHO Class II

WHO Class I

Control 12.1

20.2

151.7

388.4

470.0

424.7

472.7

545.6

632.1

644.8

BNP (pg/mL)

N=85.

Garcia-Badillo EV. CHEST 2008; October 25-30, 2008, Philadelphia, PA. Session AP2217.

63

6-Minute Walk Distance CorrelatesWith IPAH Disease Severity

Miyamoto S, et al. Am J Respir Crit Care Med. 2000;161:487-492.

0

100

200

300

400

500

600

700

800

Dis

tan

ce W

alke

d in

6 M

inu

tes

(m)

Control NYHA II

*P<0.05 versus control.†P<0.05 versus NYHA Class II.‡P<0.05 vs NYHA Class III.

NYHA III NYHA IV

**†

*†‡

64

Impact of Baseline6-Minute Walk Distance on Survival

Barst RJ, et al. N Engl J Med. 1996;334:296-302.

6-minute walk distance at baseline was the only independent predictor of survival (P<0.003)

6-MinuteWalk Distance

Survivors(n=73)

305 + 14

Deaths (n=8)

195 + 63

Epoprostenol (n=41)Conventional Therapy (n=40)

100

80

60

40

20

00 2 4 6 8 10 12

Week

Per

cen

t S

urv

iva

l

Epoprostenol Versus Placebo

65

Assessment of PH Severity: WHO Functional Classification (NYHA Modification for PH)

WHO Class Description

I No limitation of usual activities

II Mild limitation of usual activitiesNo discomfort at restNormal physical activity causes increased dyspnea, fatigue, chest pain, or presyncope

III Marked limitation of physical activityNo discomfort at restLess than ordinary activity causes increased dyspnea, fatigue, chest pain, or presyncope

IV Patient unable to perform any physical activity at rest and may have signs of right ventricular failureDyspnea and/or fatigue and/or syncope/near-syncope may be present at rest, and symptoms are increased by almost any physical activity

Rich S. World Health Organization. 1998.

66

Prognostic Factors for Risk ofPAH Disease Progression

McLaughlin VV, et al. Circulation. 2006;114:1417-1431.

Lower Risk Higher Risk

Evidence of RV failure No Yes

Progression Gradual Rapid

WHO Class II, III IV

6-minute walk distance >380 m <325 m

Brain natriuretic peptide <180 pg/mL >180 pg/mL

Echo findings Minimal RV dysfunction

Pericardial effusion; significant RV dysfunction

Hemodynamics Normal/near normal RAP and CI

High RAP, Low CI

67

Clinical and HemodynamicPredictors of Survival in PAH

Co

nco

rdan

ce In

dex

(C s

tati

stic

)

0.8

0.7

0.6

0.5

P<0.005

P<0.001

NS

Other Clinical Factors

RHC

Age, Sex, WHO Class

ECHO & PFTs

N = 657.

Kane GC. CHEST 2008; October 25-30, 2008, Philadelphia, PA. Session AP2217.

68

Role of the Internist or Pediatrician in Diagnosis and Management of PH

● Recognize possible PH in patient with unexplained dyspnea on exertion

● Initiate screening

● Facilitate referral

● Provide regular local follow-up

- Assess volume status, vital signs, and oxygenation

- Monitor laboratory tests

- Manage anticoagulation with warfarin, if indicated

● Provide local emergency careRubin LJ, et al. Ann Intern Med. 2005;143:282-292.

69

Summary:PAH Epidemiology and Diagnosis

● PAH is rare, serious, and progressive

● PAH/PH has a wide range of etiologies

● Symptoms of PAH are nonspecific

● Screening for suspected PH can be done in local communities

● Consider referral to specialty centers for PAH confirmation by right heart catheterization and initiation of PAH specific therapy

teresa de marco, md professor of clinical medicine director, heart failure and pulmonary...

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