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Screening, Referral and Diagnosis of Pulmonary Arterial Hypertension TERESA DE MARCO, MD Professor of Clinical Medicine Director, Heart Failure and Pulmonary Hypertension Program Medical Director, Heart Transplantation University of California, San Francisco Medical Center San Francisco, California Learning Objectives (CME, CE, CPE) ● At the completion of this educational activity, participants should be able to: 2 - Identify patients who are at higher risk for PAH - Identify when a patient with suspected PAH should be referred to a PAH-specific specialty center Identify the common presenting symptoms for PAH Discuss the diagnostic workup for symptoms suggestive of PAH, and the appropriate use of various tests Clinical Classification of Pulmonary Arterial Hypertension (PAH) PAH: Definition on Right Heart Catheterization Increased mean pulmonary arterial pressure (mPAP) Normal pulmonary artery wedge pressure (PAWP) Increased pulmonary vascular resistance (PVR) Gaine SP, et al. Lancet. 1998;352:719-725. 4 >25 mm Hg at rest or >30 mm Hg during exercise <15 mm Hg >3 Wood units 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. 5 Revised Clinical Classification of Pulmonary Hypertension: 2003 Venice ● 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 Simonneau G, et al. J Am Coll Cardiol. 2004;43:5S-12S. 6 Revised Clinical Classification of Pulmonary Hypertension: 2003 Venice ● 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) Simonneau G, et al. J Am Coll Cardiol. 2004;43:5S-12S. 7 Pulmonary Hypertension Connection Registry: Etiology of PAH Portal Hypertension, 7.0% Anorexigens, 3.0% HIV, 1.0% Congenital Heart, 11.0% Connective Tissue, 30.0% n=578; female-male ratio: 77% - 33% Calcium channel blocker use at referral: 80% Thenappan T. Eur Respir J. 2007;30:1103-1110. 8 Idiopathic/familial, 48.0% REVEAL Database: Most Frequent Symptoms at Diagnosis 11% 11% Dyspnea at rest IPAH APAH 13% 13.0% Cough 14% 16.0% Dizzy/lightheaded 20% 23.0% Presyncope/syncope Edema 20% 21.0% Chest pain/discomfort 20% 23% 27% 24.0% Other 29% 26.0% Fatigue 83% 84.0% Dyspnea on exertion 0 25 n=1479. Elliott CG, et al. Chest. 2007;132(4 suppl):631S. 9 50 Incidence (%) 75 100 Survival in PAH by Etiology 1.0 0.9 Congenital Heart Disease Percent Survival 0.8 0.7 Portopulmonary 0.6 0.5 IPAH 0.4 Connective Tissue Disease 0.3 0.2 HIV 0.1 0 0 1 2 3 Years IPAH, idiopathic pulmonary arterial hypertension. McLaughlin VV, et al. Chest. 2004;126:78S-92S. 10 4 5 Screening for PAH in At-Risk Populations Screening for PAH Requires High 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. 12 Alternative Diagnoses of Patients Referred to PAH Specialty Clinic ILD 5.0% VTE 5.0% Other Structural Heart Disease OSA 12.0% 13.0% 19% LV Dysfunction Obstructive Lung Disease 22.0% 24.0% All Alternative Diagnoses n=268, all patients referred to PAH specialty center. Moghbelli MH, et al. Am J Respir Crit Care Med. 2008;177:A923. 13 85.0% 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. 14 Screening for PAH Associated With Connective 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. 15 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. 16 Diagnosis of PAH PAH Diagnostic Guidelines: Decision Analysis Unexplained Symptoms of Dyspnea on Exertion, Syncope/Near Syncope, Fatigue Clinical History, Examination, Chest X-Ray, ECG McGoon M, et al. Chest. 2004;126:14S-34S. 18 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 McGoon M, et al. Chest. 2004;126:14S-34S. 19 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 McGoon M, et al. Chest. 2004;126:14S-34S. 20 Other Physical Findings: Differential Diagnosis/PAH Etiology Finding Differential Diagnosis/PAH Etiology Cyanosis Right-to-left shunt Clubbing Rare in IPAH Congenital 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 McGoon M, et al. Chest. 2004;126:14S-34S. 21 Screening Tests for PAH: Electrocardiogram ● Insufficiently sensitive as a screening tool ● May indicate right-heart disease ● May provide prognostic information McGoon M, et al. Chest. 2004;126:14S-34S. 22 Electrocardiogram Associated With Right Ventricular Hypertrophy (RVH) Image courtesy of Vallerie McLaughlin, MD 23 Electrocardiogram Associated With Right Bundle Branch Block Plus RVH Image courtesy of Vallerie McLaughlin, MD 24 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 xray ● Chest x-ray may reveal underlying causes of PH McGoon M, et al. Chest. 2004;126:14S-34S. 25 Chest X-Ray Consistent With PH Image courtesy of Vallerie McLaughlin, MD 26 MRI of Pulmonary Artery Distensibility: Preliminary Findings Potential Noninvasive Surrogate Marker of Acute Vasodilator Challenge 40 mPAD (%) 30 20 10 0 Nonresponders Responders 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. 27 PAH Diagnostic Guidelines: Decision Analysis Clinical History, Examination, Chest X-Ray, ECG Is There a Reason to Suspect PH? Yes No Echocardiography Work-Up for Other Conditions McGoon M, et al. Chest. 2004;126:14S-34S. 28 PAH Diagnostic Guidelines: Decision Analysis Echocardiography for Suspected PH RH Analysis CHD Analysis LH Analysis TRV to Estimate RVSP, RVE, RAE, RV Dysfunction Abnormal Morphology, Shunt LV Systolic Diastolic Dysfunction Valvular Dysfunction McGoon M, et al. Chest. 2004;126:14S-34S. 29 Echocardiograph: Parasternal Long Axis Image courtesy of Vallerie McLaughlin, MD 30 Echocardiograph: Parasternal Short Axis Image courtesy of Vallerie McLaughlin, MD 31 Echocardiograph: Apical Four Chamber Image courtesy of Vallerie McLaughlin, MD 32 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. Image courtesy of Vallerie McLaughlin, MD 33 Calculations of Estimated Pulmonary Artery 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 4 x (pulmonary regurgitation enddiastolic pressure diastolic velocity)2 + “RAP” TR=tricuspid regurgitant jet velocity m/sec. “RAP”=estimated right atrial pressure. RVOT AT=right ventricular outflow tract acceleration time. Bossone E, et al. Chest. 2005;127:1836-1843. 34 Limitations of Echocardiography in 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. 35 Accuracy of PH Diagnosis by Echocardiography in Advanced Lung Disease Diagnosis of PH ● Cohort study of lung transplant patients (n=374) 70 ● 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 50 Studies (%) - 60 40 30 20 10 0 Arcasoy SM, et al. Am J Respir Crit Care Med. 2003;167:735-740. 36 Overestimation Accurate Underestimation No Pulmonary Hypertension Pulmonary Hypertension Doppler Echo Overestimates PAH in Patients With Scleroderma-Related Lung Disease No PH PH 100 Percent (%) 80 60 60 40 40 38 25 25 20 0 0 Overestimate PASP Underestimate PASP Accurate PASP N = 13. Chan KM. CHEST 2008; October 25-30, 2008, Philadelphia, PA. Session AP2217. 37 PAH Diagnostic Guidelines: Further Evaluation of Patients Echocardiography Indicates PH Evaluate for Associated Causes V/Q scan PFTs Arterial Saturation HIV Infection, Scleroderma, Parenchymal Lung Suspected SLE, Other CTD, Liver Chronic Pulmonary Disease, Hypoxemia, Disease, CHD, Drugor Sleep Disorder Emboli Associated McGoon M, et al. Chest. 2004;126:14S-34S. 38 Association Between Stimulant Use and IPAH Patients Reporting Use (n=340) 40 Patients (%) 30 28.9% 20 10 0 Idiopathic 3.8% 4.3% PAH with Known Risk Factors Thromboembolic PH Retrospective analysis at single PH center of adults with PH. Chin KM, et al. Chest. 2006;130:1657-1663. 39 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 McGoon M, et al. Chest. 2004;126:14S-34S. 40 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. 41 Pulmonary Function Testing for PAH 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. 42 PH in Patients With Obstructive 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. 43 PAH Diagnostic Guidelines: Confirmation of PAH Echocardiography Indicates PH Refer to PAH Specialty Center for Right Heart Catheterization Adapted from McGoon M, et al. Chest. 2004;126:14S-34S. 44 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 McGoon M, et al. Chest. 2004;126:14S-34S. 45 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. 46 Hemodynamic Classification of PH (mean PAP >25 mmHg) Post-Capillary PH VC RA RV PA PV PC Mixed PH Pre-capillary PH 47 LA LV Ao High-Flow PH (O2 sat run) Hemodynamic Classification of PH (mean PAP >25 mmHg) Post-Capillary PH PAWP>15 mmHg; PVR nl VC 48 RA RV PA PC LA PV Ao LV PVP LAP LVEDP Hemodynamic Classification of PH (mean PAP >25 mmHg) Post-Capillary PH PAWP>15 mmHg; PVR nl MR VC RA RV PA PC LA PV Ao LV PVP LAP LVEDP Systemic HTN AoV Disease Myocardial Disease Dilated CMP-ischemic/non-ischemic Hypertrophic CMP Restrictive/infiltrative CMP Pericardial disease 49 Hemodynamic Classification of PH (mean PAP >25 mmHg) Post-Capillary PH PAWP>15 mmHg; PVR nl Atrial Myxoma Cor Triatriatum VC RA RV PA PC LA PV PVP LAP LV MV Disease 50 Ao Hemodynamic Classification of PH (mean PAP >25 mmHg) Post-Capillary PH PAWP>15 mmHg; PVR nl VC RA RV PA PC PV PVP PV compression 51 LA LV Ao Hemodynamic Classification of PH (mean PAP >25 mmHg) PAH Lung Diseases +/- Hypoxemia CTEPH { RA RV PA PV PC { VC Pre-capillary PH PAWP < 15 mmHg; PVR > 3 Wu 52 LA LV Ao Hemodynamic Classification of PH (mean PAP >25 mmHg) VC RA RV PA PC LA PV Ao LV PVP LAP LVEDP Mixed PH (“Reversible” vs. “Fixed”) PAWP >15 mmHg PVR > 3 Wu 53 Measuring Pulmonary Artery Wedge Pressure Pulmonary Artery Pressure Decay Curve 160 Balloon Occlusion Pressure (mm Hg) 140 120 ARDS IPAH 100 80 60 40 20 0 0 2 4 6 Time (seconds) 8 10 12 Time Steady State Is Longer in IPAH Than In ARDS ARDS: acute respiratory distress syndrome. Souza R, et al. Crit Care. 2005;9:R132-R138. 54 Correct and Incorrect Readings of PAWP PA and RV Recordings in Patient With PAH Oudiz RJ, et al. Advances Pulm Hypertens. 2005;4:15-25. 55 PA Pressure Tracing Erroneously Labeled As PAWP Misclassification of PAH and PVH Through Use of PAWP Versus LVEDP 100 90 Percent (%) 80 70 50.2 60 37.4 50 40 30 20 10 0 Misclassification of PAH by PAWP Misclassification of PVH by PAWP n=4,666, all patients undergoing LHC and RHC over 10 years at single center. Halpern SD, et al. Am J Respir Crit Care Med. 2008;177:A259. 56 PAH and the Right Ventricle Neurohormonal and other mediator activation RV remodeling Pulmonary hypertension Pressure overload Adaptive RV hypertrophy Decreased wall stress Maladaptive RV hypertrophy & fibrosis Diastolic dysfunction RV dilation & systolic failure RV ischemia: ] Compensated Phase ] Decompensating Phase Wall stress & heart rate Coronary perfusion gradient Tricuspid regurgitation Preload-afterload mismatch Decreased LV compliance/preload: Inter-ventricular septal shift Intrapericardial pressure DeMarco T, et al. Adv Pulm Hypertens. 2005;4:16-26. 57 Normal CO, normal RAP Higher RAP to maintain adequate CO Decompensated Phase CO, RAP AV-DO2 Hypoxemia, acidosis, life-threatening dysrhythmias Measuring Diastolic Dysfunction Mitral Inflow Velocity (m/s) Normal Diastolic Function DT>140 ms 0.75<E/A<2 Mild Diastolic Dysfunction Moderate Diastolic Dysfunction Severe Diastolic Dysfunction E/A≤0.75 DT>140 ms 0.75<E/A<2 DT<140 ms E/A>2 2.0 E A Doppler Tissue Imaging of Mitral Annular Motion Velocity (m/s) 0 E/e’<10 0 1.5 e1 a1 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. Bursi F, et al. JAMA. 2006;296:2209-2216. 58 E/e’<10 E/e’≥10 E/e’≥10 PAH Diagnostic Workup Right Heart Catheterization Confirms PAH 6-Minute Walk Borg Score Functional Class Establish Baseline, Prognosis, and Document Progression/Response to Treatment With Serial Re-Assessment McGoon M, et al. Chest. 2004;126:14S-34S. 59 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. 60 NT-proBNP Elevations Correlate With Right Ventricular Dysfunction in PH NT-proBNP (ng/L) 5000 4000 4127 3000 2000 1000 354 0 With RVSD N = 25. Threshold value RVD detection: 1,685 ng/L. Sensitivity for RVD 100%; specificity 94%. Blyth KG, et al. Eur Respir J. 2007;29:737-744. 61 Without RVSD BNP Predictive Value For Adverse Outcomes BNP (pg/mL) 644.8 Death Cardiogenic shock 632.1 Inpatient heart failure 545.6 Outpatient heart failure 472.7 Ventricular dysfunction 424.7 WHO Class IV 470.0 WHO Class III 388.4 WHO Class II 151.7 WHO Class I 20.2 Control 12.1 N=85. Garcia-Badillo EV. CHEST 2008; October 25-30, 2008, Philadelphia, PA. Session AP2217. 62 Distance Walked in 6 Minutes (m) 6-Minute Walk Distance Correlates With IPAH Disease Severity 800 700 600 500 * 400 *† 300 200 *†‡ 100 0 Control NYHA II NYHA III *P<0.05 versus control. †P<0.05 versus NYHA Class II. ‡P<0.05 vs NYHA Class III. Miyamoto S, et al. Am J Respir Crit Care Med. 2000;161:487-492. 63 NYHA IV Impact of Baseline 6-Minute Walk Distance on Survival Epoprostenol Versus Placebo 6-Minute Walk Distance 100 Percent Survival 80 305 + 14 Deaths (n=8) 195 + 63 60 40 6-minute walk distance at baseline was the only independent predictor of survival (P<0.003) Epoprostenol (n=41) Conventional Therapy (n=40) 20 0 0 2 4 6 8 10 Week Barst RJ, et al. N Engl J Med. 1996;334:296-302. 64 Survivors (n=73) 12 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 activities No discomfort at rest Normal physical activity causes increased dyspnea, fatigue, chest pain, or presyncope III Marked limitation of physical activity No discomfort at rest Less 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 failure Dyspnea 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. 65 Prognostic Factors for Risk of PAH Disease Progression Lower Risk Higher Risk No Yes Progression Gradual Rapid WHO Class II, III IV >380 m <325 m Brain natriuretic peptide <180 pg/mL >180 pg/mL Echo findings Minimal RV dysfunction Pericardial effusion; significant RV dysfunction Normal/near normal RAP and CI High RAP, Low CI Evidence of RV failure 6-minute walk distance Hemodynamics McLaughlin VV, et al. Circulation. 2006;114:1417-1431. 66 Clinical and Hemodynamic Predictors of Survival in PAH NS Concordance Index (C statistic) P<0.001 0.8 P<0.005 0.7 0.6 0.5 Age, Sex, WHO Class Other Clinical Factors ECHO & PFTs RHC N = 657. Kane GC. CHEST 2008; October 25-30, 2008, Philadelphia, PA. Session AP2217. 67 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 care Rubin LJ, et al. Ann Intern Med. 2005;143:282-292. 68 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 69