An Inconvenient Choice Pulmonary Artery Systolic Pressure of 43 mmhg: Is a Work Up for Pulmonary Hypertension Warranted?

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1 An Inconvenient Choice Pulmonary Artery Systolic Pressure of 43 mmhg: Is a Work Up for Pulmonary Warranted? Michael D. McGoon, MD Professor of Medicine Consultant in Cardiovascular Diseases Mayo Clinic Evaluation of Suspected PAH 42-year old woman with dyspnea on exertion Exam: RV lift, loud P2, JVP 12 cm with V-waveV CXR: prominent central PA s ECG: RAD, RVH Echocardiogram: RVE, RAE, RVSP 91 mmhg On-piste Management of PAH On-piste: On-piste is a regular run or route down a mountain in snow sports, which would be marked on a map for access to the public. On-piste April 29 June 29 August 29 Pivotal Tests History Exam CXR Contingent Tests Contribute to Assessment of: Index of Suspicion of PH Off-piste Management of PAH Clinical Evaluation: General Approach ECG Echocardiogram VQ Scan PFTs Overnight Oximetry HIV ANA LFTs Functional Test And Biomarkers TEE Exercise Echo Pulmonary Angiography Chest CT Angiogram Coagulopathy Profile ABGs Polysomnography Other CTD Serologies RVE, RAE, RVSP, RV Function Left Heart Disease VHD, CHD Chronic PE Ventilatory Function Gas Exchange Sleep Disorder HIV Infection Scleroderma, SLE, RA Portopulmonary Htn Establish Baseline Prognosis 62 year-old man with dyspnea on exertion Echocardiogram: RVSP 43 mmhg Why is this problematic? RH Cath Vasodilator Test Exercise RH Cath Volume Loading Left Heart Cath Confirmation of PH Hemodynamic Profile Vasodilator Response

2 Off-piste Management of PAH Further Information is Required Off-piste: Existing or taking place on snow that has not been compacted into tracks On-piste What is the definition of PH? Who is the patient? Is the measurement of PH reliable? Is PH present and serious? Off-piste Definition of PH PH is mpap 25 mm Hg Normal is 14 mm Hg Upper limit of normal 2 mm Hg mm Hg: unclear Requires invasive measurement Doppler-echocardiography echocardiography PH unlikely PH possible PH likely Definition of PH TRV 2.8, PASP 36, and no other echo features* suggesting PH TRV 2.8, PASP 36, but other echo features* suggest PH TRV , PASP 37 5, with/without other echo features* TRV >3.4, PASP >5, with/without other echo features* *RV enlargement/dysfunction, RA enlargement, IVC dilation/collapse, coronary sinus dilation ESC/ERS Guidelines Who is the Patient? Who is the Patient? Demographics Demographics Weight, age Symptomatic? Alternative explanation? Comorbidity Sleep disturbed breathing Lung disease Left heart disease Pulmonary thromboembolism Associated findings Right ventricular function McQuillan et al: Circulation 21;14: MGH Cardiac US study: 379 normal TTE s s with measured PASP 2,472 normal TTE s: Mean PASP 33.1±7.7 mm Hg, 6% >3 mm Hg People over 5 yo: 22% >4 mm Hg People with BMI >3 Kg/m2: 2% >4 mm Hg Milani et al (Ochsner), ACC 23

3 Who is the Patient? Symptoms Incidental finding or search for cause of symptoms Any alternative explanation for symptoms? Symptoms compatible with a PH substrate Who is the Patient? Comorbidities Type 1: Pulmonary Arterial Type 2: PH due to left heart disease Type 3: PH due to lung disease and/or hypoxia Type 4: Chronic thromboembolic PH Type 5: Miscellaneous, unclear or multifactorial mechanisms Adventitia Media Intima CO (>5 L) Who is the Patient? RV Function Smooth Muscle Hypertrophy Early Intimal Thickening PVR (<3 U) mpap (<25 mmhg) RV stress, compensation, enlargement maintained Smooth Muscle Hypertrophy Adventitial, Intimal Proliferation Thrombosis Plexiform Lesions failure Who is the Patient? RV Function Qualitative Right Ventricular Size (Echo) Survival, % moderate RV enlargement p< Follow-up, years Mayo data, unpublished Courtesy of G.Kane, H.Maradit Kremers Normal RV size or mild enlargement Who is the Patient? RV Function Tricuspid Annular Plane Systolic Excursion (TAPSE) end-systole end-diastole diastole Doppler-Echo Estimation of PASP LV RV ES LV RV Lateral TV annulus at end-diastole diastole Longitudinal shortening: Normal >15-2 mm Easy way to assess & follow RV function Sensitive indicator of impaired function Can be reduced before a a change in radial function ED TAPSE Lateral TV annulus at end- systole TAPSE < vs 18mm CI 1.9 vs 2.7 RV%AC 24 vs 33% RAE 17 vs 12 cm 3 /m 2 yr survival 5% vs 88% Forfia PR, Fisher MR, Mathai SC, et al. Tricuspid Annular Displacement Predicts Survival in Pulmonary. Am J Respir Crit Care Med 26;174: ED ES Oh, et al. The Echo Manual 26 The The peak systolic TR velocity (V) reflects the peak systolic pressure gradient (PSG) between the RV and the RA PSG PSG = 4 x V 2 RVSP RVSP = PSG + mrap RVSP = PASP (assuming no PS)

4 RVSP at time of RHC r 2.93, p< RVSP by echo RVSP by echo r.69, p< RVSP at time of RHC REVEAL Registry 136 adult patients having echo and RHC within 12 months Spearman correlation.56; p<.1 Simultaneous RHC and Doppler Currie et al: JACC 6:75, 1985 RHC and Doppler within 72 hours Arcasoy et al; AJRCCM 23; 167:735-4 Farber HW, Foreman AJ, Miller DP, McGoon MD. Congestive Heart Failure 211;17: Difference Between DE and RHC Measurement Pulmonary Arterial Systolic Pressure Average of DE and RHC Measurement Excellent and Good Quality Doppler Signal Fair and Poor Quality Doppler Signal Bias Upper and Lower Limits of Agreement Fisher MR, et al. Am J Respir Crit Care Med 29 epub. RVSP at time of RHC RVSP by echo r 2.89, p< Echo preceded RHC by days Real world practice, my last 6 patients (28) - Garvan Kane MD Reasons for failure of echo-cath cath RVSP correlation Underestimating TR velocity not getting peak signal, poor alignment Overestimating TR velocity taking overgained shaggy signals Failing to adequately assess right atrial pressure Changing conditions changing pressures Cath inaccuracies Implantable Hemodynamic Monitor: Chronicle RV pressure sensor measures: Systolic and diastolic pressure Estimates PA diastolic pressure RV dp/dt = Echo PASP Frantz RP et al. J Heart Lung Transplant 28 McGoon unpublished data

5 Echo and RHC Complementary Info Echo Other substrates Estimated RVSP, Pam, Pad, SV/PP, RV dp/dt, PVR RV Function Visual RV-IMP TDI TAPSE Strain, Strain Rate Isovolumic Accel Screening & Follow-up RHC Full hemodynamics PCWPPCWP PBFPBF RAPmRAPm Response to Vasodilators Oxygen saturations Shunt Shunt evaluation Definitive Diagnosis & Confirmation Reliability of Right Heart Catheterization Accurate at one point of time under a unique set of circumstances Hemodynamics fluctuate over short periods of time Hemodynamics fluctuate under varying circumstances RHC RHC is to FPG as RV function is to Hgb A1C RHC RHC hemodynamics diagnostic confirmation and immediate treatment adjustment ( insulin( insulin ) RV RV functional indices how the patient is doing Reliability of Right Heart Catheterization Pulmonary Arterial Pulmonary Venous Hyperdynamic Pulmonary Pre-capillary Post-capillary High Cardiac Output Passive Reactive PH PH (mpap 25 mmhg) Normal Normal PCWP ( ( 15 mmhg) High High TPG (>12 mmhg) PHPH High High PCWP (> 15 mmhg) Normal Normal TPG ( ( 12 mmhg) High High TPG (>12 mmhg) Normal Normal total pulmonary resistance Severity is more than just the degree of PH Symptomatic Status Functional Class, 6MWD, peak VO2 Indicators of RV (dys)function( Echo: : RA size, effusion, eccentricity index, RV- IMP, TAPSE Cath: : RAP, CO Biomarkers: : BNP or NT-proBNP probnp,, Troponin RV/PA coupling: : Capacitance Composite Functional Class and Exercise Capacity 6-Minute Walk Test Functional Class and Exercise Capacity Functional Classification FC at baseline FC after 3 months treatment Miyamoto et al. Clinical correlates and prognostic significance of six- minute walk test in patients with primary pulmonary hypertension: comparison with cardiopulmonary exercise testing. AJRCCM 2;161: Sitbon et al. Long-term intravenous epoprostenol infusion in primary pulmonary hypertension: prognostic factors and survival. JACC 22;4(4):78-8

6 Nagaya et al. Plasma brain natriuretic peptide as a Biomarkers RV/PA Coupling Pulmonary Vascular Capacitance SV/PP prognostic indicator in patients with primary pulmonary hypertension. Circulation 2;12(8): Also: NT-proBNP Troponin T Serum [Na] Uric acid * * Days Lowest Next Lowest Next Highest Highest Mahapatra et al. The Prognostic Value of Pulmonary Vascular Capacitance Determined D by Doppler Echocardiography in Patients with Pulmonary Arterial. JASE 26;19(8): Mahapatra et al. Relationship of Pulmonary Arterial Capacitance and Mortality in Idiopathic Pulmonary Arterial. JACC 26;47(4): Composite A risk calculator has been developed which uses information available on most patients, but doesn t t require that every variable is available in order to work Composite Risk scores can distinguish different probabilities of survival for 1 year. Benza et al (REVEAL): ATS 29 Benza et al (REVEAL): ATS 29 Pulmonary Association Pulmonary Clinicians and Researchers Is there corroborative evidence of PH? Is the patient at risk of PH? Is there an alternative source of symptoms? What is the patient s s functional status and RV function? What is the patient s s prognosis if PH present? If likelihood or risk is present: RHC and treat appropriately If not: observe, treat other causes of symptoms, repeat echo and exam in 6 months