PULMONARY HYPERTENSION AND EXERCISE-CARDIO-PULMONARY REHABILITATION

1. PULMONARY HYPERTENSION AND EXERCISE-CARDIO-PULMONARY REHABILITATION Rajan Joshi MD,FCCP, FAASM Assistant Professor, Pulmonary Critical care, Sleep Medicine at UKHC Medical Director, Pulmonary Rehabilitation, UKHC, Lexington. KY TLC & Sleep Center-PR, Richmond, KY

2. EXERCISE & PULMONARY HYPERTENSION • YES they can. • Progress has been made in Treatment of PAH. Well defined treatment options available now. • Early Identification and Treatment of PH is suggested since advanced disease is less responsive to therapy and Lung Transplantation may be needed • 14 FDA approved drugs are available for PH • Interventional and Surgical therapies available for CTEPH • If untreated high Mortality with in 2.8 years • Better QOL is now possible • Role of Cardio-Pulmonary Rehabilitation in PH

3. INTRODUCTION TO PULMONARY HYPERTENSION • Definition and Classification of PH • Epidemiology of PH • Pathophysiology of PH • Prognosis in PH • Diagnosis: Assessing patient with PH • Treatment of PH

4. HEMODYNAMICS & O2 SATURATIONS IN NORMAL HEART

5. DEFINITION OF PH • Screening test: Echocardiogram: Mean PAP > 25 mm HG at rest • Confirmation test: Right Heart Catheterization (RHC) • PH vs PAH • Only sub-population of patients with PH have PAH • Hemodynamically: Pre-capillary PH with mean PA pressure >25mmHG • End expiratory PCWP (PAOP) < 15 mm HG • Pulmonary Vascular Resistance (PVR) >3 wood units

6. S & S OF PULMONARY HYPERTENSION

7. CAUSES OF PULMONARY HYPERTENSION

8. CLASSIFICATION OF PH

9. WHO PH CLINICAL CLASSIFICATION

10. ECHOCARDIOGRAM IN PH

11. ECHOCARDIOGRAM FOR DIAGNOSIS PH

12. ECHOCARDIOGRAM IN PH • Qualitative & Quantitative information • Dilation of RV/RA, Septal deformity, Pulmonic regurgitation • Tricuspid jet velocity: Estimates PASP • Correlates reasonably well with RHC PA pressures • Variability in techniques and fluid status leads to false results • Poor Tricuspid regurgitation signal • PASP in ALD difficult to measure

13. PATHOPHYSIOLOGY OF PAH

14. CAUSES AND TREATMENT OPTIONS PH

15. DIAGNOSIS OF PH • Symptoms: Most common symptom is Dyspnea, Fatigue, Dizziness, Chest pains, Near syncope, Syncope • Focused family Hx: Sudden cardiac death, congenital heart disease, OSA, PF, COPD • Social Hx: Drug use or abuse • Screening tests: BNP, Liver functions, Thyroid functions, HIV, Hemolytic disorders, Thrombo-embolic disorders • ECG: RAD, Rt. Heart strain, RVH • PFTs, 6-MWT Oximetry, ABGs, PSG • CXR, HRCT Lungs, V/Q Lung scan • CPET • Echocardiogram • Right Heart Catheterization (RHC)

16. DIAGNOSIS OF PH

17. RIGHT HEART CATHETERIZATION (RHC)

18. CPET IN PH DIAGNOSIS • Very helpful and relatively simple-performed by Specialist • Sensitive, specific and comprehensive measure of Exercise capacity (6-MWT) • Gold standard for assessment of Exercise capacity and maximal cardio-pulmonary response • Findings in PAH: Reduced peak VO2, low VO2AT, Plateau effect of O2-Pulse with exercise • This correlates well with Prognosis • CPET is not suitable for more severe PAH

19. TREATMENT OF PULMONARY HYPERTENSION • Traditional treatments: O2, Diuretics, Digoxin, Anti-coagulants, Treat underlying disorders: OSA, IPF, COPD, Hypoventilation disorders, HF • General measures: Vaccinations, avoid pregnancy • Supportive therapy: Psycho-social support, family education • Expert referral • Supervised exercise training (1A), Avoid strenuous exercises (1A) and heavy load lifting, avoid Valsalva maneuvers • Interventional & surgical treatment for CTEPH: Endovascular catheter based thrombectomy, surgical thrombo-embolectomy, Atrial septoplasty, Lung and Heart-Lung transplantation

20. DETERMINANTS OF DISEASE SEVERITY

21. FDA APPROVED TREATMENT OF PAH

22. WHO FUNCTIONAL CLASSIFICATION

23. DIAGNOSIS OF PH

24. PROGNOSIS IN PAH • Troponin elevation predicts Mortality in PAH • Meta-analysis of 8 cohort studies • 49% with increased cardiac troponin died vs 18.6% with normal serum cardiac troponin • (Clinical Resp. journal Jan.2019)

25. SUMMARY OF PAH

26. EXERCISE IN PAH • Exercise training appears to be beneficial for patients with PAH • Meta-analysis of 5 randomized trial: PAH WHO GR. 1, Exercise programs 3-15 weeks resulted in improved exercise capacity( Increase in 6MWD by 60 m, peak VO2 increase by 2.4 ml/kg/min) and improved HRQL • Randomized cross over trial: 15 weeks of exercise training improved 6-MWD compared to sedentary controls(96m vs -15m) • Following cross over: sedentary group also improved mean 6-MWD by 74 m • Exercise training improved WHO Fc class and VO2 max. • Exercise training did not improve Hemodynamic abnormalities like PASP

27. EXERCISE IN PAH • Risk of exercise highest in unstable patient • Before exercise program each patient should consult their PH and Rehabilitation physician • Unsupervised training is not recommended • If difficulty getting into program CPET may help documenting limitation but also safety of exercise program and easy prescription start into exercise program • Slow start and consistent exercises (Turtle vs Rabbit) • Avoid extremes of cold and heat • Avoid straining and heavy loads lifting, Valsalva maneuvers, Avoid stooping and bending ( Near syncope and worsening SOA) • Pacing and endurance build over longer time • Use O2 liberally • Type of Programs: Cardiac vs Pulmonary rehabilitation

28. EXERCISE & PAH • Symptoms of disease make exercise more difficult • Deconditioning • Improvements come from improving muscle strength, co-ordination of breathing with muscle activity, improved breathing techniques • Efficient use of respiratory muscles with improved O2 consumption • Exercise reduces risk of other chronic diseases like Metabolic syndrome, DM but also reduces exacerbation • Improve Fc status, QOL and reduces progression of disease

29. EXERCISE & PAH • Exercise and respiratory training improve Exercise capacity and HRQOL • 15 patients each in training and control groups • VO2 AT and VO2 max improved in training group • Dyspnea and VE improved with exercise in PAH patients • Ref: Circulation 2006:114(1482-89)

30. SELECTION OF PAH PATIENTS FOR EXERCISE TRAINING • WHO Fc class 2 or 3 • Stable PAH patients (on stable medications for 3 months) • Able to use supplementary O2 in program and outside during activities too • Contraindications: Syncopal episodes or RHF patients • Avoid exercises during acute illnesses • Avoid bending over, straining and lifting heavy loads • Ref: Review Resp. Med. 2018: 12(11) 965. Lavender et al

31. POINTS TO CONSIDER:PAH and EXERCISE TRAINING • Some patients may not fit Cardiac or Pulmonary Rehabilitation • Role of Respiratory muscles training in PH is unclear unless documented Respiratory muscle dysfunction (MVV,MIP, MEP,SNIP) or other Pulmonary disorders • Strength and Endurance training is important • Weight training should target single muscle groups at low weights • Low work loads at short intervals starts ( TM or Cycle ergometers are OK)

32. POINTS TO CONSIDER: EXERCISES TRAINING IN PH • Continuous Heart rate, cardiac rhythm, O2 sat. monitoring • CPET with maximum HR or 6-MWT/6-MWD max. HR measurements • 70% of maximal HR may be good start point • Frequent symptoms monitoring: Dyspnea and perceived effort and fatigue monitoring by BORG scale • Monitoring helps safety assessment and guides training intensity • Maintain O2 sat. >88% and use O2 liberally • Stop exercises with Diaphoresis, Palpitations, Dysrhythmias, lightheadedness, pre-syncope, Hypotension or Syncope • Supported Ventilation with NPPV during Exercise training may be helpful • Comprehensive Care Center for PH or Center of Excellence for PAH

33. TREATMENT OPTIONS

34. LIVE THE LIFE YOU ALWAYS IMAGINED EXERCISE YOUR RIGHT TO BREATHE BETTER BREATHING BETTER LIVING IMPROVING LIFE ONE BREATHE AT A TIME DO NOT GIVE UP,YOU ARE CLOSER THAN YOU THINK

35. CARDIO-PULMONARY REHABILITATION UKHC

36. UKHC, LEXINGTON. KY