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  • In PAH, the pulmonary artery becomes stiff and narrow. The right ventricle has to work harder and harder to push blood through the narrow artery. When this happens, less and less blood is able to flow out into the body, and more and more symptoms of PAH begin to appear
  • Due to diseases that localize to small pulmonary arterioles
  • RVH, tall R in V1, tall S waves in V5-6, inverted T V1-3, peaked T in II
  • Uses sound waves to show the function of the right ventricle, to measure blood flow through the heart valves, and then calculate the systolic pulmonary artery pressure.
  • Modeled after New York Heart Failure functional assessment
  • Modeled after New York Heart Failure functional assessment
  • stroke volume / PA pulse pressure)

Dr. Gonzales's Power Point Presentation Transcript

  • 1. Pulmonary Hypertension: Recognize the Signs Denise A. Gonzales, MD, MS Pulmonary and Critical Care Medicine Presbyterian Medical Group Albuquerque, NM
  • 2. What is Pulmonary Arterial Hypertension?
    • PAH is an abnormal increase in the blood pressure in the pulmonary arteries
    • Normal mean pressure 8-20 mmHg at rest
    Normal Cardiac Chamber Pressures
  • 3. Normal Oxygenation by Chamber
    • Desaturated blood enters RA
    • The RV delivers blood to lungs
    • Fully saturated blood returns to LA
    • The LV delivers oxygenated blood to body
  • 4. Chamber Pressures in PAH 5 >30 Mean >24 at rest, >30 with exercise PCWP <15 mmHg
  • 5. Pathophysiology of PHTN
    • Hypoxemia causes vasoconstriction
    • Decreased perfusion worsens hypoxemia
    • Hypoxic alveoli cause arterioles to vasocontrict further to preserve VQ matching
    • Pulmonary pressures increase and become sustained
    • Right ventricle works harder, develops hypertrophy and overload
  • 6. Physiologic Effects of PAH
  • 7. Old Classification
    • Idiopathic pulmonary arterial hypertension (IPAH)
      • Or primary pulmonary hypertension (PPH)
    • Secondary pulmonary hypertension
  • 8. Current WHO Classification
  • 9. Group 1 PAH
    • Connective tissue diseases
      • 12% SSc have PAH
    • HIV infection
      • 0.5% have PAH
    • Portal hypertension
      • 2-6% have PAH
    • Congenital heart disease
    • Chronic hemolytic anemia
    • Pulmonary veno-occlusive disease
    • Pulmonary capillary hemangiomatosis
    • Idiopathic (BMPR2 gene abnormality)
  • 10. Pathologic Vascular Changes
    • Walls of small pulmonary arteries thicken.
    • Fibrous, or scarred, tissue appears on inner wall of small pulmonary artery.
    • Bands of scarred tissue build up on inner wall of small pulmonary artery, substantially narrowing the blood vessel.
    1 2 3 Normal
  • 11. Group 1 PAH Drug- and Toxin-Induced
    • Aminorex
    • Fenfluramine/Dexfenfluramine
    • Toxic rapeseed oil
    • Amphetamines methamphetamines, cocaine
    • L-tryptophan
    • Phenylpropanolamine
    • St. John’s Wort
    • Chemotherapeutic agents
    • Selective serotonin reuptake inhibitors
  • 12. Group 2 Pulmonary Hypertension (PH)
    • Group 2
    • Due to left heart disease
      • systolic dysfunction
      • diastolic dysfunction
      • valvular heart disease
  • 13. Group 3 PH Lung Diseases or Hypoxemia
    • COPD
    • interstitial lung disease (ILD)
    • sleep-disordered breathing (OSA 15% have PAH)
    • alveolar hypoventilation disorders (OHV)
    • other causes of hypoxemia
    Central OHV COPD/ILD CHF OSA
  • 14. Group 3 PH COPD
    • 5-year survival
      • < 10% if mean PAP > 45 mmHg
      • > 90% if mean PAP < 25 mmHg
    • PH more strongly associated with hypoxemia than lung function
  • 15. Group 4 PH
    • Chronic
    • Thromboembolic
    • Disease
  • 16. Group 5 PH
    • Secondary to unclear multifactorial causes
      • hematologic (myeloproliferative disease)
      • systemic disorders (sarcoidosis)
      • metabolic disorders (glycogen storage disease)
  • 17. What Are the Signs of PHTN? What should prompt an evaluation?
  • 18. Diagnostic Tests: Bloodwork
    • Complete metabolic panel
      • liver and kidney function
    • Autoantibody tests (ANA, ESR, etc)
      • collagen vascular diseases
    • Thyroid stimulating hormone
    • HIV
    • Arterial blood gases
    • Complete blood count
      • infection, elevated hemoglobin, and anemia
    • B-type natriuretic peptide (BNP)
      • heart failure
  • 19. Diagnostic Tests: CXR
    • Enlarged right ventricle and pulmonary arteries.
  • 20. Diagnostic Testing: EKG
    • Right-axis deviation
    • R/S>1 in V1
    • Deep S-wave in V5, V6
    • Tall P-wave in lead II
    • Right bundle branch block
  • 21. Diagnostic Tests Doppler echocardiogram
  • 22. Diagnostic Tests: CT Scan
    • Pulmonary emboli
    • Interstitial lung disease
    • PA/Ao > 2/3 can indicate PHTN
  • 23. Diagnostic Testing
    • Ventilation perfusion scan (V/Q)
    Q: Perfusion V: Ventilation
  • 24. Diagnostic Testing: Right Heart Catheterization
    • Heart pressures
      • RA, RV, PA, LVEDP
    • Cardiac output
    • Valve competence
    • Septal defects
  • 25. Diagnostic Testing
    • Pulmonary Angiography
  • 26. Diagnostic Tests: Physiology
    • 6 minute walk test
      • track effects of therapy
    • Pulmonary function tests
      • Diagnose COPD, ILD, hypoventilation
    • PSG or overnight oximetry
      • Diagnose hypoxemia due to sleep disordered breathing
  • 27. Functional Assessment
    • Class I
      • Patients with PHTN without limitation of physical activity
      • Ordinary physical activity does not cause undue dyspnea or fatigue, chest pain or near syncope
    • Class II
      • Slight limitation of physical activity, comfortable at rest
      • Ordinary physical activity causes undue dyspnea or fatigue, chest pain or near syncope
  • 28. Functional Assessment
    • Class III
      • Marked limitation of physical activity, comfortable at rest
      • Less than ordinary physical activity causes undue dyspnea or fatigue, chest pain or near syncope
    • Class IV
      • Inability to perform any physical activity without symptoms
      • Dyspnea and/or fatigue may be present at rest, and discomfort is increased by any physical activity
    • * Modified from the New York Heart Association classification of patients with cardiac disease
  • 29. PAH/PH Natural History
    • Group 1 symptomatic patients who do not receive treatment
      • Median survival ~3 years
      • If mean RAP ≥20 mmHg, median survival ~1 month!
    • Groups 2-5 symptomatic patients have a worse prognosis
      • If severe PHTN or RHF, death within one year
    • Patients with cardiac arrest rarely survive
      • 3000 patients with PAH who required CPR had a 6% 90-day survival (180 people lived to 90 days)
  • 30. Risk Factors for Poor Prognosis
    • Age at presentation > 45 years
    • Functional class III or IV
    • Pericardial effusion
    • Large right atrial size
    • Elevated right atrial pressure
    • Septal shift during diastole
    • Increased BNP level
    • Failure to improve to a lower functional class during treatment
  • 31. Routine Medical Treatments
    • Continuous oxygen
      • Pulmonary artery vasodilator
    • Anticoagulants
      • Treat and prevent thrombosis
    • Diuretics / Low Salt Diet
      • Decrease RA pressures
      • Treat right heart failure
    • Digoxin
      • Increases cardiac output
  • 32. Routine Medical Treatments
    • Calcium channel blockers
      • Lowers pulmonary artery pressure
      • May improve right heart cardiac output
    • Exercise
    • Immunization
      • Prevent pulmonary infections
    • Opportunistic Infection Prophylaxis
      • Patients on immunosuppression (ILD)
  • 33. Surgical Therapies
    • Pulmonary thromboendarterectomy
    • Correct mitral stenosis
    • Repair left to right shunt (ASD, VSD)
    • Atrial septosotomy to relieve RHF
      • when PA pressure exceeds systemic pressure
    • Lung transplantation
      • for advanced PHTN not responsive to medical therapy
      • ~1,000 lung transplants/year in US
    • Heart / lung transplantation
  • 34. Who Should Receive Advanced Medical Therapy?
    • Group 1 (PAH) rarely respond to primary therapies
    • Group 2 (cardiac abnormalities) usually worsen with advanced therapies
    • Group 3 (COPD) respond to oxygen therapy
    • Group 4 (thromboembolic) respond to thrombectomy and anticoagulation
    • Group 5 unknown effects of advanced therapies, primary therapy indicated
  • 35. Before Starting Advanced Therapies
    • Vasoreactivity test
      • IV adenosine or epoprostenol
      • inhaled nitric oxide 
      • patients with Type 1 PAH are most likely to respond
    • Vasoreactivity test is positive if
      • mean PAP decreases at least 10 mmHg and to a value < 40 mmHg
      • increased or unchanged cardiac output
      • minimally reduced or unchanged systemic blood pressure
  • 36. Step-up Therapy
    • CCB therapy is the first-line treatment for patients with +vasoreactivity test
  • 37. Prostanoids
    • IV epoprostenol (Prostacyclin), treprostinil (Remodulin)
    • Subcutaneous treprostinil (Ventavis)
    • Inhaled treprostinil, iloprost
    • Prostacyclin delivered continuously through a central venous catheter
      • initiated at doses of 1 to 2 ng/kg /min
      • increase by 1-2 ng/kg/min every 1-2 days
      • doses ~150-200 ng/kg/min
      • side effects: jaw pain, diarrhea, arthralgias
    • Iloprost (Ventavis)
      • Inhaled but administered 6-9 times daily
  • 38. Endothelin Receptor Antagonists
    • Endothelin-1
      • potent vasoconstrictor and smooth muscle mitogen
      • high concentrations found in group 1 PAH
      • Bosentan (Tracleer) orally administered
      • nonselective endothelin receptor antagonist
    • Ambrisentan (Letaris) and sitaxsentan (Thelin)
      • selective type A endothelin-1 receptor antagonist
  • 39. Phosphodiesterase Inhibitors
    • Sildenafil (Viagra, Revatio)
    • Tadalafil  (Cialis, Adcirca)
    • Vardenafil (Levitra)
      • orally administered
      • cyclic GMP phosphodiesterase type 5 (PDE5) inhibitors
      • prolong vasodilatory effect of nitric oxide
    • Substantial treatment effect differences in PDE5 inhibitors
      • rate of onset
      • pulmonary vascular selectivity
      • only sildenafil associated with improved oxygenation
  • 40. Combination Medical Therapy
    • Bosentan added to epoprostenol or treprostinil
      • BREATHE-2 trial randomized 22 patients
    • Treprostinil added to bosentan or sildenafil
      • TRIUMPH trial randomized 235 patients
      • improved 6 minute walk
    • Sildenafil added to epoprostenol
      • 267 patients with group 1 PAH
      • improved hemodynamics, exercise capacity, quality of life, and time to worsening
    • Sildenafil added to bosentan
      • 25 patients with group 1 PAH
      • clinical improvement of symptoms, exercise capacity, and WHO functional class
    • Sildenafil added to iloprost
      • 73 patients with group 1 PAH
      • improvement in exercise capacity, WHO functional class, and hemodynamics
  • 41. Nitric Oxide (NO)
    • Endogenous NO
      • endothelium-derived relaxing factor (EDRF)
      • naturally occurring vasodilator
      • produced from L-arginine in endothelial cells by endothelial nitric oxide synthase (eNOS)
      • induces increased cGMP which causes smooth muscle relaxation
      • T 1/2 0.1 to 5 seconds
    • Pharmacology
      • threshold for pulmonary vasodilation ~10 ppm
      • No clear dose-effect curve
      • suppresses smooth muscle proliferation
      • Decreases platelet aggregation
      • T 1/2 15 to 30 seconds at a dose of 5 to 80 ppm
      • rapidly inactivated to nitrosylmethemoglobin
  • 42. NO Vasoreactivity Test
    • Start at 5 ppm
    • Titrate up by 10 ppm every 10 minutes
    • +Vasoreactivity test at 80 ppm predicts response to nifedipine with
      • 94% predictive accuracy
  • 43. Other Uses for NO
    • PAH worsened due to acute illness
    • PH after lung transplant
    • PH after MVR in chronic PH patients
    • Acute right heart syndrome causing systemic hypotension
      • pulmonary embolism
      • ARDS
  • 44. Mechanism of Action: NO in VQ Mismatched States Adapted from Up to Date
  • 45. Will My Patient Respond to NO?
    • Less likely with septic shock (33 vs 64%)
    • Yes if high pulmonary vascular resistance
    • Maybe if responsive to PEEP
    • Dosing
      • 1.25 -40 ppm
      • continuously for days to weeks
      • interruptions result in desaturation
      • patients may become sensitized and require lower doses
  • 46. Potential Harm of NO
    • Inhaled NO may produce toxic radicals
      • But so does high FiO 2 !
    • Methemoglobin and NO 2 may increase
      • both should be monitored
      • only sustained doses of 80ppm cause increases
    • Immunosuppressant properties could increase risk of infection
  • 47. NO for ARDS-Induced PH The Studies
    • Multicenter trial (n=385), P/F ratio ≤ 250 mmHg
      • NO at 5 ppm
      • short-term improvement of oxygenation
      • no improvement in mechanical ventilation or mortality
    • Multicenter RCT (n=177) with ARDS
      • modestly improved oxygenation
      • no difference in 28-day mortality
    • Meta-analysis of 10 RCTs (n=1237 patients)
      • NO versus conventional management
      • no improvement in mortality or ventilator-free days
      • increased P/F ratio without effect on mean PA pressure
  • 48. Why Use NO in ARDS?
    • Hypothetical
      • antiinflammatory properties
      • antiplatelet activity
      • Improved vascular permeability
    • Treatment effects comparable to
      • proning
      • oscillation
      • surfactant
      • ECMO
      • antioxidants including nutritionals
      • when standard treatment is limited tidal volume ventilation
  • 49. Summary
    • The signs and symptoms of PAH/PH you can see as a respiratory therapist
      • Dyspnea, fatigue, extremity edema
      • ILD by CXR or PFTs
      • COPD by CXR of PFTs
      • Hypoventilation by PFTs, PSG
      • Typical EKG changes
  • 50. Summary
    • The tests and therapies you provide as a respiratory therapist for patients with PAH/PH
      • PFTs, PSG, 6-minute walk
      • NO vasoreactivity test
      • NO therapy in the ICU