Pulmonary Hypertension:
Recognize the Signs
Denise A. Gonzales, MD, MS
Pulmonary and Critical Care Medicine
Presbyterian M...
What is Pulmonary Arterial
Hypertension?
Normal Cardiac
Chamber Pressures
 PAH is an
abnormal
increase in the
blood press...
Normal Oxygenation by Chamber
 Desaturated blood
enters RA
 The RV delivers
blood to lungs
 Fully saturated
blood retur...
Chamber Pressures in PAH
55
>30>30
Mean >24 at rest, >30 with exercise
PCWP <15 mmHg
Pathophysiology of PHTN
 Hypoxemia causes vasoconstriction
 Decreased perfusion worsens
hypoxemia
 Hypoxic alveoli caus...
Physiologic Effects of PAH
Old Classification
 Idiopathic pulmonary arterial
hypertension (IPAH)
 Or primary pulmonary hypertension
(PPH)
 Seconda...
Current WHO Classification
Group 1 PAH
 Connective tissue diseases
 12% SSc have PAH
 HIV infection
 0.5% have PAH
 Portal hypertension
 2-6% h...
Pathologic Vascular Changes
1. Walls of small pulmonary arteries
thicken.
2. Fibrous, or scarred, tissue
appears on inner ...
Group 1 PAH
Drug- and Toxin-Induced
 Aminorex
 Fenfluramine/Dexfenfluramine
 Toxic rapeseed oil
 Amphetamines
methamph...
Group 2
Pulmonary Hypertension (PH)
 Group 2
 Due to left
heart disease
 systolic
dysfunction
 diastolic
dysfunction
...
Group 3 PH
Lung Diseases or Hypoxemia
 COPD
 interstitial lung
disease (ILD)
 sleep-disordered
breathing (OSA 15%
have ...
Group 3 PH COPD
 5-year survival
 < 10% if mean PAP
> 45 mmHg
 > 90% if mean PAP
< 25 mmHg
 PH more strongly
associate...
Group 4 PH
Chronic
Thromboembolic
Disease
Group 5 PH
 Secondary to unclear
multifactorial causes
 hematologic
(myeloproliferative
disease)
 systemic disorders
(s...
What Are the
Signs of PHTN?
What should
prompt an
evaluation?
Diagnostic Tests: Bloodwork
 Complete metabolic panel
 liver and kidney function
 Autoantibody tests (ANA, ESR, etc)
 ...
Diagnostic Tests: CXR
 Enlarged right
ventricle and
pulmonary
arteries.
Diagnostic Testing: EKG
 Right-axis
deviation
 R/S>1 in V1
 Deep S-wave in
V5, V6
 Tall P-wave in
lead II
 Right bund...
Diagnostic Tests
Doppler echocardiogram
Diagnostic Tests: CT Scan
 Pulmonary
emboli
 Interstitial lung
disease
 PA/Ao > 2/3 can
indicate PHTN
Diagnostic Testing
 Ventilation perfusion scan (V/Q)
Q: Perfusion V: Ventilation
Diagnostic Testing:
Right Heart Catheterization
 Heart pressures
 RA, RV, PA, LVEDP
 Cardiac output
 Valve competence
...
Diagnostic Testing
 Pulmonary Angiography
Diagnostic Tests: Physiology
 6 minute walk test
 track effects of therapy
 Pulmonary function tests
 Diagnose COPD, I...
Functional Assessment
 Class I
 Patients with PHTN without limitation of
physical activity
 Ordinary physical activity ...
Functional Assessment
 Class III
 Marked limitation of physical activity,
comfortable at rest
 Less than ordinary physi...
PAH/PH Natural History
 Group 1 symptomatic patients who do
not receive treatment
 Median survival ~3 years
 If mean RA...
Risk Factors for Poor Prognosis
 Age at presentation > 45 years
 Functional class III or IV
 Pericardial effusion
 Lar...
Routine Medical Treatments
 Continuous oxygen
 Pulmonary artery vasodilator
 Anticoagulants
 Treat and prevent thrombo...
Routine Medical Treatments
 Calcium channel blockers
 Lowers pulmonary artery pressure
 May improve right heart cardiac...
Surgical Therapies
 Pulmonary thromboendarterectomy
 Correct mitral stenosis
 Repair left to right shunt (ASD, VSD)
 A...
Who Should Receive Advanced
Medical Therapy?
 Group 1 (PAH) rarely respond to
primary therapies
 Group 2 (cardiac abnorm...
Before Starting
Advanced Therapies
 Vasoreactivity test
 IV adenosine or epoprostenol
 inhaled nitric oxide
 patients ...
Step-up Therapy
 CCB therapy is the first-line treatment
for patients with +vasoreactivity test
Prostanoids
 IV epoprostenol (Prostacyclin), treprostinil
(Remodulin)
 Subcutaneous treprostinil (Ventavis)
 Inhaled tr...
Endothelin Receptor Antagonists
 Endothelin-1
 potent vasoconstrictor and smooth muscle mitogen
 high concentrations fo...
Phosphodiesterase Inhibitors
 Sildenafil (Viagra, Revatio)
 Tadalafil (Cialis, Adcirca)
 Vardenafil (Levitra)
 orally ...
Combination Medical Therapy
 Bosentan added to epoprostenol or treprostinil
 BREATHE-2 trial randomized 22 patients
 Tr...
Nitric Oxide (NO)
 Endogenous NO
 endothelium-derived relaxing factor (EDRF)
 naturally occurring vasodilator
 produce...
NO Vasoreactivity Test
 Start at 5 ppm
 Titrate up by 10 ppm
every 10 minutes
 +Vasoreactivity test at
80 ppm predicts
...
Other Uses for NO
 PAH worsened due to
acute illness
 PH after lung transplant
 PH after MVR in chronic
PH patients
 A...
Mechanism of Action: NO in VQ
Mismatched States
Adapted from Up to Date
Will My Patient Respond to NO?
 Less likely with septic shock (33
vs 64%)
 Yes if high pulmonary vascular
resistance
 M...
Potential Harm of NO
 Inhaled NO may produce toxic radicals
 But so does high FiO2!
 Methemoglobin and NO2 may increase...
NO for ARDS-Induced PH
The Studies
 Multicenter trial (n=385), P/F ratio ≤ 250
mmHg
 NO at 5 ppm
 short-term improvemen...
Why Use NO in ARDS?
 Hypothetical
 antiinflammatory properties
 antiplatelet activity
 Improved vascular permeability
...
Summary
 The signs and symptoms of
PAH/PH you can see as a
respiratory therapist
 Dyspnea, fatigue, extremity
edema
 IL...
Summary
 The tests and therapies
you provide as a
respiratory therapist for
patients with PAH/PH
 PFTs, PSG, 6-minute wa...
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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

    1. 1. Pulmonary Hypertension: Recognize the Signs Denise A. Gonzales, MD, MS Pulmonary and Critical Care Medicine Presbyterian Medical Group Albuquerque, NM
    2. 2. What is Pulmonary Arterial Hypertension? Normal Cardiac Chamber Pressures  PAH is an abnormal increase in the blood pressure in the pulmonary arteries  Normal mean pressure 8-20 mmHg at rest
    3. 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. 4. Chamber Pressures in PAH 55 >30>30 Mean >24 at rest, >30 with exercise PCWP <15 mmHg
    5. 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. 6. Physiologic Effects of PAH
    7. 7. Old Classification  Idiopathic pulmonary arterial hypertension (IPAH)  Or primary pulmonary hypertension (PPH)  Secondary pulmonary hypertension
    8. 8. Current WHO Classification
    9. 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. 10. Pathologic Vascular Changes 1. Walls of small pulmonary arteries thicken. 2. Fibrous, or scarred, tissue appears on inner wall of small pulmonary artery. 3. Bands of scarred tissue build up on inner wall of small pulmonary artery, substantially narrowing the blood vessel. 1 2 3 Normal
    11. 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. 12. Group 2 Pulmonary Hypertension (PH)  Group 2  Due to left heart disease  systolic dysfunction  diastolic dysfunction  valvular heart disease
    13. 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. 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. 15. Group 4 PH Chronic Thromboembolic Disease
    16. 16. Group 5 PH  Secondary to unclear multifactorial causes  hematologic (myeloproliferative disease)  systemic disorders (sarcoidosis)  metabolic disorders (glycogen storage disease)
    17. 17. What Are the Signs of PHTN? What should prompt an evaluation?
    18. 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. 19. Diagnostic Tests: CXR  Enlarged right ventricle and pulmonary arteries.
    20. 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. 21. Diagnostic Tests Doppler echocardiogram
    22. 22. Diagnostic Tests: CT Scan  Pulmonary emboli  Interstitial lung disease  PA/Ao > 2/3 can indicate PHTN
    23. 23. Diagnostic Testing  Ventilation perfusion scan (V/Q) Q: Perfusion V: Ventilation
    24. 24. Diagnostic Testing: Right Heart Catheterization  Heart pressures  RA, RV, PA, LVEDP  Cardiac output  Valve competence  Septal defects
    25. 25. Diagnostic Testing  Pulmonary Angiography
    26. 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. 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. 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. 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. 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. 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. 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. 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. 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. 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. 36. Step-up Therapy  CCB therapy is the first-line treatment for patients with +vasoreactivity test
    37. 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. 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. 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. 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. 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  T1/2 0.1 to 5 seconds  Pharmacology  threshold for pulmonary vasodilation ~10 ppm  No clear dose-effect curve  suppresses smooth muscle proliferation  Decreases platelet aggregation  T1/2 15 to 30 seconds at a dose of 5 to 80 ppm  rapidly inactivated to nitrosylmethemoglobin
    42. 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. 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. 44. Mechanism of Action: NO in VQ Mismatched States Adapted from Up to Date
    45. 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. 46. Potential Harm of NO  Inhaled NO may produce toxic radicals  But so does high FiO2!  Methemoglobin and NO2 may increase  both should be monitored  only sustained doses of 80ppm cause increases  Immunosuppressant properties could increase risk of infection
    47. 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. 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. 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. 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

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