dr. gonzales's power point
DESCRIPTION
TRANSCRIPT
![Page 1: Dr. Gonzales's Power Point](https://reader033.vdocuments.mx/reader033/viewer/2022050905/54c902814a795979348b45eb/html5/thumbnails/1.jpg)
Pulmonary Hypertension:
Recognize the Signs
Denise A. Gonzales, MD, MS
Pulmonary and Critical Care Medicine
Presbyterian Medical Group
Albuquerque, NM
![Page 2: Dr. Gonzales's Power Point](https://reader033.vdocuments.mx/reader033/viewer/2022050905/54c902814a795979348b45eb/html5/thumbnails/2.jpg)
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
![Page 3: Dr. Gonzales's Power Point](https://reader033.vdocuments.mx/reader033/viewer/2022050905/54c902814a795979348b45eb/html5/thumbnails/3.jpg)
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
![Page 4: Dr. Gonzales's Power Point](https://reader033.vdocuments.mx/reader033/viewer/2022050905/54c902814a795979348b45eb/html5/thumbnails/4.jpg)
Chamber Pressures in PAH
55
>30>30
Mean >24 at rest, >30 with exercise PCWP <15 mmHg
![Page 5: Dr. Gonzales's Power Point](https://reader033.vdocuments.mx/reader033/viewer/2022050905/54c902814a795979348b45eb/html5/thumbnails/5.jpg)
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
![Page 6: Dr. Gonzales's Power Point](https://reader033.vdocuments.mx/reader033/viewer/2022050905/54c902814a795979348b45eb/html5/thumbnails/6.jpg)
Physiologic Effects of PAH
![Page 7: Dr. Gonzales's Power Point](https://reader033.vdocuments.mx/reader033/viewer/2022050905/54c902814a795979348b45eb/html5/thumbnails/7.jpg)
Old Classification Idiopathic pulmonary arterial
hypertension (IPAH) Or primary pulmonary hypertension
(PPH)
Secondary pulmonary hypertension
![Page 8: Dr. Gonzales's Power Point](https://reader033.vdocuments.mx/reader033/viewer/2022050905/54c902814a795979348b45eb/html5/thumbnails/8.jpg)
Current WHO Classification
![Page 9: Dr. Gonzales's Power Point](https://reader033.vdocuments.mx/reader033/viewer/2022050905/54c902814a795979348b45eb/html5/thumbnails/9.jpg)
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)
![Page 10: Dr. Gonzales's Power Point](https://reader033.vdocuments.mx/reader033/viewer/2022050905/54c902814a795979348b45eb/html5/thumbnails/10.jpg)
Pathologic Vascular Changes1. 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
![Page 11: Dr. Gonzales's Power Point](https://reader033.vdocuments.mx/reader033/viewer/2022050905/54c902814a795979348b45eb/html5/thumbnails/11.jpg)
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
![Page 12: Dr. Gonzales's Power Point](https://reader033.vdocuments.mx/reader033/viewer/2022050905/54c902814a795979348b45eb/html5/thumbnails/12.jpg)
Group 2 Pulmonary Hypertension (PH)
Group 2 Due to left
heart disease systolic
dysfunction diastolic
dysfunction valvular heart
disease
![Page 13: Dr. Gonzales's Power Point](https://reader033.vdocuments.mx/reader033/viewer/2022050905/54c902814a795979348b45eb/html5/thumbnails/13.jpg)
Group 3 PHLung 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/ILDCHF
OSA
![Page 14: Dr. Gonzales's Power Point](https://reader033.vdocuments.mx/reader033/viewer/2022050905/54c902814a795979348b45eb/html5/thumbnails/14.jpg)
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
![Page 15: Dr. Gonzales's Power Point](https://reader033.vdocuments.mx/reader033/viewer/2022050905/54c902814a795979348b45eb/html5/thumbnails/15.jpg)
Group 4 PHChronicThromboembolic Disease
![Page 16: Dr. Gonzales's Power Point](https://reader033.vdocuments.mx/reader033/viewer/2022050905/54c902814a795979348b45eb/html5/thumbnails/16.jpg)
Group 5 PH Secondary to unclear
multifactorial causes hematologic
(myeloproliferative disease)
systemic disorders (sarcoidosis)
metabolic disorders (glycogen storage disease)
![Page 17: Dr. Gonzales's Power Point](https://reader033.vdocuments.mx/reader033/viewer/2022050905/54c902814a795979348b45eb/html5/thumbnails/17.jpg)
What Are the
Signs of PHTN?
What should prompt an evaluation?
![Page 18: Dr. Gonzales's Power Point](https://reader033.vdocuments.mx/reader033/viewer/2022050905/54c902814a795979348b45eb/html5/thumbnails/18.jpg)
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
![Page 19: Dr. Gonzales's Power Point](https://reader033.vdocuments.mx/reader033/viewer/2022050905/54c902814a795979348b45eb/html5/thumbnails/19.jpg)
Diagnostic Tests: CXR Enlarged right
ventricle and pulmonary arteries.
![Page 20: Dr. Gonzales's Power Point](https://reader033.vdocuments.mx/reader033/viewer/2022050905/54c902814a795979348b45eb/html5/thumbnails/20.jpg)
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
![Page 21: Dr. Gonzales's Power Point](https://reader033.vdocuments.mx/reader033/viewer/2022050905/54c902814a795979348b45eb/html5/thumbnails/21.jpg)
Diagnostic Tests
Doppler echocardiogram
![Page 22: Dr. Gonzales's Power Point](https://reader033.vdocuments.mx/reader033/viewer/2022050905/54c902814a795979348b45eb/html5/thumbnails/22.jpg)
Diagnostic Tests: CT Scan Pulmonary
emboli Interstitial lung
disease PA/Ao > 2/3 can
indicate PHTN
![Page 23: Dr. Gonzales's Power Point](https://reader033.vdocuments.mx/reader033/viewer/2022050905/54c902814a795979348b45eb/html5/thumbnails/23.jpg)
Diagnostic Testing Ventilation perfusion scan (V/Q)
Q: Perfusion V: Ventilation
![Page 24: Dr. Gonzales's Power Point](https://reader033.vdocuments.mx/reader033/viewer/2022050905/54c902814a795979348b45eb/html5/thumbnails/24.jpg)
Diagnostic Testing: Right Heart Catheterization
Heart pressures RA, RV, PA, LVEDP
Cardiac output Valve competence Septal defects
![Page 25: Dr. Gonzales's Power Point](https://reader033.vdocuments.mx/reader033/viewer/2022050905/54c902814a795979348b45eb/html5/thumbnails/25.jpg)
Diagnostic Testing Pulmonary Angiography
![Page 26: Dr. Gonzales's Power Point](https://reader033.vdocuments.mx/reader033/viewer/2022050905/54c902814a795979348b45eb/html5/thumbnails/26.jpg)
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
![Page 27: Dr. Gonzales's Power Point](https://reader033.vdocuments.mx/reader033/viewer/2022050905/54c902814a795979348b45eb/html5/thumbnails/27.jpg)
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
![Page 28: Dr. Gonzales's Power Point](https://reader033.vdocuments.mx/reader033/viewer/2022050905/54c902814a795979348b45eb/html5/thumbnails/28.jpg)
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
![Page 29: Dr. Gonzales's Power Point](https://reader033.vdocuments.mx/reader033/viewer/2022050905/54c902814a795979348b45eb/html5/thumbnails/29.jpg)
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)
![Page 30: Dr. Gonzales's Power Point](https://reader033.vdocuments.mx/reader033/viewer/2022050905/54c902814a795979348b45eb/html5/thumbnails/30.jpg)
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
![Page 31: Dr. Gonzales's Power Point](https://reader033.vdocuments.mx/reader033/viewer/2022050905/54c902814a795979348b45eb/html5/thumbnails/31.jpg)
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
![Page 32: Dr. Gonzales's Power Point](https://reader033.vdocuments.mx/reader033/viewer/2022050905/54c902814a795979348b45eb/html5/thumbnails/32.jpg)
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)
![Page 33: Dr. Gonzales's Power Point](https://reader033.vdocuments.mx/reader033/viewer/2022050905/54c902814a795979348b45eb/html5/thumbnails/33.jpg)
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
![Page 34: Dr. Gonzales's Power Point](https://reader033.vdocuments.mx/reader033/viewer/2022050905/54c902814a795979348b45eb/html5/thumbnails/34.jpg)
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
![Page 35: Dr. Gonzales's Power Point](https://reader033.vdocuments.mx/reader033/viewer/2022050905/54c902814a795979348b45eb/html5/thumbnails/35.jpg)
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
![Page 36: Dr. Gonzales's Power Point](https://reader033.vdocuments.mx/reader033/viewer/2022050905/54c902814a795979348b45eb/html5/thumbnails/36.jpg)
Step-up Therapy CCB therapy is the first-line treatment
for patients with +vasoreactivity test
![Page 37: Dr. Gonzales's Power Point](https://reader033.vdocuments.mx/reader033/viewer/2022050905/54c902814a795979348b45eb/html5/thumbnails/37.jpg)
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
![Page 38: Dr. Gonzales's Power Point](https://reader033.vdocuments.mx/reader033/viewer/2022050905/54c902814a795979348b45eb/html5/thumbnails/38.jpg)
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
![Page 39: Dr. Gonzales's Power Point](https://reader033.vdocuments.mx/reader033/viewer/2022050905/54c902814a795979348b45eb/html5/thumbnails/39.jpg)
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
![Page 40: Dr. Gonzales's Power Point](https://reader033.vdocuments.mx/reader033/viewer/2022050905/54c902814a795979348b45eb/html5/thumbnails/40.jpg)
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
![Page 41: Dr. Gonzales's Power Point](https://reader033.vdocuments.mx/reader033/viewer/2022050905/54c902814a795979348b45eb/html5/thumbnails/41.jpg)
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
![Page 42: Dr. Gonzales's Power Point](https://reader033.vdocuments.mx/reader033/viewer/2022050905/54c902814a795979348b45eb/html5/thumbnails/42.jpg)
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
![Page 43: Dr. Gonzales's Power Point](https://reader033.vdocuments.mx/reader033/viewer/2022050905/54c902814a795979348b45eb/html5/thumbnails/43.jpg)
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
![Page 44: Dr. Gonzales's Power Point](https://reader033.vdocuments.mx/reader033/viewer/2022050905/54c902814a795979348b45eb/html5/thumbnails/44.jpg)
Mechanism of Action: NO in VQ Mismatched States
Adapted from Up to Date
![Page 45: Dr. Gonzales's Power Point](https://reader033.vdocuments.mx/reader033/viewer/2022050905/54c902814a795979348b45eb/html5/thumbnails/45.jpg)
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
![Page 46: Dr. Gonzales's Power Point](https://reader033.vdocuments.mx/reader033/viewer/2022050905/54c902814a795979348b45eb/html5/thumbnails/46.jpg)
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
![Page 47: Dr. Gonzales's Power Point](https://reader033.vdocuments.mx/reader033/viewer/2022050905/54c902814a795979348b45eb/html5/thumbnails/47.jpg)
NO for ARDS-Induced PHThe 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
![Page 48: Dr. Gonzales's Power Point](https://reader033.vdocuments.mx/reader033/viewer/2022050905/54c902814a795979348b45eb/html5/thumbnails/48.jpg)
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
![Page 49: Dr. Gonzales's Power Point](https://reader033.vdocuments.mx/reader033/viewer/2022050905/54c902814a795979348b45eb/html5/thumbnails/49.jpg)
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
![Page 50: Dr. Gonzales's Power Point](https://reader033.vdocuments.mx/reader033/viewer/2022050905/54c902814a795979348b45eb/html5/thumbnails/50.jpg)
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