Lectures6-10 Flashcards
Asthma is a chronic inflammatory disorder of the airways characterized by:
Paroxysmal symptoms of cough, wheezing, dyspnea and chest tightness, usually related to triggers
Airway narrowing that is partially or completely reversible
Why does asthma have increasing prevalence?
Improved hygiene, increased indoor air pollution, increased incidence of early-onset respiratory viral infections, survival or premature infants, increased awareness & recognition of asthma but pts and clinicians
What are some risk factors for asthma?
M>F, Low SES, urban dwellers, food allergies, family hx, atopy
What is atopy?
A genetic disposition to develop an allergic reaction and produce elevated levels of IgE upon exposure to an environmental antigen and especially one inhaled or ingested
What are some examples of atopy?
Atopic dermatitis, allergic rhinitis, asthma
Patho of asthma
Smooth muscle constriction around airways, airway wall edema, intra-luminal mucus accumulation, inflammatory cell infiltration of submucosa and basement membrane thickening
What immune cells are involved in asthma?
Eosinophils, activated helper T cells, mast cells, neutrophils
Fatal asthma
Severe collagen deposition of basement membrane, desquamation of epithelial lining with loss of ciliated cells, mucosal edema, airway smooth muscle hypertrophy, luminal plugging with inflammatory cells
What are the 3 main physiological consequences of asthma?
- Chronic airways inflammation
- Reversible or partially reversible bronchoconstriction
- Increased airways hyperresponsiveness to a variety of stimuli
What are the “classic’ sign and symptoms of asthma
Intermittent dyspnea, persistent cough, sudden onset or persistent wheezing
Additional features of asthma
Chest tightness, cold that take >10 days to resolve, apparent triggers, symptoms awake pt from sleep, exertional symptoms, seasonal, poor school performance and fatigue
The cough of asthma
Usually dry hacking, nocturnal, seasonal, response to specific exposures, lasts >3 weeks, frequently the sole complaint
What are some common asthma triggers?
Pollen, viral URIs, exercise, changes in air temp, perfumes, pets, molds, NSAIDs
What can be seen on PE for asthma?
Tachypnea, hypoxia if acute flare, cant speak full sentences without stopping to breath*, high-pitched musical wheeze
The wheeze of asthma
Initially with expiration but in severe cases also with inspiration
Critically severe asthma causes what?
Decreased breath sounds “silent chest/absent breath sounds”
Medical emergency: “the patient is very tight, not moving air”
Extrapulmonary PE findings associated with asthma
Pale, swollen nasal turbinates suggestive of allergic rhinitis, nasal polyps, atopic dermatitis
What diagnostic studies are used for asthma?
PFTs, spirometry with bronchodilator response testing, bronchoprovocation challenge, peak flow, CXR, allergy skin test
PFTs
Measurement of lung volumes, quantitation of diffusion capacity, measurement fo forced inspiratory and expiratory flow rates
What will PFTs show for an airway obstruction?
FEV1 decreased, FEV1/FVC ratio <70%
Vital capacity
Maximum amount of air a person can expel form the lungs after a maximum inhalation
What should you do if the baseline spirometry demonstrates an airway obstruction?
Administer albuterol 400mcg by MDI -> repeat spirometry 10 mins after
What suggests acute bronchodilator responsiveness?
Increase in FEV1 of >12%
What does the peak flow meter measure?
Peak expiratory flow
Peak flow meter
Effort dependent, useful for monitoring daily function during treatment of acute flare
What is needed to diagnose asthma?
Airflow obstruction symptoms, reversibility or obstruction, symptoms worse at night or early AM, prolonged expiration and wheezes on PE, limitation of airflow on PFT
Intermittent asthma severity
<2 days week symptoms and use of rescue inhaler
Mild persistent asthma
> 2 days/week symptoms and use of rescue inhaler (but not daily)
Moderate persistent asthma
Daily symptoms and use of rescue inhaler, some limitation to normal activity
Severe persistent asthma
throughout the day symptoms and use of rescue inhaler, extremely limited activity
What will the PFTs show for intermittent asthma?
Normal FEV1 between exacerbations, FEV1/FVC >.85
What will PFT show for mild persistent asthma?
FEV1 normal, FEV1/FVC >.8
What will PFT show for moderate persistent asthma?
FEV1 60-80%
FEV1/FVC 0.75-0.8
What will PFT show for severe persistent asthma?
FEV1 <60%
FEV1/FVC
What are the components of asthma management?
Routine monitoring of symptoms and lung fxn, patient education, controlling environmental triggers, pharmacological therapy
What is the treatment for intermittent asthma?
SABA prn
What is the treatment for persistent asthma (step 2)
Low dose ICS
What is step 3 treatment of asthma
either low dose ICS + LABA or med dose ICS + LABA
What is step 4 treatment of asthma
Med dose ICS + LABA
What is step 5 treatment of asthma
High dose ICS + LABA
What is step 6 for treatment of asthma?
High dose ICS +LABA + short course of oral systemic steroids
What is the treatment for acute asthma exacerbation?
02 between 90-96%, Methylprednisolone or prednisone, Albuterol (short acting bronchodilator), Magnesium IV
Can COPD and asthma co-exist?
Yes
What are some conditions that can exacerbate asthma?
Allergic rhinitis, GERD, cigarette smoking, obesity
COPD
A common preventable and treatable disease characterized by persistent respiratory symptoms and airflow limitation that is due to airway and/or alveolar abnormalities usually caused by significant exposure to noxious particles or gases
What are 3 subtypes of COPD
- Emphysema
- Chronic bronchitis
- Chronic obstructive asthma
What is Emphysema?
Enlargement of air spaces and destruction of lung tissue
What is chronic bronchitis?
Obstruction of small airways
COPD differences from asthma
Usually from smoking, diagnosed at 50-60YO, obstruction is either irreversible or partially reversible with bronchodilator therapy
Asthma differences from COPD
Associated with atopy, diagnosed in childhood usually, reversible with bronchodilator therapy
Pathophys of COPD
Inflammation and fibrosis of bronchial wall, hypertrophy of submucosal glands, hypersecretion, loss of elastic fibers and alveolar tissue
What does COPD result in?
Airways obstruction, decreased surface area for gas exchange and mismatching of ventilation and perfusion
What does loss of elastic fibers cause in COPD?
Impairs expiratory flow, leads to air trapping, predisposes to alveolar collapse
What are common triggers of exacerbations for COPD?
Pulmonary infections
What are some risk factors for COPD?
Cigarette smoking*** airway hyperresponsiveness, biomass fuel exposure, 2nd hand smoke, ambient air pollution, genetics- alpha-1-anti-trypsin deficiency for COPD in young pts
Chronic bronchitis
Excessive secretion of bronchial mucus, chronic daily cough for 3mos
What may proceed or follow development of airflow limitation?
Chronic Bronchitis
What is an abnormal and permanent enlargement of the airspace’s that is accompanied by the destruction of the airspace walls & capillary beds?
Emphysema
Loss of elasticity
Emphysema
What are the two types of emphysema?
- Proximal acinar
2. Panacinar
Proximal acinar emphysema
Initial preservation of alveolar ducts and sacs
Panacinar emphysema involves what?
Involves both bronchioles and alveoli
What type of emphysema has an abnormal dilation or destruction of the respiratory bronchiole?
Proximal acinar (centrilobular)
What is proximal acinar emphysema commonly associated with?
Cigarette smoking
What can be seen in coal workers pneumoconiosis?
Proximal acinar emphysema
Which type of emphysema has enlargement or destruction of all parts of the acinus?
Panacinar emphysema
Panacinar emphysema is associated with that?
Alpha-1 antitrypsin deficiency
What can be seen on CXR for emphysema?
Hyperexpanded lungs, flattening of the diaphragm, volume is much longer looking
What else can be seen in CXR for emphysema that can lead to pneumothorax?
Blebs
What are some symptoms of COPD?
Chronic cough, sputum production, exertional dyspnea, wheezing, chest tightness, weight gain or weight loss
When do patients typically present with COPD symptoms?
5th or 6th decade of life, symptoms usually present at that point for around 10 years
PE findings for COPD
Prolonged expiratory phase, wheezing, barrel chest, enlarged lung volumes, respiratory distress in severe exacerbation, tripod position, pursed lips, cyanosis, weight loss, signs of RHF
Who are the pink puffers?
Emphysema
Who are the blue bloaters?
Chronic bronchitis
Pink puffer si/sxs
Dyspnea major complaint* scant clear mucus, thin, accessory muscle use, chest is quiet or soft-pitched wheeze
Blue bloater si/sxs
Chronic cough major complaint* mucopurulent sputum, frequent COPD exacerbations due to infections, mild dyspnea, noisy chest with Rhonchi and wheezing
What can be used to diagnose COPD?
Hx of SOB, cough, wheeze, smoking, labs: bicarb, CXR to rule out other causes, PFTs
What does serum bicarb identify for COPD pts?
Chronic hypercapnia in chronic disease
What else can be drawn for labs to test for COPD?
AAT deficiency, Hgb and BNP to rule out other causes of dyspnea
What PFTs should be used to diagnose COPD?
Spirometry, measurement of lung volumes, DLCO, forced inspiratory and expiratory flow rates
FEV1 measures what?
Forced expiratory volume in one second
FVC measures what?
Forced vital capacity
What is the vital capacity?
Maximum amount of air a person can expel from lungs after a max inhalation
What is the FRC?
Functional residual capacity, the volume of gas w/in the lungs at end of expiration during normal tidal breathing at rest
Conditions that decrease the DLCO
Anemia, emphysema, pulmonary HTN, recurrent PEs, interstitial lung disease
DLCO helps distinguish between when for obstructive lung diseases?
Between emphysema and chronic bronchitis or asthma
What will the PFTs show for obstructive lung disease?
FEV1 and FEV1/FVC ratio decreased, FVC normal, FEV1 improves with bronchodilators
What are the non-pharmacological treatments for COPD?
Stop smoking, reduce risk factors, vaccinations, oxygen therapy, pulm rehab
What is the 1st line treatment for newly diagnosed COPD?
Rescue inhaler! SABA: Albuterol or Levalbuterol, short acting anticholinergic: Ipratropium
Combo SABA + Ipratropium: Combivent**
What is most commonly prescribed for new diagnosis of COPD?
Combivent*** Combo of SABA + Ipratropium
What are the si/sx for an acute exacerbation of COPD
SOB, frequent cough with sputum, wheezing, associated with URI or pulm infection, minimal to no improvement with rescue inhaler
PE findings for COPD acute exacerbation
Respiratory distress, accessory muscle use, tripod position, pursed lips, hypoxia, tachypnea, wheezing, poor air movement, crackles if pneumonia
What is used to treat an acute COPD exacerbation?
O2 between 90-96, Prednisone PO or Methyprednisolone IV, Ipratropium and Albuterol nebulizer, Abx
What Abxs can be used for an acute exacerbation of COPD?
Levo and Azithromycin; want to cover atypicals
Who should be hospitalized for COPD flare?
Increased dyspnea, inability to eat or sleep, new cyanosis or hypoxia, acute respiratory distress, mental status change, insufficient home support, frequent exacerbations, high risk comorbidities
What can be seen when a pt is in acute respiratory distress?
Accessory muscle use, tachypnea, tripod position
Hospital admission for COPD
O2, Prednisone daily with a taper, Duoneb, Albuterol nebulizer, Levofloxacin or Azithromycin daily
When should you consider adding maintenance meds for COPD?
Frequent chronic symptoms, frequent exacerbations, disease progression
Which types of meds are used for maintenance for COPD?
LABA, LABA + ICS, LAMA, chronic steroids, chronic O2
What LABA is used for maintenance for COPD?
Salmeterol inhaler
What LABA + ICS is used for COPD maintenance?
Salmeterol/Fluticasone (Advair) inhaler
What LAMA is used for COPD maintenance?
Triotropium (Spirivia)
When are chronic steroids and oxygen used for COPD maintenance?
End stage disease
What is 1st line treatment as “rescue inhaler” to manage COPD symptoms?
Short acting bronchodilators
What is the most common cause of bronchiectasis?
Cystic fibrosis causes about 50% of cases
What else can cause bronchiectasis?
Infections: TB, fungal, lung abscess, localized airway obstructions
What is bronchiectasis?
Disorder of the large bronchi where there is permanent, abnormal dilation and destruction of the bronchial walls, localized or diffuse
What disease does bronchiectasis share clinical features?
COPD
What has a chronic daily cough and viscid sputum production?
Bronchiectasis
What other clinical features are seen with bronchiectasis?
Bronchial wall thickening, luminal dilatation, frequent office visits and hospitalizations
What is the pathophys belong bronchiectasis?
Infection -> host response -> transmural inflammation, mucosal edema, cratering, ulceration, neovascularization -> impaired drainage, permant dilatation and destruction of bronchi and walls
Recurrent infections are common with bronchiectasis, leading to what?
Further damage of airways
Which disease involves the abnormal transport of chloride and sodium across epithelium leading to thick, viscous secretions?
Cystic fibrosis
What type of infections are most common for cystic fibrosis patients?
Pseudomonas aeruginosa infections
How is cystic fibrosis diagnosed?
Sweat chloride test
Clinical features of bronchiectasis
Cough, daily production or mucopurulent and tenacious sputum x mos-yrs, repeated respiratory tract infections
Symptoms of bronchiectasis
Cough w/sputum, dyspnea, rhinosinusitis, hemoptysis, recurrent pleurisy, fatigue, urinary incontinence (stress incontinence)
PE of bronchiectasis
Chronic pulmonary crackles, wheezing, rarely digital clubbing
Lab tests utilized for bronchiectasis
CBCD, IgG, IgM, IgA to look for immunodeficiency, antibody tigers after pneumococcal vaccine, sputum gram stain and CX, sweat chloride testing
Why are the antibody titers drawn after a pneumococcal vaccination?
To assess the immune response to vaccination
What imaging studies are good for bronchiectasis?
CRX, CT
What will CXR show for bronchiectasis?
Liner atelectasis, dilated, thickened airways, irregular peripheral opacities (mucopurulent plugs)
What will be seen on chest CT for bronchiectasis?
Airway dilatation, bronchial wall thickening, mucopurulent plus, cysts of bronchial wall (destructive bronchiectasis)
What will PFTs show for bronchiectasis?
Reduced FEV1/FVC, low FVC in advanced disease, 6 min walk test
What is the treatment for bronchiectasis?
Treat underlying disease, pneumonia or other infection, HIV, prevent aspirations if possible, immunizations
How do you treat an acute exacerbation of bronchiectasis?
Oral Abxs, nebulizer: hypertonic saline to thin secretions, chest physiotherapy, PEP device to loosen secretions, bronchodilators, pulm rehab
What is the PEP device?
Oscillatory Positive expiratory pressure device used to loosen secretions for bronchiectasis
What is virchow’s triad?
- Alterations in blood flow (stasis)
- Vascular endothelial injury
- Alterations in constituents of blood (inherited or acquired hypercoagulable states)
What are the 3 classifications of pulmonary embolisms?
Acute, subacute, and chronic
Acute pulmonary embolism
Signs and symptoms develop immediately after obstruction of pulmonary vessels prompting evaluation
Subacute pulmonary embolisms
Patient present days or weeks following initial event
Chronic pulmonary embolisms
Slowly develop symptoms of pulmonary HTN over many years
What can a massive or submassive PE cause?
Hemodynamic instability, hypotension, severe RV failure, death
What will a patient who is hemodynamically unstable need?
Fibrinolytic therapy (thrombolytic)
What are the possible locations of pulmonary embolisms?
Saddle, Segmental, Subsegmental
Saddle PE
In both right and left bronchi
Segmental PE
Small branch effected
Subsegmental PE
Small branch off of a branch is effected
What are some common risk factors for PE?
Post op, sedentary state, malignancy, hx of VTE, pregnancy, oral contraceptives, obesity, smoking, HTN, hypercoagulable disorders
What are some examples of inherited thrombophilia?
Factor V Leiden mutations, prothrombin gene mutation, deficiency of proteins C and S, amtithrombin 3 deficiency, anticardiolipin antibodies
Venous thrombi dislodge and travel where?
To lungs, arterial system if there is a patent foramen ovals or atrial septal defect, can cause stroke or arterial embolization
Symptoms of PE
Sudden SOB, pleuritic chest discomfort, heart palpitations
PE findings for pulmonary embolism
DVT: unilateral swelling, erythema, calf tenderness, tachypnea, hypoxia, tachycardia
What is used to determine the likelihood of a PE?
Wells Score
What is your change of a PE if the Wells score is <2?
15%
What is your change of PE if the Wells score is 2-5?
29%
What is your chance of PE if your Wells score is >6?
59%
Only use D-Dimer when?
For low probability patients when ruling out DVT and low to mod probability when ruling out PE
What imaging modalities are used to diagnose PE?
Chest CTA with contrast* CXR, VQ scan, pulm angiography
“Hampton’s Hump Sign”
Wedge shaped infarction from PE seen on CXR
CTA advantages for diagnosing PE
Readily available, fast, minimally invasive, can detect alternative diagnosis
CTA disadvantages
Expensive, radiation exposure, C/I️ in pts with renal failure, contrast allergies and pregnancy
Who is a CTA C/I️ in?
Renal failure, contrast allergies and pregnancy
Venous ultrasonography relies on what?
The loss of vein compressibility, occasionally the thrombus can be directly visualized
EKD features associated with PE
sinus tachycardia, S1, Q3, T3, complete or incomplete RBBB, non-specific ST or T wave changes
What can an echocardiogram show for a PE?
Evidence of right heart strain, RV enlargement or RV dysfunction
Approach to treatment of venous thromboembolism
Primary therapy: clot dissolution with thrombolytic or removal by embolectomy
Secondary: anticoagulation or placement of IVC filter
Who are high risk patients for PE?
Hemodynamic instability, RV dysfunction, RV enlargement, elevated troponin
What is the anticoagulation treatment for VTE?
Parenteral drug, can bridge to warfarin
Which anticoag should cancer patients stay on?
LMWH long term
Provoked clot antiocagulation period
3-6 mos
Unprovoked clot anticoagulation period?
Indefinite anticoagulation
What are the indications for an IVC filter?
Active bleeding, recurrent venous thrombosis despite intensive anticoags, high risk pts who cannot have fibrinolytics
What are the complications for IVC filters?
Caval thrombosis causing marked bilateral leg swelling, double DVT rate
Prevention of VTE
Heparin or LMWH at lower doses for hospitalized pts, warfarin used peri-operatively
What are some major adverse outcomes of a PE?
Recurrent thromboembolism, pulmonary HTN, death
What is defined as the abnormal elevation in pulmonary artery pressure?
Pulmonary HTN
What is the normal PAP (pulmonary artery pressure)
8-20 mmHg measured by R heart catheterization
Pulmonary HTN =
MEan pulmonary artery pressure >25mmHg
PAH is characterized by what?
Dyspnea, chest pain, syncope
Pulmonary HTN can be caused by which 3 things?
- Increased pulmonary vascular resistance
- Elevated L arterial pressure
- Increased pulmonary blood flow
What is the patho behind PAH?
Progressive increase in RV afterload results in RV hypertrophy -> eventually RV dilates resulting in decreased contractility, CO will fall
What is the first study to order for PAH?
Transthoracic echocardiogram
PH is likely if the PASP is estimated at what?
> 50mmHg and tricuspid regurg velocity is >3.4 sec
PH is unlikely is PASP is estimated what?
<36mmHg and tricuspid regurg is <2.8 sec
What is the gold standard and necessary to diagnose PH?
R heart catheterizaiton
What is R heart catheterization?
Catheter guided to right side and passes into pulmonary artery, measure pressure
What does the R heart catheter measure the pressures of?
R atria, R ventricle and pulmonary artery
What is the pulmonary capillary wedge pressure? (PCWP)
Indirect measurements of L heart pressures by inflating a balloon in the pulmonary artery
What is group 1 pulmonary HTN?
Secondary to diseases that localize to small pulmonary arteries/arterioles
What is group 2 PH?
Secondary to L heart disease
What is group 3 PH?
Secondary to lung disease or hypoxemia
What is group 4 PH?
Secondary to thromboembolic disease
What is group 5 PH?
Unclear multifactorial mechanisms
What is WHO?
World Health Organization, functional classification for pulmonary HTN
What is the WHO 1 classification for pulmonary HTN?
No limitations of physical activity. No fatigue or dyspnea, chest pain, or heart syncope
What is the WHO 2 classification for pulmonary HTN?
Slight limitation of physical activity, comfortable at rest. Ordinary physical activity results in undue fatigue or dyspnea, chest pain, or heart syncope
What is the WHO 3 classification for pulmonary HTN?
Marked limitation of physical activity. They are comfortable at rest. Less than ordinary physical activity causes undue fatigue or dyspnea, chest pain, or heart syncope
What is the WHO 4 classification for pulmonary HTN?
Inability to carry on any physical activity without symptoms, signs of RHF. Dyspnea and/or fatigue may be present even at rest.
Group 1 characteristics
Abnormalities in pulmonary endothelial and smooth muscle cells: vasoconstriction, vascular proliferation, thrombosis, inflammation
What type of dysfunction effects the layers of vascular wall in group 1PH?
Vasoconstriction, vascular proliferation, thrombosis, inflammation
What criteria must be met for someone to be in PH group 1?
Mean PAP >25 mmHg at rest, mean pulmonary capillary wedge pressure <15mmHg, chronic lung disease is mild to absent, VTE disease is absent, other disorders absent
Causes of group 1 PH
Idiopathic, hereditary, drugs or toxins, connective tissue diseases, HIV, portal HTN, congenital heart disease
What types of drugs or toxins cause group 1 PH?
Appetite suppressants, cocaine, amphetamines, meds
What type of connective tissue diseases cause group 1 PH?
Systemic sclerosis, narrowing arteries/arterioles, interstitial fibrosis
Group 1 PH caused by what?
LH disease: L atrial HTN requires increased pulm artery systolic pressure to maintain adequate driving force across pulm vasculature
Causes of PH group 3?
COPD, interstitial lung disease, obstructive sleep apnea, other causes of hypoxemia
Group 4 PH causes
Chronic thromboembolic disease, multiple small PE, decrease in function or blood vessels
Group 5 PH causes
Pulm HTN with unclear mechanisms: hematologist disorders, systemic disorders, metabolic disorders, miscellaneous
What type of hematologic disorders can cause PH group 5?
Myeloproliferative disorders
Which systemic disorder can cause PH group 5?
Sarcoidosis
What metabolic disorder can cases PH group 5?
Glycogen storage disease
What miscellaneous disease can cause PH group 5?
Sickle cell disease
What is the definition of cor pumonale?
Altered structure and/or impaired function of the RV that results from pulmonary HTN
What are some causes of cor pulmonale?
Pulm disease (COPD), vasculature (PAH),, upper airway (OSA), chest wall (kyphoscoliosis)
What is the patho of cor pulmonale?
RH failure from longstanding pulmonary HTN, RV hypertrophy develops due to high pressure, eventually RV loses contractility
What is the main cause of death in patients with PAH?
Circulatory collapse (existence in group 1 PAH is bad prognosis)
Si/sx of pulmonary HTN
DOE, dyspnea at rest in advanced disease, fatigue
Si/sx of right heart failure
Angina, exertional syncope, peripheral edema, abdominal discomfort due to hepatic congestion
Why is angina a sign of right heart failure?
R heart requires more oxygen
Why does exertional syncope happen with right hear failure?
Inability to increase cardiac output
What are some PE findings for pulmonary HTN?
Increased intensity of S2 in pulmonic area, RV failure widens S2 split, S3, high pitched sytsolic murmur of tricuspid regurg, elevated JVP, hepatomegaly, peripheral edema, ascites
What diagnostic tests are used for pulm HTN?
CXR, EKG, Echo, PFTs, overnight oximetry, polysomnography, V/Q scan
What is assessed in the echo for pulmonary HTN?
Assess PASP, R heart size and assess for L to R shunts, can diagnose LV dysfunction
What are some echo results that would point to pulmonary HTN?
Enlarged RV, small D-shaped LV, flattened septum
Treatment for pulmonary HTN?
Early id and treatment ideal, assess disease severity, primary: directed at underlying cause
Advanced: direct at PH itself
What is the treatment for group 1 PAH?
No effective primary therapy, advanced therapy often needed
PAH treatment
Diuretics, anticoagulation, CCBs, endothelin receptor blockers, PDE-5 inhibitors, prostacyclin, lung transplant
What is the primary treatment for group 2 PH?
HF treatment
What is the primary treatment for group 3 PH?
Treat underlying cause of hypoxemia and correction of hypoxemia with supplemental O2
What is the primary treatment for group 4 PH?
Anticoagulation, surgical thromboendarterectomy for selected pts
What is the primary treatment for group 5 PH?
Target specific underlying cause
What are some things to consider in all groups for PH?
Diuretics treat fluid retention, O2 can benefit all groups, exercise
Which groups of PH can anticoagulation be used for?
IPAH, hereditary PAH, drug-induced PAH or group 4 PH
Digoxin improves what?
RV EF of pts with group 3 PH and helps control HR in pts who have SVTs associated with RV dysfunction
Who is advanced therapy for?
Persistent PH and WHP class 2 3 or 4, should be administered at specialized centers where clinicians are experienced
Group 1 PAH advanced therapy
First line
Group 2 PH advanced therapy
Possibly harmful, L heart unable to handle increased flow
Advanced therapy group 3 PH
Not FDA approved, not recommended, occasionally considered
Advanced therapy in group 4 PH
Can be considered
Advanced therapy in group 5 PH
Favorable response in sarcoidosis
What is the vasoreactivity test?
Administration of short-acting vasodilator, measure hemodynamic response using RH catheter
What agents are commonly used for the vasoreactivity test?
Epoprostenol, Adenosine, and inhaled Nitric Oxide
What is the vasoreactivity test done for?
Pts who are selected for advanced therapy to determine if they will benefit from CCBs
What is the treatment if the vasoreactivity test is positive?
Trial of oral CCBs, they are less expensive and fewer side effects than other therapies
What is the treatment if the vasoreactivity test is negative?
Prostanoid formulations, endothelin receptor antagonists, PDE-5 inhibitors
What are the benefits of using CCBs?
Prolonged survival, sustained functional improvement, Hemodynamic improvement
Epoprostenol benefits
Improves hemodynamics, functional capacity, and survival in IPAH
What is the only therapy that has been shown to prolong survival?
Epoprostenol
What are the prostanoid formulations?
Epoprostenol, Treprostinil, Iloprost
Endothelin receptor antagonists
Endothelin is a potent vasoconstrictor found in high concentrations in lungs of pts with IPAH and other group 1 PAH
What are the endothelin receptor antagonists?
Bosentan, Macitentan, Ambrisentan, and Sitaxsentan
What are the benefits for endothelin receptor antagonists?
Improve exercise capacity, dyspnea, and Hemodynamic measures
What are the PDE-5 inhibitors?
Sildenafil, Tadalafil
What are the benefits of the PDE-5 inhibitors?
Prolong vasodilatory effect of nitric oxide, also used to treat ED, improves pulmonary hemodynamics and exercise capacity
What is the function of the guanylate cyclase stimulant?
NO receptor stimulators, increase sensitivity of receptor to NO, directly stimulate the receptor to mimic action of NO
What is the example of the Guanylate cyclase stimulant?
Riociguat
