L22 - Pulmonary HTN Flashcards
Pulmonary Hypertension is defined as:
- Increase in resting mean pulmonary arterial pressure ≥ ___ mmHg by right heart catheterization
- Normal pulmonary capillary wedge or left atrial pressure (less than or equal to ___ mm Hg)
- 25mmHg
2. less than or equal to 15mmHg
Pulmonary HTN is a (pathophysiological/hemodynamic) condition found in many clinical conditions
Both pathophysiological and hemodynamic
List the 5 groups in the WHO classification of PH
- Pulmonary arterial Hypertension
- PH due to LH disease
- Chronic lung disease
- Chronic thromboembolic PH (CTEPH)
- Unclear mechanisms
Group 2 is the most common cause of PH
Which group in the WHO classification of PH?
idiopathic, heritable, drug/toxin-induced, CT dz, HIV, portal HTN, CHD, schistosomiasis, hemolytic anemia, persistent PH of the new born
Group 1 PAH
Which group in the WHO classification of PH? (“pulmonary venous HTN”)
most common cause of PH
Group 2 (PH due to LH disease) is the most common cause of PH
Which group in the WHO classification of PH?
COPD, ILD, OSA, high altitude
Group 3 - Chronic Lung disease
Which group in the WHO classification of PH?
small vessel arteriopathy distal to thrombosis, persistent PH 6 mo after PE
Group 4 - Chronic thromboembolic PH
prevents clot formation in vessels by inhibiting platelet activation and vasodilates
prostacyclin - involved with vasoconstriction in the pathogenesis of PAH
This protein constricts or narrow blood vessels and increase pressure in the vessels.
endothelin - involved with vasoconstriction in the pathogenesis of PAH
3 processes that cause narrowing in PAH
- Vasoconstriction
- Smooth mm proliferation
- thrombosis
Which is NOT a symptom of PAH? A. Unexplained dyspnea B. Tachycardia C. Syncope D. Atypical chest pain E. Fatigue / exercise intolerance
B
Which 2 physical exam findings are characteristic of early stage PH?
- Diastolic murmur of PR
- Holosystolic murmur of TR
- Right ventricular gallop (right ventricular heave???)
- Accentuated P2 - second heart sound
- JVD, hepatojugular reflux and pulsatile liver
- Peripheral edema/ascites
- RV heave = left parasternal lift
4 and 7
The rest are advanced stage PH signs
A. What may be found on the 3 tests initially done in diagnosis of PAH are:
- ECG
- CXR
- Echocardiogram
B. Which is the best test?
A.
1. ECG – RV hypertrophy, right axis deviation , R atrial enlargement
2. CXR – Prominent vasculature in the hilum
3. Echocardiogram - Estimate PA systolic pressure; ID conditions that contribute to PH. D shaped septum, septum shifted L
• Best test
What is the Gold Standard for diagnosis of PAH?
Right Heart Cardiac Catheterization • Establish diagnosis differentiating pulmonary (arterial vs. venous) hypertension • Determine etiology • Exclude L heart disease • Assess L to R shunt • Establish severity & prognosis • Evaluate vasoreactivity • Adenosine, Flolan, NO • Guide treatment
- RH Catheterization pathway starting w/internal jugular.
- What do you expect to be the values of the following when doing a RHC on a patient with PH?
A. Wedge
B. RAP
C. RVP
D. mPAP
- through internal jugular –> RA –> RV –> PA –> inflate balloon and wedge it to get a sense of the LEFT sided heart pressures
2.
A. Normal (
Aside from ECG, CXR, echo, RHC, what are other diagnostic tests that can be done to diagnose PAH? (5)
- Serology (Gp 1) – ANA, HIV, liver function tests
• For CT disease - Sleep study (polysomnogram) (Gp 3)
- Pulmonary function tests, Chest CT scan, Arterial Blood Gas (Gp 3)
• Look for elevated PCO2 - VQ scan, pulmonary angiogram (Gp 4)
- Abdominal ultrasound
Which is not a predictor of poor survival in the ACCP IPAH Guidelines?
A. Poor reaction to PH therapies (PDE-5 inhibiotrs, endothelin receptor antagonists, prostanoids)
B. Advanced functional class
C. Poor exercise endurance (6 minute walk distance)
D. Syncope (sign of advanced disease)
E. Presence of pericardial effusion
F. Signs of RH failure (3)
A is not a predictor
Signs of RH failure
○ High Right Atrial Pressure
○ Low cardiac index - RVP so big that you are starting to have HF
○ High BNP (brain natriuretic peptide) level in blood
Determinants of Risk in patients with (lower risk/higher risk) of PH
- Clinical evidence of RV failure - No
- Progression - Gradual
- WHO class - II, III
- 6 min walk distance - longer (400+m)
- BNP - minimally elevated
- Echo findings - minimal RV dysfunction
- Hemodynamics - normal/near normal RAP and Cl
lower risk of PH
Determinants of Risk in patients with (lower risk/higher risk) of PH
- Clinical evidence of RV failure - yes
- Progression - rapid
- WHO class - IV
- 6 min walk distance - shorter (less than 300m)
- BNP - very elevated
- Echo findings - pericardial effusion, significatn RV dysfunction
- Hemodynamics high RAP, low Cl
higher risk of PH
List the WHO Functional class I-IV
Class I • No limitation of usual physical activity.
Class II • Mild limitation of physical activity. Normal physical activity causes dyspnea, fatigue, chest pain, or presyncope.
Class III • Marked limitation of physical activity. Less than ordinary activity causes dyspnea, fatigue, chest pain, or presyncope.
Class IV • Unable to perform any physical activity and who may have signs of RV failure. Dyspnea and/or fatigue may be may be present at rest.
T/F PAH Survival Without Treatment results in half the patients dying in 10 years.
False - 3 years
It is a very serious disease.
Which is not a goal of therapy in PAH treatment?
A. Improve hemodynamics - hemodynamics typically causes your symptoms
B. Improve exercise capacity
C. Improve functional class
D. Prevent clinical worsening
E. Improve survival
All are goals
What kind of therapy is this for PAH?
- oxygen
- diuretics - to help with RHF
- Anticoagulants
• Supportive therapies
What kind of therapy is this for PAH?
- Given if vasoreactivity during RHC
- Response: 10-20% adults
- High dose: Amlodipine, Diltiazem, or Nifedipine – limited by side effects
- Impressive survival when compared to patients without response
Ca Channel Blockers
What kind of therapy is this for PAH?
- Avoid overexertion
- Avoid pregnancy (strictly) during therapy - IPAH usually occurs during childbearing age
- Avoid high altitude (>4000 ft)
- Low sodium diet
- Fluid restriction
- Daily weight to guide diuretic therapy
Lifestyle changes
What kind of therapy is this for PAH?
- Atrial Septostomy
- Lung transplantation
Heroic measures
- Sildenafil
2. tadalafil
PDE-5 Inhibitors
- Bosentan
2. ambrisentan
Endothelin Receptor Antagonists
- iloprost (inhaled)
- epoprostenol (IV)
- treprostinil (SQ/IV/inhaled)
Prostanoids
T/F PH is most commonly due to LH disease
True
T/F PAH often is overdiagnosed
False - under diagnosed due to lack of awareness –> delayed treatment
Air flow stops for 10+ seconds
Apnea
Reduction in airflow for 10 seconds
Hypopnea
Severity of OSA
- mild = ___ Apnea +hypopnea/hour
- moderate = ____ Apnea +hypopnea/hour
- severe = ____ Apnea +hypopnea/hour
- Mild: 5-15 Apnea + Hypopnea / Hour
- Moderate: 16-30 Apnea + Hypopnea / Hour
- Severe: > 30 Apnea + Hypopnea /hour
Sleep-disordered breathing plus daytime hypersomnolence
Obstructive sleep apnea - in 2% F and 4% M
Which is not a symptom of OSA? 1. Snoring (not specific), Gasping or Choking (specific) ○ Dry mouth ○ Sore throat 2. Restless Sleep / Insomnia 3. Mood disturbances 4. Poor memory 5. Impaired work performance 6. Depression 7. Morning headaches (hypoventilation) 8. Nighttime sweating 9. Gastroesophageal reflux
6
T/F OSA is associated with HTN
True! It’s a major cause of PH
Diagnosis/Examination for OSA
- Epworth Sleepiness Scale
- Decreased weight
- High blood pressure
- Obstructed Nasal patency
- Oropharnyngeal findings
- Sleep study (polysomnogram)
- Increased weight (most are obese)
- Obstructed Nasal patency (e.g. allergic rhinitis, septal deviation)
- Oropharnyngeal findings (e.g. long soft palate/uvula, large tongue, large tonsils, small jaw)
Which is false on a person with OSA on polysomnogram (sleep study)?
- Arrhythmias
- Respiratory effort gets smaller and abdominal effort gets smaller
- Saturation goes down
- Respiratory effort gets bigger and abdominal effort gets bigger
What is this treatment for OSA? • Treatment of choice • Functions as an air splint • Effective in 90% • Long term compliance rates: 46% - 60 %
Nasal continuous positive airway pressure (CPAP)
- Benefits of nasal CPAP
2. SE
Benefits: • Improved alertness • Fewer traffic accidents • Fewer nocturnal cardiac arrhythmias • Lower blood pressure • Fewer depressive symptoms • Side effects: • Dry nose or throat, Heated humidification improves compliance
Obstructive Sleep Apnea Non-surgical Treatment: Anything that increases pharnygeal tone (4)
- Avoid Alcohol: – Alcohol causes airway narrowing AND increases apnea duration
- Avoid Sleep deprivation: – Sleep deprivation prolongs apneas
- Lose weight
- Positional Therapy:
○ Head of bed elevation
○ Avoid Supine Position
List surgeries available for OSA according to site:
- nose
- Oropharynx
- Trachea
- deviated septum correction
- UPPP (uvulopalatopharyngoplasty); genioglossus advancement; maxillary and mandibular advancement
- Tracheostomy bypasses the upper airway obstruction
Why is there no abdominal effort in central apnea?
The brain is not sensing that there is no airflow .
Central apnea is idiopathic or secondary to heart or lung disease
Which is false regarding sleep apnea and CHF
A. About 50 % of CHF patients have sleep-disordered breathing
B. Most have obstructive sleep apnea.
C. Crescendo-decrescendo respiratory pattern (Cheyne-Stokes)
D. Breathe fast then slow
E. Independent risk (four-fold increase) for death
B. central
Central VS Obstructive Apnea
- Airflow
- Respiratory effort
- absent in both
2. central (none), obstructive (active)
OSA in children
A. Peak age is 1 year old (due to lymphoid hyperplasia)
B. 2nd peak in adolescence (obesity a major risk factor)
C. Symptoms: loud snoring, daytime sleepiness, hyperactive, aggressive, attention problems, shy, learning problems
D. Diagnose with polysomnogram
E. Tx: tonsillectomy/adenoidectomy, weight loss, nasal steroids, decongestants, antihistamines, CPAP
A. 2-7 years
Which is NOT a risk factor of SIDS (sudden infant death syndrome)? A. Prone sleeping position B. Soft bedding C. maternal smoking D. drug abuse E. high body weight F. Siblings with SIDS
E. low body weight/premature
