Pulmonary Hypertension Lecture Powerpoint Flashcards
Pulmonary hypertension definition and value
- Disease state characterized by elevated pulmonary arterial pressure resulting over time in right ventricular failure due to the increased resistance
- MAP greater than 20mmHg at rest (normal 8-20)
Classification of pulmonary hypertension (5)
Group 1: pulmonary arterial hypertension
Group 2: Pulmonary venous hypertension (left sided heart failure)
Group 3: pulmonary hypertension assoc. with lung dz/hypoxemia (COPD patients)
Group 4: pulmonary hypertension due to chronic thrombotic or embolitic dz
Group 5: pulmonary hypertension with unclear multifactoral mech (sickle cell, sarcoidosis)
Pulmonary arterial hypertension (group 1) subclassifications
Includes idiopathic/familial hypertension, congenital heart disease, medication induced pulmonary hypertension, and pulmonary hypertension due to collagen vascular disease (any condition with known localizations of lesions of the small pulmonary artery vessels)
Idiopathic pulmonary artery hypertension and what group does it effect?
- Incurable disease characterized by progressive pulmonary vasoconstriction and remodeling by no underlying cause
- very rare and most commonly affects females in 30’s and 50’s, can see FPAH gene mutation in some patients
Associated Pathobiologic mechanisms with group 1 pulmonary hypertension (pulmonary arterial hypertension) (3)
- relative prostacyclin deficiency
- nitric oxide deficiency
- endothelin excess
Group 2 pulmonary hypertension (pulmonary venous hypertension)
Pulmonary hypertension owing to left sided myocardial disease, valvular disease, or compression of pulmonary veins associated with immediate increase in pulmonary arterial pressure to maintain forward blood flow thru lungs despite increase in pulmonary venous pressure
Group 3 pulmonary hypertension (pulmonary hypertension associated with lung disease)
Associated with lung disease such as COPD, impaired respiration such as hypoventilation disorders, or long term high altitude exposure
Group 4 pulmonary hypertension (pulmonary hypertension due to chronic thrombotic or embolic disease)
Occur in a patient with repeated pulmonary embolisms sometimes due to coagulopathy, as well as PE due to tumor or parasite being a possibility as well
Most common cause of sudden onset pulmonary hypertension with acute right ventricle failure
Acute pulmonary embolism
How is chronic thromboembolic disease treated? (2)
- Long term anticoagulants
- surgical removal
Group 5 pulmonary hypertension (pulmonary hypertension with unclear multifactoral mechanisms)
Includes conditions such as sarcoidosis, metabolic disorders, etc.
Cor pulmonale
Defined as heart disease 2ndary to lung disease of many causes (may be vascular or parenchymal), but ultimately results in pulmonary hypertension that leads to right ventricular failure
- Cor pulmonale is the ____ most common cardiac disease in patients greater than 50
- Many deaths from COPD actually result secondary to…
- 3rd
- ….to right ventricular failure
Symptoms of pulmonary hypertension (5)
- mostly asymptomatic in mild to moderate disease
- Dyspnea on exertion***
- Syncope on exertion
- cough or hemoptysis
- Hoarseness secondary to compression of left recurrent laryngeal nerve by dilated pulmonary artery (Ortner syndrome)
Physical exam findings of pulmonary hypertension (4)
- Increased intensity of pulmonic component of S2 (P2) louder than A2
- systolic ejection murmur from tricuspid regurgitation
- JVD
- hepatomegaly or ascites
Functional classification system of pulmonary hypertension (4)
Class I - no limitation on activity
Class II - mild discomfort with activity
Class III - marked limitation on activity
Class IV - dyspnea even at rest
Key diagnostic test to determine pulmonary hypertension, as well as some other ones (4)
- 2D echocardiography with doppler flow study
- chest x ray (often nothing is found)
- pulmonary function tests
- cardiac catheterization
- EKG
Right heart catheterization vs left heart
Right heart begin in the venous system (jugular, subclavian, femoral) and trace to the right side of the heart (can also get left sided thru pulmonary wedge pressure) while left side go thru arterial system to coronary arteries and left side of heart
EKG findings of pulmonary hypertension
- Initial + biphasic p wave in V1
- tall R wave in V1
- P >3mm in II
- Right axis deviation (neg lead I pos lead AVF)
- Bunny ears V1 or V2 (right bundle branch block)
- S1Q3T3 (S wave in lead I, Q wave and inverted T in lead III) super diagnostic for RV strain pattern in acute pulmonary emboli*******
Diagnostic study of choice to determine presence of thromboembolic disease
VQ (ventilation perfusion) scan to see areas that are being ventilated but not perfused due to clot followed by pulmonary angiography to find specific location
Why is pulmonary capillary wedge pressure useful?
In determining if a patient is a group 2 pulmonary hypertension because capillary wedge pressure measures left atrial pressure indirectly across the pulmonary vasculature
Pulmonary hypertension treatment options (3)
- diuretics (used with caution as CO is already low generally)
- o2 supplementation
- anticoagulants
Advanced therapy for pulmonary hypertension
Often used in group I patients that are deemed to be vasoresponders (tested via adenosine or nitric oxide administration during catheterization to see if drop occurs), if positive then can try a high dose Ca2+ channel blocker or PDE5 inhibitors
