Pulmonary Hypertension Lecture Powerpoint

Question 1

Pulmonary hypertension definition and value

Answer 1

• 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)

Question 2

Classification of pulmonary hypertension (5)

Answer 2

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)

Question 3

Pulmonary arterial hypertension (group 1) subclassifications

Answer 3

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)

Question 4

Idiopathic pulmonary artery hypertension and what group does it effect?

Answer 4

• 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

Question 5

Associated Pathobiologic mechanisms with group 1 pulmonary hypertension (pulmonary arterial hypertension) (3)

Answer 5

• relative prostacyclin deficiency
• nitric oxide deficiency
• endothelin excess

Question 6

Group 2 pulmonary hypertension (pulmonary venous hypertension)

Answer 6

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

Question 7

Group 3 pulmonary hypertension (pulmonary hypertension associated with lung disease)

Answer 7

Associated with lung disease such as COPD, impaired respiration such as hypoventilation disorders, or long term high altitude exposure

Question 8

Group 4 pulmonary hypertension (pulmonary hypertension due to chronic thrombotic or embolic disease)

Answer 8

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

Question 9

Most common cause of sudden onset pulmonary hypertension with acute right ventricle failure

Answer 9

Acute pulmonary embolism

Question 10

How is chronic thromboembolic disease treated? (2)

Answer 10

• Long term anticoagulants

- surgical removal

Question 11

Group 5 pulmonary hypertension (pulmonary hypertension with unclear multifactoral mechanisms)

Answer 11

Includes conditions such as sarcoidosis, metabolic disorders, etc.

Question 12

Cor pulmonale

Answer 12

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

Question 13

• Cor pulmonale is the ____ most common cardiac disease in patients greater than 50
• Many deaths from COPD actually result secondary to…

Answer 13

• 3rd

- ….to right ventricular failure

Question 14

Symptoms of pulmonary hypertension (5)

Answer 14

• 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)

Question 15

Physical exam findings of pulmonary hypertension (4)

Answer 15

• Increased intensity of pulmonic component of S2 (P2) louder than A2
• systolic ejection murmur from tricuspid regurgitation
• JVD
• hepatomegaly or ascites

Question 16

Functional classification system of pulmonary hypertension (4)

Answer 16

Class I - no limitation on activity
Class II - mild discomfort with activity
Class III - marked limitation on activity
Class IV - dyspnea even at rest

Question 17

Key diagnostic test to determine pulmonary hypertension, as well as some other ones (4)

Answer 17

• 2D echocardiography with doppler flow study
• chest x ray (often nothing is found)
• pulmonary function tests
• cardiac catheterization
• EKG

Question 18

Right heart catheterization vs left heart

Answer 18

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

Question 19

EKG findings of pulmonary hypertension

Answer 19

• 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*******

Question 20

Diagnostic study of choice to determine presence of thromboembolic disease

Answer 20

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

Question 21

Why is pulmonary capillary wedge pressure useful?

Answer 21

In determining if a patient is a group 2 pulmonary hypertension because capillary wedge pressure measures left atrial pressure indirectly across the pulmonary vasculature

Question 22

Pulmonary hypertension treatment options (3)

Answer 22

• diuretics (used with caution as CO is already low generally)
• o2 supplementation
• anticoagulants

Question 23

Advanced therapy for pulmonary hypertension

Answer 23

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