Pulmonary Hypertension

Question 1

What test should be used to screen for pulmonary HTN?

Answer 1

TTE

Question 2

Who should be screened for pulmonary hypertension?

Answer 2

Patients with:

1. scleorderma
2. Liver transplant candidate w portal hypertension
3. First degree relatives w familial PAH
4. Congenital heat disease with systemic to pulmonary shunts

Question 3

What is diagnostic criteria of pulmonary hypertension?

Answer 3

Mean pulmonary arterial pressure >=25 mmHg

Question 4

What is the most common causes of pulmonary hypertension?

Answer 4

1. Left sides heart disease

2. Hypoxia respiratory disease

Question 5

What is group 1 pulmonary HTN?

Answer 5

Localized to small pulmonary arterioles resulting in high pulmonary resistance

Question 6

What are causes of pulmonary HTN in groups 2-5?

Answer 6

1. Left sided heart failure
2. Respiratory disorders (copd, interstitial lung disease, portal hypertension, sleep-disordered breathing)
3. CTEPH
4. Amphetamines and cocaine use
5. Raynauds (SLE and SSc)

Question 7

What are findings of heart failure?

Answer 7

Symptoms of right heart failure

Unexplained dyspnea
Decreased exercise tolerance
Syncope
Chest pain
Lower extremity edema
Increased jvd (large a wave)
TR murmur

Question 8

What tests are run to check for pulmonary HTN?

Answer 8

1. ECHO (check pulm artery pressure >=40)
2. Right heart cath to confirm and quantify degree of PH

Possibly:
3. Left heart cath and coronary angiography to exclude LV dysfunction

Question 9

If suspect Pulmonary HTN in a or w history of congenital heart disease, what test should be run w ECHO?

Answer 9

Bubble contrast echo or tee to evaluate for intracardiac shunt (ASD)

Question 10

If diagnosis of pulmonary HTN is confirmed, what is the next step in management?

Answer 10

Vasoreactivity test using vasodilation agents to measure changes in pulmonary artery pressure w right heart cath in place.

Question 11

What are the diagnostics criteria for CTEPH?

Answer 11

1. Pulmonary arterial pressure (>=25) in absence of left sides failure
2. Imaging c/w chronic thromboembolism by V/Q scan.

Question 12

How does one treat pulmonary HTN of groups 2-5?

Answer 12

Treat underlying condition….can try group 1 treatment for symptomatic relief.

Question 13

A woman w unexplained dyspnea, decreased exercise tolerance, and lower extremity edema was found to have elevated pulmonary artery pressures on right heart cath with response to vasodilation from agents, what is best treatment?

Answer 13

CCBs

Question 14

A woman w dyspnea, near syncope, s3 sound, wide split s2 sound, found to have pulmonary artery pressure of systolic pressure 45, no response to vasodilation agent, what so the best therapy?

Answer 14

PDE-5 inhibitors (sildanefil, tadlafil)

Or endothelin receptor antagonists (bosentan, ambrisentan) — for mild to moderate disease

Question 15

What is the treatment for severe pulmonary hypertension?

Answer 15

Continuous effusion of epoprostenol (prostacyclin)

Question 16

Patient on continuous epoprostenol with no improvement, what is the next step?

Answer 16

Lung or heart transplantation

Question 17

If it w pulmonary HTN and o2 sat in office is 88%, what should be done?

Answer 17

Provide oxygen therapy

Question 18

Patients with pulmonary HTN, what are they At high risk for and what should be given for prevention?

Answer 18

Risk for pulmonary vascular thrombosis or embolism, anticoagulants!!!

Question 19

A person found to have multiple pulmonary perfusion and ventilation defects in v/q scan, what is definitive therapy?

Answer 19

Pulmonary thromboendartrecromy

And should be on lifelong anticoagulation