Pulmonary Hypertension

Question 1

What group of PH is defined by an mPAP at rest > 25 mmHg, a PCWP of < 15 mmHg, an mPAP with exercise of > 30 mmHg, and a PVR > 3 wood units?

Answer 1

Pulmonary arterial hypertension (PAH)

Question 2

What group of PH is defined by an mPAP at rest of > 25 mmHg and a PCWP of > 15 mmHg along with underlying heart dysfunction?

Answer 2

WHO group 2: pulmonary venous hypertension

Question 3

An mPAP at rest of > 20 mmHg, and COPD, apnea, or ILD fall under which PH group?

Answer 3

Group 3: PH associated with lung disease

Question 4

What group of PH is thought to be driven by overexpression of endothelin or underexpression of prostacyclin and NO?

Answer 4

Group 1 PAH, all of which cause a rise in PVR

Question 5

Along with abnormal vasoconstriction, the rise of PVR in PAH is associated with what 3 things?

Answer 5

remodeling of the pulmonary vessel wall
in situ thrombosis
inflammation

Question 6

smooth muscle hypertrophy, smooth muscle dysfunction, impaired potassium channels and early intimal proliferation are hallmarks of reversible or irreversible PAH?

Answer 6

reversible

Question 7

What is thought to cause “out of proportion” PH in group 2?

Answer 7

fixed vascular remodeling (i.e. continued PH after diuresis and decreasing filling pressures in left heart

Question 8

a. Hypoxic vasoconstriction
b. Endothelial cell dysfunction with imbalance of vasodilators/vasoconstrictors
c. Destruction of capillary bed
These are all thought to be pathophysiologic for which group of PH?

Answer 8

group 3 PH

Question 9

Which group of PH is thought to be caused by chronic clotting of the pulmonary arterial bed?

Answer 9

group 4 CTEPH

Question 10

What is the gold standard for diagnosing PH?

Answer 10

right heart catheterization***

Question 11

Other than catheterization, how do you evaluate for CTEPH/

Answer 11

Ct angio, Pa gram, hypercoag serologies, VQ scan

Question 12

Endothelin receptor antagonists, phosphodiesterase inhibitors and prostacyclins treat what kind of PH?

Answer 12

Group 1

Question 13

Can you use pulmonary vasodilators in treating PH caused by underlying heart pathology?

Answer 13

It is not contraindicated, but not great

Question 14

How do you treat CTEPH?

Answer 14

thromboendoarterectomy CURATIVE

Question 15

What is used in bridging patients with CTEPH to curative surgery?

Answer 15

pulmonary vasodilators

Question 16

In addition to surgery and vasodilators, what should CTEPH patients receive?

Answer 16

anticoagulation**

Question 17

57 yo WM with history of coronary artery disease s/p CABG 5 years ago presents for evaluation of progressive dyspnea on exertion. His exam is characterized by 3+ LE edema. His echocardiogram reveals LV ejection fraction of 30%, his right ventricle is severely dilated with decreased function. His PASP is 75mmHg. He undergoes right heart catheterization with the following hemodynamics. Right atrial pressure 15mmHg, pulmonary artery pressure 87/30 (49), pulmonary capillary wedge pressure 23mmHg, Cardiac output 4 L/min.

1. What is this patients pulmonary vascular resistance?
   a. 1.75 Wood or 140 dynes
   b. 16 Wood or 1280 dynes
   c. 6.5 Wood or 520 dynes
   d. -2 wood or -160 dynes

Answer 17

The answer is c : (mpap – pcwp)/CO = X wood units * 80 = X dynes/cm/sec5

Question 18

57 yo WM with history of coronary artery disease s/p CABG 5 years ago presents for evaluation of progressive dyspnea on exertion. His exam is characterized by 3+ LE edema. His echocardiogram reveals LV ejection fraction of 30%, his right ventricle is severely dilated with decreased function. His PASP is 75mmHg. He undergoes right heart catheterization with the following hemodynamics. Right atrial pressure 15mmHg, pulmonary artery pressure 87/30 (49), pulmonary capillary wedge pressure 23mmHg, Cardiac output 4 L/min.
In approaching this patient with pulmonary hypertension what would be the most appropriate first step in management?
a. Start diuresis with a loop diuretic for heart failure
b. Start IV epoprostenol for pulmonary vasodilation to acutely decrease mean pulmonary artery pressure
c. Start the endothelin receptor antagonist, bosentan, to decrease pulmonary vascular resistance and decrease RV strain
d. Start full anticoagulation with coumadin

Answer 18

The answer is A; this patient has pulmonary venous hypertension with elevated left heart filling pressures.