Pulmonary HTN

Question 1

What are the 5 WHO classifications of Pulmonary Hypertension (PH)?

Answer 1

1. Idiopathic PAH (pulmonary arterial hypertension)
2. Pulmonary Venous HTN (CHF)
3. P. HTN w/ associated resp. disorders or hypoxemia
4. P. HTN due to chronic thrombotic or embolic disease
5. P. HTN due directly to pulmonary vascular disease

Question 2

Main etiology associated with class 1 PAH.

Answer 2

Familial Disorders, mostly collagen diseases

• SLE
• scleroderma

Question 3

What does an EKG show with PAH and why?

Answer 3

Right Axis Deviation

-due to RVH

Question 4

Most important presenting symptom of PAH?

Answer 4

Dyspnea on exertion

-most PAH are non-specific but this one brings patients into the office

Question 5

What are the 4 WHO functional classifications of PAH?

Answer 5

1. No limit on physical activity
2. Slight limit of physical activity
3. Major limit on physical activity but no problems at rest
4. Dyspnea at rest and severe impaired ability to perform daily activities without symptoms

Question 6

Why does PAH lead to death?

Answer 6

In the early stages, the disease is asymptomatic and only manifests during exercise or during unusually demanding activities. However, over time there is a progressive reduction in cardiac output and increasing PVR. This eventually progresses to cardiac failure, leading to death.

Question 7

What is the best way to manage PAH?

Answer 7

Early detection: Family Hx is most indicative

Question 8

Best test to initially assess for PAH

Answer 8

Echocardiogram

-can evaluate RV systolic pressure (symptoms arise if pressure rises above 50mmHg, normal is about 20-25)

Question 9

What two conditions are associated with PAH and how can they be detected?

Answer 9

SLE and Scleroderma

• can be detected with blood tests
  1. SLE: Anti-nuclear-antibodies
  2. Scleroderma: Anti-Scl-70 antibodies

Question 10

What is the definition of PAH as measured on catheter study?

Answer 10

Pulmonary Artery Pressure above 25mmHg at rest or 30mmHg with exercise

AND

Normal pulmonary capillary wedge pressure

Question 11

What is the MOA of Prostacyclines?

Answer 11

• Direct vasodilation of pulmonary and systemic arterial vascular beds
• Inhibition of platelet aggregation

Question 12

What is a major complication of Prostacyclines?

Answer 12

rebound pulmonary hypertension with abrupt discontinuation of drug

Question 13

What is the MOA of Endothelial Receptor Antagonists?

Answer 13

A neurohormone, endothelin-1 mediates its effects by binding to endothelial receptors in the endothelium and vascular smooth muscle causing muscle relaxation

Question 14

MOA of Phosphodiesterase Inhibitors.

Answer 14

Inhibits PDE leading to high levels of cAMP or cGMP and vasodilation