7. MTB Step 3 - Pulmonary Hypertension

Question 1

ETIOLOGY

What is the Cause of PRIMARY Pulmonary Hypertension?

Answer 1

IDIOPATHIC

Question 2

ETIOLOGY

What are (5) possible causes of SECONDARY Pulmonary Hypertension?

Answer 2

1. Mitral Stenosis
2. COPD
3. Polycythemia Vera
4. Chronic Pulmonary Emboli
5. Interstitial Lung Disease

Question 3

PRESENTATION

What is the Typical Presentation to look for in a patient with PRIMARY Pulmonary Hypertension?

Answer 3

Shortness of Breath (SOB), in a Young Woman, from overgrowth and obliteration of pulmonary vasculature, leading to decreased flow out of the right ventricle.

Question 4

PRESENTATION

What are (4) Physical Exam Findings with Pulmonary Hypertension?

Answer 4

1. Loud P2
2. Tricuspid Regurgitation
3. Right Ventricular Heave
4. Raynaud Phenomenon

Question 5

DIAGNOSIS

What are (2) Findings on TTE with Pulmonary Hypertension?

Answer 5

1. Right Ventricular Hypertrophy
2. Right Atrial Hypertrophy

Question 6

DIAGNOSIS

What are (3) Findings on ECG with Pulmonary Hypertension?

Answer 6

1. Right Ventricular Hypertrophy
2. Right Atrial Hypertrophy
3. Right Axis Deviation

Question 7

DIAGNOSIS

What is the Most ACCURATE Diagnostic Test for Pulmonary Hypertension?

Answer 7

Right Heart Catheterization

shows Increased Pulmonary Artery Pressure

Question 8

TREATMENT

What are (3) Endothelin Inhibitors used in Pulmonary Hypertension, and what is the Mechanism of Action (MOA)?

Answer 8

1. Bosentan
2. Ambrisentan
3. Macitentan

​Mechanism of Action (MOA) = competitive antagonist of endothelin-1 at the endothelin-A (ET-A) and endothelin-B (ET-B) receptors. Under normal conditions, endothelin-1 binding of ET-A receptors causes constriction of the pulmonary blood vessels.

Question 9

TREATMENT

What are (3) Prostacyclin Analogs used in Pulmonary Hypertension and what is their Mechanism of Action (MOA)?

Answer 9

1. Epoprostenol
2. Treprostinil
3. Selexipag - first PO prostacyclin agonist

Mechanism of Action (MOA) = exert their effects by promoting direct arterial vasodilation and inhibiting platelet aggregation.

Question 10

TREATMENT

What Medication used to treat Pulmonary Hypertension increases nitric oxide by stimulating guanylate cyclase; increases vasodilation by generating cGMP?

Answer 10

Riociguat