Pulmonary HTN

Question 1

What is the definition for pulmonary hypertension?

Answer 1

Defined as mean pulmonary artery pressure > 20 mmHg and pulmonary vascular resistance (PVR) > 3 WU on right heart catheterization.

Question 2

What are the categories of pulmonary artery hypertension?

Answer 2

Group 1: PAH
Group 2: Due to LH disease
Group 3: Due to lung disease/hypoxia
Group 4: Chronic thromboembolic disease
Group 5: Miscellaneous

Question 3

What are the causes of Group I Pulmonary HTN?

Answer 3

• Idiopathic
• Heritable
• Drug/toxin Induced
• HIV
• CTD
• Schistosomiasis

Question 4

How do you treat Group I PAH?

Answer 4

• Vasoreactivity testing to determine whether they are a candidate for CCB
• PDE5 inhibitors (tadalafil)
• Riociguat (Soluble guanylate cyclase inhibitor)
• Endothelin receptor antagonist
• Prostanoid

Question 5

In which CTD do you need to screen annually for pulmonary HTN?

Answer 5

Scleroderma

Question 6

How do you screen patients with scleroderma for pulmonary HTN?

Answer 6

Yearly DLCO & echocardiogram

Question 7

When would you assess for Group 4 pulmonary HTN post-pulmonary embolism?

Answer 7

If there is residual dyspnea or exercise intolerance after at least 3 months of anticoagulation -> V/Q and CTPE

Question 8

What are the Class I investigations to consider in the workup of pulmonary HTN?

Answer 8

(1) Echocardiogram
(2) Routine biochem, CBC, immunology, HIV testing and TSH
(3) Abdominal U/S to screen for portal HTN
(4) PFTs & DLCO
(5) HRCT in all patients
(6) VQ scan in unexplained pulmonary HTN