Pulmonary HTN

Question 0

What is normal pulmonary artery pressure?

Answer 0

15-30mmHg systolic

4-12mmHg diastolic

Question 1

How is Pulmonary HTN defined?

Answer 1

a mean pulmonary arterial pressure (mPAP) ≥25 mmHg at rest confirmed by right heart catheterization

Question 2

What are the 5 types of pulmonary hypertension?

Answer 2

Type 1: Pulmonary artery hypertension
Type 1': Pulmonary vena-occlusive disease and/or pulmonary haemangiomatosis
Type 2: Due to left heart disease
Type 3: Due to lung disease or hypoxia
Type 4: Due to chronic VTE
Type 5: Multifactorial

Question 3

What are the symptoms of Pulmonary HTN?

Answer 3

Lethargy, fatigue, SOB, Dizziness, angina

Question 4

What are the clinical signs of Pulmonary HTN?

Answer 4

Split S2, Loud P2, Graham Steell murmur (PR), Cyanosis, Cor pulmonale: Raised JVP, pedal oedema, pulsatile liver, ascites

Question 5

What is the most common mutation that causes heritable pulmonary arterial hypertension?

Answer 5

BMPR2 - 80% of heritable cases

- autosomal dominant with incomplete penetrance

Question 6

What should be the first investigation to work up pulmonary HTN?

Answer 6

Echo

Question 7

What is the gold standard test for pulmonary HTN?

Answer 7

Right heart catheter study

Question 8

What treatment options should be considered for pulmonary HTN?

Answer 8

Oxygen, diuretics, anticoagulation, exercise, management of underlying condition

Question 9

How is pulmonary artery pressure measured with Echo?

Answer 9

By measuring the tricuspid regurgitation gradient + RA pressure

PASP = (4 x [TRV]2) + RAP

Question 10

What advanced treatments can be used to treat pulmonary hypertension?

Answer 10

Pulmonary vasodilators - calcium channel blockers
Sildenafil
Prostacyclins - e.g. inhaled iloprost
Endothelin receptor antagonists e.g bosenten

Question 11

What PASP makes a diagnosis of pulmonary hypertension likely?

Answer 11

PH is likely if the PASP is >50 and the TRV is >3.4

Question 12

What PASP makes a diagnosis of pulmonary hypertension unlikely?

Answer 12

PH is unlikely if the PASP is ≤36, the TRV is ≤2.8, and there are no other suggestive findings

Question 13

Drugs for treatment of PAH:

Sildenafil

Answer 13

Phosphodiesterase type 5 inhibitor.

Nitric oxide acts as a vasodilator & inhibits platelet aggregation + smooth muscle proliferation.

Question 13

Drugs for treatment of PAH:

Bosentan

Answer 13

Endothelin receptor antagonist.

Endothelin 1 is a vasoconstrictor & stimulates proliferation of pulmonary artery smooth muscle cells.

Question 14

Define pulmonary hypertension?

Answer 14

Pulmonary pressures >25mmHg at rest or >30mmHg on exertion

Pulmonary Artery Hypertension = capillary wedge pressure less than 15 (except in Group 2)

Question 15

Drugs for treatment of PAH:

Iloprost

Answer 15

Arachadonic acid metabolite

Prostaglandin is a potent vasodilator inhibiting platelet aggregation, + antiproliferative.

Question 17

Classes of pulmonary hypertension?

Answer 17

1) pulmonary artery hypertension
2) pulmonary vein hypertension
3) pulmonary hypertension associated with hypoxia
4) pulmonary hypertension secondary to embolic disease
5) miscellaneous