Pulmonary Hypertension

Question 1

What is pulmonary hypertension.

Answer 1

It is defined as a mean pulmonar artery pressure of >25mmHg at rest, as measured on right heart catheterization.

Question 2

What are the causes of pulmonary hypertension. (5)

Answer 2

Pulmonary arterial hypertension.
Pulmonary hypertension owing to left heart disease.
Pulmonary hypertension owing to lung diseases and/or hypoxia.
Chronic thromboembolic pulmonary hypertension.
Pulmonary hypertension with unclear and/or multifactorial mechanisms.

Question 3

What are some causes of pulmonary arterial hypertension. (5)

Answer 3

Idiopathic. 
Drugs and toxins. 
Genetic (ALK1, BMPR2). 
Connective tissue disorders. 
HIV infection.

Question 4

What are some hypoxic causes of pulmonary hypertension. (4)

Answer 4

COPD.
Sleep disordered breathing.
Chronic exposure to high altitudes.
Developmental abnormalities.

Question 5

What are some systemic causes of pulmonary hypertension. (4)

Answer 5

Sarcoidosis.
Thyroid disease.
Chronic kidney disease.
Myeloproliferative disorders.

Question 6

What do patients with progressive PH develop. (3)

Answer 6

Right ventricular hypertrophy.
Right ventricular dilatation.
Death.

Question 7

What is the most common cause of pulmonary hypertension.

Answer 7

Idiopathic.

Question 8

What are the symptoms of pulmonary hypertension. (6)

Answer 8

Dyspnoea. 
Weakness. 
Angina. 
Syncope. 
Abdominal distention.

Question 9

What are the clinical signs of pulmonary hypertension and R heart hypertrophy. (10)

Answer 9

Left parasternal heave.
Loud P2.
Cyanosis.
Soft systolic murmur with tricuspid regurgitation.
Early diastolic murmur with pulmonary regurgitation.

Right heart failure leads to:
Raised JVP. 
Ascites. 
Peripheral oedema. 
Hepatomegaly. 

Clinical signs of associated systemic diseases.

Question 10

What does CXR show in pulmonary hypertension. (3)

Answer 10

Enlargement of pulmonary arteries.
Lucent lung fields.
May be right atrial and ventricular enlargement.

Question 11

What is seen on the ECG of a patient with pulmonary hypertension. (2)

Answer 11

Right ventricular hypertrophy and right atrial enlargement (p pulmonale).

Question 12

What is cor pulmonale.

Answer 12

Right heart failure caused by chronic pulmonary arterial hypertension.

Question 13

What are some causes of cor pulmonale. (4)

Answer 13

Chronic lung disease (COPD, bronchiectasis).
Pulmonary vascular disease (PE, sickle cell, pulmonary vasculitis).
Neuromuscular disease (myasthenia gravis, MND).
Skeletal disease. (kyphosis, scoliosis).

Question 14

What is seen on the blood tests of a patient with cor pulmonale. (2)

Answer 14

Raised Hb.
Raised haemataocrit.
(secondary polycythaemia).