Pulmonary Hypertension

Question 1

What is pulmonary hypertension defined as?

Answer 1

Mean pulmonary pressure of more than 25 mmHG at rest or more than 30 mmHg during exercise

Question 2

What are the causes of Pulmonary hypertension?

Answer 2

Increased Pulmonary Vascular resistance
Hypoixa
Embolism
Back pressure in venous system

(Mostly a secondary disease to other respiratory diseases)

Question 3

What are the different types of Pulmonary hypertension?

Answer 3

Group 1 : Disease directing affecting vasculature - interstitial lung diseae.
Group 2: Pulmonary Venous hypertension
Group 3: Secondary to Respiratory disease
Group 4: Secondary to Thrombotic disease

Question 4

Describe how group 3 pulmonary hypertension commonly occurs?

Answer 4

Group 3 pulmonary hypertension - Secondary to respiratory disease.
Hypoxic pulmonary vasoconstriction
e.g. COPD

Question 5

How does group 2 pulmonary hypertension occur?

Answer 5

Group 2: Pulmonary venous hypertension
Increased left trail pressure.
Associated with congestive heart failure

Question 6

How does group 4 pulmonary hypertension occur?

Answer 6

Secondary to thrombotic disease

e.g. embolism causing obstruction

Question 7

How does group 1 pulmonary hypertensions occur?

Answer 7

Group 1: Interstitial lung diseases

e.g. Pulmonary fibrosis/Sarcoid/ Sclerodema

Question 8

What is scleroderma?

Answer 8

Tightening of the skin
Hardening of the skin
Skin looks shiny

Question 9

What are the clinical features of pulmonary hypertension?

Answer 9

Calcinosis
Raynoids
Eosophageal dysmotility
Sclerodactyly - thickening of skin on fingers and toes
Telangiectasia - dilates vessels near the surface of the skin/eye

Question 10

What is the pathology of Pulmonary hypertension?

Answer 10

Remodelling of the pulmonary vasculature leading to thickening

Question 11

What are the symptoms of pulmonary hypertension?

Answer 11

Palpitations 
Chest Pains 
Light headedness 
Syncope 
Right ventricular dysfunction 
Jugular vein distension

Question 12

How is Pulmonary hypertension diagnosed?

Answer 12

Echocardiography
CXR
Pulmonary function testing

Question 13

How is Pulmonary hypertension treated?

Answer 13

O2 therapy for hyperaemic patients
Anticoagulants for patient with thrombi
Type 5 Phosphodiesterase (Sidenafil)
Nebulise Prostacyclin

Question 14

How does a type 5 phosphodiesterase work?

Answer 14

Increase cGMP causing Vasorelaxation

Reduces vascular remodelling