Pulmonary hypertension

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What are the 5 groups of pulmonary hypertension (from WHO)
Group 1: pulmonary arterial hypertension (idiopathic, heritable, connective tissue disease related etc)
Group 2: pulmonary hypertension owing to left heart disease
Group 3: pulmonary hypertension owing to lung disease and/or hypoxia
Group 4: chronic thromboembolic pulmonary hypertension (CTEPH)
Group 5: pulmonary hypertension with unclear multifactorial mechanisms
How high does the pulmonary arterial pressure have to be to meet criteria for pulmonary arterial hypertension?
>25mmHg
What are the causes of group 1 pulmonary hypertension?
Idiopathic pulmonary hypertension
Heritable pulmonary hypertension
Drugs and toxins
Connective tissue disease
Congenital heart disease
Schistosomiasis
What do you see on examination of a pt with pulmonary hypertension?
Signs of R heart failure
Pulmonic component of 2nd heart sound has increased intensity and is widely split
Graham Steell murmur: pulmonic regurgitation murmur
Signs of R ventricular strain: R sided S3 gallop, tricuspid regurgitation murmur
How high does the estimate of pulmonary artery systolic pressure have to be on echocardiography to qualify for pulmonary hypertension?
>40mmHg
How high would the capillary wedge pressure be in a pt with pulmonary hypertension due to L heart disease?
>15mmHg
What's the gold standard investigation for pulmonary hypertension?
R heart catheterization
Which therapies should be considered in all pts with pulmonary hypertension, regardless of the group?
Diuretics
Oxygen
Anticoagulation
Digoxin
Exercise training
What are the advanced therapies that might be useful (particularly in group 1)?
Calcium channel blockers: nifedipine, amlodipine, diltiazem
Prostacyclin analogues: epoprostenol, trepostinil, iloprost
Endothelin 1 receptor antagonists: bosentan, ambrisentan
Phosphodiesterase inhibitors: sildenafil
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