Pulmonary hypertension is defined as a mean pulmonary artery pressure at cardiac catheterisation of 25mmHg or more.
Presentation tends to be progressive breathlessness, and as the condition worsens exertional dizziness and syncope. Oedema and ascites are late signs.
On examination you might find a loud P2, a systolic murmur of TR, raised JVP, oedema or ascites.
It is classified into 5 groups (NB not all subsets of each of the groups are listed below, just the main ones):
1. Pulmonary arterial hypertension
- idiopathic (=IPAH)
- heritable - including gene BMPR2
- drug/toxin induced - eg fenfluramine, amphetamines, possibly interferon alpha and beta
- CTD
- HIV infection
- portal HTN
- schistosomiasis
- pulmonary veno-occlusive disease
2. Pulmonary hypertension due to left heart dysfunction
3. Pulmonary hypertension due to lung disease/chronic hypoxia
- COPD
- interstitial lung disease
- sleep-disordered breathing
4. Chronic thromboembolic pulmonary hypertension (CTEPH)
- between 0.5 and 4% of patients develop CTEPH after PE
5. Pulmonary hypertension with unclear multifactorial mechansms
- haematological disorders - chronic haemolytic anaemia
- sarcoidosis
Prevalence is high in:
- systemic sclerosis (7-12%)
- congenital heart disease (up to 10%)
- portal hypertension (up to 6%)
- HIV (0.5%)
Investigations include: cardiac echo, CXR (cardiomegaly and prominent pulmonary vasculature), BNP, and the definitive investigation is right heart catheterisation. Investigations would also be undertaken to look for the cause.
Treatment:
- phosphodiesterase-5 inhibitors such as sildenafil or tadalafil
- endothelin receptor antagonists such as bosentan
- prostanoid infusions such as epoprostenol (avoid stopping as this can cause death)
- possibly pulmonary endarerectomy in CTEPH
- possibly high dose calcium channel blockade in IPAH
References
Humbert, M. et al. Guidelines for the diagnosis and management of pulmonary hypertension. European Heart Journal. 2009; 20: 2493-2537.
Kiely, D. et al. Pulmonary hypertesnion: diagnosis and management. BMJ. 2013; 346:f2028.
Simonneau, G. et al. Updated Clinical Classification of Pulmonary Hypertension. J Am Coll Cardiol. 2013; 62(25 Suppl):D34-41.
Humbert, M. et al. Guidelines for the diagnosis and management of pulmonary hypertension. European Heart Journal. 2009; 20: 2493-2537.
Kiely, D. et al. Pulmonary hypertesnion: diagnosis and management. BMJ. 2013; 346:f2028.
Simonneau, G. et al. Updated Clinical Classification of Pulmonary Hypertension. J Am Coll Cardiol. 2013; 62(25 Suppl):D34-41.
