Pulmonary Hypertension Flashcards
Definition
Impact
Pulmonary hypertension is defined as raised pulmonary arterial pressures ≥25mmHg at rest.
Remodelling of the pulmonary vasculature leads to right heart strain with subsequent right heart failure.
Causes v
Idiopathic or inherited
Connective tissue disease/autoimmune (systemic sclerosis, Sjogren’s syndrome, SLE, Rheumatoid arthritis)
Left ventricular dysfunction: valve disease, cardiomyopathy
Infection: HIV, Schistosomiasis
Lung disease: COPD, interstitial lung disease, obstructive sleep apnea, high altitude
CTEPH: chronic thromboembolic pulmonary hypertension
Drug/toxin induced
Clinical Features
progressive shortness of breath, fatigue, pre-syncope/syncope.
In advanced stages, patients experience peripheral oedema.
Examination findings
loud second heart sound (pulmonary component),
right parasternal heave,
raised JVP,
systolic murmur (tricuspid regurgitation),
peripheral oedema.
Investigations
Non-invasive testing with ventilation/perfusion scans is of significant value in diagnosing thromboembolic causes - CTEPH (chronic thromboembolic pulmonary hypertension).
The gold standard investigation is CT pulmonary angiogram.
Right heart catheterisation directly assess the pulmonary pressures is required for a definitive diagnosis.
Echocardiography is used to determine right ventricular function.
Other investigations (CXR, ECG, Lung function test, autoimmune screen, HRCT, MRI, V/Q scan) are all useful tests to help determine the aetiology.
Management
treating any underlying condition and supportive measures such as supplemental oxygen
Pharmacological treatment involves calcium channel blockers or prostacyclin analogues (e.g. iloprost), endothelin-A antagonists (e.g. bosentan) and PDE-5 inhibitors (eg. sildenafil).
Monitoring needed for bosentan
LFTS - contraindicated in moderate or severe hepatic impairment
