Pulmonary Hypertension Flashcards
What is the normal mean pulmonary artery pressure? (mPAP)
At rest = 14 +- 3 mmHg with an upper limite of 20 mmHg.
What is the normal mean capillary wedge pressure (mPCWP)?
6 +- 2 mmHg
What is normal cardiac output?
5 L/min
What is transpulmonary gradient?
Fall in pressure across the lung circulation; the difference between mPAP and mPCWP.
This is normally 6 +- 2 mmHg.
How is the pulmonary vascular resistance calculated?
(mPAP - mPCWP) / CO
What is pulmonary hypertension defined as?
mPAP of more than 25 mmHg at rest as measured on right heart catheterisation..
Symptoms and signs of pulmonary hypertension.
Dyspnoea
Fatigue
Wekaness
Angina
Syncope
Abdominal distension
Bipedal oedema
Findings on examination of pulmonary hypertension.
Right heart hypertrophy with left parasternal heave
Loud P2 heart sound
Soft pansystolic murmur with tricuspid regurg
Early diastolic murmur with pulmonary regurg
Raised JVP
Ascites
Hepatomegaly
Investigations done in PH.
Bloods - FBC, renal function, LFTs, TFTs, serology of autoimmune rheumatic disease, HIV and hepatitis.
CXR
ECG
Echocardiography with tricuspid regurgitation
CMR
Abdominal liver ultrasound
Right heart catheterisation - this might be indicated as part of the clinical assessment to confirm the diagnosis.
Why is abdominal liver ultrasound done in PH?
To exclude liver cirrhosis and portal hypertension.
ECG changes in PH.
Right ventricular hypertrophy
Right atrial enlargement - P pulmonale
Right axis deviation
Tall R waves in V1
Right ventricular strain
RBBB
CXR findings in PH.
Enlargement of the pulmonary arteries and major branches.
Major tapering of peripheral arteries.
Right atrial and right ventricular enlargement.
Classification of PH.
Group 1 – Primary pulmonary hypertension or connective tissue disease such as systemic lupus erythematous (SLE)
Group 2 – Left heart failure usually due to myocardial infarction or systemic hypertension
Group 3 – Chronic lung disease such as COPD
Group 4 – Pulmonary vascular disease such as pulmonary embolism
Group 5 – Miscellaneous causes such as sarcoidosis, glycogen storage disease and haematological disorders
Management of PH.
Primary pulmonary hypertension can be treated with:
IV prostanoids (e.g. epoprostenol)
Endothelin receptor antagonists (e.g. macitentan)
Phosphodiesterase-5 inhibitors (e.g. sildenafil)
Secondary pulmonary hypertension is managed by treating the underlying cause such as pulmonary embolism or SLE.
Supportive treatment for complications such as respiratory failure, arrhythmias and heart failure.
