Pulmonary hypertension (CV) Flashcards
Define pulmonary hypertension.
An elevated mean pulmonary arterial pressure (mPAP) >20mmHg at rest
What are the causes of pulmonary hypertension? (5)
- idiopathic pulmonary arterial hypertension (group 1)
- left heart disease - congestive HF, valvular diseases e.g. AS, MS (group 2)
- chronic lung disease - COPD, OSA, ILD (group 3)
- pulmonary artery obstruction e.g. chronic thromboembolic occlusion of pulmonary vessels - thromboses/emboli in lungs (group 4)
- unclear multifactorial mechanisms (group 5)
What left heart diseases can cause pulmonary hypertension? (2+2)
- congestive heart failure
- valvular diseases
- aortic stenosis
- mitral stenosis
What chronic lung diseases can cause pulmonary hypertension? (3)
- COPD
- OSA
- ILD
What is cor pulmonale?
Right-sided heart failure secondary to pulmonary artery hypertension caused by long-standing pulmonary disease
What are the five groups of pulmonary hypertension?
- group 1 - idiopathic
- group 2 - secondary to left heart disease, valvular disease, restrictive cardiomyopathy
- group 3 - secondary to chronic lung disease and environmental hypoxaemia
- group 4 - due to chronic thrombotic disease, embolic disease or both
- group 5 - metabolic, systemic, haematological disorders and other miscellaneous causes
Describe idiopathic pulmonary artery hypertension.
Rare, frequently fatal, most commonly seen in women
What are the clinical features of pulmonary hypertension? (7)
- progressive dyspnoea or syncope/dizziness on exertion
- weakness/fatigue
- late stage –> oedema and ascites
- angina, tachyarrhythmia
- parasternal heave (sign of RV hypertrophy)
- loud S2
- symptoms of RHF - raised JVP, peripheral oedema, hepatomegaly
What might you see on examination in pulmonary hypertension? (8)
- parasternal heave (RV hypertrophy)
- loud S2
- murmur - pulmonary regurgitation, tricuspid regurgitation (high-pitched pansystolic murmur)
- raised JVP
- peripheral oedema
- hepatomegaly
- ascites
- tachyarrhythmia
What are some risk factors for pulmonary hypertension? (4)
- family history
- female sex
- bone morphogenetic protein receptor type 2 mutations (BMPR2)
- appetite suppressants
What are the first-line investigations for pulmonary hypertension? (4)
- CXR
- ECG
- transthoracic doppler echocardiography
- right heart catheterisation
What is the 1st line investigation for all patients with suspected pulmonary hypertension?
Transthoracic echocardiogram
What is a confirmatory test for pulmonary hypertension?
Right heart catheterisation - directly measure pulmonary pressure: mPAP>20mmHg
What would an ECG show in pulmonary hypertension? (1+3)
Findings of RVH and strain:
- tall R wave in V1
- P-pulmonale –> peaked P wave (due to RA enlargement)
- right axis deviation
What would a CXR show in pulmonary hypertension?
Right heart hypertrophy; exclude other lung diseases
What are some differential diagnoses for pulmonary hypertension? (12)
- cor pulmonale –> secondary pulmonary hypertension
- cardiomyopathies
- primary RV failure (following MI)
- congestive heart failure
- mitral/tricuspid stenosis
- pulmonary stenosis
- tricuspid regurgitation
- recurrent PE
- portal hypertension
- OSA
- hypothyroidism
- sickle cell disease
How do we approach management of pulmonary hypertension?
Management of underlying cause
What is the first-line pulmonary vasodilator therapy for patients with idiopathic pulmonary hypertension and no signs of right HF?
CCB - for fluid overload (nifedipine / amlodipine)
What are some supportive treatments for pulmonary hypertension? (4)
- digoxin
- diuretics - furosemide
- anticoagulants - warfarin
- oxygen
What lifestyle changes can be made in pulmonary hypertension? (3)
- restrict salt intake
- exercise
- avoid pregnancy
What anticoagulation should patients with pulmonary hypertension be on?
Warfarin - adjust dose according to INR
What are some specialist treatments for pulmonary hypertension?
- prostacyclins - epoprostenol, iloprost
- Bosentan - non-selective endothelin receptor antagonist
- Sitaxentan - selective endothelin A receptor antagonist
- Sildenafil - PD-5 inhibitor
When is atrial septostomy done for pulmonary hypertension?
- palliative procedure
- may provide benefit to patients who are deteriorating
What are some complications of pulmonary hypertension? (4)
- cor pulmonale - RHF resulting from chronic pulmonary hypertension, commonly due to COPD
- supraventricular tachyarrhythmias
- RV failure - diuretics, digoxin, oxygen
- treatment-related headache - prostanoids, endothelin receptor antagonists and phosphodiesterase-5 inhibitors