Pulmonary Hypertension Flashcards
Key Features
- Endothelin thought to play a key role in pathogenesis of PAH.
- Primary
It is more common in females and typically presents between the ages of 30-50 years - Secondary
cardiac disease- congenital heart defects such as ASD;
heart valve problems such as mitral stenosis
Lung disease - COPD, pulmonary fibrosis
Symptoms
- Breathlessness - progressive exertional dyspnoea is the classical presentation
- Weakness / lethargy
- Exertional dizziness
- Syncope
- Anginal chest pain
Signs
- Raised JVP with large V wave
- Right ventricular heave
- Loud P2
- Murmur of Tricuspid Regurgitation (pan-systolic murmur at the left sternal edge)
- Fixed or reverse split 2nd heart sound
- Peripheral oedema
Sometimes: Murmur of pulmonary or mitral regurgitation Pulsatile hepatomegaly Ascites Cyanosis
5 Groups
Group 1: Pulmonary arteriolar hypertension (PAH), which can be idiopathic (IPAH) or associated with other conditions, notably systemic sclerosis and CHD
Group 2: Pulmonary hypertension owing to left heart disease (PH-LHD)
Group 3: Pulmonary hypertension owing to lung disease or hypoxia (PH-Lung), or both
Group 4: Chronic thromboembolic pulmonary hypertension (CTEPH)
Group 5: Unclear or multifactorial mechanisms
Which investigations would you do?
- vitals: esp RR, O2 sats
- ECG
- CXR
- ABGs
- PFTs - reduced transfer factor
- Echo
- Right Heart Catheter
Also consider: CTPA, exercise test, BNP, HRCT
ECG - possible signs
- right atrial enlargement: lead II peaked/tented p wave
- right axis deviation
- right ventricular hypertrophy
- ST depression and T-wave inversion in the anterior leads
- RBBB
CXR - possible findings
- elevated cardiac apex
- enlarged right atrium
- prominent pulmonary outflow tract
- enlarged pulmonary arteries
- pruning of peripheral pulmonary vessels
(blood vessels are more prominent and dilated centrally, and less visible peripherally)
What is the mean pulmonary artery pressure and systolic PAP to diagnose pulmonary hypertension?
- mean pulmonary artery pressure (PAP) > 25 mmHg at rest or 30 mm Hg with exercise
- most echo reports will give systolic PAP
systolic PAP 40 mmHg implies mean PAP > 25 mmHg
Echo - possible findings
- systolic PAP
- dilated Pulmonary Artery
- dilated Right Atrium
- bulging of septum into the LV cavity causing diastolic dysfunction
- presence of at least a trivial tricuspid regurgitant jet
What can the right heart catheter show?
- measure pulmonary artery pressure directly
- determine pulmonary vasoreactivity: In patients with idiopathic and hereditary pulmonary arterial hypertension (IPAH and HPAH) acute vasodilator testing* during right heart catheterization is done to assess need for CCB - if they test positive CCB are offered
*other ways to test for this include using NO, epoprostenol (prostacyclin) and adenosine
Treatment - overview
- Anticoagulation - esp If CTEPH or idiopathic
- Diuretics - if oedematous/ HF symptoms
- Oxygen
Acute vasodilator testing
+ve –> offer CCB*
-ve –> Specific medical therapies (IPAH)
Patients with progressive symptoms should be considered for a heart-lung transplant.
*amlodipine/nifedipine are more selective of the vasculature
What are Specific medical therapies?
Prostanoids (synthetic prostacyclin = vasodilation)
such as iloprost (inh), Epoprostenol (IV)
Endothelin receptor antagonists
such as bosentan
Phosphodiesterase-5 inhibitors - reduction in pulmonary vascular tone - such as sildenafil
What level of PaO2 do pulmonary hypertension patients need to be referred for Long Term Oxygen Therapy?
PaO2 ≤ 8 kPa
Criteria for LTOT
- Stable COPD, ILD or Cystic Fibrosis
- PaO2 ≤ 7.3 kPa at rest
- PaO2 ≤ 8 kPa if peripheral oedema, polycythaemia (haematocrit ≥55%) or pulmonary hypertension - Patients with pulmonary hypertension
PaO2 ≤ 8 kPa
- Use oxygen for at least 15 h per day
How are patients assessed for need of LTOT?
- ABG measurements
Measurements should be taken on 2 occasions at least 3 weeks apart to demonstrate clinical stability, and not sooner than 4 weeks after an acute exacerbation of the disease
