Pulmonary HTN Flashcards
How is a positive vasoreactivity test defined when investigating pulmonary HTN?
Reduction in mPAP (Pulm arterial pressure) >= 10mmHg to reach an absolute value <=40mmHg
Positive tests can indicate that a person may respond well to CCBs
What are the contraindications to right heart catheterisation?
Recent pacemaker insertion (<1 month)
RA/RV thrombus or tumour
Mechanical right heart valve
Current infection
What is the cut-off TRV for pulmonary HTN probability on ECHO?
TRV >2.8 m/s
What is one of the main genes associated with Pulmonary HTN? What is the inheritance pattern?
BMPR2. Autosomal dominant inheritance. Lifetime risk of Pulmonary HTN ~20%
Incomplete penetrance but higher in female carriers than males
Annual screening ECHO is recommended
What is the prevalence of Pulmonary Hypertension in patients with systemic sclerosis?
5-19%
What is deemed as a satisfactory response to CCB in Pulmonary HTN?
Maintenance of WHO-FC I/II, ideally with mPAP <30 and PVR <4WU
BNP<50 or NT-proBNP <300
They should not be prescribed if vasoreactivity not performed or is negative due to the risk of side effects
What is the correct treatment for PAH in the presence of cardiopulmonary comorbiditiess?
Initial monotherapy with ERA or PDE5i
Ambrisentan, Bositentan, Macitentan OR Sildenafil/Tadalafil
What are the characteristics of Low, Intermediate-Low, Intermediate-High and High risk in Pulmonary Hypertension?
Low- 6MWT >440m, NT-proBNP <300
WHO-FC I/II
Intermediate-Low: 6MWT 320-440m, NT-proBNP 300-649
Intermediate-High: WHO-FC III, 6MWT 165-319m, NT-proBNP 650-1100
High: WHO-FC IV, 6MWT <165 NT-proBNP >1100
What are the initial treatment options for patients with PAH without cardiopulmonary comorbidities?
Combination therapy with ERA and PDE5i.
Usually ambrisentan & tadalafil
OR macitentan & tadalafil
What treatments should be started in patients with CTEPH?
Lifelong anticoagulation
Test for anti phospholipid syndrome
Pulmonary Endartectomy is the treatment of choice
If inoperable/persistence after PEA then can perform balloon angioplasty
Can give medical therapy if inoperable such as riociguat
What is a possible side effect of ERAs?
Liver dysfunction. Around 10% will experience a rise in LFTs. In severe cases it can result in cirrhosis or liver failure.
Should avoid them in patients with existing liver pathology
Bosentan, ambrisentan, macitentan
Which follow up investigations are recommended in stable pulmonary HTN?
WHO Functional class, NT pro-BNP, 6MWT and pulse oximetry.
If deterioration may consider ECHO or right heart catheterisation.
In the developing world, which infection is commonly associated with Pulmonary HTN? What is the prevalence?
Schistosomiasis (snail fever). Often caught from parasites in fresh water sources from infected snails.
May get fever and lymphadenopathy plus bloody diarrhoea.
The prevalence of pulmonary HTN is ~6.1%
In patients with Pulmonary HTN secondary to IPF (class III), which (off-label) treatment may be added?
Inhaled treprostinil (vasodilator) can be added if other treatments have been optimised
Roughly what proportion of patients with PE will go on to develop CTEPH?
~3.8%
Which medical treatment may be trialled in inoperable CTEPH? What is the mechanism of action?
Riociguat. Soluble Guanylate Cyclase simulator
Which agents may be used in pulmonary vasoreactivity testing?
Inhaled NO
Inhaled iloprost
IV epoprostenol
