HOW TO TREAT 25
ausdoc . com . au 19 APRIL 2024
HOW TO TREAT 25
Hughes , M ., et . Al . Raynaud phenomenon and digital ulcers in systemic sclerosis . Nature Reviews Rheumatology , 16 ( 4 ), 208-221 , 2020 , Springer Nature
How to Treat Quiz .
1 . Which THREE statements regarding pulmonary hypertension ( PH ) are correct ? a One in 10 Australian adults reports clinically important dyspnoea . b About 8.5 % of patients presenting to their GP with dyspnoea do not have a cause identified . c Early identification and treatment of pulmonary arterial hypertension ( PAH ) can prevent irreversible damage to the pulmonary vascular bed and dramatically improve prognosis . d PH is the usual cause of breathlessness .
2 . Which TWO statements regarding PH are correct ? a In Australia and New Zealand , the median time between symptom onset and diagnosis is 2.8 years . b PH is an umbrella term to describe all patients with an elevated mean pulmonary artery pressure . c The aetiology of PH has significant implications for the prognosis and treatment decisions . d The terms PH and PAH are synonymous .
3 . Which ONE is the leading cause of PH worldwide ? a Lung disease . b Left heart disease . c Pulmonary embolism . d Sarcoidosis .
4 . Which THREE may be features of abnormal vessels in PH ? a Reduced nitric oxide production . b Smooth muscle proliferation / hypertrophy . c Reduced endothelin-1 ( ET-1 ) production . d Perivascular inflammation .
5 . Which THREE are red flag symptoms in PH ? a Exertional chest pain . b Exertional pre-syncope . c Palpitations . d Syncope .
6 . Which ONE is the gold standard test to diagnose PH ? a Transthoracic echocardiograph . b Chest X-ray . c Right heart catheterisation .
Figure 9 . Hand manifestations of systemic sclerosis including Raynaud ’ s phenomenon , digital ulceration / gangrene , calcinosis and flexion contractures .
Reproduced with permission from Hughes M et al . 29
one-year history of Raynaud ’ s phenomenon , associated with sclerodactyly ( see figure 9 ), joint pain , gastro-oesophageal reflux and progressive exertional dyspnoea . Blood tests reveal an antinuclear antibody greater than 1:2560 with a speckled pattern and anti-centromere antibodies .
TTE reveals a TRV of 3.4 metres / second , with mild RV dilation and normal systolic function .
An RHC demonstrates pre-capillary PH ( see table 5 ). V / Q scan is normal .
Bernard ’ s final diagnosis is SSc-related PAH ( Group 1 ) and he is started on tadalafil ( PDE5i ) and macitentan ( ERA ).
Case study three
Arlene , a 67-year-old female , is referred by her GP to the cardiology outpatient clinic . Arlene has an 18-month history of progressive exertional dyspnoea that has previously been managed as poorly controlled asthma .
TTE reveals a TRV of 3.1 metres / second with normal RV size and systolic function . An RHC demonstrates pre-capillary PH ( see table 6 ).
A V / Q scan reveals multiple segmental and subsegmental mismatched perfusion defects in both lungs , consistent with widespread bilateral pulmonary emboli . Anticoagulation with apixaban is started .
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d Ventilation / perfusion scan .
7 . Which TWO statements regarding the investigation of PH are correct ? a The single most useful investigation a GP can arrange in a patient with suspected PH is a TTE . b A normal chest X-ray and normal ECG rule out PH . c Pulmonary function tests are always abnormal in PAH . d A V / Q scan is the imaging of choice to exclude chronic thromboembolic pulmonary hypertension .
8 . Which TWO statements regarding the investigation of PH are correct ? a The need for contrast limits the utility of RHC in those with renal impairment . b RHC is high risk because it is performed under general anaesthetic . c Non-contrast high resolution CT chest is the best modality
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PULMONARY HYPERTENSION
Table 4 . Eden ’ s results Measure Result Normal range Mean pulmonary artery pressure 53mmHg Less than 20mmHg Mean pulmonary arterial wedge pressure 4mmHg Less than 15mmHg Pulmonary vascular resistance 9 Wood units Less than 2 Wood units Cardiac index 1.85L / min / m 2 2.5-4L / min / m 2
Table 5 . Bernard ’ s results Measure Result Normal range Mean pulmonary artery pressure 29mmHg Less than 20mmHg Mean pulmonary arterial wedge pressure 12mmHg Less than 15mmHg Pulmonary vascular resistance
Table 6 . Arlene ’ s results Measure Result Normal range Mean pulmonary artery pressure 48mmHg Less than 20mmHg Mean pulmonary arterial wedge pressure 9mmHg Less than 15mmHg Pulmonary vascular resistance 8 Wood units Less than 2 Wood units Cardiac index 2.61L / min / m 2 2.5-4L / min / m 2
There is no history of acute PE or DVT . An invasive pulmonary angiogram is performed and the thrombi are too distal for PEA .
Arlene ’ s final diagnosis is inoperable CTEPH ( Group 4 ). Riociguat and warfarin are started ( a change from apixaban ), followed by six sessions of BPA that significantly improve pulmonary pressures and functional status .
for identifying interstitial lung disease and other parenchymal abnormalities . d The most feared complication of RHC is pulmonary artery perforation .
9 . Which THREE statements regarding the specific management of PAH are correct ? a PAH therapies target the pathways of the pulmonary vasodilator drugs , nitric oxide , endothelin and prostacyclin . b Low-intensity exercise is safe . c Vaccination against influenza , Streptococcus pneumoniae and SARS-CoV-2 is recommended . d Anticoagulation is indicated in all patients with PAH .
10 . Which THREE statements regarding the management of Groups 2 , 3 and 4 PH are correct ? a It is vital to treat the underlying heart and lung conditions in patients with Groups 2 and 3 PH . b CTEPH is the only form of PH where interventional and / or surgical treatment may be indicated . c PH persists or recurs after PEA in 17-31 % of patients . d ET-1 receptor antagonists are first-line in patients with Groups 2 and 3 PH .
2.4 Wood units
Less than 2 Wood units
Cardiac index 3.16L / min / m 2 2.5-4L / min / m 2
CONCLUSION
OVERALL , PH is a common condition . It is typically seen in patients with underlying cardiac disease or pulmonary disease and is usually mild . In contrast , PAH and CTEPH are rare and potentially life-threatening subtypes of PH . These can significantly benefit from targeted treatments such as pulmonary vasodilator drugs and surgery respectively .
The functional state of the right heart is the major determinant of the patient ’ s prognosis ; early detection before irreversible pathophysiological changes occur is key to improving outcomes . GPs play a pivotal role in the diagnosis and longterm management of patients with PH and it is therefore important for GPs to know when to suspect PH and which initial investigations to arrange . If the results suggest an intermediate or high probability of PH , urgently refer patients to the nearest specialist PH centre .
RESOURCES
• 2022 ESC / ERS Guidelines for the diagnosis and treatment of pulmonary hypertension bit . ly / 3SnCUDE
• The ‘ Ten Commandments ’ of the 2022 ESC / ERS Guidelines for the diagnosis and treatment of pulmonary hypertension bit . ly / 3vT5pkZ
• Pulmonary Hypertension Society of Australia and New Zealand phsanz . org — Expert referral centres phsanz . org / expert-referralcentres
Declaration of interest statement Ramey Bajwa — nil declarations . Dr Claire Ellender — Has received sponsorship from GSK and Janssen . Dr Cerys Jones — nil declarations .
References Available on request from howtotreat @ adg . com . au