Pulmonary hypertension — structured written assessment

SAQ 1 (10 marks)

Practice scenario. A 63-year-old woman has systemic sclerosis of 6 years’ duration and new exertional breathlessness. Her FVC is 85% predicted and her DLCO is 48% predicted. Echocardiography shows a peak tricuspid regurgitation velocity of 3.2 m/s, an RV/LV basal diameter ratio above 1.0 and a right atrial area of 20 cm². Lung function testing and chest CT show no other explanation for her breathlessness.^[2][1]

1. Which Class I ESC/ERS 2022 screening rows apply to patients with systemic sclerosis, and which one fits her now? (2)^[2]
2. Which echocardiographic variable does ESC/ERS 2022 use to assign the probability of PH, and how do her other signs count? (2)^[1]
3. What test confirms the diagnosis, and what haemodynamic findings characterise PAH? (2)^[2][1]
4. RHC shows pre-capillary PH and no other cause is found. Should she have vasoreactivity testing? (2)^[2][1]
5. Give two general measures from ESC/ERS 2022 Recommendation Table 5, and state what the guideline says about anticoagulation in her form of PAH. (2)^[2][1]

Model answers — SAQ 1

1. In SSc an annual evaluation of the risk of having PAH is recommended (ESC/ERS 2022, Class I, Level B), and in adults with SSc of more than 3 years’ duration, FVC of 40% or more and DLCO below 60%, the DETECT algorithm is recommended to identify asymptomatic patients with PAH (ESC/ERS 2022, Class I, Level B) (1 mark).^[2] She is symptomatic and her breathlessness remains unexplained after non-invasive assessment, so the row that fits is: in SSc, where breathlessness remains unexplained following non-invasive assessment, RHC is recommended to exclude PAH (ESC/ERS 2022, Class I, Level C) (1 mark).^[2]
2. ESC/ERS 2022 uses the peak TRV, not the estimated sPAP, as the key variable; a peak TRV above 2.8 m/s may suggest PH, but TRV alone cannot reliably determine whether PH is present (1 mark).^[1] Signs from at least two of the Table 10 categories (A/B/C) must be present to alter the probability; her RV/LV ratio above 1.0 is a ventricle sign (A) and her RA area above 18 cm² is an IVC and RA sign (C) (1 mark).^[1]
3. RHC is recommended to confirm the diagnosis of PH (especially PAH or CTEPH) and to support treatment decisions (ESC/ERS 2022, Class I, Level B) (1 mark).^[2] PAH is pre-capillary PH (mPAP >20 mmHg, PAWP ≤15 mmHg, PVR >2 WU) in the absence of other causes of pre-capillary PH, such as CTEPH and PH associated with lung diseases (1 mark).^[1]
4. No: vasoreactivity testing for CCB candidacy is not recommended in PAH other than idiopathic, heritable or drug-associated PAH (ESC/ERS 2022, Class III, Level C), and PAH associated with SSc is not one of these (1 mark).^[2][1] ESC/ERS 2022 explains that an acute vasodilator response does not predict a favourable long-term CCB response in other forms of PAH (1 mark).^[1]
5. Any two recommended general measures from ESC/ERS 2022 Recommendation Table 5, such as supervised exercise training under medical therapy (Class I, Level A); immunisation against SARS-CoV-2, influenza and Streptococcus pneumoniae (Class I, Level C); psychosocial support (Class I, Level C); or diuretic treatment with signs of RV failure and fluid retention (Class I, Level C) (1 mark).^[2][1] Anticoagulation is not generally recommended in PAH but may be considered on an individual basis (ESC/ERS 2022, Class IIb, Level C), and in SSc-associated PAH registry data and meta-analyses uniformly indicated that anticoagulation may be harmful (1 mark).^[2][1]

SAQ 2 (10 marks)

Practice scenario. A 61-year-old man had an acute pulmonary embolism 5 months ago and has taken therapeutic anticoagulation since then. He still has exertional dyspnoea.^[2][4]

1. What do the ESC/ERS 2022 and 2026 AHA/ACC rows say about evaluating him, and about his anticoagulation meanwhile? (2)^[2][4]
2. Which imaging test is most effective at excluding CTEPD, and why is a negative CTPA not enough? (2)^[1]
3. Define CTEPD and say when the term CTEPH is used. (2)^[1]
4. RHC confirms CTEPH. The CTEPH team judges him technically inoperable, with distal obstructions. Give the ESC/ERS 2022 treatment rows that apply. (2)^[2]
5. What long-term anticoagulation is recommended, and what changes if he has antiphospholipid syndrome? (2)^[2]

Model answers — SAQ 2

1. ESC/ERS 2022: with persistent or new-onset dyspnoea or exercise limitation after PE, further diagnostic evaluation to assess for CTEPH/CTEPD is recommended (Class I, Level C); AHA/ACC 2026: with ongoing dyspnoea and/or functional impairment after 3 months or more of therapeutic anticoagulation after acute PE, a diagnostic evaluation for CTEPD is recommended (COR 1, LOE B-NR) (1 mark).^[2][4] AHA/ACC 2026: patients being evaluated for CTEPD should continue anticoagulation until the evaluation is complete, to prevent recurrent VTE and/or CTEPD progression, unless contraindicated by high bleeding risk (COR 1, LOE B-NR) (1 mark).^[4]
2. ESC/ERS 2022 says V/Q scintigraphy remains the most effective tool to exclude CTEPD (1 mark).^[1] A negative CTPA, even if high quality, does not exclude CTEPD, because distal disease can be missed (1 mark).^[1]
3. CTEPD describes symptomatic patients with mismatched perfusion defects on V/Q scan and signs of chronic, organised, fibrotic clots on CTPA or DSA after at least 3 months of therapeutic anticoagulation (1 mark).^[1] Patients whose symptoms can be attributed to post-thrombo-embolic fibrotic obstructions have CTEPD with or without PH, and CTEPH remains the preferred term when PH is present (1 mark).^[1]
4. ESC/ERS 2022: BPA is recommended in patients who are technically inoperable or have residual PH after PEA, with distal obstructions amenable to BPA (Class I, Level B), and riociguat is recommended for symptomatic inoperable CTEPH (Class I, Level B) (1 mark).^[2] In CTEPH candidates for BPA, medical therapy should be considered before the intervention (ESC/ERS 2022, Class IIa, Level B; GRADE: very low quality, conditional); the same guideline says patients with PVR above 4 WU should be treated before BPA because medical pre-treatment can reduce interventional complications (1 mark).^[2][1]
5. ESC/ERS 2022: lifelong, therapeutic doses of anticoagulation are recommended in all patients with CTEPH (Class I, Level C), and antiphospholipid syndrome testing is recommended (Class I, Level C) (1 mark).^[2] With antiphospholipid syndrome, anticoagulation with VKAs is recommended (ESC/ERS 2022, Class I, Level C) (1 mark).^[2]