Clinical Medicine 2023 Vol 23, No 5: 449–54 CME CARDIOLOGY

Pulmonary hypertension – the latest updates for

physicians

Authors: Ruta Virsinskaite,A Nina Karia,B Tushar Kotecha,C Benjamin E Schreiber,D J Gerry CoghlanE and Daniel S KnightF

Pulmonary hypertension (PH) is common, with an estimated an awareness of how to identify and manage PH will improve
ABSTRACT

prevalence of approximately 1% that increases with age. care for the general medical patient and also help identify the
Prompt and accurate diagnosis is key to institute timely and rarer, directly treatable forms of PH such as pulmonary arterial
appropriate therapy to improve symptoms and prognosis. The hypertension (PAH, Group 1) and chronic thromboembolic PH
international guidelines for the diagnosis and management (CTEPH, Group 4). 2 Identifying and differentiating these patients
of PH have recently been updated, with a lowering of the
haemodynamic threshold for diagnosis to a mean pulmonary Key points
artery pressure >20 mmHg. New diagnostic algorithms and
Pulmonary hypertension is common, with multiple subtypes
revised indications for screening in at-risk groups have been
and aetiologies that have individualised management
developed to facilitate early referral to specialist PH centres.
recommendations.
This includes fast-track referral pathways for patients who are
either clinically high-risk or are at-risk for pulmonary arterial The haemodynamic definition of PH by right heart
hypertension (PAH) or chronic thromboembolic pulmonary catheterisation (RHC) has changed to a lower mean
hypertension (CTEPH). This review summarises key changes in pulmonary arterial pressure (mPAP) at rest of >20 mmHg
the PH guidelines for general physicians who are, most often, based upon the upper normal limit in healthy individuals
the first healthcare professionals to encounter these patients along with supportive prognostic data. However, there
and consequently have a key role as referrers into specialist is currently no evidence for the efficacy of targeted PH
PH services. medications in patients with mPAP <25 mmHg and
pulmonary vascular resistance (PVR) <3 WU, which remains
an evidence gap.
Introduction
Pulmonary hypertension (PH) is commonly encountered in Early detection and referral to a specialist PH centre is
daily clinical practice. It has an estimated prevalence of 1% crucial to institute targeted management that can improve
of the global population, which considerably increases in symptoms and optimise prognosis. Physicians should be
people aged over 65 years owing to the higher burden of familiar with the streamlined pathway for suspicion, detection
cardiopulmonary comorbidities encountered with age.1 Among and confirmation of PH, the latter step being performed at
the many aetiologies of PH (Table 1), left heart disease (Group PH centres. Fast-track referral pathways should be taken for
2) is the most common, followed by lung disease (Group 3), with clinically high-risk patients and for patient cohorts ‘at-risk’ of
treatment in both aimed at the underlying cause. Consequently, developing pulmonary arterial hypertension (PAH) or chronic
thromboembolic pulmonary hypertension (CTEPH).

Annual systematic screening using the DETECT algorithm

Authors: Apulmonary hypertension clinical research fellow, is a class 1 recommendation in asymptomatic adults with
National Pulmonary Hypertension Service, Royal Free London systemic sclerosis of more than 3 years’ duration, forced vital
NHS Foundation Trust, London, UK; Bconsultant cardiologist, capacity (FVC) ≥40%, and a transfer factor (DLCO) <60%.
National Pulmonary Hypertension Service, Royal Free London
NHS Foundation Trust, London, UK; Cconsultant cardiologist, Patients with ongoing dyspnoea after 3 months of
National Pulmonary Hypertension Service, Royal Free London anticoagulation in the setting of a history of previous
NHS Foundation Trust, London, UK; Dconsultant rheumatologist, thromboembolic disease require further evaluation for chronic
National Pulmonary Hypertension Service, Royal Free London thromboembolic pulmonary disease (CTEPD) or CTEPH.
NHS Foundation Trust, London, UK; Econsultant cardiologist,
National Pulmonary Hypertension Service, Royal Free London KEYWORDS: Pulmonary hypertension, chronic thromboembolic
NHS Foundation Trust, London, UK; Fdirector, National Pulmonary pulmonary hypertension, pulmonary arterial hypertension,
Hypertension Service, Royal Free Hospital. consultant cardiologist, screening, systemic sclerosis
National Pulmonary Hypertension Service, Royal Free London NHS
DOI:10.7861/clinmed.2023-23.5.Cardio4
Foundation Trust, London, UK

© Royal College of Physicians 2023. All rights reserved. 449

Ruta Virsinskaite, Nina Karia, Tushar Kotecha et al

Table 1. Clinical classification of PH3

Group 1: Pulmonary arterial hypertension (PAH)
1.1 Idiopathic
> 1.1.1 Non-responders at vasoreactivity testing
> 1.1.2 Acute responders at vasoreactivity testing
1.2 Heritable
1.3 Associated with drugs and toxins
1.4 Associated with:
> 1.4.1 Connective tissue disease
> 1.4.2 HIV infection
> 1.4.3 Portal hypertension
> 1.4.4 Congenital heart disease
> 1.4.5 Schistosomiasis
1.5 PAH with features of venous/capillary (PVOD/PCH) involvement
1.6 Persistent PH of the newborn
Group 2: PH associated with left heart disease
2.1 Heart failure:
> 2.1.1 with preserved ejection fraction
> 2.1.2 with reduced (≤40%) or mildly reduced (41–49%) left ventricular ejection fraction
2.2 Valvular heart disease
2.3 Congenital/acquired cardiovascular conditions leading to post-capillary PH
Group 3: PH associated with lung diseases and/or hypoxia
3.1 Obstructive lung disease or emphysema
3.2 Restrictive lung disease
3.3 Lung disease with mixed restrictive/obstructive pattern
3.4 Hypoventilation syndromes
3.5 Hypoxia without lung disease (eg high altitude)
3.6 Developmental lung disorders
Group 4: PH associated with pulmonary artery obstructions
4.1 Chronic thrombo-embolic PH
4.2 Other pulmonary artery obstructions (including sarcomas, other malignant or non-malignant tumours, arteritis without connective
tissue disease, congenital pulmonary arterial stenoses, and hydatidosis)
Group 5: PH with unclear and/or multifactorial mechanisms (discussion with +/− referral to PH centres should be considered)
5.1 Haematological disorders (including inherited and acquired chronic haemolytic anaemia and chronic myeloproliferative disorders)
5.2 Systemic disorders (including sarcoidosis, pulmonary Langerhans’s cell histiocytosis, and neurofibromatosis type 1)
5.3 Metabolic disorders (including glycogen storage diseases and Gaucher’s disease)
5.4 Chronic renal failure with or without haemodialysis
5.5 Pulmonary tumour thrombotic microangiopathy
5.6 Fibrosing mediastinitis
HIV = human immunodeficiency virus; PAH = pulmonary arterial hypertension; PCH = pulmonary capillary haemangiomatosis; PH = pulmonary hypertension;
PVOD = pulmonary veno-occlusive disease.

who may benefit from targeted PH therapies is complicated The recently updated European Society of Cardiology/European
by the non-specific symptoms and signs of PH, which overlap Respiratory Society (ESC/ERS) guidelines for the diagnosis and
across all PH subtypes and with other common cardiopulmonary treatment of PH include many changes relevant to physicians
conditions. Therefore, a key focus for physicians is ensuring early across a range of specialties.3 Early recognition and detection of
referral to specialist PH centres when directly treatable PH is who to refer, fast-track referral pathways to PH centres, and a focus
suspected, thereby avoiding worse outcomes associated with on screening at-risk populations are key features. Importantly,
delays in initiating PH therapies. the diagnostic criteria and thresholds for PH have been lowered

450 © Royal College of Physicians 2023. All rights reserved.

 Pulmonary hypertension

along with the recommendation for haemodynamic evaluation of right heart failure, all of which suggest advanced PH, should
to be undertaken at expert centres. This review aims to provide prompt an expedited referral directly to a specialist PH centre.
physicians with an overview of the key advances in the diagnosis Echocardiography remains the first-line non-invasive
and management guidelines of known or suspected PH and the investigation for suspected PH. An echocardiographic probability
evidence that underpins these changes. of PH is given according to the peak tricuspid regurgitation
velocity (TRVmax) measured by continuous wave Doppler along
Changes to diagnostic thresholds with the presence of any accompanying features suggestive of
PH: abnormalities of right heart size and function, deleterious
The haemodynamic definition of PH has changed to a lower mean interventricular septal dynamics suggesting pressure overload
pulmonary arterial pressure (mPAP) at rest of >20 mmHg by right of the right ventricle, pulmonary arterial dilatation, and the
heart catheterisation (RHC). This change is based upon the upper ratio of right ventricular longitudinal function to estimated
normal limit in healthy individuals along with prognostic data systolic pulmonary arterial pressure (sPAP). These measurements
in patients beyond this threshold. However, there is currently no are all within the remit of routine echocardiography studies
evidence for the efficacy of targeted PH medications in patients and are not limited to expert centres. Of note, the TRVmax
with mPAP <25 mmHg and pulmonary vascular resistance thresholds to estimate the probability of PH are unchanged from
(PVR) <3 WU (Wood units), which remains an evidence gap. previous guidelines as there are no data to recalibrate them
Nevertheless, patients at risk for developing PAH (such as those against the new RHC-derived diagnostic criteria. Importantly,
with systemic sclerosis (SSc) or portal hypertension) but with echocardiography only provides a probability estimate for PH
haemodynamics in the new milder PH range should be closely and cannot be used alone to diagnose PH - this can only be made
followed up in a specialist PH centre. by RHC. Echocardiography can also offer clues to PH aetiology,
Measuring pulmonary arterial wedge pressure (PAWP) and PVR especially due to left heart disease in cases of left ventricular
is pivotal for the accurate haemodynamic classification of PH systolic dysfunction, severe left-sided valvular heart disease, or
(Table 2). Hence, RHC is recommended to be performed only in isolated left atrial dilatation implying diastolic dysfunction.
experienced centres following standardised protocols. Thus, the
key focus for referring physicians should be early referral if PH is Screening in ‘at risk’ patients
suspected and screening at-risk patient populations.
Approximately three-quarters of patients with connective tissue
disease (CTD)-associated PAH have SSc as the underlying CTD.4
Diagnostic pathway, early referral and the role of
The prevalence of PAH in patients with SSc ranges from 7%
echocardiography
to 19% and carries a poor prognosis, which can be improved
A new diagnostic algorithm, including a fast-track referral with early detection and initiation of targeted PH therapy.5 The
pathway, has been proposed to encourage earlier detection of PH benefits of systematic screening for PAH in SSc are well known but
in patients (Fig 1). Step 1 is the initial evaluation for suspecting the screening methods used vary between institutions.6 The latest
PH, including clinical review, ECG and blood test for brain PH guidelines recommend annual systematic screening for PH in
natriuretic peptide (BNP or NT-proBNP). Step 2 involves first-line patients with SSc, with the DETECT algorithm recommended for
investigations, with the investigation pathway diverging according screening asymptomatic adults with SSc of more than 3 years’
to the suspicion of lung disease versus cardiac disease/PH as the duration, forced vital capacity (FVC) ≥40%, and a transfer
most likely cause. The final step suggests which patients to refer factor (DLCO) <60%.7 This is an evidence-based, non-invasive,
on to a PH centre. These include patients with intermediate or multiparametric clinical screening tool that has a high sensitivity
high probability of PH and patients at risk of PH, such as those and high negative predictive value for PAH detection in SSc.7 Using
with a prior history of pulmonary embolism (PE), connective tissue this tool minimises missed diagnoses, identifies milder PH, and
disease, portal hypertension, HIV, or a family history of PAH. Any addresses resource utilisation, particularly for echocardiography.
warning signs such as syncope, tachycardia, hypotension, or signs Overall, physicians should have a low threshold to investigate any
patient complaining of dyspnoea with an associated condition
Table 2. Haemodynamic definitions of PH3,21 deeming them at-risk for PH, including SSc, portal hypertension, or
HIV. In breathless patients with SSc in whom no other identifiable
Definitions Characteristics Clinical groups
cause is found, referral to a PH centre for evaluation by RHC is
Pre-capillary PH mPAP >20 mmHg 1, 3, 4 and 5 recommended, irrespective of the echocardiographic probability
PAWP ≤15 mmHg of PH. Unlike patients with SSc, screening asymptomatic
patients with portal hypertension or HIV is not recommended
PVR >2 WU
given the lower prevalence of PH in these conditions. However,
Isolated post-capillary mPAP >20 mmHg 2 and 5 patients under consideration for liver transplantation or a
PH (IpcPH) transjugular portosystemic shunt (TIPS) should routinely undergo
PAWP >15 mmHg
echocardiography screening for portopulmonary hypertension.
PVR ≤2 WU
Patients with dyspnoea and a history of previous thromboembolic
Combined pre- and mPAP >20 mmHg 2 and 5 disease require further evaluation for chronic thromboembolic
post-capillary PH pulmonary disease (CTEPD) or chronic thromboembolic
PAWP >15 mmHg
(CpcPH) pulmonary hypertension (CTEPH). For example, patients with
PVR >2 WU
ongoing dyspnoea after 3 months of anticoagulation for an
mPAP = mean pulmonary arterial pressure; PAWP = pulmonary arterial wedge acute pulmonary embolism (PE) in conjunction with mismatched
pressure; PVR = pulmonary vascular resistance; WU = Wood units.
perfusion defects should be referred to a PH centre after

© Royal College of Physicians 2023. All rights reserved. 451

Ruta Virsinskaite, Nina Karia, Tushar Kotecha et al

Paents with unexplained dyspnoea and/or suspected PH

Step 1: suspicion Fast-track referral

General praconer Warning signs
or physician ● Rapid progression of symptoms
● Severely reduced exercise capacity
● History ● Pre-syncope or syncope on mild exeron
● Examinaon ● Signs of right heart failure
● O₂ saturaon
● ECG PAH suspected
● BNP/NT-proBNP
Risk factors include:
● CTD (especially SSc)
● Portal hypertension
● HIV infecon
Suspected causes ● Family history of PAH

CTEPH suspected

Step 2: detecon Risk factors include:

● History of PE
Lung disease PH or cardiac disease ● Permanent intravascular devices
● Inflammatory bowel diseases
Lung invesgaons Cardiac invesgaons ● Essenal thrombocythaemia
Splenectomy
● CXR Rapid cross- ● Echocardiogram ● High-dose thyroid hormone replacement
● PFT referral as ● CPET ● Malignancy
● CT chest (CTPA ideally) needed
● ABG
● CPET

Step 3: confirmaon

Causes other than PH Low PH probability Intermediate/high Refer to PH centre

idenfied
(class I
recommendaon)
Y N
Complete a
comprehensive PH
Risk factors for
Manage accordingly Further work up work-up
PAH or CTEPH
RHC when
indicated

Fig 1. Diagnostic algorithm for patients with unexplained dyspnoea and/or suspected pulmonary hypertension. Adapted from the 2022
ESC/ERS guidelines for the diagnosis and treatment of pulmonary hypertension.3 ABG = arterial blood gas; BNP = brain natriuretic peptide; CPET =
cardiopulmonary exercise testing; CT = computed tomography; CTEPH = chronic thromboembolic pulmonary hypertension; ECG = electrocardiogram;
HIV = human immunodeficiency virus; N = no; NT-proBNP = N-terminal pro-brain natriuretic peptide; PAH = pulmonary arterial hypertension;
PE = pulmonary embolism; PFT = pulmonary function tests; PH = pulmonary hypertension; Y = yes.

considering the results of an echocardiogram, which is the and onward referral should be considered when disproportionate
preferred first-line investigation for suspected CTEPH. right heart abnormalities are found on echocardiography (Fig 2).
Moreover, RHC still has a role in patients with LHD if it will assist
Left heart PH (Group 2) – when to refer? in the patient’s management but, as discussed previously, should
be performed at PH centres.3 In suspected PH-LHD, this enables
PH due to left heart disease (PH-LHD), also known as group 2 or relevant provocative testing when required, including fluid
post-capillary PH, is the most common type of PH and is defined challenge or exercise testing.
by mPAP >20 mmHg in the presence of an elevated PAWP
(>15 mmHg) and normal PVR (≤2 WU). In general, it reflects
Lung PH (Group 3) – evidence for targeted treatment?
the severity of LHD, and treatment should be directed at the
underlying cause before further assessing the PH itself. Targeted PH due to lung disease (Group 3 PH) is the second commonest
PH therapies are not recommended in isolated PH-LHD, with PH subtype. Like PH-LHD, it reflects the severity of the lung
studies showing potentially harmful outcomes.8 disease with treatment of the underlying cause being the focus
However, referral to a PH centre and RHC should still be of management. This includes, where indicated, management
considered in specific cases. For example, features of predominant of hypoxaemia, alveolar hypoventilation or sleep-disordered
right heart failure or disproportionately severe PH in patients with breathing. Referral to PH centres should be considered in patients
LHD should be referred to PH services for diagnostic evaluation. with potential dual diagnosis, in particular, patients with CTD who
These patients may have PAH (Group 1) or CTEPH (Group 4) may have interstitial lung disease (ILD) as well as an independent
with LHD comorbidities or combined pre- and post-capillary vasculopathy. Referral for RHC might also assist in management
PH, for which individualised management in a PH service is decisions such as referral for transplantation or when the
recommended. Even after assigning a clinical pre-test probability suspected severity of PH gives rise to uncertainty in treatment.
of LHD, differentiating heart failure with preserved ejection Despite an individualised approach being recommended for the
fraction (HFpEF) from PAH with comorbidity can be challenging management of group 3 PH, there is a lack of robust evidence

452 © Royal College of Physicians 2023. All rights reserved.

 Pulmonary hypertension

Pre-test probability of HFpEF-associated PH, based upon:

● Risk factors for LHD: age, obesity, hypertension, dyslipidaemia, glucose intolerance/diabetes mellitus
● History of known LHD, including AF, structural LHD or cardiac intervenon
● ECG features: le axis deviaon, LVH, LBBB
● Echocardiography features: Le atrial dilataon, LVH, significant (≥grade2) diastolic dysfuncon
● Other invesgaons including CPET or CMR

Intermediate High

No RV abnormality RV abnormality

Consider RHC for RHC recommended Management of LHD

paents with:
● SSc
● CTEPH risk factors
● Unexplained dyspnoea

Fig 2. When to refer patients with possible HFpEF-associated pulmonary hypertension (PH) to specialist centres for further evaluation by right-
heart catheterisation (RHC). RHC is recommended in cases with an intermediate probability of HFpEF-associated PH when risk factors of pulmonary
arterial hypertension / chronic thromboembolic pulmonary hypertension (PAH/CTEPH) are present and/or if there is evidence of right ventricular (RV)
abnormality. If the probability of HFpEF-associated PH is high, management should focus on the underlying left heart disease (LHD). Adapted from Vachiery
et al 2019.20 AF = atrial fibrillation; CMR = cardiovascular magnetic resonance; CPET = cardiopulmonary exercise testing; ECG = electrocardiogram; HFpEF
= heart failure with preserved ejection fraction; LBBB = left bundle branch block; LVH: left ventricular hypertrophy; SSc: systemic sclerosis.

for the use of targeted PH therapies currently commissioned in The presence of cardiopulmonary comorbidities in patients with
the UK for group 1 PH. Moreover, medications in PAH may have PAH confers a poorer response to targeted PH therapies with higher
detrimental effects in these patients and should be avoided in discontinuation rates, a lower likelihood of improving their risk
most instances. A recent phase 3 randomised controlled trial of profile, and a poorer prognosis. Evidence for targeted PH treatments
inhaled treprostinil in PH due to ILD demonstrated improvements in these patients is limited as patients with such comorbidities
in six-minute walk distance, NT-proBNP, and reduced clinical are often excluded from clinical trials. Initial PH monotherapy is
worsening.9 However, further evidence is required and this is recommended on an individual basis, with registry data suggesting
currently not clinically commissioned in the UK. a preference for phosphodiesterase-5 inhibitors (PDE5i).

PAH with cardiopulmonary comorbidities – ‘real Group 1 PH – current and future treatment options
world’ phenotypes and strategies
Rather than being the primary cause of PH, cardiovascular and For patients with PAH without significant comorbidity, initial
respiratory comorbidities are common and further complicate combination therapy with PDE5i (eg tadalafil or sildenafil) and
diagnosis and treatment options in PAH. The classic phenotype of endothelin receptor antagonist (eg ambrisentan or macitentan)
young, predominantly female patients without comorbidity only is the standard of care.11 If the patient is high risk at presentation,
accounted for the minority (12.6%) of patients diagnosed with intravenous prostanoid (epoprostenol) is also considered.12 The
idiopathic PAH (IPAH) in the COMPERA registry.10 The majority of aim of PH therapy is to optimise the patient’s risk profile and,
patients with IPAH (51.6%) were in the cardiopulmonary cluster, consequently, prognosis.13 Addition of the oral prostacyclin
mostly consisting of elderly men with LHD risk factors and a receptor agonist selexipag is recommended in patients with an
significant smoking history who were often hypoxaemic with a intermediate-low risk profile after initial PH therapy.14 Patients
low transfer factor. The remaining 35.8% of patients, termed the in the intermediate-high or high risk categories should be
left heart phenotype, were predominantly elderly women with risk considered for intravenous prostanoid and/or lung transplantation.
factors for HFpEF, just under one-third of whom had a history of Sotatercept, an activin receptor type IIA-Fc fusion protein, is
atrial fibrillation. a potential therapy of promise in PAH. A recent double-blind,

© Royal College of Physicians 2023. All rights reserved. 453

Ruta Virsinskaite, Nina Karia, Tushar Kotecha et al

randomised controlled trial showed significantly improved 4 Condliffe R, Kiely DG, Peacock AJ et al. Connective tissue disease-
6-minute walk distance with sotatercept in patients with PAH associated pulmonary arterial hypertension in the modern
already receiving stable background PH therapy, including patients treatment era. Am J Respir Crit Care Med 2009;179:151–7.
on parenteral prostanoid therapy.15 5 Weatherald J, Montani D, Jevnikar M et al. Screening for pulmonary
arterial hypertension in systemic sclerosis. Eur Respir Rev 2019;28:
190023.
Treatments options for CTEPH (Group 4) 6 Humbert M, Yaici A, de Groote P et al. Screening for pulmonary
arterial hypertension in patients with systemic sclerosis: clinical
Medical, percutaneous, and surgical treatment options are now
characteristics at diagnosis and long-term survival. Arthritis Rheum
available for CTEPH. In patients with anatomically suitable disease 2011;63:3522–30.
(in terms of distribution and burden), pulmonary endarterectomy 7 Coghlan JG, Denton CP, Grunig E et al. Evidence-based detection of
(PEA) is the treatment of choice following evaluation by expert pulmonary arterial hypertension in systemic sclerosis: the DETECT
centres.16 Percutaneous balloon pulmonary angioplasty (BPA) study. Ann Rheum Dis 2014;73:1340–9.
is a more recent treatment modality recommended in suitable 8 Bermejo J, Yotti R, García-Orta R et al. Sildenafil for improving
patients with CTEPH who have inoperable disease or with outcomes in patients with corrected valvular heart disease and
residual CTEPH following PEA surgery.17,18 Furthermore, BPA in persistent pulmonary hypertension: a multicenter, double-blind,
combination with medical PH therapy has been upgraded in randomized clinical trial. Eur Heart J 2018;39:1255–64.
9 Waxman A, Restrepo-Jaramillo R, Thenappan T et al. Inhaled
the CTEPH treatment algorithm. Riociguat, a soluble guanylate
treprostinil in pulmonary hypertension due to interstitial lung
cyclase inhibitor, is the recommended medical therapy in patients
disease. N Engl J Med 2021;384:325–34.
with inoperable CTEPH or with residual CTEPH following PEA.19 10 Hoeper MM, Pausch C, Grünig E et al. Idiopathic pulmonary arterial
Lifelong anticoagulation is recommended in all patients with hypertension phenotypes determined by cluster analysis from the
CTEPH. Patients with CTEPH should be tested for antiphospholipid COMPERA registry. J Heart Lung Transplant 2020;39:1435–44.
syndrome and, when found, treated with vitamin K antagonists. 11 Galiè N, Barberà JA, Frost AE et al. Initial use of ambrisentan
plus tadalafil in pulmonary arterial hypertension. N Engl J Med
2015;373:834–44.
Conclusions
12 Boucly A, Savale L, Jaïs X et al. Association between initial
The early recognition of suspected PH along with systematic treatment strategy and long-term survival in pulmonary arterial
screening of at-risk patient groups enables early diagnosis and, hypertension. Am J Respir Crit Care Med 2021;204:842–54.
consequently, early treatment. Obtaining a prompt and accurate 13 Hoeper MM, Kramer T, Pan Z et al. Mortality in pulmonary arte-
rial hypertension: prediction by the 2015 European pulmonary
diagnosis determines treatment strategies and impacts on
hypertension guidelines risk stratification model. Eur Respir J
prognosis, with referral to specialist PH centres for RHC being the
2017;50:1700740.
final step in the diagnostic algorithm. This requires a collaborative 14 Sitbon O, Channick R, Chin KM et al. Selexipag for the treatment of
multidisciplinary approach between referrers across a wide range pulmonary arterial hypertension. N Engl J Med 2015;373:2522–33.
of medical specialties, national PH centres, and their associated 15 Hoeper MM, Badesch DB, Ghofrani HA et al. Phase 3 trial of sota-
regional shared care centres. ■ tercept for treatment of pulmonary arterial hypertension. N Engl J
Med 2023;388:1478–90.
16 Kim NH, Delcroix M, Jais X et al. Chronic thromboembolic
Funding and disclosures
pulmonary hypertension. Eur Respir J 2019;53:1801915.
DSK is supported by a British Heart Foundation (BHF) Clinical 17 Kataoka M, Inami T, Hayashida K et al. Percutaneous transluminal
Research Leave Fellowship (FS/CRLF/20/23004). TK has received pulmonary angioplasty for the treatment of chronic thromboembolic
speaker fees from Janssen UK. BES has received consulting fees, pulmonary hypertension. Circ Cardiovasc Interv 2012;5:756–62.
speaker fees, and research funding from Johnson & Johnson, 18 Mizoguchi H, Ogawa A, Munemasa M et al. Refined balloon pulmo-
nary angioplasty for inoperable patients with chronic thromboembolic
and speaker fees from GSK. JGC has received consulting fees
pulmonary hypertension. Circ Cardiovasc Interv 2012;5:748–55.
from Acceleron, consulting and speaker fees from Bayer, GSK 19 Ghofrani HA, D’Armini AM, Grimminger F et al. Riociguat for the
and Johnson & Johnson, and research funding from Johnson & treatment of chronic thromboembolic pulmonary hypertension.
Johnson. DSK has received research support, speaker fees, and N Engl J Med 2013;369:319–29.
advisory board fees from Janssen UK. 20 Vachiéry JL, Tedford RJ, Rosenkranz S et al. Pulmonary hypertension
due to left heart disease. Eur Respir J 2019;53:1801897.
21 Simonneau G, Montani D, Celermajer DS et al. Haemodynamic
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454 © Royal College of Physicians 2023. All rights reserved.