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Pulmonary hypertension

Key sources
The following summarized guidelines for the evaluation and management of pulmonary hypertension are prepared by our editorial team based on guidelines from the American Thoracic Society (ATS 2023), the European Society of Cardiology (ESC/ERS 2022; 2016), the American College of Radiology (ACR 2022), the Canadian Cardiovascular Society (CCS/CTS 2020), the American College of Chest Physicians (ACCP 2019; 2014), the International Society for Heart and Lung Transplantation (ISHLT 2015), and the American Academy of Family Physicians (AAFP 2013).
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Guidelines

1.Screening and diagnosis

Indications for screening, genetic causes: provide counseling regarding the risk of PAH and obtain annual screening in patients tested positive for PAH-causing mutations and in first-degree relatives of patients with heritable PAH.
B
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  • Indications for screening (connective tissue disease)

  • Indications for screening (systemic sclerosis)

  • Indications for screening (HIV)

  • Indications for screening (liver transplant candidates)

  • Indications for screening (bronchopulmonary dysplasia)

  • Indications for testing

2.Classification and risk stratification

Severity assessment: as per ESC 2022 guidelines, assess disease severity in patients with PAH with a panel of data derived from clinical assessment, exercise tests, biochemical markers, echocardiography, and hemodynamic evaluations.
B
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3.Diagnostic investigations

Echocardiography: as per ACR 2022 guidelines, obtain a resting TTE as initial imaging in patients with suspected PH.
B

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  • Chest CT

  • CTA

  • Pulmonary angiography

  • Cardiac MRI

  • Perfusion lung scan

  • Vasoreactivity testing

  • Pulmonary function tests

  • Abdominal ultrasound

  • Laboratory evaluation

4.Diagnostic procedures

Right heart catheterization: as per ESC 2022 guidelines, perform right heart catheterization to confirm the diagnosis of PH (especially PAH or CTEPH) and to support treatment decisions.
B
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  • Left heart catheterization

  • Lung biopsy

5.Respiratory support

Supplemental oxygen
As per ESC 2022 guidelines:
Initiate long-term oxygen therapy in patients with PAH with arterial blood oxygen pressure < 8 kPa. (60 mmHg).
B
Administer in-flight oxygen in patients using oxygen or if arterial blood oxygen pressure is < 8 kPa (60 mmHg) at sea level.
B

6.Medical management

Setting of care: as per CCS 2020 guidelines, assess all patients with PAH in a recognized PH center to confirm the diagnosis, direct institution of PH-targeted therapies, and evaluate the response to treatment.
B

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  • General principles

  • CCBs

  • PAH-specific therapy (initial therapy)

  • PAH-specific therapy (sequential therapy)

  • Anticoagulant therapy

  • Management of fluid overload

  • Management of iron deficiency

7.Nonpharmacologic interventions

Exercise training: as per ESC 2022 guidelines, advise supervised exercise training in patients with PAH under medical therapy.
A

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  • Pulmonary rehabilitation

  • Psychosocial support

  • Palliative care support

8.Therapeutic procedures

Balloon pulmonary angioplasty: perform balloon pulmonary angioplasty in technically inoperable patients or in patients with residual PH after pulmonary endarterectomy and distal obstructions amenable to balloon pulmonary angioplasty.
B
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  • Extracorporeal life support

9.Perioperative care

Choice of anesthesia: consider obtaining multidisciplinary consultation at a PH center to assess the risks and benefits of interventions requiring anesthesia.
C

10.Surgical interventions

Lung transplantation
As per ESC 2022 guidelines:
Refer potential eligible candidates with an inadequate response to oral combination therapy, defined by an intermediate-high or high risk or by a REVEAL risk score > 7, for lung transplantation evaluation.
B
List patients for lung transplantation when presenting with a high risk of death or with a REVEAL risk score ≥ 10 despite receiving optimized medical therapy including SC or IV prostacyclin analogs.
B

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  • Liver transplantation

11.Specific circumstances

Pediatric patients: screen infants with bronchopulmonary dysplasia for PH.
B
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  • Pregnant patients

  • Patients with CTEPH

  • Patients with pulmonary PVOD/PCH

  • Patients with drug-induced PAH

  • Patients with lung disease-associated PH (evaluation)

  • Patients with lung disease-associated PH (referral)

  • Patients with lung disease-associated PH (management)

  • Patients with connective tissue disease

  • Patients with HIV-associated PAH

  • Patients with left heart disease (evaluation)

  • Patients with left heart disease (management)

  • Patients with Eisenmenger syndrome (risk assessment)

  • Patients with Eisenmenger syndrome (pharmacotherapy)

  • Patients with Eisenmenger syndrome (management of hematological derangements)

  • Patients with Eisenmenger syndrome (supplemental oxygen)

  • Patients with Eisenmenger syndrome (shunt closure)

  • Patients with Eisenmenger syndrome (pregnancy counseling)

  • Patients with portal hypertension

12.Patient education

Pregnancy counseling: as per ESC 2022 guidelines, counsel female patients of childbearing potential with PAH at the time of diagnosis about the risks and uncertainties associated with becoming pregnant, including advice against becoming pregnant and referral for psychological support where needed.
B
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13.Preventative measures

Routine immunizations: as per ESC 2022 guidelines, provide immunization against severe acute respiratory syndrome coronavirus-2, influenza, and S. pneumoniae in patients with PAH.
B

14.Follow-up and surveillance

Indications for specialist referral: as per ESC 2022 guidelines, refer symptomatic patients with mismatched perfusion lung defects beyond 3 months of anticoagulation or acute PE to a PH/CTEPH center, after considering the results of echocardiography, BNP/NT-proBNP, and/or cardiopulmonary exercise testing.
B
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  • Serial clinical and imaging assessment

  • Management of persistent/recurrent PH

15.Quality improvement

Hospital requirements
Ensure that PH centers:
provide care by a multidisciplinary team including a cardiologist, pneµmologist, rheumatologist, nurse specialist, radiologist, psychological and social work support, and appropriate on-call expertise
B
have direct links and quick referral patterns to other services, such as genetic counseling, pulmonary endarterectomy/balloon pulmonary angioplasty, lung transplantation, and adult congenital heart disease service
B
maintain a patient registry
B
collaborate with patient associations
B
follow-up a sufficient number of patients of at least 50 patients with PAH or CTEPH and at least 2 new referrals per month with documented PAH or CTEPH in order to maintain expertise, and consider establishing collaborations with high-volume centers.
B
Consider ensuring that PH centers have accreditation and participate in collaborative clinical research.
C