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Pulmonary hypertension

Guidelines

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 2025,2023), the American Academy of Family Physicians (AAFP 2024,2013), the British Society for Rheumatology (BSR 2024), the Society of Cardiovascular Computed Tomography (SCCT/SVM/SCMR/SCA/AHA/ASNC/HRS/ACC/ACS 2024), the American College of Radiology (ACR 2022), the European ...
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Screening and diagnosis

Indications for screening, patients at risk: as per ACCP 2019 guidelines, consider monitoring patients at increased risk for PAH (family history of PAH, known PAH-causing genetic mutation in patient or first-degree relative, limited cutaneous scleroderma, or mixed connective tissue disease, exposure to drugs or toxins known to cause PH, and congenital heart disease with surgically repaired left-to-right shunt within 3-6 months) for the development of symptoms of PAH.
E
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  • Indications for screening (genetic causes)

  • 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

Classification and risk stratification

Severity assessment: as per ERS/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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Diagnostic investigations

Echocardiography: as per AAFP 2024 guidelines, obtain echocardiography as the initial investigation 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

Diagnostic procedures

Right heart catheterization: as per AAFP 2024 guidelines, perform right heart catheterization as the test of choice to confirm PH, especially if PAH or CTEPH is suspected, and can guide treatment decisions.
B
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  • Left heart catheterization

  • Lung biopsy

Respiratory support

Supplemental oxygen: as per AAFP 2024 guidelines, initiate long-term oxygen therapy in patients with PAH with an arterial blood oxygen pressure < 60 mmHg, aiming to maintain oxygen saturation > 90% at sea level.
B
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Medical management

Setting of care: as per AAFP 2024 guidelines, refer patients with PAH to a specialty center.
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

Nonpharmacologic interventions

Exercise training: as per AAFP 2024 guidelines, offer a supervised exercise training program in patients with PAH optimized and stable on medical therapy.
A
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  • Pulmonary rehabilitation

  • Psychosocial support

  • Palliative care support

Therapeutic procedures

Balloon pulmonary angioplasty: as per ERS/ESC 2022 guidelines, 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

Perioperative care

Choice of anesthesia: as per ERS/ESC 2022 guidelines, consider obtaining multidisciplinary consultation at a PH center to assess the risks and benefits of interventions requiring anesthesia.
C
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Surgical interventions

Lung transplantation
As per ERS/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

Specific circumstances

Pediatric patients, evaluation: as per ERS/ESC 2022 guidelines, screen infants with bronchopulmonary dysplasia for PH.
B
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  • Pediatric patients (general principles of management)

  • Pediatric patients (therapeutic and surgical interventions)

  • Pregnant patients

  • Patients with CTEPH (evaluation)

  • Patients with CTEPH (setting of care)

  • Patients with CTEPH (anticoagulation)

  • Patients with CTEPH (pulmonary endarterectomy and balloon pulmonary angioplasty)

  • Patients with CTEPH (pharmacotherapy)

  • Patients with CTEPH (exercise training)

  • Patients with CTEPH (lung transplantation)

  • Patients with pulmonary veno-occlusive disease/pulmonary capillary hemangiomatosis

  • 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 (setting of care)

  • Patients with connective tissue disease (general principles)

  • Patients with connective tissue disease (pharmacotherapy)

  • Patients with connective tissue disease (anticoagulant therapy)

  • Patients with connective tissue disease (supportive care)

  • 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

  • Patients undergoing noncardiac surgery

Patient education

Pregnancy counseling: as per ERS/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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Preventative measures

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

Indications for specialist referral: as per ERS/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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  • Clinical and imaging follow-up

  • Management of persistent/recurrent PH

Quality improvement

Hospital requirements
As per ERS/ESC 2022 guidelines:
Ensure that PH centers:
provide care by a multidisciplinary team including a cardiologist, pneumologist, 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
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