Guideline sources The following summarized guidelines for the evaluation and management of pulmonary hypertension (PH) are prepared by our editorial team based on guidelines from the European Society of Cardiology (ESC/ERS 2022), the American College of Radiology (ACR 2022), the Canadian Cardiovascular Society (CCS/CTS 2020), the American College of Chest Physicians (ACCP 2019; 2014), the European Society of Cardiology (ESC/ESR 2016), the International Society for Heart and Lung Transplantation (ISHLT 2015), and the American Association of Family Physicians (AAFP 2013). 1 2 3 4 5 6 7 8 Calculator Calculator https://web.pathway.md/diseases/reckQhYSI2H7lpvit 1/13 6/29/23, 11:35 PM Pulmonary hypertension Pathway Calculator Calculator NYHA functional classification f REVEAL risk score for pulmonar Guidelines 1. Screening and diagnosis Indications for screening (genetic causes): provide counseling regarding the risk of pulmonary arterial hypertension and obtain annual screening in patients tested positive for pulmonary arterial hypertension-causing mutations and in first-degree relatives of patients with heritable pulmonary arterial hypertension. B Indications for screening (connective tissue disease): consider obtaining further diagnostic tests (echocardiography, BNP/N-terminal pro-BNP, pulmonary function tests, and/or cardiopulmonary exercise testing) to screen for pulmonary arterial hypertension in symptomatic patients with connective tissue disease. C Indications for screening, systemic sclerosis: As per ERS/ESC 2022 guidelines, obtain an annual assessment of the risk of having pulmonary arterial hypertension in patients with systemic sclerosis. B Consider obtaining an annual assessment of the risk of pulmonary arterial hypertension in patients with connective tissue disease with overlap features of systemic sclerosis. B Show 5 more As per CTS/CCS 2020 guidelines, obtain annual echocardiography and measurement of lung diffusing capacity for CO to screen for PH in all patients with systemic sclerosis. B Indications for screening (HIV): consider obtaining further diagnostic tests (echocardiography, BNP/N-terminal pro-BNP, pulmonary function tests, and/or cardiopulmonary exercise testing) to screen for pulmonary arterial hypertension in symptomatic patients with human immunodeficiency virus infection. C Indications for screening, liver transplant candidates: As per ERS/ESC 2022 guidelines: Obtain echocardiography in patients with liver disease or portal hypertension with signs or symptoms suggestive of PH and as a screening test in patients evaluated for liver transplantation or transjugular portosystemic shunt. B Consider obtaining further diagnostic tests (echocardiography, BNP/N-terminal pro-BNP, pulmonary function tests, and/or cardiopulmonary exercise testing) to screen for pulmonary arterial hypertension in symptomatic patients with portal hypertension. C As per CTS/CCS 2020 guidelines, obtain echocardiography to screen for PH in all patients with portal hypertension being assessed for liver transplantation. B Indications for screening (bronchopulmonary dysplasia): screen infants with bronchopulmonary dysplasia for PH. B https://web.pathway.md/diseases/reckQhYSI2H7lpvit 2/13 6/29/23, 11:35 PM Pulmonary hypertension Pathway Indications for testing: obtain echocardiography in patients with obesity, obstructive sleep apnea, and edema to evaluate pulmonary arterial pressures. B 2. Classification and risk stratification Severity assessment: as per ESC 2022 guidelines, assess disease severity in patients with pulmonary arterial hypertension with a panel of data derived from clinical assessment, exercise tests, biochemical markers, echocardiography, and hemodynamic evaluations. B Show 2 more 3. Diagnostic investigations Echocardiography: As per ACR 2022 guidelines, obtain a resting TTE as initial imaging in patients with suspected PH. B As per ESC 2022 guidelines, obtain echocardiography as first-line, noninvasive diagnostic investigation in patients with suspected PH. B Show 4 more As per CCS 2020 guidelines, obtain transthoracic echocardiography for the initial assessment in all patients with clinically suspected PH or unexplained dyspnea. B Show 3 more Chest computed tomography: As per ACR 2022 guidelines, obtain contrast-enhanced chest CT as initial imaging in patients with suspected PH. B As per ESC 2022 guidelines, consider obtaining chest CT in all patients with PH. C Computed tomography angiography: As per ACR 2022 guidelines, obtain chest CT angiography as initial imaging in patients with suspected PH. B As per ESC 2022 guidelines, obtain CT pulmonary angiography in the evaluation of patients with suspected chronic thromboembolic PH. B Pulmonary angiography: consider obtaining digital subtraction angiography in the evaluation of patients with chronic thromboembolic PH. C Cardiac magnetic resonance imaging: consider obtaining cardiac MRI when accessible to assess right ventricle size and function to help guide management in patients with pulmonary arterial hypertension. C Perfusion lung scan: As per ESC 2022 guidelines, obtain V/Q or perfusion lung scan to assess for chronic thromboembolic PH in patients with unexplained PH. B As per CCS 2020 guidelines, obtain an initial V/Q scanning to assess the possibility of chronic thromboembolic PH in patients being evaluated for PH. B Vasoreactivity testing: https://web.pathway.md/diseases/reckQhYSI2H7lpvit 3/13 6/29/23, 11:35 PM Pulmonary hypertension Pathway As per ESC 2022 guidelines, obtain vasoreactivity testing in patients with idiopathic, heritable, drug-associated pulmonary arterial hypertension to identify patients who can be treated with high doses of CCBs. B Show 4 more As per CCS 2020 guidelines: Obtain acute vasodilator testing (with inhaled nitro oxygen, IV epoprostenol or adenosine) in selected patients with pulmonary arterial hypertension (idiopathic, heritable, or drug- associated pulmonary arterial hypertension). B View marked vasodilator responsiveness (decrease in mean pulmonary arterial pressure 10 mmHg to < 40 mmHg and stable/increased cardiac output) as predictive of responsiveness to high-dose CCBs (to be initiated in PH expert centers). B Pulmonary function tests: obtain pulmonary function tests with lung diffusion capacity for CO in the initial evaluation of patients with PH. B Abdominal ultrasound: obtain abdominal ultrasound to screen for portal hypertension. B Laboratory evaluation: as per ESC 2022 guidelines, obtain routine biochemistry, hematology, immunology, human immunodeficiency virus testing, and thyroid function tests to identify associated conditions in all patients with pulmonary arterial hypertension. B 4. Diagnostic procedures Right heart catheterization: As per ESC 2022 guidelines, perform right heart catheterization to confirm the diagnosis of PH (especially pulmonary arterial hypertension or chronic thromboembolic PH) and to support treatment decisions. B Show 2 more As per CCS 2020 guidelines, perform right heart catheterization to confirm the hemodynamic diagnosis of precapillary PH and to assess the severity of PH in all patients with suspected pulmonary arterial hypertension or chronic thromboembolic PH. B Show 2 more Left heart catheterization: Consider performing left heart catheterization to measure LV end-diastolic pressure. C Consider left heart catheterization to measure LV end diastolic pressure when measurement of pulmonary arterial wedge pressure is unreliable. C Lung biopsy: do not perform open or thoracoscopic lung biopsy in patients with pulmonary arterial hypertension. D 5. Respiratory support Supplemental oxygen: As per ESC 2022 guidelines: Initiate long-term oxygen therapy in patients with pulmonary arterial hypertension with arterial blood oxygen pressure < 8 kPa. (60 mmHg). B https://web.pathway.md/diseases/reckQhYSI2H7lpvit 4/13 6/29/23, 11:35 PM Pulmonary hypertension Pathway Administer in-flight oxygen in patients using oxygen or if arterial blood oxygen pressure is < 8 kPa (60 mmHg) at sea level. B As per CCS 2020 guidelines, consider administering supplemental oxygen for resting hypoxemia in patients with pulmonary arterial hypertension. C 6. Medical management Setting of care: As per CCS 2020 guidelines, assess all patients with pulmonary arterial hypertension in a recognized PH center to confirm the diagnosis, direct institution of PH-targeted therapies, and evaluate the response to treatment. B As per ACCP 2014 guidelines, consider evaluating all patients with pulmonary arterial hypertension at a center with expertise in the diagnosis of pulmonary arterial hypertension, whenever possible, ideally before initiating treatment. E Show 2 more General principles: as per ESC 2022 guidelines, achieve and maintain a low-risk profile on optimized medical therapy as a treatment goal in patients with pulmonary arterial hypertension. B Show 3 more Calcium channel blockers: as per ESC 2022 guidelines, initiate high doses of CCBs in patients with idiopathic, heritable, or drug-associated pulmonary arterial hypertension responding to acute vasoreactivity testing. B Show 5 more PAH-specific therapy, initial therapy: As per ERS/ESC 2022 guidelines, initiate combination therapy with a PDE5 inhibitor and an endothelin receptor antagonist (such as ambrisentan and tadalafil, or macitentan and tadalafil) in patients with idiopathic, heritable, or drug-associated pulmonary arterial hypertension with a low or intermediate risk of death. B Show 3 more As per CTS/CCS 2020 guidelines, initiate oral monotherapy (an endothelin receptor antagonist, PDE5, or soluble guanylate cyclase stimulator) only in low-risk, treatment-naive patients with pulmonary arterial hypertension. A Show 3 more PAH-specific therapy, sequential therapy: as per ERS/ESC 2022 guidelines, base treatment escalation decisions on risk assessment and general treatment strategies. B Show 9 more Anticoagulant therapy: As per ESC 2022 guidelines, consider initiating anticoagulation therapy in patients with pulmonary arterial hypertension on an individual basis. C Show 3 more As per CCS 2020 guidelines: Consider initiating systemic anticoagulation with warfarin in selected patients with pulmonary arterial hypertension (idiopathic, heritable, or drug-associated pulmonary arterial https://web.pathway.md/diseases/reckQhYSI2H7lpvit 5/13 6/29/23, 11:35 PM Pulmonary hypertension Pathway hypertension) in the absence of elevated bleeding risk. C Do not initiate systemic anticoagulation in patients with pulmonary arterial hypertension associated with connective tissue disease, congenital heart disease, portal hypertension, or human immunodeficiency virus infection. D Management of fluid overload: As per ESC 2022 guidelines, administer diuretics in patients with pulmonary arterial hypertension with signs of RV failure and fluid retention. B As per CCS 2020 guidelines, consider administering diuretics for volume overload in patients with pulmonary arterial hypertension. C Management of iron deficiency: Correct iron status in patients with pulmonary arterial hypertension in the presence of iron deficiency anemia. B Consider initiating iron repletion in patients with pulmonary arterial hypertension with iron deficiency in the absence of anemia. C 7. Nonpharmacologic interventions Exercise training: As per ESC 2022 guidelines, advise supervised exercise training in patients with pulmonary arterial hypertension under medical therapy. A As per CCS 2020 guidelines, consider offering supervised exercise rehabilitation to improve functional capacity and health-related quality of life in patients with pulmonary arterial hypertension. B Psychosocial support: As per ESC 2022 guidelines: Provide psychosocial support to pulmonary arterial hypertension patients. B Provide psychosocial support in patients with pulmonary arterial hypertension. B As per CCS 2020 guidelines, provide general supportive measures (education, psychosocial support, contraception) to improve understanding and self-management in all patients with pulmonary arterial hypertension. B Palliative care support: consider incorporating palliative care services in the management of patients with pulmonary arterial hypertension. E 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 Show 2 more Extracorporeal life support: As per ESC 2022 guidelines, consider initiating mechanical circulatory support as an option in selected patients as a bridge to transplantation or recovery, and consider making an https://web.pathway.md/diseases/reckQhYSI2H7lpvit 6/13 6/29/23, 11:35 PM Pulmonary hypertension Pathway interhospital transfer if such resources are unavailable on site. C As per CCS 2020 guidelines, consider initiating extracorporeal life support as a bridge to definitive PH therapy in patients with refractory RV failure and/or hemodynamic instability. C 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 As per CCS 2020 guidelines, refer patients with PH (especially pulmonary arterial hypertension and chronic thromboembolic PH) with persistent severe PH (NYHA functional class III or IV, and/or RV failure) despite maximal medical therapy for lung transplantation assessment. B As per ATS 2015 guidelines, refer for lung transplantation evaluation if: NYHA Functional Class III or IV symptoms during escalating therapy rapidly progressive disease use of parenteral targeted pulmonary arterial hypertension therapy regardless of symptoms or NYHA Functional Class known or suspected pulmonary VOD or pulmonary capillary hemangiomatosis. Liver transplantation: as per ESC 2022 guidelines, consider performing liver transplantation in patients with pulmonary arterial hypertension associated with portal hypertension on an individual basis, as long as pulmonary vascular resistance is normal or near normal with pulmonary arterial hypertension therapy. C 11. Specific circumstances Pediatric patients: screen infants with bronchopulmonary dysplasia for PH. B Show 10 more Pregnant patients: as per ESC 2022 guidelines, counsel female patients of childbearing potential with pulmonary arterial hypertension 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 https://web.pathway.md/diseases/reckQhYSI2H7lpvit 7/13 6/29/23, 11:35 PM Pulmonary hypertension Pathway Show 5 more Patients with CTEPH: As per ESC 2022 guidelines, obtain diagnostic evaluation to assess for chronic thromboembolic PH/chronic thromboembolic pulmonary disease in patients with persistent or new-onset dyspnea or exercise intolerance after PE. B Show 20 more As per CCS 2020 guidelines, assess for chronic thromboembolic PH with echocardiography and V/Q lung scan in patients with residual dyspnea or exercise intolerance after at least 3 months of uninterrupted anticoagulation post-acute PE. B Show 5 more Patients with pulmonary PVOD/PCH: elicit a combination of clinical and radiological findings, obtain arterial blood gas analysis, pulmonary function tests, and genetic testing for the diagnosis of pulmonary arterial hypertension with signs of venous and/or capillary involvement (pulmonary VOD/pulmonary capillary hemangiomatosis). A Show 4 more Patients with drug-induced PAH: diagnose drug- or toxin-associated pulmonary arterial hypertension in the setting of relevant exposure when other causes of PH have been excluded. B Show 3 more Patients with lung disease-associated PH, evaluation: As per ERS/ESC 2022 guidelines: Obtain echocardiography when PH is suspected in patients with lung disease, and interpret results in conjunction with arterial blood gas analysis, pulmonary function tests (including lung diffusion capacity for CO), and CT. B Perform right heart catheterization if the results are expected to aid management decisions in patients with lung disease and suspected PH. B As per CTS/CCS 2020 guidelines, do not perform routine right heart catheterization in patients with mild-to-moderate, lung disease-associated PH, in the absence of RV failure. D Patients with lung disease-associated PH, referral: As per ERS/ESC 2022 guidelines: Refer patients with lung disease and suspected severe PH, or where there is uncertainty regarding the treatment of PH, to a PH center. B Refer eligible patients with lung disease and PH for evaluation of lung transplantation. B As per CTS/CCS 2020 guidelines, consider referring patients with moderate-to-severe, lung disease-associated PH (with or without features of RV failure) to a PH center. B Patients with lung disease-associated PH (management): optimize treatment of the underlying lung disease and, where indicated, hypoxemia, sleep-disordered breathing, and/or alveolar hypoventilation in patients with lung disease and suspected PH. B Show 7 more Patients with connective tissue disease: Treat the underlying condition in patients with pulmonary arterial hypertension associated with connective tissue disease, according to the current published guidelines. A https://web.pathway.md/diseases/reckQhYSI2H7lpvit 8/13 6/29/23, 11:35 PM Pulmonary hypertension Pathway Follow the same treatment algorithm in patients with pulmonary arterial hypertension associated with connective tissue disease as for patients with idiopathic pulmonary arterial hypertension. B Patients with HIV-associated PAH: consider obtaining diagnostic tests (echocardiography, BNP/N-terminal pro-BNP, pulmonary function tests, and/or cardiopulmonary exercise testing) to screen for pulmonary arterial hypertension in symptomatic patients with human immunodeficiency virus infection. C Show 2 more Patients with left heart disease (evaluation): optimize treatment of the underlying condition before obtaining an assessment of suspected PH in patients with left heart disease. A Show 5 more Patients with left heart disease, management: As per ERS/ESC 2022 guidelines, offer an individualized approach to treatment in patients with left heart disease and combined post- and pre-capillary PH with a severe pre-capillary component (such as pulmonary vascular resistance > 5 Wood units). B Show 3 more As per CTS/CCS 2020 guidelines: Optimize ventricular filling pressures and treat the underlying causes of and contributors to left heart disease for the management of patients with PH associated with left heart disease. B Do not use pulmonary arterial hypertension-specific therapies in patients with PH associated with left heart disease. D Patients with Eisenmenger syndrome (risk assessment): Obtain risk assessment in patients with persistent pulmonary arterial hypertension after defect closure. B Consider obtaining risk assessment in patients with Eisenmenger syndrome. C Patients with Eisenmenger syndrome (pharmacotherapy): initiate bosentan to improve exercise capacity in symptomatic patients with Eisenmenger syndrome. B Show 4 more Patients with Eisenmenger syndrome (management of hematological derangements): Consider initiating iron therapy in patients with iron deficiency. C Do not perform routine phlebotomy to lower elevated hematocrit in patients with Eisenmenger syndrome. D Patients with Eisenmenger syndrome (supplemental oxygen): consider administering supplemental oxygen therapy in cases where it consistently increases SaO and reduces symptoms in patients with Eisenmenger syndrome. C Patients with Eisenmenger syndrome (shunt closure): perform shunt closure in patients with an atrial septal defect, ventricular septal defect, or patent ductus arteriosus and a pulmonary vascular resistance < 3 Wood units. B Show 4 more Patients with Eisenmenger syndrome (pregnancy counseling): advise avoiding pregnancy in female patients with Eisenmenger syndrome. B https://web.pathway.md/diseases/reckQhYSI2H7lpvit 9/13 6/29/23, 11:35 PM Pulmonary hypertension Pathway Patients with portal hypertension: obtain abdominal ultrasound to screen for portal hypertension in patients with pulmonary arterial hypertension. B Show 6 more 12. Patient education Pregnancy counseling: as per ESC 2022 guidelines, counsel female patients of childbearing potential with pulmonary arterial hypertension 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 Show 3 more 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 pulmonary arterial hypertension. 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/chronic thromboembolic PH center, after considering the results of echocardiography, BNP/N-terminal pro-BNP, and/or cardiopulmonary exercise testing. B Show 3 more As per CCS 2020 guidelines: Refer all patients with suspected chronic thromboembolic PH to a local expert PH center for the establishment of a formal diagnosis of chronic thromboembolic PH and assessment for the most appropriate treatment. B Consider referring patients with moderate-to-severe, lung disease-associated PH (with or without features of RV failure) to a PH center. B Serial clinical and imaging assessment: As per CCS 2020 guidelines, obtain regular reassessment using a panel of measures (clinical, functional, hemodynamic, and/or right ventricle size/function) in all patients with pulmonary arterial hypertension. B As per ESC 2016 guidelines, follow-up stable patients every 3-6 months. B Management of persistent/recurrent PH: As per ESC 2022 guidelines, consider offering a multimodality approach in patients with persistent PH after pulmonary endarterectomy. C Show 2 more As per CCS 2020 guidelines: https://web.pathway.md/diseases/reckQhYSI2H7lpvit 10/13 6/29/23, 11:35 PM Pulmonary hypertension Pathway Initiate riociguat monotherapy in all patients with residual/recurrent chronic thromboembolic PH post-pulmonary endarterectomy. B Refer patients with PH (especially pulmonary arterial hypertension and chronic thromboembolic PH) with persistent severe PH (NYHA functional class III or IV, and/or RV failure) despite maximal medical therapy for lung transplantation assessment. B 15. Quality improvement Hospital requirements: Ensure that PH centers: provide care by a multidisciplinary team including a cardiologist, pne mologist, 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 pulmonary arterial hypertension or chronic thromboembolic PH and at least 2 new referrals per month with documented pulmonary arterial hypertension or chronic thromboembolic PH 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 Clinical findings Symptoms Past medical history Chest pain Atrial septal defect Dizziness COPD Fainting Congenital heart disease Fatigue Human immunodeficiency virus infection Lightheadedness Hypertension Palpitations ILD Shortness of breath Obstructive sleep apnea Medication history Patent ductus arteriosus Portal hypertension Aminorex Pulmonary VOD Dasatinib Pulmonary capillary hemangiomatosis Fenfluramine Methamphetamine Raynaud's phenomenon Schistosomiasis Cardiac exam Sickle cell disease https://web.pathway.md/diseases/reckQhYSI2H7lpvit 11/13 6/29/23, 11:35 PM Pulmonary hypertension Pathway Graham Steell murmur Systemic sclerosis Holosystolic murmur Vascular exam Jugular venous distention Palpable P2 Peripheral edema Parasternal heave Abdominal exam Split S2 heart sound Third heart sound Abdominal distension Ascites Imaging findings Hepatomegaly Enlarged pulmonary artery Enlarged right atrium Tricuspid regurgitation Ventricular septal defect Studies 2023 STELLAR In adult patients with pulmonary arterial hypertension who were receiving stable background therapy, sotatercept was superior to placebo with respect to improvement in the median 6- minute walk distance at week 24. Marius M Hoeper et al. N Engl J Med. 2023 Apr 20. 2021 PULSAR (low-dose sotatercept) In adults who were receiving background therapy for pulmonary arterial hypertension, low-dose sotatercept was superior to placebo with respect to pulmonary vascular resistance reduction at week 24. Marc Humbert et al. N Engl J Med. 2021 Apr 1. Show 1 more References 1. Marc Humbert, Gabor Kovacs, Marius M Hoeper et al. 2022 ESC/ERS Guidelines for the diagnosis and treatment of pulmonary hypertension. Eur Heart J. 2022 Oct 11;43 38 3618 3731. Open 2. Klinger JR, Elliott G, Levine DJ et al. Therapy for Pulmonary Arterial Hypertension in Adults 2018 Update of the CHEST Guideline and Expert Panel Report. Chest. 2019 Jan 17. pii: S0012 3692 19 30002 9. Open 3. Naushad Hirani, Nathan W Brunner, Ali Kapasi et al. Canadian Cardiovascular Society/Canadian Thoracic Society Position Statement on Pulmonary Hypertension. Can J Cardiol. 2020 Jul;36 7 977 992. Open 4. Gali N, Humbert M, Vachiery JL et al. 2015 ESC/ERS Guidelines for the Diagnosis and Treatment of Pulmonary Hypertension. Rev Esp Cardiol Engl Ed). 2016 Feb;69 2 177. Open 5. Expert Panel on Thoracic Imaging, Arlene Sirajuddin, S Mojdeh Mirmomen et al. ACR Appropriateness Criteria Suspected Pulmonary Hypertension: 2022 Update. J Am Coll Radiol. 2022 Nov;19 11S S502 https://web.pathway.md/diseases/reckQhYSI2H7lpvit 12/13 6/29/23, 11:35 PM Pulmonary hypertension Pathway S512. Open 6. Taichman DB, Ornelas J, Chung L et al. Pharmacologic therapy for pulmonary arterial hypertension in adults: CHEST guideline and expert panel report. Chest. 2014 Aug;146 2 449 475. Open 7. Weill D, Benden C, Corris PA et al. A consensus document for the selection of lung transplant candidates: 2014 an update from the Pulmonary Transplantation Council of the International Society for Heart and Lung Transplantation. J Heart Lung Transplant. 2015 Jan;34 1 1 15. Open 8. Kathryn P Trayes, James S Studdiford, Sarah Pickle et al. Edema: Diagnosis and Management. Am Fam Physician. 2013 Jul 15;88 2 102 10. Open 9. B Bradley, H M Branley, J J Egan Irish Thoracic Society) et al. Interstitial lung disease guideline: the British Thoracic Society in collaboration with the Thoracic Society of Australia and New Zealand and the Irish Thoracic Society. Thorax. 2008 Sep;63 Suppl 5:v1 58. Open 10. Hossein-Ardeschir Ghofrani, Andrea M D'Armini, Friedrich Grimminger et al. Riociguat for the treatment of chronic thromboembolic pulmonary hypertension. N Engl J Med. 2013 Jul 25;369 4 319 29. Open 11. Regitz-Zagrosek V, Roos-Hesselink JW, Bauersachs J et al. 2018 ESC Guidelines for the management of cardiovascular diseases during pregnancy. Eur Heart J. 2018 Sep 7;39 34 3165 3241. Open https://web.pathway.md/diseases/reckQhYSI2H7lpvit 13/13