ESC 2022 2022 ESC/ERS Guidelines for the diagnosis and treatment of pulmonary hypertension
European Society of Cardiology｜循環器 / 肺循環｜114 ページ｜チャンク 1080｜推奨 231 件｜current
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| 頁 | クラス | レベル | 推奨文 | 表 |
|---|---|---|---|---|
| p.18 | I | B | Low Conditional | |
| p.18 | III | C | Low Conditional | |
| p.18 | IIb | C | Very low Conditional | |
| p.18 | III | C | The use of PDE5i in patients with ILD and non-severe PH is not recommended Very low Conditional | |
| p.18 | IIa | B | Very low Conditional | |
| p.31 | I | B | Right heart catheterization It is recommended that RHC is performed to confirm the diagnosis of PH (especially PAH or CTEPH) and to support treatment decisions25,26 | Recommendation Table 1 — Recommendations for right heart catheterization and vasoreactivity testing |
| p.31 | I | C | In patients with suspected or known PH, it is recommended that RHC is performed in experienced centres125 | Recommendation Table 1 — Recommendations for right heart catheterization and vasoreactivity testing |
| p.31 | I | C | It is recommended that RHC comprises a complete set of haemodynamics and is performed following standardized protocols25,26,145 | Recommendation Table 1 — Recommendations for right heart catheterization and vasoreactivity testing |
| p.31 | I | B | Vasoreactivity testing Vasoreactivity testing is recommended in patients with I/H/DPAH to detect those who can be treated with high doses of a CCB129,146 | Recommendation Table 1 — Recommendations for right heart catheterization and vasoreactivity testing |
| p.31 | I | C | It is recommended that vasoreactivity testing is performed at PH centres It is recommended to consider a positive response | Recommendation Table 1 — Recommendations for right heart catheterization and vasoreactivity testing |
| p.31 | I | C | to vasoreactivity testing by a reduction in mPAP ≥10 mmHg to reach an absolute value of mPAP ≤40 mmHg with an increased or unchanged | Recommendation Table 1 — Recommendations for right heart catheterization and vasoreactivity testing |
| p.31 | I | C | COc129 Inhaled nitric oxide, inhaled iloprost, or i.v. epoprostenol are recommended for performing vasoreactivity testing129–132 | Recommendation Table 1 — Recommendations for right heart catheterization and vasoreactivity testing |
| p.31 | III | C | Vasoreactivity testing, for identifying candidates for CCB therapy, is not recommended in patients with PAH other than I/H/DPAH, and in PH groups 2, 3, 4, and 5124,129 | Recommendation Table 1 — Recommendations for right heart catheterization and vasoreactivity testing |
| p.35 | I | C | screening of portal hypertension164 | |
| p.35 | I | B | Echocardiography Echocardiography is recommended as the first-line, non-invasive, diagnostic investigation in suspected PH82,84,91 | Recommendation Table 2 — Recommendations for diagnostic strategy |
| p.35 | I | B | It is recommended to assign an echocardiographic probability of PH, based on an abnormal TRV and the presence of other echocardiographic signs suggestive of PH (see Table 10)91,92,162 | Recommendation Table 2 — Recommendations for diagnostic strategy |
| p.35 | I | C | It is recommended to maintain the current threshold for TRV (.2.8 m/s) for echocardiographic probability of PH according to the updated haemodynamic definition88 | Recommendation Table 2 — Recommendations for diagnostic strategy |
| p.35 | IIa | B | Based on the probability of PH by echocardiography, further testing should be considered in the clinical context (i.e. symptoms and risk factors or associated conditions for PAH/ CTEPH)92 | Recommendation Table 2 — Recommendations for diagnostic strategy |
| p.35 | IIb | C | In symptomatic patients with intermediate echocardiographic probability of PH, CPET may be considered to further determine the likelihood of PH123,163 | Recommendation Table 2 — Recommendations for diagnostic strategy |
| p.35 | I | C | PH Imaging Ventilation/perfusion or perfusion lung scan is recommended in patients with unexplained PH to assess for CTEPH105 | |
| p.35 | I | C | CT pulmonary angiography is recommended in the work-up of patients with suspected CTEPH104 | |
| p.35 | I | C | Routine biochemistry, haematology, immunology, HIV testing, and thyroid function tests are recommended in all patients with PAH, to identify associated conditions | |
| p.35 | IIa | C | Abdominal ultrasound is recommended for the Chest CT should be considered in all patients with PH | |
| p.35 | IIa | C | considered in the work-up of patients with CTEPH | Recommendation Table 2 — Recommendations for diagnostic strategy |
| p.35 | I | C | Other diagnostic tests Pulmonary function tests with DLCO are recommended in the initial evaluation of patients with PH78 | Recommendation Table 2 — Recommendations for diagnostic strategy |
| p.35 | III | C | Open or thoracoscopic lung biopsy is not recommended in patients with PAH | Recommendation Table 2 — Recommendations for diagnostic strategy |
| p.37 | I | B | risk of having PAH is recommended183,186 | Recommendation Table 3 — Recommendations for screening and improved detection of pulmonary arterial hypertension and chronic thrombo-embolic pulmonary hypertension |
| p.37 | I | B | In adult patients with SSc with .3 years’ disease duration, an FVC ≥40%, and a DLCO ,60%, the DETECT algorithm is recommended to identify asymptomatic patients with PAH173,186 | |
| p.37 | I | C | In patients with SSc, where breathlessness remains unexplained following non-invasive assessment, RHC is recommended to exclude PAH185–187 | |
| p.37 | IIa | B | Assessing the risk of having PAH based on an evaluation of breathlessness, in combination with echocardiogram or PFTs and BNP/NT-proBNP, should be considered in patients with | |
| p.37 | IIa | C | SSc172,173,186,188,190 Policies to evaluate the risk of having PAH should be considered in hospitals managing patients with SSc | |
| p.37 | IIb | C | In symptomatic patients with SSc, exercise echocardiography or CPET, or CMR may be considered to aid decisions to perform RHC | |
| p.37 | IIb | C | In patients with CTD with overlap features of SSc, an annual evaluation of the risk of PAH may be considered CTEPH/CTEPD | |
| p.37 | I | C | In patients with persistent or new-onset dyspnoea or exercise limitation following PE, further diagnostic evaluation to assess for CTEPH/ CTEPD is recommended103 | |
| p.37 | I | C | For symptomatic patients with mismatched perfusion lung defects beyond 3 months of anticoagulation for acute PE, referral to a PH/ CTEPH centre is recommended after considering the results of echocardiography, BNP/ NT-proBNP, and/or CPET203,206 | |
| p.37 | I | B | Other Counselling regarding the risk of PAH and annual screening are recommended in individuals who test positive for PAH-causing mutations and in first-degree relatives of patients with HPAH33 | |
| p.37 | I | C | In patients referred for liver transplantation, echocardiography is recommended as a screening test for PH | |
| p.37 | IIa | B | Further tests (echocardiography, BNP/ NT-proBNP, PFTs, and/or CPET) should be considered in symptomatic patients with CTD, portal hypertension, or HIV to screen for PAH172 Continued | |
| p.43 | I | B | It is recommended to evaluate disease severity in patients with PAH with a panel of data derived from clinical assessment, exercise tests, biochemical markers, echocardiography, and haemodynamic | Recommendation Table 4 — Recommendations for evaluating the disease severity and risk of death in patients with pulmonary arterial hypertension |
| p.43 | I | B | evaluations212,213,216,249,292,293,295,296,302,307 Achieving and maintaining a low-risk profile on optimized medical therapy is recommended as a treatment goal in patients with PAH210,212,213,216,298,300,303,309,310 | Recommendation Table 4 — Recommendations for evaluating the disease severity and risk of death in patients with pulmonary arterial hypertension |
| p.43 | I | B | For risk stratification at the time of diagnosis, the use of a three-strata model (low, intermediate, and high risk) is recommended, taking into account all available data, including haemodynamics292,293,295 | Recommendation Table 4 — Recommendations for evaluating the disease severity and risk of death in patients with pulmonary arterial hypertension |
| p.43 | I | B | For risk stratification during follow-up, the use of a four-strata model (low, intermediate–low, intermediate–high, and high risk) based on WHO-FC, 6MWD, and BNP/NT-proBNP is recommended, with additional variables taken into account as necessary280,308 | Recommendation Table 4 — Recommendations for evaluating the disease severity and risk of death in patients with pulmonary arterial hypertension |
| p.43 | IIa | B | In some PAH aetiologies and patients with comorbidities, optimization of therapy should be considered on an individual basis, while acknowledging that a low-risk profile is not always achievable293,294,299,311 | Recommendation Table 4 — Recommendations for evaluating the disease severity and risk of death in patients with pulmonary arterial hypertension |
| p.45 | I | A | General measures Supervised exercise training is recommended in patients with PAH under medical therapy314,315,317 | Recommendation Table 5 — Recommendations for general measures and special circumstances |
| p.46 | I | C | Psychosocial support is recommended in patients with PAH | |
| p.46 | I | C | Immunization of patients with PAH against SARS-CoV-2, influenza, and Streptococcus pneumoniae is recommended | |
| p.46 | I | C | Diuretic treatment is recommended in patients with PAH with signs of RV failure and fluid retention | |
| p.46 | I | C | Long-term oxygen therapy is recommended in patients with PAH whose arterial blood oxygen pressure is ,8 kPa (60 mmHg)c | |
| p.46 | I | C | In the presence of iron-deficiency anaemia, correction of iron status is recommended in patients with PAH | |
| p.46 | IIb | C | In the absence of anaemia, iron repletion may be considered in patients with PAH with iron deficiency | |
| p.46 | IIb | C | Anticoagulation is not generally recommended in patients with PAH but may be considered on an individual basis | |
| p.46 | III | C | The use of ACEis, ARBs, ARNIs, SGLT-2is, beta-blockers, or ivabradine is not recommended in patients with PAH unless required by comorbidities (i.e. high blood pressure, coronary artery disease, left HF, or arrhythmias) | |
| p.46 | I | C | Special circumstances In-flight oxygen administration is recommended for patients using oxygen or whose arterial blood oxygen pressure is ,8 kPa (60 mmHg) at sea level | |
| p.46 | IIa | C | For interventions requiring anaesthesia, multidisciplinary consultation at a PH centre to assess risk and benefit should be considered | |
| p.46 | I | C | It is recommended that women of childbearing potential with PAH are counselled at the time of diagnosis about the risks and uncertainties associated with becoming pregnant; this should include advice against becoming pregnant, and referral for psychological support where needed | Recommendation Table 6 — Recommendations for women of childbearing potential |
| p.46 | I | C | It is recommended to provide women of childbearing potential with PAH with clear contraceptive advice, considering the individual needs of the woman but recognizing that the implications of contraceptive failure are significant in PAH | |
| p.46 | I | C | It is recommended that women with PAH who consider pregnancy or who become pregnant receive prompt counselling in an experienced PH centre, to facilitate genetic counselling and shared decision-making, and to provide psychological support to the patients and their families where needed | |
| p.46 | I | C | For women with PAH having a termination of pregnancy, it is recommended that this be performed in PH centres, with psychological support provided to the patients and their families | |
| p.46 | IIb | C | For women with PAH who desire to have children, where available, adoption and surrogacy with pre- conception genetic counselling may be considered | |
| p.46 | III | B | As teratogenic potential has been reported in pre-clinical models for endothelin receptor antagonists and riociguat, these drugs are not recommended during pregnancy359,377 © ESC/ERS 2022 | |
| p.47 | I | C | High doses of CCBs are recommended in patients with IPAH, HPAH, or DPAH who are responders to acute vasoreactivity testing | Recommendation Table 7 — Recommendations for the treatment of vasoreactive patients with idiopathic, heritable, or drug-associated pulmonary arterial hypertension |
| p.47 | I | C | Close follow-up with complete reassessment after 3–4 months of therapy (including RHC) is recommended in patients with IPAH, HPAH, or DPAH treated with high doses of CCBs | Recommendation Table 7 — Recommendations for the treatment of vasoreactive patients with idiopathic, heritable, or drug-associated pulmonary arterial hypertension |
| p.47 | I | C | Continuing high doses of CCBs is recommended in patients with IPAH, HPAH, or DPAH in WHO-FC I or II with marked haemodynamic improvement (mPAP ,30 mmHg and PVR ,4 WU) | Recommendation Table 7 — Recommendations for the treatment of vasoreactive patients with idiopathic, heritable, or drug-associated pulmonary arterial hypertension |
| p.47 | I | C | Initiating PAH therapy is recommended in patients who remain in WHO-FC III or IV or those without marked haemodynamic improvement after high doses of CCBs | Recommendation Table 7 — Recommendations for the treatment of vasoreactive patients with idiopathic, heritable, or drug-associated pulmonary arterial hypertension |
| p.47 | IIa | C | In patients with a positive vasoreactivity test but insufficient long-term response to CCBs who require additional PAH therapy, continuation of CCB therapy should be considered | Recommendation Table 7 — Recommendations for the treatment of vasoreactive patients with idiopathic, heritable, or drug-associated pulmonary arterial hypertension |
| p.47 | III | C | CCBs are not recommended in patients without a vasoreactivity study or non-responders, unless prescribed for other indications (e.g. Raynaud’s phenomenon) Starting | Recommendation Table 7 — Recommendations for the treatment of vasoreactive patients with idiopathic, heritable, or drug-associated pulmonary arterial hypertension |
| p.51 | IIa | C | Recommendations for initial therapy In patients with IPAH/HPAH/DPAH who present at high risk of death, initial combination therapy with a PDE5i, an ERA, and i.v./s.c. prostacyclin analogues should be consideredd | Recommendation Table 8 — Recommendations for the treatment of non-vasoreactive patients with idiopathic, heritable, or drug-associated pulmonary arterial hypertension who present without cardiopulmonary comorbiditiesa |
| p.51 | IIa | B | In patients with IPAH/HPAH/DPAH who present at intermediate–low risk of death while receiving ERA/PDE5i therapy, the addition of selexipag should be considered419 | Recommendation Table 8 — Recommendations for the treatment of non-vasoreactive patients with idiopathic, heritable, or drug-associated pulmonary arterial hypertension who present without cardiopulmonary comorbiditiesa |
| p.51 | IIa | C | In patients with IPAH/HPAH/DPAH who present at intermediate–high or high risk of death while receiving ERA/PDE5i therapy, the addition of i.v./ s.c. prostacyclin analogues and referral for LTx evaluation should be considered | Recommendation Table 8 — Recommendations for the treatment of non-vasoreactive patients with idiopathic, heritable, or drug-associated pulmonary arterial hypertension who present without cardiopulmonary comorbiditiesa |
| p.51 | IIb | B | In patients with IPAH/HPAH/DPAH who present at intermediate–low risk of death while receiving ERA/PDE5i therapy, switching from PDE5i to riociguat may be considered429 | Recommendation Table 8 — Recommendations for the treatment of non-vasoreactive patients with idiopathic, heritable, or drug-associated pulmonary arterial hypertension who present without cardiopulmonary comorbiditiesa |
| p.52 | I | B | tadalafil is recommended166,420,423 | Recommendation Table 9 — Recommendations for initial oral drug combination therapy for patients with idiopathic, heritable, or drug-associated pulmonary arterial hypertension without cardiopulmonary comorbidities |
| p.52 | I | B | tadalafil is recommended421,430 | Recommendation Table 9 — Recommendations for initial oral drug combination therapy for patients with idiopathic, heritable, or drug-associated pulmonary arterial hypertension without cardiopulmonary comorbidities |
| p.52 | IIa | B | PDE5is should be considered430 | Recommendation Table 9 — Recommendations for initial oral drug combination therapy for patients with idiopathic, heritable, or drug-associated pulmonary arterial hypertension without cardiopulmonary comorbidities |
| p.52 | III | B | tadalafil, and selexipag is not recommended421 | Recommendation Table 9 — Recommendations for initial oral drug combination therapy for patients with idiopathic, heritable, or drug-associated pulmonary arterial hypertension without cardiopulmonary comorbidities |
| p.52 | I | B | GRADE Recommendations Quality Strength of of recommendation evidence Recommendations for initial therapy In patients with IPAH/ HPAH/DPAH who present at low or intermediate risk of death, initial combination therapy with a PDE5i and an ERA is recommended166 Low Conditional | Recommendation Table 8B |
| p.53 | I | B | recommended to improve exercise capacity392,438 | Recommendation Table 10 — Recommendations for sequential drug combination therapy for patients with idiopathic, heritable, or drug-associated pulmonary arterial hypertension |
| p.53 | IIa | B | considered to improve exercise capacity395,440 | Recommendation Table 10 — Recommendations for sequential drug combination therapy for patients with idiopathic, heritable, or drug-associated pulmonary arterial hypertension |
| p.53 | IIb | C | considered to improve exercise capacity393 | |
| p.53 | IIb | B | be considered to improve exercise capacity441,442 | |
| p.53 | IIb | C | considered to improve exercise capacity443 | |
| p.53 | IIb | C | considered to improve exercise capacity419,444 | |
| p.53 | IIb | C | considered to improve exercise capacity444–446 | |
| p.53 | III | B | Combining riociguat and PDE5is is not recommendedd 389 | Recommendation Table 10 — Recommendations for sequential drug combination therapy for patients with idiopathic, heritable, or drug-associated pulmonary arterial hypertension |
| p.53 | I | C | It is recommended to base treatment escalations on risk assessment and general treatment strategies (see Figure 9) | Recommendation Table 10 — Recommendations for sequential drug combination therapy for patients with idiopathic, heritable, or drug-associated pulmonary arterial hypertension |
| p.53 | I | B | endpoint as the primary outcome measure The addition of macitentan to PDE5is or oral/ inhaled prostacyclin analogues is recommended to reduce the risk of morbidity/mortality events167,168,437 | Recommendation Table 10 — Recommendations for sequential drug combination therapy for patients with idiopathic, heritable, or drug-associated pulmonary arterial hypertension |
| p.53 | I | B | The addition of selexipag to ERAsc and/or PDE5is is recommended to reduce the risk of morbidity/ mortality events418,419 | Recommendation Table 10 — Recommendations for sequential drug combination therapy for patients with idiopathic, heritable, or drug-associated pulmonary arterial hypertension |
| p.53 | I | B | The addition of oral treprostinil to ERA or PDE5i/ riociguat monotherapy is recommended to reduce the risk of morbidity/mortality events412,413,415 | Recommendation Table 10 — Recommendations for sequential drug combination therapy for patients with idiopathic, heritable, or drug-associated pulmonary arterial hypertension |
| p.53 | III | B | The addition of bosentan to sildenafil is not recommended to reduce the risk of morbidity/ mortality events419a outcome measure | Recommendation Table 10 — Recommendations for sequential drug combination therapy for patients with idiopathic, heritable, or drug-associated pulmonary arterial hypertension |
| p.53 | IIa | B | The addition of sildenafil to epoprostenol is The addition of inhaled treprostinil to sildenafil or bosentan monotherapy should be considered to improve exercise capacity411,439 The addition of riociguat to bosentan should be | Recommendation Table 10 — Recommendations for sequential drug combination therapy for patients with idiopathic, heritable, or drug-associated pulmonary arterial hypertension |
| p.53 | IIb | C | The addition of tadalafil to bosentan may be The addition of inhaled iloprost to bosentan may The addition of ambrisentan to sildenafil may be The addition of bosentan to sildenafil may be The addition of sildenafil to bosentan may be Other sequential double- or triple-combination therapies may be considered to improve exercise capacity and/or alleviate PH symptoms the primary outcome measure | |
| p.54 | IIa | C | Recommendations for initial therapy In patients with IPAH/HPAH/DPAH and cardiopulmonary comorbidities, initial monotherapy with a PDE5i or an ERA should be considered | Recommendation Table 11 — Recommendations for the treatment of non-vasoreactive patients with idiopathic, heritable, or drug-associated pulmonary arterial hypertension who present with cardiopulmonary comorbiditiesa |
| p.54 | IIb | C | In patients with IPAH/HPAH/DPAH with cardiopulmonary comorbidities who present at intermediate or high risk of death while receiving PDE5i or ERA monotherapy, additional PAH medication may be considered on an individual basis | Recommendation Table 11 — Recommendations for the treatment of non-vasoreactive patients with idiopathic, heritable, or drug-associated pulmonary arterial hypertension who present with cardiopulmonary comorbiditiesa |
| p.55 | I | C | When managing patients with right HF in the ICU, it is recommended to involve physicians with expertise, treat causative factors, and use supportive measures, including inotropes and vasopressors, fluid management, and PAH drugs, as appropriate | Recommendation Table 12 — Recommendations for intensive care management for pulmonary arterial hypertension |
| p.55 | IIa | C | Mechanical circulatory support may be an option for selected patients as a bridge to transplantation or recovery, and interhospital transfer should be considered if such resources are unavailable on site | Recommendation Table 12 — Recommendations for intensive care management for pulmonary arterial hypertension |
| p.56 | I | C | It is recommended that potentially eligible candidates are referred for LTx evaluation when they have an inadequate response to oral combination therapy, indicated by an intermediate– high or high risk or by a REVEAL risk score .7 | Recommendation Table 13 — Recommendations for lung transplantation |
| p.56 | I | C | It is recommended to list patients for LTx who present with a high risk of death or with a REVEAL risk score ≥10 despite receiving optimized medical therapy including s.c. or i.v. prostacyclin analogues i.v., intravenous; LTx, lung transplantation; s.c., subcutaneous. aClass of recommendation. bLevel of evidence. | Recommendation Table 13 — Recommendations for lung transplantation |
| p.57 | I | C | It is recommended to make a diagnosis of drug- or toxin-associated PAH in patients who had relevant exposure and in whom other causes of PH have been excluded | Recommendation Table 14 — Recommendations for pulmonary arterial hypertension associated with drugs or toxins |
| p.57 | I | C | In patients with suspected drug- or toxin-associated PAH, it is recommended to immediately discontinue the causative agent whenever possible | Recommendation Table 14 — Recommendations for pulmonary arterial hypertension associated with drugs or toxins |
| p.58 | IIa | C | Immediate PAH therapy should be considered in patients who present with intermediate-/high-risk PAH at diagnosis | |
| p.58 | IIb | C | Patients with low-risk PAH should be re-evaluated 3–4 months after discontinuing the suspected drug or toxin, and PAH therapy may be considered when the haemodynamics have not normalized | |
| p.58 | I | A | In patients with PAH associated with CTD, treatment of the underlying condition according to current guidelines is recommended166,167,419,524 | Recommendation Table 15 — Recommendations for pulmonary arterial hypertension associated with connective tissue disease |
| p.59 | I | C | In patients with PAH associated with CTD, the same treatment algorithm as for patients with IPAH is recommended | |
| p.59 | I | A | In patients with PAH associated with HIV infection, antiretroviral treatment according to current guidelines is recommended541,542 | Recommendation Table 16 — Recommendations for pulmonary arterial hypertension associated with human immunodeficiency virus infection |
| p.59 | IIa | C | In patients with PAH associated with HIV infection, initial monotherapy should be considered, followed by sequential combination if necessary, taking into consideration comorbidities and drug–drug interactions | Recommendation Table 16 — Recommendations for pulmonary arterial hypertension associated with human immunodeficiency virus infection |
| p.60 | I | C | Echocardiography is recommended in patients with liver disease or portal hypertension with signs or symptoms suggestive of PH, and as a screening tool in patients evaluated for liver transplantation or transjugular portosystemic | Recommendation Table 17 — Recommendations for pulmonary arterial hypertension associated with portal hypertension |
| p.60 | I | C | shunt It is recommended that patients with PAH associated with portal hypertension are referred to centres with expertise in managing both conditions | Recommendation Table 17 — Recommendations for pulmonary arterial hypertension associated with portal hypertension |
| p.60 | IIa | C | In patients with PAH associated with portal hypertension, initial monotherapy should be considered, followed by sequential combination if necessary, taking into consideration the underlying liver disease and indication for liver | Recommendation Table 17 — Recommendations for pulmonary arterial hypertension associated with portal hypertension |
| p.60 | IIa | C | transplantation Liver transplantation should be considered on an individual basis in patients with PAH associated with portal hypertension, as long as PVR is normal or near normal with PAH therapy | Recommendation Table 17 — Recommendations for pulmonary arterial hypertension associated with portal hypertension |
| p.60 | III | C | Drugs approved for PAH are not recommended for patients with portal hypertension and unclassified PH (i.e. elevated mPAP, high CO, and a normal PVR) | Recommendation Table 17 — Recommendations for pulmonary arterial hypertension associated with portal hypertension |
| p.63 | I | C | In patients with an ASD, VSD, or PDA and a PVR ,3 WU, shunt closure is recommended | Recommendation Table 18 — Recommendations for shunt closure in patients with pulmonary–systemic flow ratio .1.5:1 based on calculated pulmonary vascular resistance |
| p.63 | IIa | C | In patients with an ASD, VSD, or PDA and a PVR of 3–5 WU, shunt closure should be considered | Recommendation Table 18 — Recommendations for shunt closure in patients with pulmonary–systemic flow ratio .1.5:1 based on calculated pulmonary vascular resistance |
| p.63 | IIb | C | In patients with an ASD and a PVR .5 WU that declines to ,5 WU with PAH treatment, shunt closure may be considered | Recommendation Table 18 — Recommendations for shunt closure in patients with pulmonary–systemic flow ratio .1.5:1 based on calculated pulmonary vascular resistance |
| p.63 | IIb | C | In patients with a VSD or PDA and a PVR .5 WU, shunt closure may be considered after careful evaluation in specialized centres | Recommendation Table 18 — Recommendations for shunt closure in patients with pulmonary–systemic flow ratio .1.5:1 based on calculated pulmonary vascular resistance |
| p.63 | III | C | In patients with an ASD and a PVR .5 WU despite PAH treatment, shunt closure is not recommended | Recommendation Table 18 — Recommendations for shunt closure in patients with pulmonary–systemic flow ratio .1.5:1 based on calculated pulmonary vascular resistance |
| p.63 | I | C | Risk assessment Risk assessment is recommended for patients with persistent PAH after defect closure | Recommendation Table 19 — Recommendations for pulmonary arterial hypertension associated with adult congenital heart disease |
| p.63 | IIa | C | Risk assessment should be considered in patients with Eisenmenger syndrome Treatment | Recommendation Table 19 — Recommendations for pulmonary arterial hypertension associated with adult congenital heart disease |
| p.63 | I | B | Bosentan is recommended in symptomatic patients with Eisenmenger syndrome to improve exercise capacity574 | Recommendation Table 19 — Recommendations for pulmonary arterial hypertension associated with adult congenital heart disease |
| p.63 | IIa | C | In patients with Eisenmenger syndrome, the use of supplemental oxygen therapy should be considered in cases where it consistently increases | Recommendation Table 19 — Recommendations for pulmonary arterial hypertension associated with adult congenital heart disease |
| p.63 | IIa | C | arterial oxygen saturation and reduces symptoms Supplemental iron treatment should be considered in patients with iron deficiency | Recommendation Table 19 — Recommendations for pulmonary arterial hypertension associated with adult congenital heart disease |
| p.63 | IIa | C | In patients with adult CHD, including Eisenmenger syndrome, other ERAs, PDE5is, riociguat, prostacyclin analogues, and prostacyclin receptor agonists should be considered | Recommendation Table 19 — Recommendations for pulmonary arterial hypertension associated with adult congenital heart disease |
| p.63 | IIa | C | combination therapy including i.v./s.c. prostacyclin analogues should be considered for patients at high risk In patients with adult CHD, including Eisenmenger syndrome, sequential combination therapy should be considered if patients do not meet treatment goals | Recommendation Table 18 — Recommendations for shunt closure in patients with pulmonary–systemic flow ratio .1.5:1 based on calculated pulmonary vascular resistance |
| p.63 | IIb | C | In the absence of significant haemoptysis, oral anticoagulant treatment may be considered in patients with Eisenmenger syndrome with | Recommendation Table 18 — Recommendations for shunt closure in patients with pulmonary–systemic flow ratio .1.5:1 based on calculated pulmonary vascular resistance |
| p.63 | III | C | pulmonary artery thrombosis © ESC/ERS 2022 In women with Eisenmenger syndrome, pregnancy is not recommended | Recommendation Table 18 — Recommendations for shunt closure in patients with pulmonary–systemic flow ratio .1.5:1 based on calculated pulmonary vascular resistance |
| p.63 | III | C | In patients with Eisenmenger syndrome, routine phlebotomy to lower elevated haematocrit is not recommended | Recommendation Table 18 — Recommendations for shunt closure in patients with pulmonary–systemic flow ratio .1.5:1 based on calculated pulmonary vascular resistance |
| p.64 | I | A | A combination of clinical and radiological findings, ABG, PFTs, and genetic testing is recommended to diagnose PAH with signs of venous and/or capillary involvement (PVOD/PCH)591 | Recommendation Table 20 — Recommendations for pulmonary arterial hypertension with signs of venous/ capillary involvement |
| p.64 | I | A | Identification of biallelic EIF2AK4 mutations is recommended to confirm a diagnosis of heritable PVOD/PCH158,591 | Recommendation Table 20 — Recommendations for pulmonary arterial hypertension with signs of venous/ capillary involvement |
| p.64 | I | C | Referral of eligible patients with PVOD/PCH to a transplant centre for evaluation is recommended as soon as the diagnosis is established | Recommendation Table 20 — Recommendations for pulmonary arterial hypertension with signs of venous/ capillary involvement |
| p.64 | IIb | C | In patients with PVOD/PCH, the use of drugs approved for PAH may be considered with careful monitoring of clinical symptoms and gas exchange | Recommendation Table 20 — Recommendations for pulmonary arterial hypertension with signs of venous/ capillary involvement |
| p.64 | III | C | Lung biopsy is not recommended to confirm a diagnosis of PVOD/PCH | Recommendation Table 20 — Recommendations for pulmonary arterial hypertension with signs of venous/ capillary involvement |
| p.68 | I | B | It is recommended to screen infants with bronchopulmonary dysplasia for PH628,629 | Recommendation Table 21 — Recommendations for paediatric pulmonary hypertension |
| p.68 | I | C | Children It is recommended to perform the diagnostic work-up, including RHC and acute vasoreactivity testing, and treat children with PH at centres with specific expertise in paediatric PH | Recommendation Table 21 — Recommendations for paediatric pulmonary hypertension |
| p.68 | I | C | In children with PH, a comprehensive work-up for confirming diagnosis and specific aetiology is recommended (similar to that in adults, but | Recommendation Table 21 — Recommendations for paediatric pulmonary hypertension |
| p.68 | I | C | adapted for age) For confirming PH diagnosis, RHC is recommended, preferably before initiating any PAH therapy | Recommendation Table 21 — Recommendations for paediatric pulmonary hypertension |
| p.68 | I | C | In children with IPAH/HPAH, acute vasoreactivity testing is recommended to detect those who may benefit from CCB therapy | Recommendation Table 21 — Recommendations for paediatric pulmonary hypertension |
| p.68 | I | C | It is recommended to similarly define a positive response to acute vasoreactivity testing in children and adults by a reduction in mPAP ≥10 mmHg to reach an absolute value of mPAP ≤40 mmHg, with an increased or unchanged CO | Recommendation Table 21 — Recommendations for paediatric pulmonary hypertension |
| p.68 | I | C | In children with PAH, a therapeutic strategy based on risk stratification and treatment response is recommended, extrapolated from that in adults but adapted for age | Recommendation Table 21 — Recommendations for paediatric pulmonary hypertension |
| p.68 | I | C | It is recommended to monitor the treatment response in children with PAH by serially assessing a panel of data derived from clinical assessment, echocardiographic evaluation, biochemical markers, and exercise tolerance tests | Recommendation Table 21 — Recommendations for paediatric pulmonary hypertension |
| p.68 | IIa | C | Achieving and maintaining a low-risk profile should be considered as an adequate treatment response for children with PAH Infants | Recommendation Table 21 — Recommendations for paediatric pulmonary hypertension |
| p.68 | I | B | In infants with (or at risk of) bronchopulmonary dysplasia and PH, treating lung disease—including hypoxia, aspiration, and structural airway disease —and optimizing respiratory support is recommended before initiating PAH therapy630 | Recommendation Table 21 — Recommendations for paediatric pulmonary hypertension |
| p.68 | IIa | C | In neonates and infants, a diagnostic and therapeutic approach to PH distinct from that in older children and adults should be considered, given the frequent association with developmental vascular and parenchymal lung disease | |
| p.73 | I | A | In patients with LHD, optimizing treatment of the underlying condition is recommended before considering assessment of suspected PH27,28 | Recommendation Table 22A |
| p.73 | I | C | RHC is recommended for suspected PH in patients with LHD, if it aids management decisions | Recommendation Table 22A |
| p.73 | I | C | RHC is recommended in patients with severe tricuspid regurgitation with or without LHD prior to surgical or interventional valve repair | Recommendation Table 22A |
| p.73 | I | C | to a PH centre for a complete diagnostic work-up is recommended29,47,142 | Recommendation Table 22A |
| p.73 | IIb | C | may be considered to uncover post-capillary PH133,143 | Recommendation Table 22A |
| p.73 | III | C | Low Conditional | Recommendation Table 22 — Recommendations for pulmonary hypertension associated with left heart disease |
| p.73 | III | A | GRADE Strength of evidence recommendation | Recommendation Table 22 — Recommendations for pulmonary hypertension associated with left heart disease |
| p.76 | I | C | In patients with lung disease and suspected severe PH, or where there is uncertainty regarding the treatment of PH, referral to a PH centre is recommendedd | Recommendation Table 23A |
| p.76 | I | C | In patients with lung disease and severe PH, an individualized approach to treatment is recommended | Recommendation Table 23A |
| p.76 | I | C | It is recommended to refer eligible patients with lung disease and PH for LTx evaluation | Recommendation Table 23A |
| p.76 | I | C | In patients with lung disease and suspected PH, RHC is recommended if the results are expected to aid management decisions | Recommendation Table 23A |
| p.76 | IIb | C | Very low Conditional | Recommendation Table 23 — Recommendations for pulmonary hypertension associated with lung disease and/or hypoxia |
| p.76 | III | C | The use of PDE5is in patients with ILD and non-severe PH is not recommended Very low Conditional | Recommendation Table 23B |
| p.76 | III | C | GRADE Strength of evidence recommendation | Recommendation Table 23 — Recommendations for pulmonary hypertension associated with lung disease and/or hypoxia |
| p.81 | I | C | Antiphospholipid syndrome testing is recommended in patients with CTEPH | Recommendation Table 24A |
| p.81 | I | B | surgery54,102 | Recommendation Table 24A |
| p.81 | I | B | BPA54,102,783,784,789,793,798,811 | Recommendation Table 24A |
| p.81 | IIb | C | In patients with inoperable CTEPH, a combination of sGC stimulator/PDE5i, ERA,777 or parenteral prostacyclin analogues776 may be considered | Recommendation Table 24A |
| p.81 | IIb | C | BPA may be considered for technically operable patients with a high proportion of distal disease and an unfavourable risk:benefit ratio for PEA | Recommendation Table 24A |
| p.81 | IIa | C | PEA or BPA should be considered in selected symptomatic patients with CTEPD without PH | Recommendation Table 24A |
| p.82 | IIa | B | Very low Conditional | Recommendation Table 24B |
| p.85 | I | C | It is recommended that PH centres provide care by a multidisciplinary team (cardiologist, pneumologist, rheumatologist, nurse specialist, radiologist, psychological and social work support, and appropriate on-call expertise) | Recommendation Table 25 — Recommendations for pulmonary hypertension centres |
| p.85 | I | C | It is recommended that PH centres have direct links and quick referral patterns to other services (such as genetic counselling, PEA/BPA, LTx, and | Recommendation Table 25 — Recommendations for pulmonary hypertension centres |
| p.85 | I | C | adult congenital heart disease service) It is recommended that PH centres maintain a patient registry | Recommendation Table 25 — Recommendations for pulmonary hypertension centres |
| p.85 | I | C | It is recommended that PH centres collaborate with patient associations | Recommendation Table 25 — Recommendations for pulmonary hypertension centres |
| p.85 | IIa | C | Accreditation of the PH centres should be considered (e.g. https://ec.europa.eu/health/ern/ assessment_en) | Recommendation Table 25 — Recommendations for pulmonary hypertension centres |
| p.85 | IIa | C | PH centres’ participation in collaborative clinical research should be considered PH centres should follow-up a sufficient number | Recommendation Table 25 — Recommendations for pulmonary hypertension centres |
| p.85 | IIa | C | of patients to maintain expertise (at least 50 patients with PAH or CTEPH and at least two new referrals per month with documented PAH or CTEPH), and consider establishing collaborations with high-volume centres | Recommendation Table 25 — Recommendations for pulmonary hypertension centres |
| p.87 | I | C | In patients with suspected or known PH, it is recommended that RHC is performed in experienced centres | |
| p.87 | I | C | It is recommended that RHC comprises a complete set of haemodynamics and is performed following standardized protocols | |
| p.87 | I | B | Vasoreactivity testing is recommended in patients with I/H/DPAH to detect those who can be treated with high doses of a CCB | |
| p.87 | I | C | It is recommended that vasoreactivity testing is performed at PH centres | |
| p.87 | I | C | value of mPAP ≤40 mmHg with an increased or unchanged COc | |
| p.87 | I | C | Inhaled nitric oxide, inhaled iloprost, or i.v. epoprostenol are recommended for performing vasoreactivity testing | |
| p.88 | I | B | Echocardiography is recommended as the first-line, non-invasive, diagnostic investigation in suspected PH | |
| p.88 | I | C | Ventilation/perfusion or perfusion lung scan is recommended in patients with unexplained PH to assess for CTEPH | |
| p.88 | I | C | CT pulmonary angiography is recommended in the work-up of patients with suspected CTEPH | |
| p.88 | I | C | Abdominal ultrasound is recommended for the screening of portal hypertension | |
| p.88 | I | C | Pulmonary function tests with DLCO are recommended in the initial evaluation of patients with PH | |
| p.88 | III | C | Open or thoracoscopic lung biopsy is not recommended in patients with PAH | |
| p.88 | I | B | In patients with SSc, an annual evaluation of the risk of having PAH is recommended | |
| p.88 | I | C | In patients referred for liver transplantation, echocardiography is recommended as a screening test for PH | |
| p.88 | I | B | Achieving and maintaining a low-risk profile on optimized medical therapy is recommended as a treatment goal in patients with PAH | |
| p.89 | I | A | Supervised exercise training is recommended in patients with PAH under medical therapy | |
| p.89 | I | C | Psychosocial support is recommended in patients with PAH | |
| p.89 | I | C | Immunization of patients with PAH against SARS-CoV-2, influenza, and Streptococcus pneumoniae is recommended | |
| p.89 | I | C | Diuretic treatment is recommended in patients with PAH with signs of RV failure and fluid retention | |
| p.89 | I | C | In the presence of iron-deficiency anaemia, correction of iron status is recommended in patients with PAH | |
| p.89 | I | C | High doses of CCBs are recommended in patients with IPAH, HPAH, or DPAH who are responders to acute vasoreactivity testing | |
| p.89 | I | B | Initial combination therapy with ambrisentan and tadalafil is recommended | |
| p.89 | I | B | Initial combination therapy with macitentan and tadalafil is recommended | |
| p.89 | III | B | Initial combination therapy with macitentan, tadalafil, and selexipag is not recommended | |
| p.90 | I | C | It is recommended to base treatment escalations on risk assessment and general treatment strategies (see treatment algorithm) | |
| p.90 | I | B | The addition of selexipag to ERAsf and/or PDE5is is recommended to reduce the risk of morbidity/mortality events | |
| p.90 | III | B | The addition of bosentan to sildenafil is not recommended to reduce the risk of morbidity/mortality events | |
| p.90 | I | B | The addition of sildenafil to epoprostenol is recommended to improve exercise capacity | |
| p.90 | I | A | In patients with PAH associated with CTD, treatment of the underlying condition according to current guidelines is recommended | |
| p.90 | I | C | In patients with PAH associated with CTD, the same treatment algorithm as for patients with IPAH is recommended | |
| p.90 | I | A | In patients with PAH associated with HIV infection, antiretroviral treatment according to current guidelines is recommended | |
| p.91 | I | C | In patients with an ASD, VSD, or PDA and a PVR ,3 WU, shunt closure is recommended | |
| p.91 | III | C | In patients with an ASD and a PVR .5 WU despite PAH treatment, shunt closure is not recommended | |
| p.91 | I | C | Risk assessment is recommended for patients with persistent PAH after defect closure | |
| p.91 | I | B | Bosentan is recommended in symptomatic patients with Eisenmenger syndrome to improve exercise capacity | |
| p.91 | III | C | In women with Eisenmenger syndrome, pregnancy is not recommended | |
| p.91 | III | C | In patients with Eisenmenger syndrome, routine phlebotomy to lower elevated haematocrit is not recommended | |
| p.91 | I | A | Identification of biallelic EIF2AK4 mutations is recommended to confirm a diagnosis of heritable PVOD/PCH | |
| p.91 | I | C | Referral of eligible patients with PVOD/PCH to a transplant centre for evaluation is recommended as soon as the diagnosis is established | |
| p.91 | III | C | Lung biopsy is not recommended to confirm a diagnosis of PVOD/PCH | |
| p.91 | I | C | For confirming PH diagnosis, RHC is recommended, preferably before initiating any PAH therapy | |
| p.91 | I | C | In children with IPAH/HPAH, acute vasoreactivity testing is recommended to detect those who may benefit from CCB therapy | |
| p.91 | I | B | It is recommended to screen infants with bronchopulmonary dysplasia for PH | |
| p.91 | I | C | RHC is recommended for suspected PH in patients with LHD, if it aids management decisions | |
| p.92 | I | C | RHC is recommended in patients with severe tricuspid regurgitation with or without LHD prior to surgical or interventional valve repair | |
| p.92 | I | C | centre is recommendedj | |
| p.92 | I | C | In patients with lung disease and severe PH, an individualized approach to treatment is recommended | |
| p.92 | I | C | It is recommended to refer eligible patients with lung disease and PH for LTx evaluation | |
| p.92 | I | C | In patients with lung disease and suspected PH, RHC is recommended if the results are expected to aid management decisions | |
| p.92 | III | B | The use of ambrisentan is not recommended in patients with PH associated with IPF | |
| p.92 | III | B | The use of riociguat is not recommended in patients with PH associated with IIP | |
| p.92 | I | C | Lifelong, therapeutic doses of anticoagulation are recommended in all patients with CTEPH | |
| p.92 | I | C | Antiphospholipid syndrome testing is recommended in patients with CTEPH | |
| p.92 | I | C | In patients with CTEPH and antiphospholipid syndrome, anticoagulation with VKAs is recommended | |
| p.92 | I | C | It is recommended that all patients with CTEPH are reviewed by a CTEPH team for the assessment of multimodality management | |
| p.92 | I | B | BPA is recommended in patients who are technically inoperable or have residual PH after PEA and distal obstructions amenable to BPA | |
| p.92 | I | B | Riociguat is recommended for symptomatic patients with inoperable CTEPH or persistent/recurrent PH after PEA | |
| p.92 | I | C | Long-term follow-up is recommended after PEA and BPA, as well as for patients with CTEPH established on medical therapy | |
| p.93 | I | C | It is recommended that PH centres maintain a patient registry | |
| p.93 | I | C | It is recommended that PH centres collaborate with patient associations | |
| p.93 | I | B | Low Conditional | |
| p.93 | III | C | Low Conditional | |
| p.93 | III | C | The use of PDE5i in patients with ILD and non-severe PH is not recommended Very low Conditional |