Incorrect. The current value is > 20mmHg

Correct! This is consistent with the data suggesting that values above 20 mmHg are associated with increased mortality.

Incorrect. The current value is > 20mmHg

Incorrect. The current value is > 20mmHg

Incorrect. Peak TVR is recommended in assigning echocardiographic probablity of PH.

Incorrect. Estimating the value of sPAP requires both the values of TR pressure gradient and estimated right atrial pressure (RAP). Considering the inaccuracies in estimating RAP and the amplification of measurement errors by using derived variables, current guidelines recommend using the peak TRV.

Incorrect. Peak TVR is recommended in assigning echocardiographic probablity of PH.

Correct! Current guidelines recommend using the peak TRV (and not the estimated sPAP) as the key variable for assigning the echocardiographic probability of PH. A peak TRV >2.8 m/s may suggest PH; however, the presence or absence of PH cannot be reliably determined by TRV alone. Additional variables related to RV morphology and function are needed.

Incorrect. Current thershold for peak TRV is >2.8 m/s

Incorrect. Current thershold for peak TRV is >2.8 m/s

Correct! A peak TRV >2.8 m/s may suggest PH; however, the presenceor absence of PH cannot be reliably determined by TRV alone. TRV may underestimate (e.g. in patients with severe TR) or overestimate (e.g. in patients with high CO sickle cell disease) pressure gradients. Hence, additional variables related to RV morphology and function are used to define the echocardiographic probability of PH.

Incorrect. Current thershold for peak TRV is >2.8 m/s

Correct! In patients with PAH, PFTs are usually normal or may show mild restrictive, obstructive, or combined abnormalities. More severe PFT abnormalities are occasionally found in patients with PAH associated with CHD (congenital heart disease), and those with group 3 PH.

Incorrect. The DLCO may benormal in patients with PAH, although it is usually mildly reduced. A severely reduced DLCO (<45% of the predicted value) in the presence of otherwise normal PFTs can be found in PAH associated with systemic sclerosis (SSc). A low DLCO is also associated with a poor prognosis in several forms of PH.

Incorrect. Patients with PAH usually have normal or slightly reduced PaO2. Severe reduction of PaO2 might raise suspicion for patent foramen ovale, hepatic disease, other abnormalities with right-to-left shunt (e.g. septal defect), or low-DLCO-associated conditions.

Incorrect. Elevated PaCO2 reflects alveolar hypoventilation, not hyperventilation. Elevated PaCO2 is very unusual in PAH, and overnight oximetry or polysomnography should be performed if there is suspicion of sleep-disordered breathing or hypoventilation.

Incorrect. Interlobular septal thickening ´Kerley B´ lines are a sign of pulmonary congestion seen in left heart disease, not specific for PH.

Incorrect. This is seen in patients with COPD/emphysema. It is not specific for pulmonary hypertension, however, lung disease can be an underlying cause of PH.

Correct!

Correct! It may be present in patients with PH with advanced right ventricular failure and moderate pericardial effusion.

Incorrect. V/Q scan is recommended in the diagnostic work-up of patients with suspected or newly diagnosed PH, to rule out or detect signs of CTEPH.

Correct! In the absence of parenchymal lung disease, a normal perfusion scan excludes CTEPH with a negative predicted value of 98%. The V/Q SPECT is superior to planar imaging and is the methodology of choice.

Incorrect. CTPA is mainlyused to detect direct or indirect signs of CTEPH, however, the diagnostic accuracy of CTPA for CTEPH is limited (at the patient level, sensitivity and specificity are 76% and 96%, respectively).

Incorrect. V/Q scan is recommended in the diagnostic work-up of patients with suspected or newly diagnosed PH, to rule out or detect signs of CTEPH.

Correct! In a validation study assesing the value of different echocardiographic signs of pulmonary hypertension, PA diameter >25 mm was found to be a strong predictor of PH

Correct! In a validation study assesing the value of different echocardiographic signs of pulmonary hypertension, at univariate analysis RV/LV basal diameter area ratio >1.0 was found to be a strong predictor of PH.

Incorrect. RVOT AT less than 105 ms increases the echocardiograpic probablity. Another sign increasing the probablity is the mid-systolic ´notch´ of the pulmonary valve Doppler signal, which represents an earlier reflected systolic pulse due to decreased compliance and/or increased resistance within the pulmonary vascular bed.

Incorrect.

Correct!

Incorrect. The normal values are 8-20 mmHg.

Incorrect. The noraml values are 2.5–4.0 L/min·m2.

Correct! It is derived from blood sample taken from the pulmonary artery; compartmental oximetry to exclude an intracardiac shunt is recommended when SvO2 >75%.

Incorrect. The purpose of vasoreactivity testing in PAH is to identify acute vasoresponders who may be candidates for treatment with high-dose calcium channel blockers (CCBs)

Incorrect. It is no longer recommended due to frequent side effects.

Correct! Inhaled iloprost is also recommended.

Correct! In other groups of PH, like PH associated with left heart disease, vasoreactivity testing is restricted to evaluating heart transplantation candidacy.

Correct!

Incorrect. Reduction in mPAP by ≥10 mmHg to reach an absolutevalue ≤40 mmHg is the correct answer.

Correct! Inhaled nitric oxide or inhaled iloprost are the recommended test compounds for vasoreactivity testing. There is similar evidence for i.v. epoprostenol, but due to incremental dose increases and repetitive measurements, testing takes much longer and is therefore less feasible.

Correct!

Incorrect. CT is not used for screening, rather for situations like preprocedural planning or concerns for extrahepatic obstruction.

Correct! This is a class I recommendation. A major objective is to search for liver disease and/or portal hypertension, or portocaval shunt (Abernethy malformation).

Incorrect. MRI is not used for screening, rather for situations like preprocedural planning or concerns for extrahepatic obstruction.

Incorrect.

Correct! Data suggest that PVR > 2 WU is associated with clinically significant PVD (pulmonary vascular disease) and reduced survival.

Incorrect. PVR > 2 WU is correct.

Incorrect. PVR > 2 WU is correct.

Incorrect. PVR > 2 WU is correct.

Incorrect. This procedure is not recommended. This is a class III, level C recommendation.

Incorrect. This procedure is not recommended. This is a class III, level C recommendation.

Incorrect. This procedure is not recommended. This is a class III, level C recommendation.

Correct! This procedure has class III recommendation, meaning, it is not recommended.

Correct! This is considered a warning sign associated with worse outcomes, warranting attention and immediate intervention. Such cases must be immediately managed as inpatients for initial work-up at a nearby hospital or PH centre.

Correct! CTEPH should be suspected in patients with a history of PE, permanent intravascular devices, inflammatory bowel diseases, essential thrombocythaemia, splenectomy, high-dose thyroid hormone replacement, and malignancy.

Correct!

Incorrect.

Correct! In SSc, the prevalence of PAH is 5–19%, with an annual incidence of developing PAH of 0.7–1.5%. A screening programme showed less severe haemodynamic impairment and better survival in screened patients compared with a contemporaneous, non-screened cohort, providing a strong rationale for screening for PAH in patients with SSc.

Correct! BMPR2 mutation carriers carry a lifetime risk of developing PAH of approximately 20%. At present, based on expert consensus, asymptomatic relatives who screen positive for PAH-causing mutations are often offered yearly screening echocardiography.

Incorrect. Routine follow-up of patients after PE by imaging methods of the pulmonary vascular tree is not recommended. However, patients with persistent dyspnoea after 3 months of anticoagulation for acute PE should be referred to PH/CTEPH center.

Correct! An estimated 1–2% of patients with liver disease and portal hypertension develop PH. Echocardiography is recommended in these patients, even in the absence of symptoms. In agreement with the International Liver Transplant Society, for patients awaiting liver transplantation, it is recommended to reassess for PAH annually, although the optimal interval remains unclear.

Incorrect. EstimatedsPAP at rest is not prognostic and irrelevant to therapeutic decision-making. An increase in sPAP does not necessarily reflect disease progression and a decrease in sPAP does not necessarily reflect improvement.

Correct! TAPSE/sPAP ratio is tightly linked to RV–PA coupling and predicts outcome. A cut-off value of <0.55 mm/mmHg is considered as one measure contributing to the probability of PH. A cut-off of >0.32 mm/mmHg may indicate a low-risk status, whereas a cut-off of >0.19 mm/mmHg may indicate a high mortality risk.

Incorrect. The mPAP provides little prognostic information, except in acute vasodilator responders.

Correct! A recent study from France, which combined clinical and haemodynamic parameters, found that WHO-FC, 6MWD (6-minute walking distance), RAP, and SVI (stroke volume index) (but not SV and SvO2) were independent predictors of outcome.

Correct! Change in 6MWD is one of the most commonly used parameters in PAH clinical trials. A recent investigation showed that the best absolute-threshold values for 1 year mortality and 1 year survival, respectively, were 165 m and 440 m, respectively. However, 6MWT results must always be interpreted in the clinical context .

Incorrect. 6MWD >440 m is a threshold for low-risk patients.

Incorrect. 6MWD >440 m is a threshold for low-risk patients.

Incorrect. 6MWD >440 m is a threshold for low-risk patients. Values between 165-440 m are associated with intermediate risk (5-20%). However, 6MWT results must always be interpreted in the clinical context.

Incorrect. High risk (estimated 1-year mortality >20%) patients usually present with signs of right HF, rapid progression of symptoms and repeated episodes of syncope even with little or regular physical activity.

Correct!

Incorrect. Patients without signs of right HF, no progression of symptoms and no syncope are considered to be low risk (estimated 1-year mortality <5%) .

Incorrect.

Incorrect. Emerging proteins related to PAH and vascular remodellinf include BMP (bone morphogenetic proteins) 9 and 10 and translationally controlled tumour proteins. However, none of these biomarkers have been introduced in clinical practice.

Incorrect. A study found that early development of SSc-associated PAH (PAH-SSc) was predicted by high circulating levels of C-X-C motif chemokine 4 in patients with SSc. However, these biomarkers have not been introduced in clinical practice.

Correct! BNP and NT-proBNP remain the only biomarkers routinely used in clinical practice at PH centres, correlating with myocardial stress and providing prognostic information.

Correct! BNP and NT-proBNP remain the only biomarkers routinely used in clinical practice at PH centres, correlating with myocardial stress and providing prognostic information.

Correct! WHO-FC, 6MWD, and BNP/NT-proBNP were found to be the variables with the highest predictive value.

Correct! WHO-FC, 6MWD, and BNP/NT-proBNP were found to be the variables with the highest predictive value.

Correct! WHO-FC, 6MWD, and BNP/NT-proBNP were found to be the variables with the highest predictive value.

Incorrect. Peak VO2 value may be used for the initial risk stratification according to the three-strata model. The simplified four-strata model uses WHO-FC, 6MWD, and BNP/NT-proBNP for risk assesment at follow-up.

Incorrect. Routine anticoagulation is not recommended. Even though a survival benefit was observed in two recent meta-analyses in patients with idiopathic PAH, data from RCTs (randomized controlled trials) are lacking and registry data are rather conflicting.

Correct! It is not generally recommended but may be considered on an individual basis. In the absence of robust data, no general recommendation has been made for or against the use of anticoagulants in patients with PAH.; therefore, individual decision-making is required.

Incorrect. In PAH associated with SSc, registry data and meta-analyses uniformly indicated that anticoagulation may be harmful.

Correct! This is a class IIb, level C recommendation. As anticoagulation is associated with an increased bleeding risk,and in the absence of robust data, no general recommendation has been made for or against the use of anticoagulants in patients with PAH.; therefore, individual decision-making is required.

Incorrect. Diuretic treatment is recommended in patients with PAH with signs of RV failure and fluid retention.

Correct! In the absence of robust data on the use of oxygen in patients with PAH, guidance is based on evidence in patients with COPD; when PaO2 is <8 kPa (60 mmHg; alternatively, SaO2 <92%) on at least two occasions, patients are advised to take oxygen to achieve a PaO2 >8 kPa.

Correct! Iron deficiency is common in patients with PAH and is associated with impaired myocardial function, aggravated symptoms, and increased mortality risk. Based on these data, regular monitoring of iron status (serum iron, ferritin, transferrin saturation, soluble transferrin receptors) is recommended in patients with PAH.

Incorrect! These drugs are not recommended in patients with PAH unless required by comorbidities (i.e. high blood pressure, coronary artery disease, left HF, or arrhythmias). Inthis group of patients, these drugs may lead to potentially dangerous drops in blood pressure, heart rate, or both.

Incorrect. The current thershold is PAWP ≤ 15 mmHg.

Incorrect. The current thershold is PAWP ≤ 15 mmHg.

Correct! It is recommended keeping PAWP ≤15 mmHg as the threshold for pre-capillary PH However, PAWP threshold is arbitrary. The patient phenotype, risk factors, and echocardiographic findings need to be considered when distinguishing pre- from post-capillary PH.

Incorrect. The current thershold is PAWP ≤ 15 mmHg.

Correct! Despite limited evidence, these drugs are considered safe. Another group of drugs that can be used in this population are PDE5is (phosphodiestarase 5 inhibitors).

Incorrect. As teratogenic potential has been reported in pre-clinical models for endothelin receptor antagonists and riociguat, these drugs are notrecommended during pregnancy. Class III, level B recommendation.

Incorrect. Previous ESC/ERS Guidelines for the diagnosis and treatment of PH have recommended that patients with PAH should avoid pregnancy. The 2022 guidelines recommend that women of childbearing potential with PAH are counselled at the time of diagnosis about the risks and uncertainties associated with becoming pregnant, this shouldinclude advice against becoming pregnant, and referral for psychological support where needed.

Incorrect. Historically, pregnancy in women with PAH and other forms of severe PH has been associated with maternal mortality rates of up to 56%. With improved treatment of PAH, maternal mortality has declined but remains high, ranging 11–25%.

Incorrect. Patients with negative test or those that have not undergone a vasoreactivity study should not be started on CCBs because of potentially severe side effects (e.g. severe hypotension, syncope, and RV failure), unless prescribed at standard doses for other indications. Class III recommendation.

Incorrect. Close follow-up after 3-4 months is recommended.

Correct! Close follow-up with complete reassessment after 3–4 months of therapy (including right heart catheterization) is recommended in patients with IPAH, HPAH, or DPAH treated with high doses of CCBs. Class I recommendation.

Correct! In these patients, continuation of high doses of CCBs is recommended. Class I recommendation.

Incorrect. Sildenafil or Tadalafil are phosphodiesterase 5 inhibitors.

Incorrect. Prostacyclin analogues are Beraprost, Treprostinil Epoprostenol or Iloprost.

Incorrect. Selexipag is a prostacyclin receptor agonist.

Correct! Ambrisentan is none of the options. It is an endothelin receptor antagonist. Some other drugs from the same class are bosentan and macitentan.

Correct! Endothelial receptor antagonists have teratogenic effects and should not be used during pregnancy.

Incorrect. ERAs are a group of drugs that are administered orally.

Correct! Dose-dependent increases in liver transaminases can occur in circa 10% of treated patients (reversible after dose reduction or discontinuation). Thus, liver function testing should be performed monthly in patients receiving bosentan.

Incorrect. There was no increased incidence of abnormal liver function with ambrisentan use, however an increased incidence of peripheral oedema was reported.

Incorrect. Initial oral triple-combination therapyis not recommended, given the current lack of evidence supporting this strategy. In patients presenting at high risk, initial triple combination therapy including an i.v./s.c. prostacyclin analogue should be considered.

Correct! 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 recommended. The available data support a positive recommendation for dual-combination over monotherapy, despite the low certainty of evidence (a single randomized controlled trial). Additionally, the long-term effect on survival is uncertain.

Incorrect. However in patients with IPAH/HPAH/DPAH who present at high risk of death, initial combination therapy with a PDE5i (phosphodiaterase 5 inhibitor), an ERA (endothelin receptor antagonist), and i.v./s.c. prostacyclin analogues should be considered. This is a clas IIa recommendation.

Incorrect. Initial dual-combination therapy vs. monotherapy was assessed in the AMBITION study, which showed a benefit of combination therapy over monotherapy.

Correct! An event-driven, phase 3 randomized controlled trial that enrolled 1156 patients showed that selexipag alone or on top of mono or double therapy with an ERA and/or a PDE5i reduced the relative risk of composite morbidity/mortality events by 40%.

Incorrect. Tadalafil is a PDE5i.

Incorrect. Switching (but not the addition) from PDE5i to riociguat may be considered. This is a class IIb recommendation. Combining riociguat and PDE5is is not recommended.

Incorrect.

Incorrect. However, this would be true for patients without cardiopulmonary comorbitidies.

Incorrect. Initial monotherapy should be considered. As patients with cardiopulmonary comorbidities were under-represented in or excluded from PAH trials, no evidence-based treatment recommendations can be made for this patient population. Therefore, additional PAH medication may be considered on an individual basis.

Incorrect. Initial monotherapy should be considered for all risk categories. This is a class IIa recommendation.

Correct! Initial monotherapy is recommended for most of these patients, with PDE5is (phosphodiesterase 5 inhibitors) being the most widely used compounds according to registry data. Further treatment decisions should be made on an individual basis in collaboration with the PH centre and local physicians.

Correct! Profound systemic hypotension may occur. Combination is contraindicated.

Correct! Cyclosporine level fal 50%, bosentan levels increase four-fold. Combination is contraindicatied

Incorrect. Ambrisentan decreases the AUC (area under the curve) of hormonal contraceptives, not clinically significant.

Correct! Warfarin metabolism increases; may need to adjust warfarin dose. Intensified monitoring of warfarin recommended following initiation, but dose adjustment usually unnecessary.

Incorrect. Fluid management is of utmost importance in these patients, most of whom require a negative fluid balance to reduce RV pre-load, thereby improving RV geometry and function.

Correct! Intubation and invasive mechanical ventilation should be avoided whenever possible in patients with advanced RV failure because of a high risk of further haemodynamic deterioration and death.

Incorrect. Maintaining the mean systemic blood pressure > 60 mmHg is a key objective when treating right HF, and patients with persistent hypotension may require vasopressors such as norepinephrine or vasopressin.

Incorrect. Inotropes may be useful in some patients with severe right heart failure with decreased CO.

Correct!

Correct! These patients have 1-year mortality > 10% when estimated with established risk stratification tools.

Correct! When patients have a disease variant that poorly respondsto medical therapy, like PVOD or PCH, referral to an LTx centre should be considered early.

Incorrect.

Incorrect. They do not meet the criteria for pre-capillary pulmonary hypertension, in which PVR is more than 2 Wood units.

Incorrect. They do not meet the criteria for post-capillary pulmonary hypertension, in which PCWP is more than 15 mmHg.

Incorrect. Exercise pulmonary hypertension is defined by mPAP/CO slope between rest and exercise > 3 mmHg/L/min.

Correct! These patients are frequently characterized by elevated pulmonary blood flow and, although they have PH, they do not fulfil the criteria of pre- or post-capillary PH. Patients with unclassified PH may present with congenital heart disease (CHD), liver disease, airway disease, lung disease, or hyperthyroidism explaining their mPAP elevation.

Incorrect. Partial or full reversal of PAH has been reported after discontinuing the causative agent (at least for interferons and dasatinib). Therefore discontinuation of the causative agent is recommended whenever possible. Immediate PAH therapy should be considered in patients who present with intermediate-/high-risk PAH at diagnosis.

Correct! Pulmonary arterial hypertension therapy should be initiated in patients who do not normalize their haemodynamics after withdrawing or in patients presenting with more advanced PAH at diagnosis. Unlike in other forms of PAH, de-escalation of PAH therapy is often possible during the course of the disease.

Correct! It is recommended that these patients be observed over 3–4 months before considering PAH therapy.

Correct! In patients with suspected drug- or toxin-associated PAH, it is recommended to immediately discontinue the causative agent whenever possible (Class I, level C recommendation).

Correct!

Incorrect. In these patients, shunt closure should be considered (class IIa recommendation).

Incorrect. Shunt closure is not recommended (class III recommendation) in case of ASD (atrial septal defect), but may by considered in cases of VSD (ventricular septal defect) and PDA (patent ductus arteriosus) after careful evaluation in specialized centres (class IIb recommendation).

Incorrect. In patients with ASD and PVR >5 WU that declines to <5 WU with PAH treatment, shunt closure may be considered (class IIb recommendation).

Incorrect. Defect closure is contraindicated in all patients with Eisenmenger syndrome. This is because shunt closure may sacrifice the right-sided pressure relief role, leading to worsening right heart failure.

Correct! Defect closure is contraindicated in all patients with Eisenmenger syndrome.

Incorrect. Secondary erythrocytosis is beneficial for adequate oxygen transport and delivery, and routine phlebotomy should be avoided whenever possible (it also removes platelets/plasma).

Correct! In the absence of significant haemoptysis, oral anticoagulant treatment may be considered in patients with Eisenmenger syndrome with pulmonary artery thrombosis (Class IIb, level C recommendation).

Incorrect. This is not a class I recommendation. Other ERAs, PDE5is, riociguat, prostacyclin analogues, and prostacyclin receptor agonists should be considered (class Iia recommendation).

Incorrect. An RCT (randomized controlled trial) investigating the efficacy of macitentan found no effect on 6MWD in a mixed cohort of patients with Eisenmenger syndrome (6MWD improved in both treatment and placebo arms), although decreases in NT-proBNP and PVR were noted in the macitentan arm.

Correct! In a first placebo-controlled trial in patients with Eisenmenger syndrome, Bosentan improved 6MWD (6-minute walk distance) and decreased PVR (pulmonary vascular resistance) in patients with Eisenmenger syndrome in WHO-FC III, without compromising peripheral oxygen saturation.

Incorrect. This is not a class I recommendation. Other ERAs, PDE5is, riociguat, prostacyclin analogues, and prostacyclin receptor agonists should be considered (class Iia recommendation).

Correct!

Incorrect. The only curative therapy is LTx. This group of patients has a poor prognosis and limited response to PAH therapy, with a risk of pulmonary oedema due to pulmonary venous obstruction.

Correct! The only curative therapy is LTx, and eligible patients should be referred to a transplant centre for evaluation upon diagnosis.

Incorrect. Lung biopsy is hazardous in PH and is not recommended for the diagnosis.

Incorrect. Drugs approved for PAH (pulmonary arterial hypertension) are not recommended in PH-LHD (Class III, level A recommendation). Macitentan failed to show efficacy but rather led to more adverse events (fluid retention) vs. placebo in patients with HFpEF-associated PH.

Incorrect. Drugs approved for PAH (pulmonary arterial hypertension) are not recommended in PH-LHD (Class III, level A recommendation). Macitentan failed to show efficacy but rather led to more adverse events (fluid retention) vs. placebo in patients with HFpEF-associated PH.

Incorrect. Drugs approved for PAH are not recommended in PH associated with left heart disease. Small studies have suggested that sildenafil may improve haemodynamics and exercise capacity in PH and HFrEF, but randomized controlled trials are lacking. Similarly, in patients with HFpEF a general recommendation for or against the use of phosphodiesterase 5 inhibitors in patients with HFpEF and CpcPH (combined post- and pre-capillary PH) cannot be made, as there is no study that specifically enrolled patients with HFpEF and CpcPH. However, it is clinically relevant to make a recommendation against their use for patients with HFpEF and IpcPH (isolated post-capilary PH).

Correct! Drugs approved for PAH are not recommended in PH associated with left heart disease.

Incorrect. The currentguidelines used PVR to distinguish between non-severe PH (PVR ≤5 WU) and severe PH (PVR >5 WU)

Correct! Recent studies have demonstrated that a PVR >5 WU is a better threshold for predicting worse prognosis in patients with PH associated with both COPD (chronic obstructive lung disease) and ILD (interstitial lung disease).

Incorrect. In the 2015 ESC/ERS Guidelines for the diagnosis and treatment of PH, severe PH was defined by mPAP >35 mmHg or mPAP≥25 mmHg with CI <2.5 L/min/m2. However, two recent studies have demonstrated that a PVR >5 WU is a better threshold for predicting worse prognosis in patients with PH associatedwith both COPD and ILD.

Incorrect. In the 2015 ESC/ERS Guidelines for the diagnosis and treatment of PH, severe PH was defined by mPAP >35 mmHg or mPAP≥25 mmHg with CI <2.5 L/min/m2. However, two recent studies have demonstrated that a PVR >5 WU is a better threshold for predicting worse prognosis in patients with PH associatedwith both COPD and ILD.

Incorrect. Inhaled treprostinil may be conisdered (class IIa recommendation) in patients with PH associated with ILD.

Correct! The use of ambrisentan is not recommended in patients with PH associated with IPF (interstitial pulmonary fibrosis). Ambrisentan was associated with an increased risk of clinical worsening in patients with ILD with and without PH.

Correct! The use of riociguat is not recommended in patients with PH associated with IIP (idiopathic interstitial pneumonia). Riociguat was associated with an increased risk of clinical worsening events, including potential excess mortality, in patients with PH associated with idiopathic interstitial pneumonia.

Incorrect.

Incorrect. Ventilation/perfusion scintigraphy remains the most effective tool in excluding CTEPD.

Incorrect. Perfusion imaging is the moste effective test to rule out CTEPD. Digital subtraction angiography is still used to assess treatment options when CTPA is inconclusive.

Incorrect. Computed tomography pulmonary angiography with bi-planar reconstruction is broadly used for diagnosing CTEPD, not for excluding it.

Correct! A normal perfusion scan effectively rules out the possibility of CTEPH, as endorsed by a Canadian Thoracic Society clinical practice guideline update on the diagnosis of chronic thromboembolic pulmonary hypertension.

Incorrect. Lifelong, therapeutic doses of anticoagulation are recommended in all patients with CTEPH (class I, level C recommendation).

Incorrect. Lifelong, therapeutic doses of anticoagulation are recommended in all patients with CTEPH (class I, level C recommendation).

Incorrect. Lifelong, therapeutic doses of anticoagulation are recommended in all patients with CTEPH (class I, level C recommendation).

Correct! Lifelong therapeutic anticoagulation is recommended for patients with CTEPH, as recurrent pulmonary thrombo-embolism accompanied by insufficient clot resolution are key pathophysiological features of this disease.

Incorrect. The value mPAP/CO slope between rest and exercise is used in the current definiton.

Correct! Exercise PH, defined by an mPAP/CO slope > 3 mmHg/L/min between rest and exercise, has been reintroduced. It defines an abnormal haemodynamic response to exercise. Increase in pulmonary pressure during exercise is also associated with impaired prognosis in patients with exercise dyspnoea.

Incorrect. Even though it is not used in the definition, the PAWP/CO slope with a threshold >2 mmHg/L/min may best differentiate between pre- and post-capillary causes of exercise PH. This value may be useful as mPAP/CO slope value does not allow for differentiation between pre- and post-capillary causes.

Incorrect. The value mPAP/CO slope between rest and exercise is used in the current definiton.

Correct! PEA is recommended as the treatment of choice for patients with CTEPH and fibrotic obstructions within pulmonary arteries accessible by surgery. (Class I, level B recommendation).

Incorrect. BPA is recommended in patients who are technically inoperable or have residual PH after PEA and distal obstructions amenable to BPA BPA may be also considered for technically operable patients with a high proportion of distal disease and an unfavourable risk:benefit ratio for PEA.

Incorrect. Preliminary data on PADN point towards improved exercise capacity and pulmonary haemodynamics in patients with persistent PH after PEA; further confirmation is being awaited.

Incorrect. PEA is recommended as the treatment of choice for patients with CTEPH and fibrotic obstructions within pulmonary arteries accessible by surgery.

Incorrect. Treprostinil has a class IIb recommendation for patients in WHO-FC III–IV who have inoperable CTEPH or persistent/recurrent PH after PEA (pulmonary endarterectomy) - meaning, it may be considered.

Correct! In the phase 3 randomized controlled trial, riociguat improved 6MWD (6-minute walking distance) and reduced PVR (pulmonary vascular resistance) by 31% compared with placebo, and is approved for this indication - in patients with inoperable CTEPH or those with persistent/recurrent PH after PEA .

Incorrect. There is currently no specific class I recommendation for the use of macitentan in these patients. However, a phase 3 randomized controlled trial is ongoing to evaluate the safety and efficacy of macitentan 75 mg in inoperable or persistent/recurrent CTEPH.

Incorrect. There is no class I recommendation for all PAH drugs in this setting. Off-label use of drugs approved for PAH may be considered in symptomatic patients who have inoperable CTEPH.

Incorrect. Lung disease, especially chronic obstructive pulmonary disease (COPD), is the second most common cause.

Incorrect. Even though, the number of patients diagnosed with CTEPH (chronic thrombo-embolic pulmonary hypertension) is increasing, it is not the leading cause of PH. Registry data indicate a CTEPH incidence and prevalence of 2–6 and 26–38 cases/million adults, respectively.

Correct! Left heart disease (LHD) is the leading cause of pulmonary hypertension (PH). Post-capillary PH, is a frequent complication mainly in HFpEF, affecting at least 50% of these patients. The prevalence of PH also increases with severity of left-sided valvular diseases.

Incorrect. Idiopathic pulmonary artery hypertension (IPAH) is the most common subtype of PAH, however, it is not the leading cause of PH.

Correct! Cardinal symptom of pulmonary hypertension is dyspnoea on progressively minor exertion.

Incorrect. Exertional chest pain is not typically an early symptom. It is caused by dynamic compression of the left main coronary artery due to pulmonary artery dilation.

Correct! It is caused by inadequate increase in cardiac output during exercise because of increased pulmonary vascular resistance.

Incorrect. Hoarseness is an uncommon, usually late symptom. It is cuased by the compression of the left laryngeal recurrent nerve, due to pulmonary artery dilation.

Correct! It is usually a result of right ventricular hypertrophy. It also ahs a high predictive value for PH in adults with clinical suspicion of PH

Correct! A sign of right atrial enlargement, best seen in the inferior leads.

Correct! Although non-specific, prolonged QTc may reflect RV dysfunction and delayed myocardial repolarization. It is also an independent predictor of mortality.

Correct! A normal ECG does not exclude the presence of PH, but when combined with normal biomarkers (BNP/NTpro-BNP), it is associated with a low likelihood of PH in patients referred for suspected PH or at risk of PH.

Incorrect. These signs are not typically found in patients with CTEPH.

Correct! The mentioned signs point towards the diagnosis of systemic sclerosis (SSc). Systemic sclerosis, particularly in its limited variant, represents the main cause of PAH-CTD (pulmonary arterial hypertension associated with connective tissue disease).

Incorrect. These signs are not typically found in patients with PVOD.

Incorrect.