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Lung transplantation – Recommendation Table 13 R Lung transplantation is recommended soon after

inadequate clinical response on maximal medical therapy

I 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 I

N 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

Pulmonary arterial hypertension associated with drugs or toxins – Recommendation Table 14 N 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 I

N In patients with suspected drug- or toxin-associated PAH,

it is recommended to discontinue the causative agent immediately whenever possible I

N Immediate PAH therapy should be considered in patients

who present with intermediate/high-risk PAH at diagnosis IIa

N 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 IIb

Pulmonary arterial hypertension associated with connective tissue disease – Recommendation Table 15 N In patients with PAH associated with CTD, treatment of

the underlying condition according to current guidelines is recommended I

Pulmonary arterial hypertension associated with human immunodeficiency virus infection – Recommendation Table 16 N In patients with PAH associated with HIV infection,

antiretroviral treatment according to current guidelines is recommended I

N 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 IIa

Pulmonary arterial hypertension associated with portal hypertension – Recommendation Table 17 R Echocardiographic assessment for signs of PH is

recommended in symptomatic patients with liver disease or portal hypertension and in all candidates for liver transplantation I 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 shunt I

R It is recommended that the treatment algorithm for

patients with other forms of PAH should be applied to patients with PAH associated with portal hypertension, taking into account the severity of liver disease I 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 transplantation IIa

R Liver transplantation may be considered in selected

patients responding well to PAH therapy IIb 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 IIa

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