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R A PH diagnostic algorithm work-up is recommended for

diagnosis and definition of the specific aetiology group in paediatric PH patients I In children with PH, a comprehensive work-up for

confirming diagnosis and specific aetiology is recommended (similar to that in adults, but adapted for age) I

N For confirming PH diagnosis, RHC is recommended, preferably before initiating any PAH therapy I N In children with IPAH/HPAH, acute vasoreactivity testing is recommended to detect those who may benefit from

calcium channel blocker therapy I

N It is recommended to define a positive response to acute

vasoreactivity testing in children similar to adults by a reduction of mPAP ≥10 mmHg to reach an absolute value of mPAP ≤40 mmHg, with an increased or unchanged CO I

R A PAH-specific therapeutic algorithm is recommended in

paediatric PH patients I 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 I

R Specific paediatric determinants of risk should be considered

IIa 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 I

N Achieving and maintaining a low-risk profile should be considered as an adequate treatment response for

children with PAH IIa

N It is recommended to screen infants with

bronchopulmonary dysplasia for PH I

N 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 therapy I

N 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 IIa

Pulmonary hypertension associated with left heart disease – Recommendation Table 22 N RHC is recommended for suspected PH in patients with

LHD, if it aids management decisions I

N RHC is recommended in patients with severe tricuspid

regurgitation with or without LHD prior to surgical or interventional valve repair I

R Patients with PH-LHD and a severe pre-capillary

component as indicated by a high DPG and/or high PVR should be referred to an expert PH centre for a complete diagnostic work-up and an individual treatment decision IIa For patients with LHD and suspected PH with features of

a severe pre-capillary component and/or markers of RV dysfunction, referral to a PH centre for a complete diagnostic work-up is recommended I

N In patients with LHD and CpcPH with a severe

pre-capillary component (e.g. PVR .5 WU), an individualized approach to treatment is recommended I

N When patients with PH and multiple risk factors for LHD,

who have a normal PAWP at rest but an abnormal response to exercise or fluid challenge, are treated with PAH drugs, close monitoring is recommended I

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