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Apitz, C.; Abdul-Khaliq, H.; Albini, S.; Beerbaum, P.; Dubowy, K. O.; Gorenflo, M.; Hager, A.; Hansmann, G.; Hilgendorff, A.; Humpl, T.; Kaestner, M.; Koestenberger, M.; Kozlik-Feldmann, R.; Latus, H.; Michel-Behnke, I.; Miera, O.; Quandt, D.; Sallmon, H.; Schranz, D.; Schulze-Neick, I.; Stiller, B.; Warnecke, G.; Pattathu, J. and Lammers, A. E. (2019): Neue hämodynamische Definition der pulmonalen Hypertonie. Kommentar der Arbeitsgemeinschaft Pulmonale Hypertonie der Deutschen Gesellschaft für Pädiatrische Kardiologie und angeborene Herzfehler e. V. (DGPK). In: Monatsschrift Kinderheilkunde, Vol. 168, No. 3: pp. 252-256

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Pulmonary hypertension (PH) is a pathological elevation of pulmonary pressure and is associated with a heterogeneous spectrum of diseases affecting the pulmonary vasculature. The most common etiologies in children are idiopathic pulmonary arterial hypertension (IPAH), hereditary pulmonary arterial hypertension (HPAH) and PH associated with congenital heart disease (PH-CHD). According to international guidelines, PH used to be defined as elevation of the mean pulmonary arterial pressure (mPAP) >= 25mmHg at rest. In children, particularly with congenital heart disease and a shunt lesion, pulmonary vascular resistance (PVR, indexed to body surface area, PVRi) has been used for years as an adjunct diagnostic criterion to distinguish high-flow PH from pulmonary hypertensive vascular disease (PHVD) with an increase in PVR. A PVRi >= 3 WU center dot m(2)BSA suggests the presence of PHVD, which has a precapillary component (if a normal or reduced cardiac output is present). At the latest World Symposium on Pulmonary Hypertension (WSPH) in 2018 in Nice a new definition of PH has been introduced with a lower cut-off value for a normal mPAP from 24 to 20 mmHg. This is motivated by register studies in adult PH patients in whom a higher mortality has been shown with these lower mPAP levels. Although not uniformly welcomed by all pediatric cardiologists, this new definition (mPAP >20mmHg) has been accepted by the Pediatric Task Force of the WSPH to establish a uniform language and facilitate transition to adult services. The authors would like to emphasize, that there are no published pediatric data demonstrating, that a mild elevation of the mPAP of 21-24mmHg (according to the new definition) has a similar impact in children and leads to adverse outcomes and increased mortality. Hence no change in treatment strategies can be derived on the basis of the new definition to date. Randomized controlled studies in children with PH are sparse, evidence-based pediatric therapeutic strategies are lacking and experience is mostly adopted from adult guidelines. All available studies testing safety and efficacy of medical treatment in PAH have been performed mainly in adult patients, with inclusion criteria according to the former definition of mPAP >= 25mmHg. From the authors' point of view, the indication for using advanced treatment on the basis of the new definition (mPAP >20mmHg) is debatable. In most children and adolescents with PH advanced medical treatment is still only warranted if mPAP exceeds >= 25mmHg. Indications for treatment are often a patient-tailored decision, depending on PH etiology and other driving factors. For individual (symptomatic) patients with only mild elevation of the mPAP (21-24mmHg) and increase of PVRi >= 3 WU center dot m(2)BSA, targeted therapy may be indicated. Because of the complexity and heterogeneity of PH in childhood and adolescence, children should be referred to pediatric cardiology centers with PH expertise. Independent of clinical severity at presentation, the decision for the indication for PAH-specific therapy should be addressed by experienced PH specialists and children should be linked to PH clinics. Conclusion. Counselling patients and parents on the implications of the new PH definition (mPAP >20mmHg) is paramount. To date there are no therapeutic consequences, even though a child may fulfil contemporary criteria according to the new definition with mPAP of 21-24mmHg. Summary. The definition of PH has changed in 2018 (WSPH, Nice) to mPAP >20mmHg. Because of lacking evidence this change of definition does not necessarily result in any changes to currently applied pharmaceutical strategies in children.

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