Pulmonale Hypertonie im Kindesalter – Ein Update vom Weltsymposium Pulmonale Hypertonie 2024

Zusammenfassung

Pulmonale Hypertonie (PH) kann in jeder Lebensphase, vom Neugeborenen- bis in das Erwachsenenalter auftreten, wobei bei Kindern teils sehr spezifische, altersabhängige Besonderheiten berücksichtigt werden müssen. Pulmonale Hypertonie im Kindesalter ist kein umschriebenes Krankheitsbild, sondern beschreibt einen hämodynamischen Zustand, dem unterschiedliche Ursachen zugrunde liegen können, die eine altersgemäße, vollständige, leitliniengerechte, diagnostische Abklärung erfordern, um eine korrekte Phänotypisierung, Charakterisierung und Klassifikation sowie zielgerichtete Behandlung der zugrunde liegenden Erkrankung zu ermöglichen. In ihrem aktuellen Bericht vom Welt-Symposium zur Pulmonalen Hypertonie (WSPH) 2024 fasst die pädiatrische Arbeitsgruppe neue Daten und Entwicklungen zusammen, die zu aktualisierten Konsensempfehlungen hinsichtlich Diagnose und Behandlung der PH im Kindesalter geführt haben. Dazu gehören eine alterskorrelierte Charakterisierung und Definition der pädiatrischen PH, eine erweiterte Risikoabschätzung bei Kindern sowie ein neuer Behandlungsalgorithmus, der erstmals auch kardiopulmonale Komorbiditäten berücksichtigt. Das vorliegende Manuskript bietet ein Update über die wichtigsten Neuerungen vom WSPH, ergänzt durch Expertenkommentare unter Bezug auf rezente Literatur.

Abstract

Pulmonary hypertension (PH) can occur at any stage of life, from neonatal to adulthood, although very specific, age-dependent characteristics must be taken into account in children. Pulmonary hypertension in children is not a circumscribed clinical picture, but describes a haemodynamic condition that can be caused by different factors, which require an age-appropriate, complete, guideline-based diagnostic clarification in order to enable correct phenotyping, characterization and classification, as well as targeted treatment of the underlying disease. In their recent report from the World Symposium on Pulmonary Hypertension (WSPH) 2024, the pediatric task force summarized new data and developments that have led to updated consensus recommendations regarding the diagnosis and treatment of PH in childhood. These include an age-correlated characterization and definition of pediatric PH, expanded risk assessment in children, and a new treatment algorithm that includes cardiopulmonary comorbidities for the first time. This manuscript provides an update on the most important innovations of the WSPH, supplemented by expert commentary with reference to recent literature.

Schlüsselwörter

Pädiatrische Pulmonale Hypertonie - pulmonal arterielle Hypertonie - angeborene Herzfehler - pädiatrische Kardiologie - Risikofaktoren - Therapiealgorithmus

Keywords

pediatric pulmonary hypertension - pulmonary arterial hypertension - congenital heart disease - pediatric cardiology - risk assessment - treatment algorithm

Publication History

Article published online:
06 October 2025

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