Pulmonalarterielle Hypertonie bei angeborenen Herzfehlern: Problemstellung und Versorgungslage

Zusammenfassung

Fehlbildungen des Herzens und der herznahen Gefäße (AHF) gehören zu den häufigsten kongenitalen Anomalien. Aufgrund der verbesserten interdisziplinären Betreuung erreichen nun etwa 90 % der AHF-Patienten das Erwachsenenalter. Bei bis zu 10 % dieser Patienten besteht oder entwickelt sich eine pulmonale arterielle Hypertonie (PAH), die die Belastungsfähigkeit und Prognose verschlechtert. Daten zur Versorgungssituation der Patienten mit PAH-AHF sind nur eingeschränkt verfügbar. Das Prospective Registry of Newly Initiated Therapies for Pulmonary Hypertension (COMPERA, ClinTrials.gov Identifier NCT01347216) dokumentiert prospektiv Patienten ab 18 Jahren mit allen Formen der pulmonalen Hypertonie, sofern sie mit PAH-spezifischen Medikamenten behandelt werden (mittlere Beobachtungszeit aktuell 40 Monate). Zum 16.  November 2012 hatten 8 % von insgesamt 3642 Patienten eine PAH-AHF, von denen 104 ausführlich in einem separaten Modul beschrieben wurden. Im Mittel waren sie 39 Jahre alt (39 %) Männer, hatten eine mittlere 6-Minuten-Gehstrecke von 370 ± 102 Metern, und wurden in NYHA-Funktionsklasse I und II in 39 %, III in 59 %, und IV in 3 % eingestuft. Die mittlere Lebensqualität auf der visuellen Analogskala (EQ-5 D, 0 bis 100) lag bei 51. Die PAH-AHF-Patienten wurden zu 80 % mit einer Monotherapie behandelt, zu 9 % mit Kombinationen bzw. erhielten in 11 % keine PAH-Medikamente. Nur 20 % erhielten eine orale Antikoagulation. Die mittlere Überlebensrate nach 4 Jahren lag bei „inzidenten“ Patienten (PAH-AHF seit Registerbeginn neu diagnostiziert) bei 79 %, verglichen mit 72 % bei idiopathischer PAH (IPAH). Patienten mit PAH-AHF sind ausweislich der Registerdaten eingeschränkt belastungsfähig und haben eine stark verminderte Lebensqualität. Sie werden seltener als IPAH-Patienten mit Kombinationen bzw. oraler Antikoagulation behandelt, ihre Überlebensrate ist jedoch höher.

Abstract

Defects of the heart and associated large vessels (CHD) are among the most frequent congenital anomalies. Owing to improved interdisciplinary management, about 90 % of CHD patients reach adulthood. Up to 10 % maintain or newly develop pulmonary arterial hypertension (PAH) over time, which impairs exercise tolerance and prognosis. Data on the health care situation of patients with PAH-CHD are limited. The ongoing Prospective Registry of Newly Initiated Therapies for Pulmonary Hypertension (COMPERA, ClinTrials.gov Identifier NCT01347216) prospectively documents adult patients with all forms of pulmonary hypertension, if treated with PAH drugs (mean follow-up 40 months). As of 16 November 2012, 8 % of the 3642 patients in the database had PAH-CHD. Of the latter, 104 were documented in great detail in specific CHD report forms. These patients were on average 39 years old, men in 39 %, had a mean 6-minute walk distance of 370 ± 102 meters, and were in NYHA functional class I/ II in 39 %, III in 59 %, und IV in 3 %. Mean quality of life on the 100-point visual analogue scale (EQ-5 D) was 51. PAH-CHD patients received monotherapy in 80 %, combination therapy in 9 %, and no PAH drugs in 11 %. Only 20 % were on oral anticoagulation (OAC). Mean 4-year survival in incident patients (PAH-CHD diagnosis after start of the registry in 2007) was 79 %, compared with 72 % in patients with idiopathic PAH (IPAH). According to these registry data, patients with PAH-CHD have impaired exercise capacity, and substantially reduced quality of life. They receive combination therapy or OAC, respectively, less frequently than IPAH patients, however, their survival rate is higher.

Schlüsselwörter

angeborene Herzfehler - Links-rechts-Shunt - pulmonale arterielle Hypertonie - Versorgung - Lebensqualität - Überleben

Keywords

congenital heart disease - left-right shunt - pulmonary arterial hypertension - health care - quality of life - survival

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