Pulmonalarterielle Hypertonie (PAH) bei angeborenen Herzfehlern mit Shuntverbindung: eine heterogene und komplexe Konstellation

Zusammenfassung

Es wird erläutert, wie sich bei angeborenen Herzfehlern mit primärem Links-Rechts-Shunt über die verschiedenen Alters- und Entwicklungsstufen mit und ohne operative, interventionelle oder medikamentöse Behandlung eine pulmonalvaskuläre Erkrankung entwickeln kann. Darauf aufbauend wird diskutiert, in welchen Stadien der Erkrankung eine sog. spezifische oder „advanced therapy“ der pulmonalvaskulären Erkrankung zum Einsatz kommen kann.

Abstract

It will be explained how pulmonary vascular disease can develop in congenital heart defects with primary left-to-right shunt across the different ages and stages of development, with and without surgical, interventional or medical treatment. Furthermore it will be discussed, in which specific stages of pulmonary vascular disease “advanced therapy” can be used.

Schlüsselwörter

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

Keywords

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

• Literatur

• 1 Adatia I, Kothari SS, Feinstein JA. Pulmonary hypertension associated with congenital heart disease. Chest 2010; 137 (Suppl. 01) 53S
• 2 Beghetti M, Galiè N, Bonnet D. Can „inoperable“ congenital heart defects become operable in patients with pulmonary arterial hypertension? Dream or reality?. Congenit Heart Dis 2012; 7: 3-11
• 3 Diller GP, Alonso-Gonzalez R, Dimopoulos K et al. Disease targeting therapies in patients with Eisenmenger syndrome: Response to treatment and long-term efficiency. Int J Cardiol 2012; Mar 3. [Epub ahead of print]
• 4 Dimopoulos K, Inuzuka R, Goletto S et al. Improved survival among patients with Eisenmenger syndrome receiving advanced therapy for pulmonary arterial hypertension. Circulation 2010; 121: 20-25
• 5 European Society of Cardiology/European Respiratory Society. ESC/ERS Guidelines for the diagnosis and treatment of pulmonary hypertension. Eur Heart J 2009; 30: 2493-2537
• 6 Galiè N, Beghetti M, Gatzoulis MA et al. Bosentan Randomized Trial of Endothelin Antagonist Therapy-5 (BREATHE-5) Investigators.  Bosentan therapy in patients with Eisenmenger syndrome: a multicenter, double-blind, randomized, placebo-controlled study. Circulation 2006; 114: 48-54
• 7 Iversen K, Jensen AS, Jensen TV et al. Combination therapy with bosentan and sildenafil in Eisenmenger syndrome: a randomized, placebo-controlled, double-blinded trial. Eur Heart J 2010; 31: 1124-1131
• 8 Kaemmerer H, Mebus S, Schulze-Neick I et al. The adult patient with eisenmenger syndrome: a medical update after dana point part I: epidemiology, clinical aspects and diagnostic options. Curr Cardiol Rev 2010; 6: 343-355
• 9 Manes A, Palazzini M, Leci H et al. Current era survival of patients with pulmonary arterial hypertension associated with congenital heart disease: a comparison between clinical subgroups. Eur Heart J 2013; Mar 1. [Epub ahead of print]
• 10 Mebus S, Schulze-Neick I, Oechslin E et al. The adult patient with Eisenmenger syndrome: a medical update after Dana Point Part II: Medical treatment – study results. Curr Cardiol Rev 2010; 6: 356-362
• 11 Tay EL, Papaphylactou M, Diller GP et al. Quality of life and functional capacity can be improved in patients with Eisenmenger syndrome with oral sildenafil therapy. Int J Cardiol 2011; 149: 372-376