[I-AEPCJS-3] Modern treatment strategies for pediatric heart failure
キーワード：heart failure, dilative cardiomyopathy, inovative therapy
The incidence of pediatric heart failure due to congenital heart disease and cardiomyopathies at various stages of disease progression is poorly defined. However, the incidence of childhood cardiomyopathy (CMP) is reported at 1-1.5/ 100.000 children. Dilated cardiomyopathy (DCM) is the most common form of primary cardiomyopathies and prevailing cause of cardiac transplantation at young age. DCM is a serious diagnosis with a gloomy prognosis: reported rates of death or transplantation at 1 or 5 years are 28-35 and 37-49%, respectively. Furthermore, heart transplantation (HTx) is not curative and there is shortage of organ availability and mortality and morbidity on the waiting list, with assist devices and after transplantation. This situation creates a permanent motivation and obligation to explore alternative concepts to avoid or postpone HTx.
The Giessen approach to end-stage pediatric heart failure routinely involves ß1-receptor blockers, tissue ACE-I and mineralocorticoid receptor blockers as well as phosphodiesterase-III blocker and calcium sensitizer while avoiding diuretics and catecholamines after the initial recompensating phase. Atrial decompression by creating a restrictive ASD can benefit children with heart failure and reduced ejection fraction (HFrEF, DCM) and HFpEF (RCM). In DCM patients with preserved RV function the pulmonary artery banding (PAB) approach can be applied and the pathophysiological concept thereof as well as experimental and clinical evidence for this approach will be discussed. In addition, cardiac resynchronization therapy (especially with LBBB) is a means to improve ventriculo-ventricular interaction and LV function. Finally, cell based approaches may benefit selected patients with end-stage heart failure.
The Giessen approach to end-stage pediatric heart failure routinely involves ß1-receptor blockers, tissue ACE-I and mineralocorticoid receptor blockers as well as phosphodiesterase-III blocker and calcium sensitizer while avoiding diuretics and catecholamines after the initial recompensating phase. Atrial decompression by creating a restrictive ASD can benefit children with heart failure and reduced ejection fraction (HFrEF, DCM) and HFpEF (RCM). In DCM patients with preserved RV function the pulmonary artery banding (PAB) approach can be applied and the pathophysiological concept thereof as well as experimental and clinical evidence for this approach will be discussed. In addition, cardiac resynchronization therapy (especially with LBBB) is a means to improve ventriculo-ventricular interaction and LV function. Finally, cell based approaches may benefit selected patients with end-stage heart failure.