Volume 2 Supplement 1
Modern chronic heart failure therapy in context of pulmonary banding to avoid heart transplantation
© Recla et al. 2015
Published: 1 July 2015
Dilated cardiomyopathy (DCM) is a leading cause of cardiac death in children. Approximately 30% of children die or need cardiac transplantation in the first year after diagnosis. We established a protocol to improve the outcome in this high-risk population.
Patients and methods
We present our experience in 21 patients (mean age 8 month, mean weight 6 kg) treated in our institution from 2006 to 2015. The patients were diagnosed with DCM with a highly impaired function of the LV (mean EF 17%) and a conserved function of the RV (mean EF 52%). Our protocol for medical enhancement of left ventricular recovery in association with pulmonary artery banding involves the use of a highly specific β1-blocker, an angiotensin-converting enzyme inhibitor and an aldosterone antagonist. Our therapy aims to reduce oxygen consumption and improve oxygen delivery. Heart rate control is the most important goal; therefore clonidine is given after surgical procedure and digoxin in long-term treatment if heart rate remains high despite adequate β1-blocker therapy. To improve oxygen delivery our goal is to archive haemoglobin levels of 12-14 g/dl, therefore Erythropoietin is given as long term treatment. Additional treatments include supplementation of carnitine, coenzyme Q, riboflavin or thiamine.
At a mean follow up of 36 month (range 2-120) freedom from death was 91% and freedom from heart transplantation was 85%. Surviving patients showed a significant improvement in left ventricular ejection fraction (from 17 ± 6 to 50 ± 11%) and LVEdD (z-score from +7 ± 2 to +1.7 ± 1.9). The levels of BNP improved significantly (from 3222 ± 2756 to 70 ± 56 pg/ml).
Our data suggest that the medical and surgical approach described may result in a markedly improved medium-term outcome in children with DCM. Further studies are required to evaluate the long-term-outcome of these patients.
This article is published under license to BioMed Central Ltd. This is an Open Access article distributed under the terms of the Creative Commons Attribution License (http://creativecommons.org/licenses/by/4.0), which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited.