caa a22 4500 47578720X CHVBK 20180406123701.0 cr unu---uuuuu 170329e20001001xx s 000 0 eng 10.1007/s004310000588 doi (NATIONALLICENCE)springer-10.1007/s004310000588 Chronic cardiac failure in children due to dilated cardiomyopathy: diagnostic approach, pathophysiology and management [Elektronische Daten] [Poothirikovil Venugopalan, Ajit Kumar Agarwal, Elizabeth Ann Worthing] Dilated cardiomyopathy (DCM) is an important cause of chronic congestive cardiac failure (CCF) in infants and children. Although a variety of aetiological factors have been listed, most patients with echocardiographically documented DCM do not possess a demonstrable cause. Poor myocardial function in DCM triggers a sequence of compensatory mechanisms, mediated through the renin angiotensin system, the sympathetic system (neural and humoral) and a number of vasodilatory molecules. These favour myocardial and peripheral vascular remodelling by necrosis, fibrosis and apoptosis which ultimately does more harm than good. Management is essentially similar to that of CCF using a combination of diuretics, angiotensin converting enzyme inhibitors (ACEI) and digoxin. Use of additional diuretics, intravenous frusemide, dobutamine infusion and β-adrenergic receptor blockers help to improve quality of life in resistant patients. ACEI, β-adrenergic blockers and possibly aldosterone antagonists prolong survival. Compliance to medication can be enhanced by improving drug formulations and by assisting the family to choose the ‘best-fit' regimen (concordance). At present, optimum management of end-stage CCF is cardiac transplant. Conclusion Current understanding of the pathophysiology of chronic congestive cardiac failure resulting from dilated cardiomyopathy has shifted management strategy from steps that directly improve myocardial function to those that modulate the neuroendocrine profile and peripheral vascular reactivity. Similar advances in therapeutic applications would be assisted by controlled studies and full licensing of drugs for use in children. Medical intervention will remain the cornerstone of management until advances in surgical techniques become more widely available. Springer-Verlag Berlin Heidelberg, 2000 Keywords Apoptosis nationallicence Beta-blocker therapy nationallicence Concordance nationallicence Drug compliance nationallicence Myocardial remodelling nationallicence AbbreviationsACEI angiotensin converting enzyme inhibitors nationallicence Ang angiotensin nationallicence CCF congestive cardiac failure nationallicence DCM dilated cardiomyopathy nationallicence Venugopalan Poothirikovil Department of Cardiology, Child Ward 2, Sultan Qaboos University Hospital, PO Box 38, PC 123, Muscat, Sultanate of Oman e-mail: gopalax@omantel.net.om Fax: +968-513009, OM aut Agarwal Ajit Kumar Department of Cardiology, Child Ward 2, Sultan Qaboos University Hospital, PO Box 38, PC 123, Muscat, Sultanate of Oman e-mail: gopalax@omantel.net.om Fax: +968-513009, OM aut Worthing Elizabeth Ann Pharmacy Department, Sultan Qaboos University Hospital, Muscat, Sultanate of Oman, OM aut https://doi.org/10.1007/s004310000588 text/html Onlinezugriff via DOI 1 research-article jats NATIONALLICENCE 856 40 https://doi.org/10.1007/s004310000588 text/html Onlinezugriff via DOI NATIONALLICENCE 700 1- Venugopalan Poothirikovil Department of Cardiology, Child Ward 2, Sultan Qaboos University Hospital, PO Box 38, PC 123, Muscat, Sultanate of Oman e-mail: gopalax@omantel.net.om Fax: +968-513009, OM aut NATIONALLICENCE 700 1- Agarwal Ajit Kumar Department of Cardiology, Child Ward 2, Sultan Qaboos University Hospital, PO Box 38, PC 123, Muscat, Sultanate of Oman e-mail: gopalax@omantel.net.om Fax: +968-513009, OM aut NATIONALLICENCE 700 1- Worthing Elizabeth Ann Pharmacy Department, Sultan Qaboos University Hospital, Muscat, Sultanate of Oman, OM aut Metadata rights reserved Springer special CC-BY-NC licence nationallicence BK010053 XK010053 XK010000 NATIONALLICENCE NATIONALLICENCE NL-springer