PMID- 27812385 OWN - NLM STAT- PubMed-not-MEDLINE LR - 20210109 IS - 2055-5822 (Print) IS - 2055-5822 (Electronic) IS - 2055-5822 (Linking) VI - 3 IP - 2 DP - 2016 Jun TI - Heart failure from heart muscle disease in childhood: a 5-10 year follow-up study in the UK and Ireland. PG - 107-114 AB - AIMS: Our original study, the first national prospective study of new-onset heart failure from heart muscle disease in children, showed overall 1-year survival of 82%, and event (death or transplantation)-free survival of 66%. This study aimed to evaluate 5 + year outcomes of this important cohort. METHODS AND RESULTS: All centres in the UK and Ireland with 1-year event-free survivors participated (n = 14). Anonymised data based on last hospital attendance and echocardiograms were reviewed. The investigator was blinded to outcome at the time of echo review. Of sixty-nine 1-year event-free survivors, data were obtained on 64, with three lost to follow-up and two moved abroad. There were three deaths at 2.2, 3.3 and 9.0 years after presentation and one transplant, at 5.2 years. Overall/event-free survival was 77%/62% at 5 years and 73%/59% at 10 years, respectively. Overall and event-free survival conditional on 1-year survival was 94% at 5 years, and 89% at 10 years. For the 60 event-free survivors, median (range) follow-up duration was 9.04 (5.0-10.33) years for those still under review (n = 45), or time to discharge 5.25 (0.67-10.0) years (n = 15). Fifty-eight were in New York Heart Association (NYHA) Class 1, and two in Class 2. Forty-one out of sixty had normal echocardiograms at last follow-up. Predictors of better longer-term outcome were the same as for the original 1-year follow-up study, namely, younger age and higher fractional shortening measurement at presentation. CONCLUSIONS: Children who survive the first year following their first presentation with significant heart failure from heart muscle disease have a good longer-term outcome although there remains a small attrition rate. FAU - Andrews, Rachel E AU - Andrews RE AD - Cardiothoracic Unit Great Ormond Street Hospital for Children NHS Foundation Trust London UK. FAU - Fenton, Matthew J AU - Fenton MJ AD - Cardiothoracic Unit Great Ormond Street Hospital for Children NHS Foundation Trust London UK. FAU - Dominguez, Troy AU - Dominguez T AD - Cardiothoracic Unit Great Ormond Street Hospital for Children NHS Foundation Trust London UK. FAU - Burch, Michael AU - Burch M AD - Cardiothoracic Unit Great Ormond Street Hospital for Children NHS Foundation Trust London UK. CN - British Congenital Cardiac Association LA - eng PT - Journal Article DEP - 20160124 PL - England TA - ESC Heart Fail JT - ESC heart failure JID - 101669191 PMC - PMC5066798 OTO - NOTNLM OT - Cardiomyopathy OT - Morbidity OT - Mortality OT - Myocarditis OT - Transplantation EDAT- 2016/11/05 06:00 MHDA- 2016/11/05 06:01 PMCR- 2016/01/24 CRDT- 2016/11/05 06:00 PHST- 2015/09/28 00:00 [received] PHST- 2015/12/02 00:00 [revised] PHST- 2015/12/11 00:00 [accepted] PHST- 2016/11/05 06:00 [pubmed] PHST- 2016/11/05 06:01 [medline] PHST- 2016/11/05 06:00 [entrez] PHST- 2016/01/24 00:00 [pmc-release] AID - EHF212082 [pii] AID - 10.1002/ehf2.12082 [doi] PST - ppublish SO - ESC Heart Fail. 2016 Jun;3(2):107-114. doi: 10.1002/ehf2.12082. Epub 2016 Jan 24.