title: Severity of mitral regurgitation predicts risk of death or cardiac transplantation in children with idiopathic dilated cardiomyopathy.
abstract: Clinical outcomes among children with idiopathic dilated cardiomyopathy (IDC) are diverse, which makes the decision as to when a patient should be listed for a cardiac transplantation challenging. This study aimed to determine echocardiographic and clinical variables that can help clinicians identify those at highest risk for death or cardiac transplantation. The study was a single-center, retrospective chart review of children with IDC. Patients younger than 18 years with a diagnosis of IDC, as defined by a left ventricular end-diastolic dimension (LVEDD) z-score higher than 2, and fractional shortening of less than 28 % on the initial echocardiogram, were included in the study. Echocardiographic parameters including mitral regurgitation (MR) grade and certain clinical parameters at the time of presentation were assessed. A follow-up echocardiogram was similarly studied. The study included 49 children with IDC. Those who died or underwent cardiac transplantation were grouped as "nonsurvivors" (n = 26). The remaining children who either completely recovered or experienced chronic dilated cardiomyopathy were grouped as "survivors" (n = 23). The median age overall was 1.25 years (range 0.1-17 years). The follow-up echocardiograms of the survivors showed significant improvement in left ventricle size, systolic function, left atrial volume, and MR grade, whereas these parameters did not change in the nonsurvivor group. The use of inotropic medications at initial presentation was an independent predictor of death or cardiac transplantation (p < 0.05). The presence of moderate to severe MR at diagnosis also was predictive of a worse outcome.
authors.affiliation: Division of Pediatric Cardiology, The Carmen and Ann Adams Department of Pediatrics, Children's Hospital of Michigan, Wayne State University School of Medicine, 3901 Beaubien Boulevard, Detroit, MI, 48201, USA.
authors.forename: Amit
authors.initials: A
authors.lastname: Patange
authors.forename: Ronald
authors.initials: R
authors.lastname: Thomas
authors.forename: Robert D
authors.initials: RD
authors.lastname: Ross
mesh_terms: D000293:Adolescent; D002311:Cardiomyopathy, Dilated / Q000150:complications* / Q000401:mortality / Q000601:surgery; D002648:Child; D002675:Child, Preschool; D016757:Death, Sudden, Cardiac / Q000453:epidemiology* / Q000209:etiology; D004452:Echocardiography; D005260:Female; D005500:Follow-Up Studies; D005544:Forecasting; D016027:Heart Transplantation; D006801:Humans; D015994:Incidence; D007223:Infant; D007231:Infant, Newborn; D019028:Magnetic Resonance Imaging, Cine; D008297:Male; D008944:Mitral Valve Insufficiency / Q000150:complications / Q000175:diagnosis* / Q000601:surgery; D011379:Prognosis; D012189:Retrospective Studies; D012307:Risk Factors; D012720:Severity of Illness Index; D015996:Survival Rate / Q000639:trends; D014481:United States / Q000453:epidemiology
references.citation: Circ Heart Fail. 2010 Nov;3(6):689-97
references.pmid: 20833772
references.citation: J Am Coll Cardiol. 2000 Nov 15;36(6):1920-6
references.pmid: 11092665
references.citation: Circulation. 2012 Aug 28;126(9):1079-86
references.pmid: 22800850
references.citation: J Vet Cardiol. 2012 Mar;14(1):73-92
references.pmid: 22386719
references.citation: Arq Bras Cardiol. 2004 Jun;82(6):505-14
references.pmid: 15257368
references.citation: Circulation. 2006 Dec 12;114(24):2671-8
references.pmid: 17116768
references.citation: Chest. 1978 Jul;74(1):59-65
references.pmid: 668437
references.citation: Eur J Echocardiogr. 2008 May;9(3):351-5
references.pmid: 17658300
references.citation: Prog Pediatr Cardiol. 2007 Sep 1;23(1):25-32
references.pmid: 19701490
references.citation: Clin Cardiol. 2008 Sep;31(9):424-30
references.pmid: 18781602
references.citation: Circulation. 1994 Jun;89(6):2582-9
references.pmid: 8205668
references.citation: Eur Heart J. 1996 Aug;17(8):1233-8
references.pmid: 8869865
references.citation: J Am Soc Echocardiogr. 2003 Jul;16(7):777-802
references.pmid: 12835667
references.citation: JAMA. 2006 Oct 18;296(15):1867-76
references.pmid: 17047217
references.citation: Ann Thorac Surg. 2008 Mar;85(3):932-9
references.pmid: 18291174
references.citation: Cardiol Young. 2007 Apr;17(2):175-84
references.pmid: 17244382
references.citation: Am J Cardiol. 2011 May 15;107(10):1517-21
references.pmid: 21377646
references.citation: J Am Coll Cardiol. 1985 Nov;6(5):1126-31
references.pmid: 4045036