title: Periodic rescreening is indicated for family members at risk of developing familial dilated cardiomyopathy.
abstract: OBJECTIVE
  This study evaluated the role of clinical rescreening of family members at risk for familial dilated cardiomyopathy (FDC).
  
  
  BACKGROUND
  Familial dilated cardiomyopathy is a genetic cardiomyopathy that usually is transmitted in an autosomal dominant pattern and may underlie from one-quarter to one-half of idiopathic dilated cardiomyopathy (IDC) diagnoses. Thus, FDC may present with advanced heart failure (HF) or sudden cardiac death (SCD). Because FDC may respond to medical intervention, we have previously recommended that screening of first-degree relatives (parents, siblings, children) of patients diagnosed with IDC be undertaken to rule out FDC, and that with a diagnosis of FDC in the kindred, unaffected but at-risk family members be rescreened every three to five years. METHODS; Follow-up screening (history, examination, electrocardiogram, echocardiography) of a large family with FDC was performed six years after initial screening. Of 68 family members who underwent rescreening, two (one with left ventricular enlargement only, one with a left bundle branch block) presented with advanced HF and SCD, respectively. Two additional subjects, asymptomatic at initial screening, were also affected with FDC at follow-up.
  
  
  CONCLUSIONS
  Considerable vigilance for disease presentation and progression is indicated in at-risk members of a kindred with FDC, especially those with incipient FDC.
authors: Department of Medicine/Cardiology, Oregon Health and Science University, Portland, Oregon 97201, USA.
authors: Kathy A
authors: KA
authors: Crispell
authors: Emily L
authors: EL
authors: Hanson
authors: Kelly
authors: K
authors: Coates
authors: Warren
authors: W
authors: Toy
authors: Ray E
authors: RE
authors: Hershberger
mesh_terms: D000293:Adolescent; D000328:Adult; D000368:Aged; D002311:Cardiomyopathy, Dilated / Q000175:diagnosis* / Q000235:genetics / Q000503:physiopathology; D002648:Child; D002675:Child, Preschool; D004452:Echocardiography; D004562:Electrocardiography; D005190:Family; D005260:Female; D006801:Humans; D007223:Infant; D008297:Male; D008875:Middle Aged; D010375:Pedigree; D010641:Phenotype; D012307:Risk Factors; D018487:Ventricular Dysfunction, Left / Q000000981:diagnostic imaging*