Progression of familial and non-familial dilated cardiomyopathy: long term follow up.
BACKGROUND
It is unknown whether progression of familial idiopathic dilated cardiomyopathy differs from progression in the non-familial form. It has been suggested that familial disease indicates a worse prognosis, and that this should be considered when planning the timing of heart transplantation.


OBJECTIVE
To compare five year survival or time to heart transplantation in an unselected series of patients with dilated cardiomyopathy who had been evaluated for familial v non-familial disease through the echocardiographic investigation of first degree relatives.


METHODS
Medical records were reviewed and questionnaires were mailed to all patients who had previously participated in a family based study of dilated cardiomyopathy. Information was gathered about survival, heart transplantation, and left ventricular ejection fraction (LVEF) measurements. Survival data were censored at the time of cardiac transplantation.


RESULTS
Follow up data were obtained for 99 of 101 patients (69 with non-familial and 30 with familial disease). Five year survival was 55% for non-familial and 51% for familial patients (NS). The main predictor of mortality was an LVEF of < 30%. Familial status did not predict mortality. There was no significant difference in follow up LVEF values between the groups.


CONCLUSIONS
Five year survival is not significantly different in the familial and non-familial forms of dilated cardiomyopathy.
Michels
V V
VV
Department of Medical Genetics, Mayo Clinic/Foundation, Rochester, Minnesota 55905, USA. michels.virginia@mayo.edu
Driscoll
D J
DJ
Miller
F A
FA
Olson
T M
TM
Atkinson
E J
EJ
Olswold
C L
CL
Schaid
D J
DJ
D000293:Adolescent; D000328:Adult; D000368:Aged; D002311:Cardiomyopathy, Dilated / Q000235:genetics / Q000401:mortality*; D002648:Child; D002675:Child, Preschool; D018450:Disease Progression; D004452:Echocardiography; D005260:Female; D005500:Follow-Up Studies; D016027:Heart Transplantation; D006801:Humans; D007223:Infant; D008297:Male; D008875:Middle Aged; D010375:Pedigree; D011379:Prognosis; D012189:Retrospective Studies; D016019:Survival Analysis; D018487:Ventricular Dysfunction, Left / Q000000981:diagnostic imaging / Q000401:mortality