PMID- 20525703
OWN - NLM
STAT- MEDLINE
DCOM- 20110111
LR  - 20110608
IS  - 1879-0844 (Electronic)
IS  - 1388-9842 (Linking)
VI  - 12
IP  - 9
DP  - 2010 Sep
TI  - Prognostic impact of familial screening in dilated cardiomyopathy.
PG  - 922-7
LID - 10.1093/eurjhf/hfq093 [doi]
AB  - AIMS: Familial screening of patients with dilated cardiomyopathy (DCM) allows an 
      early diagnosis of the disease in family members. It is unclear if familial forms 
      (FDC) have a different long-term outcome compared with sporadic DCM. Our aim was 
      to compare the long-term prognosis of FDC and sporadic forms in order to assess 
      the role of familial screening. METHODS AND RESULTS: Between 1988 and 2007, 637 
      DCM patients were consecutively enrolled. Of these, 130 patients (20.4%) had FDC, 
      including 82 (12.9%) probands and 48 (7.5%) non-proband FDC patients (NP-FDC), 
      identified by family screening. We compared the 48 NP-FDC patients with a sample 
      of 96 patients with sporadic DCM, who were randomly matched by year of enrolment 
      in a 2:1 ratio. At enrolment the NP-FDC patients were younger (40 +/- 16 vs. 48 
      +/- 13 years, P = 0.002), less symptomatic [New York Heart Association, (NYHA) 
      III-IV: 8 vs. 28%, P = 0.006], had higher left ventricular ejection fraction (35 
      +/- 10 vs. 30 +/- 9%, P = 0.005) and were less intensively treated with 
      evidence-based drugs than the sporadic DCM patients. Survival free from heart 
      transplant at 2, 5 and 10 years was 93, 91 and 82%, respectively, in NP-FDC 
      patients compared with 86, 76 and 62% in sporadic forms (P = 0.04). After 
      stratification for NYHA classes, no difference in survival was observed between 
      sporadic and NP-FDC patients. CONCLUSION: Our study demonstrates that family 
      screening can effectively identify DCM patients at an earlier stage of disease 
      and can improve survival. The possibility of changing the prognosis of DCM needs 
      to be verified in patients intensively treated with tailored medical treatment. 
      Family screening should be recommended for all DCM patients.
FAU - Moretti, Michele
AU  - Moretti M
AD  - Cardiovascular Department, Azienda Ospedaliera Ospedali Riuniti and University of 
      Trieste, Trieste, Italy. michele.moretti@gmail.com
FAU - Merlo, Marco
AU  - Merlo M
FAU - Barbati, Giulia
AU  - Barbati G
FAU - Di Lenarda, Andrea
AU  - Di Lenarda A
FAU - Brun, Francesca
AU  - Brun F
FAU - Pinamonti, Bruno
AU  - Pinamonti B
FAU - Gregori, Dario
AU  - Gregori D
FAU - Mestroni, Luisa
AU  - Mestroni L
FAU - Sinagra, Gianfranco
AU  - Sinagra G
LA  - eng
PT  - Comparative Study
PT  - Journal Article
PT  - Research Support, Non-U.S. Gov't
DEP - 20100604
PL  - England
TA  - Eur J Heart Fail
JT  - European journal of heart failure
JID - 100887595
SB  - IM
MH  - Adult
MH  - Cardiomyopathy, Dilated/*diagnosis/epidemiology/genetics
MH  - Early Diagnosis
MH  - *Family
MH  - Female
MH  - Follow-Up Studies
MH  - Humans
MH  - Italy/epidemiology
MH  - Male
MH  - Mass Screening/*methods
MH  - Middle Aged
MH  - Prevalence
MH  - Prognosis
MH  - Retrospective Studies
MH  - Survival Rate/trends
EDAT- 2010/06/08 06:00
MHDA- 2011/01/12 06:00
CRDT- 2010/06/08 06:00
PHST- 2010/06/08 06:00 [entrez]
PHST- 2010/06/08 06:00 [pubmed]
PHST- 2011/01/12 06:00 [medline]
AID - hfq093 [pii]
AID - 10.1093/eurjhf/hfq093 [doi]
PST - ppublish
SO  - Eur J Heart Fail. 2010 Sep;12(9):922-7. doi: 10.1093/eurjhf/hfq093. Epub 2010 Jun 
      4.