We postulated that familial idiopathic dilated cardiomyopathy (F-IDC) is associated
with a worse prognosis than nonfamilial IDC (nonF-IDC). Patients with F-IDC had either a strong family history and/or proved genetic mutations.
We studied long-term prognosis (mean follow-up: 6.1 ± 4.1 years) of 162 patients with
IDC (age: 55.5 ± 17.9 years, men: 57.8%, 50% F-IDC) with an implantable cardioverter-defibrillator
or cardiac resynchronization therapy. The primary end point was a composite of death,
left ventricular (LV) assist device implant, or heart transplantation. The secondary
end point was a ventricular arrhythmia event. There was no significant difference
in the prevalence of diabetes, hypertension, New York Heart Association class, medical
therapy, and years of follow-up between the F-IDC and nonF-IDC groups. Patients with
F-IDC were younger than patients with nonF-IDC (49.1 ± 17.0 years vs 61.6 ± 16.5 years,
p <0.001). Mean LV ejection fraction was significantly lower in F-IDC group than in
the nonF-IDC group (26 ± 12% vs 31 ± 12%, p = 0.022). The primary end point was achieved
in 54 patients in F-IDC group (66.7%) versus 19 in the nonF-IDC group (23.5%) (p <0.001).
The Kaplan–Meier survival estimates for the composite end point and for ventricular
arrhythmia were significantly lower in the F-IDC versus nonF-IDC (log-rank p ≤0.001
and 0.04, respectively). F-IDC was the only multivariable predictor of the primary
composite end point (hazard ratio 3.419 [95% confidence interval 1.845 to 6.334],
p <0.001). The likelihood of LV remodeling manifested by LV ejection fraction improvement
(≥10%) was significantly lower in F-IDC than nonF-IDC (27.1% vs 44.8%, p = 0.042).
In conclusion, F-IDC is a predictor of mortality, need for LV assist device, or heart
transplantation. F-IDC is associated with significantly lower event-free survival
for primary end point and ventricular arrhythmia than nonF-IDC. F-IDC has significantly
lower likelihood of LV reverse remodeling than nonF-IDC.
Abbreviations:
F-IDC (Familial idiopathic dilated cardiomyopathy), LVAD (Left ventricular assist device), NonF-IDC (Nonfamilial idiopathic dilated cardiomyopathy)To read this article in full you will need to make a payment
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Article Info
Publication History
Published online: July 28, 2022
Received in revised form:
May 23,
2022
Received:
February 28,
2022
Publication stage
In Press Journal Pre-ProofIdentification
Copyright
© 2022 Elsevier Inc. All rights reserved.
