Introduction Risk stratification of nonischemic dilated cardiomyopathic (NICM) patients is challenging due to the large heterogeneity in disease phenotype, genetic background, and poorly characterized progression of disease. We postulated that patients with familial NICM (F-NICM, operationally defined as a strong family history or genetically proven) carry a significantly worse prognosis than nonfamilial/idiopathic NICM (I-NICM) patients for the composite endpoint of death, left ventricular assist device (LVAD) placement, or orthotopic heart transplantation (OHT). Methods A total of 127 consecutive patients, 52 F-NICM and 85 I-NICM, with implanted cardiac defibrillators (ICDs) or cardiac resynchronization devices (CRT) were studied. F-NICM patients were identified from the cardiovascular genetics clinic. Patients were followed for a mean of 6.1 +/- 5.1 years for cardiovascular outcomes: death, LVAD, or OHT. Results There were no significant differences regarding incidence of diabetes, hypertension, NYHA functional class, use of guideline directed medical therapy (GDMT), use of CRT, ICD shocks, or years of follow-up. Patients with F-NICM were significantly younger as compared to I-NICM group (45.8 +/- 15.8 years vs. 61.2 +/- 17 years, p=0.001). Mean ejection fraction (EF) was not significantly different between F-NICM and I-NICM; 31.2 +/- 14.5% vs. 31.6 +/- 12.2%. Thirty percent (30%) of F-NICM patients underwent LVAD implant or OHT as compared to 10.4% of I-NICM patients (p=0.008). The composite endpoint of LVAD, OHT, or death was significantly different between F-NICM and I-NICM, occurring in 45.8% vs 21.5% of patients respectively (p=0.004). After multivariate analysis, F-NICM was the only independent predictor of the composite endpoint. The Kaplan-Meier survival estimates for the composite endpoint was significantly lower in F-NICM as compared to I-NICM (Figure 1). Conclusion : In patients with NICM, the family history and/or a positive gene test for cardiomyopathy is a powerful predictor of the composite end point of death, LVAD implantation, or OHT. Risk stratification of nonischemic dilated cardiomyopathic (NICM) patients is challenging due to the large heterogeneity in disease phenotype, genetic background, and poorly characterized progression of disease. We postulated that patients with familial NICM (F-NICM, operationally defined as a strong family history or genetically proven) carry a significantly worse prognosis than nonfamilial/idiopathic NICM (I-NICM) patients for the composite endpoint of death, left ventricular assist device (LVAD) placement, or orthotopic heart transplantation (OHT). A total of 127 consecutive patients, 52 F-NICM and 85 I-NICM, with implanted cardiac defibrillators (ICDs) or cardiac resynchronization devices (CRT) were studied. F-NICM patients were identified from the cardiovascular genetics clinic. Patients were followed for a mean of 6.1 +/- 5.1 years for cardiovascular outcomes: death, LVAD, or OHT. There were no significant differences regarding incidence of diabetes, hypertension, NYHA functional class, use of guideline directed medical therapy (GDMT), use of CRT, ICD shocks, or years of follow-up. Patients with F-NICM were significantly younger as compared to I-NICM group (45.8 +/- 15.8 years vs. 61.2 +/- 17 years, p=0.001). Mean ejection fraction (EF) was not significantly different between F-NICM and I-NICM; 31.2 +/- 14.5% vs. 31.6 +/- 12.2%. Thirty percent (30%) of F-NICM patients underwent LVAD implant or OHT as compared to 10.4% of I-NICM patients (p=0.008). The composite endpoint of LVAD, OHT, or death was significantly different between F-NICM and I-NICM, occurring in 45.8% vs 21.5% of patients respectively (p=0.004). After multivariate analysis, F-NICM was the only independent predictor of the composite endpoint. The Kaplan-Meier survival estimates for the composite endpoint was significantly lower in F-NICM as compared to I-NICM (Figure 1). : In patients with NICM, the family history and/or a positive gene test for cardiomyopathy is a powerful predictor of the composite end point of death, LVAD implantation, or OHT.