Contemporary characteristics and outcomes of adults with familial dilated cardiomyopathy listed for heart transplantation.

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Abstract

BACKGROUND:

Familial dilated cardiomyopathy (FDCM) account for 20%-30% of non-ischemic cardiomyopathies (NICM). Previous published data showed that some patients with FDCM tend to have rapidly progressive disease; however, five-year mortality was not significantly different in the familial and non-familial forms of NICM with optimal medical therapy.

AIM:

To better define the characteristics and clinical outcomes of FDCM patients listed for heart transplantation (HT).

METHODS:

We queried the United Network for Organ Sharing Registry to identify FDCM patients listed for HT between January 2008 and September 2015 and compared them to NICM and ischemic cardiomyopathy (ICM) patients. We included all patients ≥ 18 years old and we separated patients to three groups: FDCM, NICM and ICM. Chi-square test was used to compare between categorical variables, the t-test was used to compare between continues variables, and Cox-proportional hazards model was used to perform time-dependent survival analyses.

RESULTS:

Of the 24809 adults listed for HT, we identified 677 patients (2.7%) with the diagnosis of FDCM. Compared to patients with NICM and ICM, FDCM patients were younger (FDCM 43.9 ± 13.5 vs NICM 50.9 ± 12.3, P < 0.001, vs ICM 58.5 ± 8.1, P < 0.001), more frequently listed as status 2 (FDCM 35.2% vs NICM 26.5%, P < 0.001), with significantly lower left ventricular assist device (LVAD) utilization (FDCM 18.4% vs NICM 25.1%, P < 0.001; vs ICM 25.6%, P < 0.001), but higher use of total artificial heart (FDCM 1.3% vs NICM 0.6%, P = 0.039; vsICM 0.4%, P = 0.002). Additionally, patients with FDCM were less frequently delisted for clinical deterioration or death and more likely to be transplanted compared to those with NICM [hazard ratio (HR): 0.617, 95% confidence interval (CI): 0.47-0.81; HR: 1.25, 95%CI: 1.14-1.37, respectively], and ICM (HR: 0.5, 95%CI: 0.38-0.66; HR: 1.18, 95%CI: 1.08-1.3, respectively). There was more frequent rejection among patients with FDCM (FDCM 11.4% vs NICM 9.8%, P = 0.28; vs ICM 8.4%, P = 0.034). One, three, and five post-transplant survival of patients with FDCM (91%, 88% and 80%) was similar to those with NICM (91%, 84%, 79%, P = 0.225), but superior to those with ICM (89%, 82%, 75%, P = 0.008), respectively.

CONCLUSION:

End-stage FDCM patients are more likely to be transplanted, more likely to have early rejection, and have similar or higher survival than patients with other cardiomyopathies.