Retrospective Study
Copyright ©The Author(s) 2019. Published by Baishideng Publishing Group Inc. All rights reserved.
World J Cardiol. Jan 26, 2019; 11(1): 38-46
Published online Jan 26, 2019. doi: 10.4330/wjc.v11.i1.38
Contemporary characteristics and outcomes of adults with familial dilated cardiomyopathy listed for heart transplantation
Mohamed Khayata, Sadeer G Al-Kindi, Guilherme H Oliveira
Mohamed Khayata, Sadeer G Al-Kindi, Guilherme H Oliveira, Cardiology Section, Department of Internal Medicine, University Hospitals Cleveland Medical Center, Harrington Heart and Vascular Institute, Cleveland, OH 44106, United States
Author contributions: All authors helped to perform the research; Khayata M and Al-Kindi SG contributed equally to the manuscript, and are co-first authors; Khayata M manuscript writing, drafting conception and design, data analysis; Al-Kindi SG manuscript writing, data analysis; Oliveira GH manuscript writing, drafting conception and design.
Institutional review board statement: Institutional review board approval was not required because only deidentified data sets were used for this analysis.
Informed consent statement: Patients were not required to give informed consent to the study because the analysis used anonymous clinical data.
Conflict-of-interest statement: All authors declare no conflicts-of-interest related to this article.
Data sharing statment: No additional data are available.
Open-Access: This article is an open-access article which was selected by an in-house editor and fully peer-reviewed by external reviewers. It is distributed in accordance with the Creative Commons Attribution Non Commercial (CC BY-NC 4.0) license, which permits others to distribute, remix, adapt, build upon this work non-commercially, and license their derivative works on different terms, provided the original work is properly cited and the use is non-commercial. See: http://creativecommons.org/licenses/by-nc/4.0/
Corresponding author: Guilherme H Oliveira, MD, FACC, Associate Professor, Section of Heart Failure and Transplant, Harrington Heart and Vascular Institute, University Hospitals Cleveland Medical Center, 11100 Euclid Avenue, Cleveland, OH 44106, United States. guilherme.oliveira@uhhospitals.org
Telephone: +1-216-844-8242 Fax: +1-216-844-8954
Received: August 23, 2018
Peer-review started: August 23, 2018
First decision: October 4, 2018
Revised: November 1, 2018
Accepted: January 3, 2019
Article in press: January 4, 2019
Published online: January 26, 2019
Processing time: 156 Days and 21.2 Hours
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; vs ICM 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.

Keywords: Familial dilated cardiomyopathy, End-stage heart failure, Wait list, Transplant, Outcomes

Core tip: Familial dilated cardiomyopathy (FDCM) can lead to end-stage heart failure requiring heart transplantation (HT). There is little contemporary information on progression, circulatory mechanical support use, and HT outcomes of these patients. We aimed to define the characteristics and outcomes of FDCM patients and to compare FDCM to non-ischemic cardiomyopathy (NICM) and ischemic cardiomyopathy (ICM) patients listed for HT. FDCM patients were younger, more frequently listed as status 2, and more likely to be transplanted. There was more frequent rejection among patients with FDCM compared to ICM. Post-transplant survival of FDCM patients was similar to NICM, but superior to ICM patients.