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
ARTICLE HIGHLIGHTS
Research background

Familial dilated cardiomyopathy (FDCM) is a sub-type of non-ischemic cardiomyopathy (NICM) that may lead to end-stage heart failure requiring heart transplantation (HT). This group of patients tends to develop heart failure at earlier age and they are more likely to have less comorbidity, which suggest they may have better outcomes after HT. Although characteristics of FDCM patients with end-stage heart failure have been reported, the outcomes of FDCM patients listed for HT were not described.

Research motivation

As the outcomes of FDCM listed for HT patients were not studied, we used a large database to compare FDCM to ischemic cardiomyopathy (ICM) and NICM patients who are listed for HT. Our results may help to better understand the clinical course of FDCM patients while they are awaiting HT and their outcomes after being transplanted.

Research objectives

The objective of this study was to compare FDCM to ICM and NICM patients who are listed for HT and describe their clinical course while awaiting HT and their post-HT outcomes.

Research methods

We identified patients who are listed for HT using the United Network for Organ Sharing Registry. We divided patients to three groups: ICM, NICM, and FDCM, and compared clinical outcomes of FDCM to ICM and NICM patients who are listed for HT.

Research results

FDCM patients were younger, less likely to be males, more likely to be listed as status 2, less likely to require mechanical support, but more likely to need total artificial heart. While awaiting HT, FDCM patients were less likely to die compared to ICM [HR 0.609 (0.429-0.864)], less likely to be delisted due to deterioration compared to ICM [0.387 (0.248-0.604)] and NICM [0.499 (0.319-0.781)], less likely to die or to be delisted due to deterioration compared ICM [0.501 (0.381-0.659)] and NICM [0.617 (0.468-0.813)], less likely to be delisted due to improvement compared to ICM [0.347 (0.163-0.735)] and NICM [0.277 (0.131-0.588)], and more likely to be transplanted compared to ICM [1.183 (1.076-1.302)] and NICM [1.248 (1.135-1.373)]. After HT, FDCM patients were more likely to have early rejection compared to ICM (FDCM 11.4% vs ICM 8.4%; P < 0.034), but more likely to survive (91%, 88%, and 80%) compared to ICM (89%, 82%, and 75%) at 1, 3, and 5 years, respectively.

Research conclusions

Patients with end-stage heart failure due to FDCM are more likely to be transplanted compared to NICM and ICM. After HT, they are more likely to develop early rejection, but more likely to survive compared to ICM patients.

Research perspectives

This study may help providers in making clinical decisions for patients with end-stage heart failure due to FDCM while waiting and after HT.