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Lack of Bridge to Recovery in Pediatric Dilated Cardiomyopathy With Left Ventricular Noncompaction
Moyu Hasegawa1, Masaki Taira1, Yuji Tominaga1
1Department of Cardiovascular Surgery, Osaka University Graduate School of Medicine, Yamadaoka, Osaka, Japan.
Insights
Pediatric patients with dilated cardiomyopathy and left ventricular noncompaction had lower recovery rates using the Berlin Heart EXCOR device. Myocardial fibrosis was higher, and cardiac function did not improve in this group.
Area of Science:
- Pediatric Cardiology
- Cardiovascular Surgery
- Histopathology
Background:
- Assessing the Berlin Heart EXCOR as a bridge to recovery in pediatric dilated cardiomyopathy.
- Investigating the impact of left ventricular noncompaction phenotype on recovery.
- Examining histologic characteristics in affected children.
Purpose of the Study:
- To evaluate the efficacy of the Berlin Heart EXCOR in pediatric patients with dilated cardiomyopathy.
- To determine if the presence of left ventricular noncompaction affects cardiac recovery.
- To compare histologic features and left ventricular function between patient groups.
Main Methods:
- Retrospective analysis of 17 pediatric patients with dilated cardiomyopathy treated with Berlin Heart EXCOR (2013-2020).
- Classification into dilated cardiomyopathy (DCM) group and DCM with left ventricular noncompaction (DCM-LVNC) phenotype group.
- Histologic examination of myocardium and comparison of left ventricular function pre- and post-implantation.
Main Results:
- No cardiac recovery observed in the DCM-LVNC group.
- 55% of patients in the DCM group achieved recovery and device explantation.
- Significantly higher myocardial fibrosis in the DCM-LVNC group (P < .05).
- Significant improvement in ejection fraction and end-diastolic diameter in the DCM group (P < .001), but not in the DCM-LVNC group.
Conclusions:
- The left ventricular noncompaction phenotype may hinder cardiac recovery in pediatric patients with dilated cardiomyopathy supported by the Berlin Heart EXCOR.
- Histologic differences, specifically increased fibrosis, may contribute to poorer outcomes in the DCM-LVNC group.
Background:
This study assessed the possibility of a bridge to recovery using the Berlin Heart EXCOR and the histologic characteristics of pediatric patients with dilated cardiomyopathy accompanied by a left ventricular noncompaction phenotype.
Methods:
Of the 17 pediatric patients with dilated cardiomyopathy who underwent Berlin Heart EXCOR implantation between 2013 and 2020, 6 were diagnosed with left ventricular noncompaction association. The patients were classified into 2 groups: the dilated cardiomyopathy group and dilated cardiomyopathy with the left ventricular noncompaction phenotype group. The histologic characteristics of the left ventricular myocardium and left ventricular function after Berlin Heart EXCOR implantation were compared.
Results:
Cardiac recovery was not observed in the dilated cardiomyopathy with left ventricular noncompaction group. In contrast, 6 patients (55%) in the dilated cardiomyopathy group achieved cardiac recovery, and the Berlin Heart EXCOR was explanted. The degree of myocardial fibrosis was significantly higher in the dilated cardiomyopathy with the left ventricular noncompaction phenotype group than in the dilated cardiomyopathy group (P < .05). The final follow-up left ventricular ejection fraction and end-diastolic diameter during Berlin Heart EXCOR support improved significantly compared with the preimplantation variables in the dilated cardiomyopathy group (both P < .001); the dilated cardiomyopathy with left ventricular noncompaction phenotype group showed no improvements (P = .84 and P = .37, respectively).
Conclusions:
The left ventricular noncompaction phenotype associated with dilated cardiomyopathy may adversely affect the rate of cardiac recovery with Berlin Heart EXCOR.
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