Related Experiment Video
Updated: May 20, 2025
Detection of Residual Donor Erythroid Progenitor Cells after Hematopoietic Stem Cell Transplantation for Patients with Hemoglobinopathies
Published on: September 6, 2017
Prevalence and Correlates of Dilated and Non-Dilated Left Ventricular Cardiomyopathy in Transfusion-Dependent
Antonella Meloni1, Laura Pistoia2, Anna Spasiano3
1Bioengineering Unit, Fondazione G. Monasterio CNR-Regione Toscana, 56124 Pisa, Italy.
Insights
Dilated cardiomyopathy (DCM) and non-dilated left ventricular cardiomyopathy (NDLVC) are prevalent in transfusion-dependent β-thalassemia (β-TDT). Detecting these conditions aids in predicting cardiac complications in β-TDT patients.
Area of Science:
- Cardiology
- Hematology
- Medical Imaging
Background:
- Transfusion-dependent β-thalassemia (β-TDT) patients face significant risks of cardiac complications.
- Myocardial iron overload (MIO) is a known complication, but the specific roles of different cardiomyopathy phenotypes require further investigation.
Purpose of the Study:
- To determine the prevalence, clinical features, and prognostic significance of dilated cardiomyopathy (DCM) and non-dilated left ventricular cardiomyopathy (NDLVC) in β-TDT patients.
- To assess the relationship between these cardiomyopathy phenotypes, MIO, myocardial fibrosis, and cardiac outcomes.
Main Methods:
- Retrospective analysis of 415 β-TDT patients undergoing cardiovascular magnetic resonance (CMR).
- CMR was used to quantify MIO, assess biventricular function, and detect myocardial fibrosis.
- Patients were categorized into no overt cardiomyopathy (NOCM), DCM, and NDLVC groups.
Main Results:
- DCM and NDLVC patients showed higher MIO and myocardial fibrosis prevalence compared to NOCM patients.
- Cardiac complications (heart failure, arrhythmias, pulmonary hypertension) occurred in 7.7% of patients during follow-up.
- Both NDLVC and DCM were significantly associated with an increased risk of cardiac complications (HR=4.26 and 8.81, respectively).
Conclusions:
- NDLVC and DCM are significant phenotypes in β-TDT, associated with increased MIO and fibrosis.
- These cardiomyopathy phenotypes are independent predictors of adverse cardiac outcomes in β-TDT.
- Identifying NDLVC and DCM via CMR is crucial for risk stratification and management in β-TDT.
Abstract:
We investigated the prevalence, clinical characteristics, and prognostic role of dilated cardiomyopathy (DCM) and non-dilated left ventricular cardiomyopathy (NDLVC) in patients with transfusion-dependent β-thalassemia (β-TDT). We retrospectively included 415 β-TDT patients who underwent cardiovascular magnetic resonance to quantify myocardial iron overload (MIO) and biventricular function parameters and to detect replacement myocardial fibrosis. Demographic and laboratory parameters were comparable among patients with no overt cardiomyopathy (NOCM; n = 294), DCM (n = 12), and NDLVC (n = 109), while cardiac size and systolic function were significantly different. Compared to NOCM patients, DCM and NDLVC patients had a higher prevalence of MIO and replacement myocardial fibrosis. During a mean follow-up of 57.03 ± 18.01 months, cardiac complications occurred in 32 (7.7%) patients: 15 heart failures, 15 supraventricular arrhythmias, and 2 pulmonary hypertensions. Compared to the NOCM group, both the NDLVC and the DCM groups were associated with a significantly increased risk of cardiac complications (hazard ratio = 4.26 and 8.81, respectively). In the multivariate analysis, the independent predictive factors were age, MIO, and the presence of DCM and NDLVC versus the NOCM phenotype. In β-TDT, the detection of NDLVC and DCM phenotypes may hold value in predicting cardiac outcomes.
More Related Videos
Related Concept Videos
01:26Imbalances in Cardiac Output
CHF can occur due to the failure of either side of the heart. Left-side failure leads to pulmonary congestion—the right side continues to send...
01:17Pathophysiology of Heart Failure