Biatrial and Biventricular Reference Ranges Based on Cardiac Magnetic Resonance in Sickle Cell Disease Patients
Laura Pistoia1,2, Antonella Meloni2,3, Vincenzo Positano2,3
1U.O.C. Ricerca Clinica, Fondazione G. Monasterio CNR-Regione Toscana, 56124 Pisa, Italy.
Insights
Cardiac magnetic resonance (CMR) provides reference ranges for sickle cell disease (SCD) patients, aiding in accurate diagnosis by accounting for anemia-related cardiac adaptations. This helps prevent misdiagnosis of cardiomyopathy in SCD individuals.
Area of Science:
- Cardiology
- Hematology
- Medical Imaging
Background:
- Sickle cell disease (SCD) can cause cardiac complications, but distinguishing them from anemia-related adaptations is challenging.
- Establishing normal cardiac parameters in SCD patients without overt heart damage is crucial for accurate diagnosis.
Purpose of the Study:
- To establish biatrial and biventricular reference ranges using cardiac magnetic resonance (CMR) in adult and pediatric sickle cell disease (SCD) patients without cardiac damage.
- To differentiate between cardiac adaptation due to anemia and true cardiomyopathy in SCD.
Main Methods:
- Compared CMR parameters (cine SSFP sequences) in 48 adult SCD patients (no cardiac involvement) to 96 healthy controls and 96 thalassemia major (TM) patients.
- Analyzed 9 pediatric SCD patients against matched TM and healthy controls.
- Defined cardiac involvement by absence of risk factors, normal ECG, and no myocardial fibrosis or iron on T2* CMR.
Main Results:
- Males in all groups had higher biventricular volumes and mass indexes than females.
- Adult SCD patients showed significantly increased left ventricular (LV) and right ventricular (RV) volumes, mass indexes, and atrial areas compared to healthy controls.
- SCD and TM patients had comparable cardiac parameters, suggesting anemia-related changes dominate over disease-specific effects in this cohort.
Conclusions:
- Established CMR-derived biatrial and biventricular reference ranges for adult SCD patients, stratified by sex.
- These reference ranges are essential for preventing misdiagnosis of cardiomyopathy in SCD by accounting for anemia-induced cardiac remodeling.
- Findings highlight the importance of considering anemia's impact on cardiac structure and function in SCD management.
Background/Objectives:
We aimed to establish biatrial and biventricular reference ranges using cardiac magnetic resonance (CMR) parameters in SCD patients without heart damage.
Methods:
This study compared CMR parameters, quantified by cine SSFP sequences, in 48 adult SCD patients without apparent cardiac involvement (defined by the absence of known risk factors, normal electrocardiogram, and no macroscopic myocardial fibrosis or significant cardiac iron on T2* CMR) to matched cohorts of 96 healthy controls and 96 thalassemia major (TM) patients without cardiac damage. Nine paediatric SCD patients were also analysed and compared to age- and gender-matched groups of nine TM patients and nine healthy subjects.
Results:
In all groups, studied males displayed higher biventricular volumes and mass indexes than females. Male SCD patients showed significantly higher left ventricular (LV) end-diastolic volume index (EDVI), LV end-systolic volume index (ESVI), LV stroke volume index (SVI), cardiac index, LV and right ventricular (RV) mass index, and atrial areas than healthy subjects. Females with SCD exhibited increased LV EDVI, LV SVI, RV mass index, and left atrial area index compared to healthy controls. SCD and TM patients showed comparable biatrial areas and biventricular volumes and function. When compared to TM, SCD males exhibited a larger mass index, while SCD females showed an increased RV mass index. CMR parameters were similar across all paediatric groups.
Conclusions:
By establishing the biatrial and biventricular reference ranges through CMR for adult male and female SCD patients, we aimed to prevent possible misdiagnosis of cardiomyopathy in this population by taking into account cardiac adaptation due to anaemia.
