Abnormal ECG Findings Prevalent Among People with Sickle Cell Disease

Erfan Taherifard, MD, MPH

Credit: LinkedIn

A recent systematic review and meta-analysis exhibited a higher prevalence of abnormal electrocardiography (ECG) findings among patients with sickle cell disease (SCD) compared to a population with the disease.¹

Data analysis of 59 studies encompassing nearly 898,000 patients revealed significantly higher odds of these abnormal ECG findings, predominantly nonspecific ST–T changes, left ventricular hypertrophy, T-wave changes, prolonged corrected QT (QTc) interval, and ischemic changes.

“This increased prevalence of these ECG abnormalities underscores the elevated risk of cardiovascular events in SCD patients, emphasizing the significance of risk stratification, close monitoring, and judicious prescription of medications guided by ECG assessments in their management,” wrote the investigative team led by Erfan Taherifard, MD, MPH, Hematology Research Center, Shiraz University of Medical Sciences.

Cardiovascular diseases are one of the leading causes of morbidity and mortality in patients with SCD—complications, including cardiomyopathy, myocardial infarction, and pulmonary hypertension have been increasing in the population with the hereditary hemoglobin (Hb) disorder.² Researchers have stressed the importance of prioritizing cardiovascular evaluations for patients with SCD to benefit their overall health and quality of life.

A noninvasive diagnostic technique, ECG can be cost-effective and easily obtainable for a cardiac structure and function assessment. Prior reported evidence has identified a range of ECG changes in patients with SCD, from rhythm abnormalities to changes in morphology.³ For the current analysis, Taherifard and colleagues sought to systematically review the available literature to determine the patterns of ECG abnormalities in SCD and identify prevalence to provide better management strategies.¹

An initial systematic search was performed using online databases of PubMed, Scopus, Web of Science, Embase, and Google Scholar in February 2023, with an update conducted before study finalization in October 2023. There were no restrictions on study design, provided that the trials reported the findings of standard ECG assessments in patients with SCD. For statistical analysis, four types of effect sizes were used and pooled using the random effects model.

Overall, 62 records in the meta-analysis corresponded to 59 studies involving data on 897,920 patients with SCD. Studies were published between 1945 and 2023, with a setting in the United States comprising the highest portion (42.4%). Upon analysis, investigators found most patients with SCD exhibited various ECG abnormalities (75%; 95% CI, 67 - 81%).

The most common abnormalities were nonspecific ST–T changes (41%), left ventricular hypertrophy (40%), T-wave changes (34%), prolonger correct QT interval (26%), and ischemic changes (25%). Taherifard and colleagues further compared the prevalence of ECG abnormalities between patients with SCD and the control group.

Patients with SCD also exhibited significantly increased odds of having any ECG abnormalities (odds ratio [OR], 17.50; 95% CI, 4.68–65.49). The patient population also exhibited higher odds of right atrial enlargement (OR, 6.09; 95% CI, 1.48–25.09), left ventricular hypertrophy (OR, 3.45; 95% CI, 1.73–6.89), right ventricular hypertrophy (OR, 7.18; 95% CI, 2.28–22.57), biventricular hypertrophy (OR, 10.11; 95% CI, 1.99–51.38), prolonged QTc interval (OR, 5.54; 95% CI, 2.44–12.59), ST depression (OR, 3.34; 95% CI, 1.87–5.97), and T-wave changes (OR, 5.41; 95% CI, 1.43–20.56).

For further analysis, the investigative team assessed the differences in mean values for P-wave duration, PR duration, QRS duration, QTc duration, and QTc dispersion between patients with SCD and controls. Overall, the mean QTc interval was significantly higher among those with SCD (23.51 milliseconds; 95% CI, 16.08–30.94).

Taherifard and colleagues indicated the need for careful consideration when prescribing medications that can potentially further prolong the QTc interval in patients with SCD.

“Therefore, given the relatively high needs of patients with SCD for medication that could potentially prolong the QTc interval, the high prevalence of prolonged QTc interval in this population, and the risk of highly serious complications, we recommend conducting screenings to evaluate these patients for a prolonged QT interval,” investigators wrote.

References

1. _{Taherifard E, Movahed H, Taherifard E, et al. Electrocardiographic abnormalities in patients with sickle cell disease: A systematic review and meta-analysis. Pediatr Blood Cancer. Published online February 13, 2024. doi:10.1002/pbc.30916}
2. _{Gladwin MT. Cardiovascular complications in patients with sickle cell disease. Hematology Am Soc Hematol Educ Program. 2017;2017(1):423-430. doi:10.1182/asheducation-2017.1.423}
3. _{Upadhya B, Ntim W, Brandon Stacey R, et al. Prolongation of QTc intervals and risk of death among patients with sickle cell disease. Eur J Haematol. 2013;91(2):170-178. doi:10.1111/ejh.12127}