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Understanding the Correlation Between GLS and Ventricular Arrhythmia in SCA

July 31, 2023

August 2023

Khylia Marshall

Khylia Marshall is a freelance journalist based in Tucson, Arizona.

Sudden death is a common yet unexplained cause of mortality in adults living with sickle cell anemia (SCA), accounting for about 20% of their deaths. Ventricular arrhythmias (VAs) are frequent in people with SCA, and left ventricular (LV) global longitudinal strain (GLS), an echocardiography parameter used to assess LV function, may be used to enhance the stratification of rhythmic risk in people with SCA, according to a study published in Blood.

“This is the first study where we have a systematic and prospective evaluation [of rhythmic disorders in individuals with SCA]. We clearly see that there are particular arrhythmic abnormalities that are associated with cardiac sudden death in non-sickle cell disease (SCD) populations, so why not in SCD?” said study author Thomas d’Humières, MD, PhD, of the Henri Mondor Teaching Hospital in France.

Researchers observed 100 individuals with SCA from November 2018 to March 2022 who had documented or suspected cardiac involvement, were referred to the ambulatory cardiology department, and were subsequently enrolled in the DREPACOEUR registry. Participants’ median age was 46, and 48% were male. Participants underwent simultaneous three-channel 24-hour electrocardiogram monitoring to measure ventricular tachycardia (VT) and transthoracic echocardiography. LV GLS was measured using automated functional imaging. Researchers aimed to determine the prevalence and predictors of VA in individuals with SCA.

VA was observed in 22% of the patient population, “a significant and worrying prevalence,” said Dr. d’Humières. Moreover, of patients with VA, 64% had at least a moderate premature ventricular contraction (PVC) burden (>1000/24h), and 41% exhibited non-sustained VT; however, researchers note these findings must be interpreted cautiously because a high load of PVCs alone may contribute to an LV systolic dysfunction.

Researchers also observed NT-proBNP (N-​terminal [NT]-pro hormone BNP), a known prognosis factor that is higher in patients with VAs. NT-proBNP greater than 160 ng/L, a mortality risk factor in SCA, was observed in more patients with VA (68% vs. 44%; p=0.047), indicating an opportunity to determine arrhythmogenic triggers in people with SCA.

Although researchers found an association between VA and decreased platelet count (p=0.02), they could not apply these data to their stable study population because of the “high prevalence of treatment interfering with thrombocytopoiesis in the study.” They did, however, note that platelet activation is associated with the release of potential arrhythmic factors.

Finally, VA affected men 4.3 times more than women. In the univariate analysis, the prevalence of VA was higher among males (81% vs. 34%; p<0.001), and in the multivariate analysis, male sex was independently associated with VA (p=0.02).

Ultimately, researchers found that alteration of GLS demonstrated good accuracy for identifying patients with VA (area under the curve = 0.76; p=0.02) with an optimal cutoff of -17.5%. In patients with VA, echocardiography revealed a decrease in GLS (-16.0% vs. -18.3%; p<0.001), despite normal LV ejection fraction and no significant increase in LV filling pressures.

“We found that when [GLS] was lower, it was associated with a higher probability of ventricular rhythmic disorders that might be in relation with some early fibrosis in the myocardial tissue,” Dr. d’Humières said. Researchers found that patients with VA showed a worse LV remodeling and a lower E/A ratio, markers that can be hallmarks of myocardial fibrosis.

Researchers recognized the advanced age, high level of organ complications, and small study population as limitations to their pilot study and thus were cautious to extend their data to all individuals living with SCA. They urged future studies to include a larger number of younger, lower-risk patients.

Dr. d’Humières emphasized that hematologists should “be aware of rhythmic disorders, their potential links to sudden cardiac death, and to look for rhythmic disorders when the strain is lower.” Researchers recommend a close collaboration between hematologists and cardiologists who are experienced in the specific cardiac challenges of people living with SCA with the intent to initiate and evaluate preventative anti-arrhythmic strategies or targeted therapies leading to a better prevention of sudden cardiac death.

Any conflicts of interest declared by the authors can be found in the original article.

Reference

d’Humières T, Saba J, Savale L, et al. Determinants of ventricular arrhythmias in sickle cell anemia: towards a better prevention of sudden cardiac death [published online ahead of print, 2023 May 22]. Blood. doi:10.1182/blood.2022019571.

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