Sickle Cell Disease with Venous Thromboembolism Linked to Higher Mortality

Credit: BU Neurology

A new study found patients with sickle cell disease (SCD) and venous thromboembolism (VTE) have increased morality, as well as longitudinal worsening of left ventricular diastolic and right-sided cardiac function when assessing for morality risk factors.¹

About 11 – 12% of SCD patients develop VTE by age 40. The condition increases mortality risk by 2- or 3-fold. A 2013 study examined the prevalence and risk factors for VTE in patients with SCD, and during the study, 30 patients died (7.4%).² The investigators found non-catheter-related VTE was independently linked to death.

Another study in 2018 looked at hospital mortality and hospital stay in patients with SCD and VTE.³ The investigators used Healthcare Cost and Utilization Project’s National Inpatient Sample data from 1999 – 2014, and over the 15 years, 217,791 SCD patients were admitted, 7896 of them having VTE. The average mortality rate for patients with SCD and VTE was 3.08% (P <.0001) while for patients with SCD alone it was 0.27%.

“Patients with SCD and VTE experience significant morbidity, mortality, prolonged hospitalization and increased cost associated with this complication of the disease as was observed in this study,” the investigators of the 2018 study wrote, led by Michael Rainone, MBBCH, of Mount Sinai St. Luke's and Mount Sinai West Hospitals in New York.

A new study, led by Matthew Spring, MD, from the department of neurology at Boston University, sought to identify mortality risk factors in patients with SCD a transthoracic echocardiography.¹ The team specifically wanted to examine longitudinal changes in left- and right-sided cardiac function and identify the impact of VTE in this scenario.

The team conducted a retrospective study of 402 patients with SCD, aged ≥18 years old. The study, which was presented at the American Society of Hematology (ASH) 2023, had 2 groups—the first group had patients with both SCD and venous thromboembolism, and the second group had patients with just SCD patients.

The investigators collected data on demographics, clinical history, laboratory data, and diagnosis evaluation from medical record, as well as echocardiographic data with coding of left ventricular and right ventricular systolic and diastolic function.

Over half (62%) had HbSS/HbSβ0, 56% were female, and 19% had a prior VTE. In the VTE group, it was more common to have prior history of acute chest syndrome, stroke, surgical splenectomy, and avascular necrosis than the non-VTE group (P <.01).

Morality was significantly higher in the VTE group (13%) than the non-VTE group (6%) (P = .04). After the 5-year echocardiography follow-up, the team observed significant declines longitudinally in medial and lateral e’ velocity and the E/A ratio, demonstrating the worsening of left ventricular diastolic dysfunction. Meanwhile, the right side also worsened with a significantly increased tricuspid regurgitant jet velocity and right ventricular base dimension. The baseline tricuspid regurgitant jet velocity was higher in the VTE group than in the non-VTE group.

In the non-VTE group, the investigators observed significant increases in baseline tricuspid regurgitant jet velocity and right ventricular and decreases in left atrial ejection fraction, medial and lateral e’, and E/A ratio.

“Our findings need to be confirmed prospectively but suggest the importance of a deeper evaluation of echocardiography to better understand the progression of SCD related cardiac dysfunction and pulmonary hypertension,” the investigators wrote.

References

1. ^{3876 Longitudinal Worsening of Left and Right Sided Cardiac Function in Patients with Sickle Cell Disease. American Society of Hematology. December 11, 2023. https://ash.confex.com/ash/2023/webprogram/Paper182118.html. Accessed December 7, 2023.}
2. ^{Naik RP, Streiff MB, Haywood C Jr, Nelson JA, Lanzkron S. Venous thromboembolism in adults with sickle cell disease: a serious and under-recognized complication. Am J Med. 2013;126(5):443-449. doi:10.1016/j.amjmed.2012.12.016}
3. ^{Rainone, M. Perimbeti, S, Shrivastav, R, et al. Sickle Cell Disease and Venous Thromboembolism: Hospital Mortality, Length of Stay and Cost. ASH Publications. November 28, 2018. https://ashpublications.org/blood/article/132/Supplement%201/2378/264356/Sickle-Cell-Disease-and-Venous-Thromboembolism. Accessed December 7, 2023.}