Cardiovascular Consequences of Sickle Cell Disease

Salem Bahashwan; Rahaf Mohammad Almuhanna; Maryam Taher Al Hazza; Reem Wajdi Baarma; Abdulrahman Yousif AlNajjar; Faris Sameer Siddiqui; Shouq Ziyad Fatani; Ahmed Barefah; Hatem Alahwal; Abdullah Almohammadi; Osman Radhwi; Alaa S Algazzar; Eman M Mansory

doi:10.2147/JBM.S455564

Cardiovascular Consequences of Sickle Cell Disease

J Blood Med. 2024 May 6:15:207-216. doi: 10.2147/JBM.S455564. eCollection 2024.

Authors

Salem Bahashwan^{1

2}, Rahaf Mohammad Almuhanna³, Maryam Taher Al Hazza¹, Reem Wajdi Baarma¹, Abdulrahman Yousif AlNajjar⁴, Faris Sameer Siddiqui¹, Shouq Ziyad Fatani⁵, Ahmed Barefah^{1

2}, Hatem Alahwal^{1

2}, Abdullah Almohammadi^{1

2}, Osman Radhwi^{1

2}, Alaa S Algazzar⁶, Eman M Mansory^{1

2}

Affiliations

¹ Hematology Department, Faculty of Medicine, King Abdulaziz University, Jeddah, 21589, Saudi Arabia.
² Hematology Research Unit, King Fahd Medical Research Center, King Abdulaziz University, Jeddah, 21589, Saudi Arabia.
³ Emergency Medicine Department, King Faisal Specialist Hospital and Research Center, Jeddah, Saudi Arabia.
⁴ Emergency Medicine Department, King Abdullah Medical Complex, Jeddah, Saudi Arab.
⁵ Physical Medicine and Rehabilitation, Prince Sultan Military Medical City, Riyadh, Saudi Arabia.
⁶ Cardiology Department,Ahmed Maher Teaching hospital, Cairo, Egypt.

Abstract

Background: Sickle cell disease is an inherited blood disorder which can lead to severe complications, particularly in the cardiovascular and respiratory systems, potentially resulting in arrhythmias, pulmonary hypertension (PH), and cardiomegaly. This study aims to investigate the risk of PH and arrhythmias in adult SCD patients.

Methods: Retrospective analysis of medical records from King Abdulaziz University Hospital (KAUH) for patients with SCD aged 15 and above between 2009 and 2021. The study included 517 patients, with echocardiograms and electrocardiograms assessed according to the European Society of Cardiology/the European Respiratory Society (ESC/ERS) guidelines for categorizing PH risk (low, moderate, high) and detecting arrhythmias. Data analysis employed the Statistical Package for the Social Sciences (SPSS), utilizing quantitative and qualitative data representation. Multivariate logistic regression identified independent risk factors with odds ratios at a 95% confidence interval (CI).

Results: Among participants, 50.3% were male, with a total sample average age of 34.45 ± 9.28 years. Results indicated that 1.4% of patients experienced arrhythmias, 3.7% had a moderate PH risk, and 3.3% were classified as high PH risk. Logistic regression revealed significant independent risk factors for PH and arrhythmia in patients with SCD, with chronic kidney disease (CKD) carrying the highest odds (26.4 times higher odds of PH and 15.36 times higher odds of arrhythmias).

Conclusion: Patients with SCD are at risk for developing PH and various arrhythmias but are often underdiagnosed. Key risk factors for PH included CKD, liver cirrhosis, and pre-existing cardiac conditions. Arrhythmias were significantly associated with CKD and pre-existing cardiac conditions. To mitigate these risks, we recommend involving a multidisciplinary healthcare team in the care of adult patients with SCD. Future prospective studies are advised for early detection of PH and arrhythmias in hemoglobinopathy patients, potentially reducing mortality.

Keywords: PH risk; arrhythmia; chronic kidney disease; hemoglobinopathies; pulmonary hypertension; sickle cell disease.