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July 21, 2026HealthcareOpen Access

Cardiac Remodeling Patterns in Pediatric and Adolescent Patients with Sickle Cell Disease and Their Association with the Genotype and Clinical Severity of the Disease: A Systematic Review

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Authors

AMAlam Eldin Musa MustafaUniversity of KordofanNANiemat Mohammed Tahir AliKing Khalid University

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Implication

Systematic review reveals cardiac remodeling's association with genotype and clinical severity in pediatric sickle cell disease.

Key Points

  • This review aims to characterize cardiac remodeling patterns and their associations with genotype and clinical severity in pediatric patients with sickle cell disease.
  • Conducted a systematic review in accordance with PRISMA 2020 guidelines.
  • Retrieved and analyzed 37 studies focused on cardiac remodeling patterns in pediatric and adolescent SCD patients.
  • Included approximately 4253 patients from 12 countries, utilizing various imaging techniques.
  • Left ventricular dilation was observed in 89.2% of studies, indicating a common cardiac issue.
  • Diastolic dysfunction reported in 48.6% of studies, while elevated TRV was noted in 37.8% as a screening marker for pulmonary hypertension.
  • The HbSS genotype correlated with the most severe cardiac changes, with inconsistencies in reporting across mixed-genotype cohorts.

Cite This Study

Mustafa et al. (2026) studied this question.

synapsesocial.com/papers/6a5f0bea86a4235cc1619720https://doi.org/10.3390/healthcare14142180
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Also Consider

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  1. 1Left atrial dysfunction in sickle cell anemia is associated with diffuse myocardial fibrosis, increased right ventricular pressure and reduced exercise capacity2020 · 24 citations
  2. 2Pulmonary Hypertension as a Risk Factor for Death in Patients with Sickle Cell Disease2004 · 1,326 citations
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  4. 4Cardiac manifestations in sickle cell disease varies with patient genotype2018 · 9 citations
  5. 5Sickle cell disease: at the crossroads of pulmonary hypertension and diastolic heart failure2019 · 48 citations