Dual-energy computed tomography to detect early pulmonary vascular changes in children with sickle cell disease: a pilot study

Raphael Joye; Julie Wacker; Duy-Anh Nguyen; Anne-Lise Hachulla; Albane B R Maggio; Laurent Cimasoni; Frederic Lador; Marc Ansari; Maurice Beghetti

doi:10.3389/fped.2023.1221977

Dual-energy computed tomography to detect early pulmonary vascular changes in children with sickle cell disease: a pilot study

Front Pediatr. 2023 Aug 30:11:1221977. doi: 10.3389/fped.2023.1221977. eCollection 2023.

Authors

Raphael Joye^{1

2}, Julie Wacker^{1

2}, Duy-Anh Nguyen¹, Anne-Lise Hachulla^{2

3}, Albane B R Maggio⁴, Laurent Cimasoni⁵, Frederic Lador^{2

6}, Marc Ansari^{5

7}, Maurice Beghetti^{1

2}

Affiliations

¹ Pediatric Cardiology Unit, Department of Woman, Child, and Adolescent Medicine, Geneva University Hospitals, Geneva, Switzerland.
² Pulmonary Hypertension Program, Geneva University Hospital, Geneva, Switzerland.
³ Division of Radiology, Diagnostic Department, Geneva University Hospitals, Geneva, Switzerland.
⁴ Health and Movement Consultation, Department of Woman, Child, and Adolescent Medicine, Geneva University Hospitals, Geneva, Switzerland.
⁵ Division of Pediatric Oncology and Hematology, Department of Women, Child and Adolescent, University Geneva Hospitals, Geneva, Switzerland.
⁶ Division of Pneumology, Geneva University Hospitals, Geneva, Switzerland.
⁷ Cansearch Research Platform for Pediatric Oncology and Hematology, Faculty of Medicine, Department of Pediatrics, Gynecology and Obstetrics, University of Geneva, Geneva, Switzerland.

Abstract

Introduction: Pulmonary hypertension (PH) is a rare but fatal complication of sickle cell disease (SCD) that is possibly reversible if treated early. Dual-energy computed tomography (DECT) is a valuable tool for diagnosing PH. We attempted to determine if DECT can detect early signs of PH in children with SCD.

Methods: This prospective observational pilot study was conducted at the Geneva University Hospitals and was approved by the local human ethics committee (CCER 2019-01975). A written informed consent was obtained from the patients and/or their legal guardian. Eight children (consisting of five girls and three boys) with homozygous SCD were included in the study. They underwent full cardiological workup using transthoracic echocardiography (TTE) and cardiopulmonary exercise test (CPET), as well as DECT.

Results: The median age of the children was 11 years old (range 8-12). All patients exhibited a normal biventricular systo-diastolic function using the TTE. The median tricuspid regurgitant jet velocity value was 2.24 m/s (range 1.96-2.98). Four children were found to have signs of vasculopathy detected on DECT. Of them, two had abnormal screening test results. They both had an increased VE/VCO₂ slope during CPET and an increased TVR of >2.5 m/s on TTE.

Conclusion: DECT is capable of identifying early signs of pulmonary vascular disease in children with SCD. Further studies are needed to understand the correlation between DECT abnormalities and hemodynamic pulmonary circulation better.

Keywords: children; dual-energy computed tomography; pulmonary hypertension; screening; sickle cell disease.

Grants and funding

This study was funded by Cansearch Foundation and open-access funding by the University of Geneva.