Pritish Mondal1, Bryan Stefek2, Ankita Sinharoy3, Binu-John Sankoorikal4, Mutasim Abu-Hasan5, Vincent Aluquin2. 1. Division of Pediatric Pulmonology and Sleep Medicine, Department of Pediatrics, Penn State College of Medicine, Hershey, PA, USA. pmondal@pennstatehealth.psu.edu. 2. Division of Pediatric Cardiology, Department of Pediatrics, Penn State College of Medicine, Hershey, PA, USA. 3. Department of Public Health, Penn State College of Medicine, Hershey, PA, USA. 4. Division of Pediatric Pulmonology and Sleep Medicine, Department of Pediatrics, Penn State College of Medicine, Hershey, PA, USA. 5. Division of Pediatric Pulmonology, Department of Pediatrics, University of Florida, Gainesville, FL, USA.
Abstract
BACKGROUND: Pulmonary hypertension (PH) is multifactorial in origin and may develop early in children with sickle cell disease (C-SCD). Potential etiologies are hemolysis-induced endothelial dysfunction, left ventricular (LV) dysfunction, and chronic hypoxia. Nocturnal hypoxia (NH) in C-SCD is known to be a sequela of obstructive sleep apnea (OSA). The primary objective of this study is to correlate polysomnographic evidence NH with echocardiographic measures of PH in C-SCD. METHODS: We performed a retrospective chart review of 20 C-SCD (Hemoglobin SS), who had polysomnography and echocardiogram performed within a narrow time interval, and 31% of them had pre-existing cardiac conditions. Tricuspid regurgitant jet velocity (TRJV) ≥ 2.5 m/s was considered as an indicator of PH. RESULTS: Twenty-five percent of the subjects had NH. Forty percent of C-SCD, predominantly male, had evidence of PH based on an elevated TRJV. Children with NH compared to non-NH had significantly worse baseline hypoxemia (p < 0.001), higher TRJV (p = 0.005), and higher LV end-diastolic diameters (p = 0.009). The severity of NH was influenced by OSA. However, PH was not associated with OSA or duration of hydroxyurea therapy. CONCLUSION: Our study indicates that NH is associated with PH in C-SCD, and that screening for NH may help to identify C-SCD with higher morbidity risk.
BACKGROUND: Pulmonary hypertension (PH) is multifactorial in origin and may develop early in children with sickle cell disease (C-SCD). Potential etiologies are hemolysis-induced endothelial dysfunction, left ventricular (LV) dysfunction, and chronic hypoxia. Nocturnal hypoxia (NH) in C-SCD is known to be a sequela of obstructive sleep apnea (OSA). The primary objective of this study is to correlate polysomnographic evidence NH with echocardiographic measures of PH in C-SCD. METHODS: We performed a retrospective chart review of 20 C-SCD (Hemoglobin SS), who had polysomnography and echocardiogram performed within a narrow time interval, and 31% of them had pre-existing cardiac conditions. Tricuspid regurgitant jet velocity (TRJV) ≥ 2.5 m/s was considered as an indicator of PH. RESULTS: Twenty-five percent of the subjects had NH. Forty percent of C-SCD, predominantly male, had evidence of PH based on an elevated TRJV. Children with NH compared to non-NH had significantly worse baseline hypoxemia (p < 0.001), higher TRJV (p = 0.005), and higher LV end-diastolic diameters (p = 0.009). The severity of NH was influenced by OSA. However, PH was not associated with OSA or duration of hydroxyurea therapy. CONCLUSION: Our study indicates that NH is associated with PH in C-SCD, and that screening for NH may help to identify C-SCD with higher morbidity risk.
Authors: Panae Noomuna; Mary Risinger; Sitong Zhou; Katie Seu; Yuncheng Man; Ran An; Daniel A Sheik; Jiandi Wan; Jane A Little; Umut A Gurkan; Francesco M Turrini; Theodosia Kalfa; Philip S Low Journal: Br J Haematol Date: 2020-04-28 Impact factor: 6.998
Authors: Ilaria Liguoro; Michele Arigliani; Bethany Singh; Lisa Van Geyzel; Subarna Chakravorty; Cara Bossley; Maria Pelidis; David Rees; Baba P D Inusa; Atul Gupta Journal: ERJ Open Res Date: 2020-10-26