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Published on: May 11, 2015
Pulmonary hypertension in children and adolescents with sickle cell disease
O C Onyekwere1, A Campbell, M Teshome
1Center for Sickle Cell Disease, Howard University, Washington, DC, USA. oonyekwere@howard.edu
Insights
Pulmonary hypertension (PHTN) affects nearly half of pediatric sickle cell disease (SCD) patients, with over 11% having significant PHTN. This complication is linked to hemolysis and the Hb-SS phenotype in children.
Area of Science:
- Pediatric Hematology
- Cardiology
- Pulmonology
Background:
- Pulmonary hypertension (PHTN) is common in adult sickle cell disease (SCD) patients.
- The prevalence and characteristics of PHTN in pediatric SCD are largely unknown.
- Pulmonary complications may increase PHTN risk in children with SCD.
Purpose of the Study:
- To determine the prevalence of PHTN in children and adolescents with SCD.
- To investigate the association between pulmonary disease history and PHTN in pediatric SCD.
- To explore correlations between PHTN and hemolysis markers in pediatric SCD.
Main Methods:
- Screening of 52 pediatric SCD patients for PHTN using tricuspid regurgitant jet velocity (TRV).
- PHTN defined as TRV ≥ 2.5 m/s; significant PHTN as TRV ≥ 3.0 m/s.
- Analysis of associations with pulmonary disease history, hemolysis markers (LDH, bilirubin, hemoglobin, hematocrit), and Hb-SS phenotype.
Main Results:
- Nearly half (46.15%) of pediatric SCD patients had elevated pulmonary artery pressures (TRV ≥ 2.5 m/s).
- 11.5% of patients had significant PHTN (TRV ≥ 3.0 m/s).
- PHTN showed a trend towards association with pulmonary disease (p=0.0795) and was correlated with hemolysis markers and Hb-SS phenotype.
Conclusions:
- PHTN is prevalent in pediatric SCD patients, similar to adults.
- Hemolysis and Hb-SS phenotype are associated with PHTN in this population.
- Further research is needed to elucidate PHTN mechanisms in pediatric SCD.
Abstract:
The prevalence of pulmonary hypertension (PHTN) in the pediatric sickle cell disease (SCD) population is not known despite its high prevalence in adult patients. Our hypothesis was that increased pulmonary artery pressures (PAPs) would be found in SCD children and adolescents, especially those with a history of pulmonary complications: acute chest syndrome, obstructive sleep apnea, asthma, and reactive airway disease. Fifty-two SCD children, 23 of whom had underlying pulmonary disease, were screened for PHTN, which was defined as a tricuspid regurgitant jet velocity (TRV) of at least 2.5 m/s. Twenty-four (46.15%) SCD patients had increased PAP (i.e., TRV > or =2.5 m/s), and 6 (11.5%) had significant PHTN (i.e., TRV > or =3.0 m/s). Pulmonary disease was marginally associated with PHTN (odds ratio 2.80 and confidence interval 0.88 to 8.86; p = 0.0795). As in adult SCD patients with PHTN, this complication was correlated with the degree of hemolysis as manifested by significantly higher lactate dehydrogenase and bilirubin, lower hemoglobin and hematocrit levels, and a strong association with Hb-SS phenotype. However, after statistical adjustment for age and sex, increased serum LDH was not associated with the development of PHTN. Further studies are needed to clarify the prevalence and mechanisms of PHTN in pediatric and adolescent patients with SCD.
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