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Pulmonary Hypertension In Children With Sickle Cell Disease

M. Khan

Erika Berman Rosenzweig

Robyn Barst

M. T. Lee

Tania Small

Mitchell Cairo

Sujit Sheth


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Conference: 2006 International PHA Conference and Scientific Sessions

Release Date: 06.23.2006

Presentation Type: Abstracts

Muhammad A Khan, MD1, Erika Berman Rosenzweig, MD1, Robyn J Barst, MD1, Margaret T Lee, MD2, Tania Small2, Mitchell S Cairo, MD2   and Sujit S Sheth, MD2.

1Pediatric Cardiology, Children’s Hospital of New York, New York, NY, United States and 2Pediatric Hematology and Oncology, Children’s Hospital of New York, New York, NY, United States.

BACKGROUNDPulmonary hypertension (PH) is increasingly recognized as a significant cause of morbidity and mortality in adult patients with sickle cell disease (SCD), with a prevalence of 20-30% and 18-month mortality rate of 16%. However, its prevalence in the pediatric population is unknown. We sought to determine the prevalence of PH in children and adolescents with SCD and identify potential risk factors associated with this complication.

METHODSWe included children ages 6 to 20 years old followed at our Comprehensive Sickle Cell Center who had doppler echocardiography done within the last two years. Tricuspid regurgitant jet velocity (TRV) was measured by doppler echocardiography. Pulmonary hypertension was defined as a TRV  2.5 m/s. A retrospective chart review was also performed. Data were analyzed to determine the prevalence of PH in this population and to identify clinical and laboratory parameters that may be associated with PH.

RESULTSFifty patients had available echocardiographic studies (Hgb SS =33, Hgb S/B Thalassemia =1, and Hgb SC =16; mean age = 13 + 4 years; males =28, females =22). Eight of 50 patients (16%) had PH (Hgb SS = 6, Hgb S/B Thalassemia =1, Hgb SC = 1; males = 7, female =1). All were teenagers except one (7y/o). PH was associated with laboratory markers of hemolysis (low hemoglobin, increased reticulocyte count, high LDH and high AST) [p < 0.05], but not markers of inflammation (WBC and platelet count), Hgb F%, renal function (creatinine), or iron overload (ferritin, iron, transferrin saturation). History  of  other sickle cell complications (VOC,  acute chest syndrome, priapism), asthma, hydroxyurea treatment, degree of hypoxemia were not associated with PH.

COMMENTS AND CONCLUSIONS: These data suggest that PH associated with SCD begins in childhood. Laboratory markers of increased hemolysis and liver disease are associated with PH. Early screening and diagnosis could enable early treatment with SCD-directed therapy such as chronic transfusion or hydroxyurea, as well as targeted treatment of the PH with the goal of decreasing associated morbidity and mortality in SCD.