{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,10,4]],"date-time":"2026-10-04T08:08:23Z","timestamp":1791101303041,"version":"4.1.0"},"reference-count":35,"publisher":"American Academy of Pediatrics (AAP)","issue":"4","content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":[],"published-print":{"date-parts":[[2008,4,1]]},"abstract":"<jats:p>OBJECTIVES. The objectives of this study were (1) to determine the prevalence and risk factors of elevated pulmonary artery pressures in children with homozygous SS or S\u03b2\u00b0 thalassemia using Doppler echocardiography and (2) to determine a correlation between abnormal transcranial Doppler examinations and elevated pulmonary artery pressures.<\/jats:p>\n                  <jats:p>METHODS. Screening echocardiograms were prospectively performed during an annual comprehensive clinic visit on children who were older than 6 years and had homozygous SS or S\u03b2\u00b0 thalassemia. Detailed history, examination, and laboratory tests were done, and transcranial Doppler examinations were obtained in children 2 to 14 years of age. Pulmonary hypertension was defined as pulmonary artery systolic pressure of at least 30 mmHg corresponding to a peak tricuspid regurgitant jet velocity of \u22652.5 m\/second. Mild pulmonary hypertension was defined as tricuspid regurgitant jet velocity \u22652.5 to 2.9 m\/second. Moderate pulmonary hypertension was defined as tricuspid regurgitant jet velocity \u22653 m\/second. Patients with pulmonary stenosis or right outflow obstruction were excluded. Characteristics were compared between patients with mild, moderate, and no pulmonary hypertension using 1-way analysis of variance for continuous variable and Fisher's exact test for categorical variables.<\/jats:p>\n                  <jats:p>RESULTS. Of the 75 patients who had homozygous SS\/S\u03b2\u00b0 thalassemia and were older than 6 years, echocardiograms were obtained for 62 (82.6%). Thirty percent (19 of 62) of patients had elevated tricuspid regurgitant jet velocity \u22652.5 m\/second. One third of these patients had tricuspid regurgitant jet velocity \u22653 m\/second. All patients with elevated tricuspid regurgitant jet velocity had SS disease. A high reticulocyte count, low oxygen saturation, and a high platelet count were significantly associated with elevated pulmonary artery pressures. There was no difference in age, gender, history of acute chest syndrome, hydroxyurea therapy, chronic blood transfusion, stroke, hemoglobin, and bilirubin between patients with and without elevated pulmonary artery pressures. A total of 47% patients with elevated tricuspid regurgitant jet velocity and 57% without elevated tricuspid regurgitant jet velocity had screening transcranial Doppler examinations. Transcranial Doppler examinations were normal for all patients.<\/jats:p>\n                  <jats:p>CONCLUSIONS. High pulmonary artery pressures do occur in children with sickle cell disease. Screening by echocardiography can lead to early detection and intervention that may potentially reverse this disease process. There was no correlation between elevated pulmonary artery pressures and abnormal transcranial Doppler examination in our study.<\/jats:p>","DOI":"10.1542\/peds.2007-0730","type":"journal-article","created":{"date-parts":[[2008,4,1]],"date-time":"2008-04-01T16:25:58Z","timestamp":1207067158000},"page":"777-782","source":"Crossref","is-referenced-by-count":81,"title":["Prevalence and Risk Factors of Elevated Pulmonary Artery Pressures in Children With Sickle Cell Disease"],"prefix":"10.1542","volume":"121","author":[{"given":"Farzana D.","family":"Pashankar","sequence":"first","affiliation":[{"name":"Department of Pediatrics, Yale University School of Medicine, New Haven, Connecticut"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Judith","family":"Carbonella","sequence":"additional","affiliation":[{"name":"Department of Pediatrics, Yale University School of Medicine, New Haven, Connecticut"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Alia","family":"Bazzy-Asaad","sequence":"additional","affiliation":[{"name":"Department of Pediatrics, Yale University School of Medicine, New Haven, Connecticut"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Alan","family":"Friedman","sequence":"additional","affiliation":[{"name":"Department of Pediatrics, Yale University School of Medicine, New Haven, Connecticut"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"417","published-online":{"date-parts":[[2008,4,1]]},"reference":[{"key":"2021120902111558600_R1","doi-asserted-by":"crossref","unstructured":"Collins FS, Orringer EP. Pulmonary hypertension and cor pulmonale in the sickle hemoglobinopathies. Am J Med.\u20081982;73(6):814\u2013821","DOI":"10.1016\/0002-9343(82)90763-X"},{"key":"2021120902111558600_R2","doi-asserted-by":"crossref","unstructured":"Simmons BE, Santhanam V, Castaner A, et al. Sickle cell heart disease: two dimensional echo and Doppler ultrasonographic findings in the heart of adult patients with sickle cell anemia. Arch Intern Med.\u20081988;148(7):1526\u20131528","DOI":"10.1001\/archinte.148.7.1526"},{"key":"2021120902111558600_R3","doi-asserted-by":"crossref","unstructured":"Sutton LL, Castro O, Cross DJ, Spencer JE, Lewis JF. Pulmonary hypertension in sickle cell disease. Am J Cardiol.\u20081994;74(6):626\u2013628","DOI":"10.1016\/0002-9149(94)90760-9"},{"key":"2021120902111558600_R4","doi-asserted-by":"crossref","unstructured":"Castro O, Hoque M, Brown BD. Pulmonary hypertension in sickle cell disease: cardiac catheterization results and survival. Blood.\u20082003;101(4):1257\u20131261","DOI":"10.1182\/blood-2002-03-0948"},{"key":"2021120902111558600_R5","doi-asserted-by":"crossref","unstructured":"Manci EA, Culberson DE, Yang YM, et al. Causes of death in sickle cell disease: an autopsy study. Br J Haematol.\u20082003;123(2):359\u2013365","DOI":"10.1046\/j.1365-2141.2003.04594.x"},{"key":"2021120902111558600_R6","doi-asserted-by":"crossref","unstructured":"Gladwin MT, Sachdev V, Jison ML, et al. Pulmonary hypertension as a risk factor for death in patients with sickle cell disease. N Engl J Med.\u20082004;350(9):886\u2013895","DOI":"10.1056\/NEJMoa035477"},{"key":"2021120902111558600_R7","doi-asserted-by":"crossref","unstructured":"Ataga KI, Sood N, Gent GD, et al. Pulmonary hypertension in sickle cell disease. Am J Med.\u20082004;117(9):665\u2013669","DOI":"10.1016\/j.amjmed.2004.03.034"},{"key":"2021120902111558600_R8","doi-asserted-by":"crossref","unstructured":"Castro O, Gladwin MT. 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Blood.\u20082001;97(11):3411\u20133416","DOI":"10.1182\/blood.V97.11.3411"},{"key":"2021120902111558600_R12","doi-asserted-by":"crossref","unstructured":"Morris CR, Kuypers FA, Kato GJ, et al. Hemolysis associated pulmonary hypertension in thalassemia. Ann N Y Acad Sci.\u20082005;1054:481\u2013485","DOI":"10.1196\/annals.1345.058"},{"key":"2021120902111558600_R13","doi-asserted-by":"crossref","unstructured":"Singer ST, Kuypers FA, Styles L, et al. Pulmonary hypertension in thalassemia: association with platelet activation and hypercoagulable state. Am J Hematol.\u20082006;81(9):670\u2013675","DOI":"10.1002\/ajh.20640"},{"key":"2021120902111558600_R14","doi-asserted-by":"crossref","unstructured":"Hayag-Barin JE, Smith RE, Tucker FC Jr. Hereditary spherocytosis, thrombocytosis, and chronic pulmonary emboli: a case report and review of the literature. 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