P>Despite the finding of elevated Tricuspid Regurgitant Velocity (TRV) in children below 5 years of age, the prevalence and evolution of Pulmonary Hypertension (PH) in young children with sickle cell disease (SCD) are unclear. In order to identify predictive factors of precocious PH development, SCD children >= 3 years old, at steady state, underwent annual echocardiography and Tissue Doppler Imaging (TDI). Patients receiving chronic transfusion were excluded. Thirty-seven of seventy-five patients were >= 3 years, with measurable TRV. In our young population (mean age 6 center dot 2 years) of mainly African, HbS/HbS patients, 8/37 (21 center dot 6%) had TRV >= 2 center dot 5 m/s, 8% being only 3 years old. Significant correlation was found between precocious TRV elevation and high platelet and reticulocyte counts and frequent acute chest syndromes (ACS). In multivariate analysis, ACS was the only variable predicting TRV >= 2 center dot 5 m/s. TDI of the 37 patients showed signs of diastolic dysfunction of the left ventricle. At follow-up all eight patients with high TRV displayed further increase and seven more developed TRV >= 2 center dot 5 m/s. PH seems to begin in children earlier than expected. Factors involved in its early onset might be different from the ones causing its development in older children or adults. African children might benefit from early screening and re-assessment once a year.
机构:
Hop Marie Lannelongue, INSERM UMR S 999 Pulm Hypertens Pathophysiol & Nov, F-92350 Le Plessis Robinson, France
Univ Paris Saclay, Fac Med Pulm Hypertens Pathophysiol & Novel Therap, F-94276 Le Kremlin Bicetre, France
Hop Bicetre, AP HP, Serv Pneumol & Soins Intens Resp, F-94270 Le Kremlin Bicetre, FranceHop Marie Lannelongue, INSERM UMR S 999 Pulm Hypertens Pathophysiol & Nov, F-92350 Le Plessis Robinson, France
Al Kahf, Salma
Roche, Anne
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Hop Marie Lannelongue, INSERM UMR S 999 Pulm Hypertens Pathophysiol & Nov, F-92350 Le Plessis Robinson, France
Univ Paris Saclay, Fac Med Pulm Hypertens Pathophysiol & Novel Therap, F-94276 Le Kremlin Bicetre, France
Hop Bicetre, AP HP, Serv Pneumol & Soins Intens Resp, F-94270 Le Kremlin Bicetre, FranceHop Marie Lannelongue, INSERM UMR S 999 Pulm Hypertens Pathophysiol & Nov, F-92350 Le Plessis Robinson, France
Roche, Anne
Baron, Audrey
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Hop Marie Lannelongue, INSERM UMR S 999 Pulm Hypertens Pathophysiol & Nov, F-92350 Le Plessis Robinson, France
Univ Paris Saclay, Fac Med Pulm Hypertens Pathophysiol & Novel Therap, F-94276 Le Kremlin Bicetre, France
Hop Bicetre, AP HP, Serv Pneumol & Soins Intens Resp, F-94270 Le Kremlin Bicetre, FranceHop Marie Lannelongue, INSERM UMR S 999 Pulm Hypertens Pathophysiol & Nov, F-92350 Le Plessis Robinson, France
Baron, Audrey
Chantalat-Auger, Christelle
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Univ Paris Saclay, Fac Med Pulm Hypertens Pathophysiol & Novel Therap, F-94276 Le Kremlin Bicetre, France
Hop Bicetre, AP HP, Serv Med Interne, F-94270 Le Kremlin Bicetre, FranceHop Marie Lannelongue, INSERM UMR S 999 Pulm Hypertens Pathophysiol & Nov, F-92350 Le Plessis Robinson, France
Chantalat-Auger, Christelle
Savale, Laurent
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Hop Marie Lannelongue, INSERM UMR S 999 Pulm Hypertens Pathophysiol & Nov, F-92350 Le Plessis Robinson, France
Univ Paris Saclay, Fac Med Pulm Hypertens Pathophysiol & Novel Therap, F-94276 Le Kremlin Bicetre, France
Hop Bicetre, AP HP, Serv Pneumol & Soins Intens Resp, F-94270 Le Kremlin Bicetre, France
Hop Bicetre, Serv Pneumol & Soins Intens Resp, 78 Rue Gen Leclerc, F-94270 Le Kremlin Bicetre, FranceHop Marie Lannelongue, INSERM UMR S 999 Pulm Hypertens Pathophysiol & Nov, F-92350 Le Plessis Robinson, France
机构:
Boston Childrens Hosp, Dept Cardiol, Boston, MA USA
Harvard Med Sch, Dept Pediat, Boston, MA 02115 USABoston Childrens Hosp, Dept Cardiol, Boston, MA USA
Harrington, Jamie K.
Krishnan, Usha S.
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Columbia Univ, Coll Phys & Surg, Med Ctr, Div Pediat Cardiol,Dept Pediat, 3959 Broadway,CHN 2-255, New York, NY 10032 USABoston Childrens Hosp, Dept Cardiol, Boston, MA USA
机构:
Columbia Univ, Coll Physicians & Surg, Div Pediat Hematol, New York, NY 10032 USAColumbia Univ, Coll Physicians & Surg, Div Pediat Hematol, New York, NY 10032 USA
Lee, Margaret T.
Small, Tania
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Columbia Univ, Coll Physicians & Surg, Div Pediat Hematol, New York, NY 10032 USAColumbia Univ, Coll Physicians & Surg, Div Pediat Hematol, New York, NY 10032 USA
Small, Tania
Khan, Muhammad A.
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Columbia Univ, Coll Physicians & Surg, Div Pediat Cardiol, New York, NY 10032 USAColumbia Univ, Coll Physicians & Surg, Div Pediat Hematol, New York, NY 10032 USA
Khan, Muhammad A.
Rosenzweig, Erika B.
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Columbia Univ, Coll Physicians & Surg, Div Pediat Cardiol, New York, NY 10032 USAColumbia Univ, Coll Physicians & Surg, Div Pediat Hematol, New York, NY 10032 USA
Rosenzweig, Erika B.
Barst, Robyn J.
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Schneider Childrens Hosp N Shore Long Isl Jewish, Dept Pediat & Med, Long Isl City, NY USAColumbia Univ, Coll Physicians & Surg, Div Pediat Hematol, New York, NY 10032 USA
Barst, Robyn J.
Brittenham, Gary M.
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Columbia Univ, Coll Physicians & Surg, Div Pediat Hematol, New York, NY 10032 USAColumbia Univ, Coll Physicians & Surg, Div Pediat Hematol, New York, NY 10032 USA
机构:
Penn State Coll Med, Dept Pediat, Div Pediat Pulmonol & Sleep Med, Hershey, PA 17033 USAPenn State Coll Med, Dept Pediat, Div Pediat Pulmonol & Sleep Med, Hershey, PA 17033 USA
Mondal, Pritish
Stefek, Bryan
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机构:
Penn State Coll Med, Dept Pediat, Div Pediat Cardiol, Hershey, PA USAPenn State Coll Med, Dept Pediat, Div Pediat Pulmonol & Sleep Med, Hershey, PA 17033 USA
Stefek, Bryan
Sinharoy, Ankita
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机构:
Penn State Coll Med, Dept Publ Hlth, Hershey, PA USAPenn State Coll Med, Dept Pediat, Div Pediat Pulmonol & Sleep Med, Hershey, PA 17033 USA
Sinharoy, Ankita
Sankoorikal, Binu-John
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Penn State Coll Med, Dept Pediat, Div Pediat Pulmonol & Sleep Med, Hershey, PA 17033 USAPenn State Coll Med, Dept Pediat, Div Pediat Pulmonol & Sleep Med, Hershey, PA 17033 USA
Sankoorikal, Binu-John
Abu-Hasan, Mutasim
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机构:
Univ Florida, Dept Pediat, Div Pediat Pulmonol, Gainesville, FL USAPenn State Coll Med, Dept Pediat, Div Pediat Pulmonol & Sleep Med, Hershey, PA 17033 USA
Abu-Hasan, Mutasim
Aluquin, Vincent
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Penn State Coll Med, Dept Pediat, Div Pediat Cardiol, Hershey, PA USAPenn State Coll Med, Dept Pediat, Div Pediat Pulmonol & Sleep Med, Hershey, PA 17033 USA