Pulmonary hypertension in sickle cell disease children under 10 years of age

被引:42
|
作者
Colombatti, Raffaella [1 ]
Maschietto, Nicola [2 ]
Varotto, Elena [2 ]
Grison, Alessandra [2 ]
Grazzina, Nicoletta [3 ]
Meneghello, Linda [4 ]
Teso, Simone
Carli, Modesto [1 ]
Milanesi, Ornella [2 ]
Sainati, Laura [1 ]
机构
[1] Univ Padua, Dept Paediat, Clin Paediat Haematol Oncol, I-35100 Padua, Italy
[2] Univ Padua, Dept Paediat, Cardiol Unit, I-35100 Padua, Italy
[3] Osped Cittadella, Dept Paediat, Padua, Italy
[4] Osped S Chiara, Dept Paediat, Trento, Italy
关键词
sickle cell disease; pulmonary hypertension; Tricuspid regurgitant jet velocity; African; child; REGURGITANT JET VELOCITY; VENTRICULAR DIASTOLIC FUNCTION; ARTERY PRESSURES; BLOOD-PRESSURE; NORMAL VALUES; RISK-FACTORS; M-MODE; DEATH; PREVALENCE; ECHOCARDIOGRAPHY;
D O I
10.1111/j.1365-2141.2010.08269.x
中图分类号
R5 [内科学];
学科分类号
1002 ; 100201 ;
摘要
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.
引用
收藏
页码:601 / 609
页数:9
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