Morrison M Louise, McMahon Corrina, Tully Riona, Enright Noelle, Pignatelli Ricardo, Towbin Jeffrey A, McMahon Colin J
Department of Pediatric Cardiology, Our Lady's Hospital for Sick Children, Crumlin, Dublin, Ireland.
Department of Hematology, Our Lady's Hospital for Sick Children, Crumlin, Dublin, Ireland.
Congenit Heart Dis. 2018 May;13(3):440-443. doi: 10.1111/chd.12592. Epub 2018 Feb 21.
Incidence of sickle cell disease (SCD) in Ireland has dramatically increased. Disease survival has also steadily improved however cardiovascular manifestations remain important causes of morbidity. These include reports of left ventricular hypertrabeculation (LVHT)/noncompaction. We sought to investigate the prevalence of LVHT among a large cohort of children with SCD.
We retrospectively reviewed the records of all patients with a diagnosis of SCD who had undergone surveillance echocardiography at Our Lady's Children's Hospital Crumlin (OLCHC) from 1998 to 2015. Demographics, hemoglobin phenotype and treatment information was recorded. LV systolic function, evidence of LVHT, and possible pulmonary arterial hypertension was assessed.
Two hundred thirty-six patients had echocardiograms available for interpretation. One hundred twenty-one (51.3%) were female; mean age was 11.3 years (± 4.1 years). Twenty-six patients (11%) had features of LVHT on echocardiography. Eleven patients (4.7%) had borderline features of LVHT. Mean LVEDD across the whole cohort was 4.2 ± 0.69 cm, LVEDD z-score of 1.44 ± 1.9, and mean LVSF was 37.3% ±15.7%. There were no significant differences in terms of age, LVEDD, LVEDD z-score, or LVSF between patients with and those without LVHT.
The prevalence of LVHT/noncompaction in children with SCD is lower than the adult population and LV systolic function is well preserved throughout our patient group. The mechanism behind the development of LVHT in this population remains speculative. Further work is required in this field. Sickle cell patients require longitudinal evaluation to ascertain changes in left ventricular function and the presence of LVHT/noncompaction.
爱尔兰镰状细胞病（SCD）的发病率急剧上升。尽管疾病生存率也在稳步提高，但心血管表现仍是发病的重要原因。这些表现包括左心室小梁增多（LVHT）/心肌致密化不全的报告。我们试图调查一大群SCD儿童中LVHT的患病率。
我们回顾性分析了1998年至2015年在克鲁姆林圣母儿童医院（OLCHC）接受监测超声心动图检查的所有确诊为SCD的患者的记录。记录人口统计学、血红蛋白表型和治疗信息。评估左心室收缩功能、LVHT证据以及可能的肺动脉高压。
236例患者有可供解读的超声心动图。121例（51.3%）为女性；平均年龄为11.3岁（±4.1岁）。26例（11%）患者在超声心动图上有LVHT特征。11例（4.7%）有LVHT的临界特征。整个队列的平均左心室舒张末期内径（LVEDD）为4.2±0.69 cm，LVEDD z评分为1.44±1.9，平均左心室短轴缩短率（LVSF）为37.3%±15.7%。有LVHT和无LVHT的患者在年龄、LVEDD、LVEDD z评分或LVSF方面无显著差异。
SCD儿童中LVHT/心肌致密化不全的患病率低于成人，且我们整个患者群体的左心室收缩功能保存良好。该人群中LVHT发生的机制仍具有推测性。该领域需要进一步研究。镰状细胞病患者需要进行纵向评估，以确定左心室功能的变化以及LVHT/心肌致密化不全的存在情况。
