Department of Pediatrics, Lillie Frank Abercrombie Section of Pediatric and Congenital Cardiology, Baylor College of Medicine/Texas Children's Hospital, Houston, Texas, USA.
Division of Cardiology, The Children's Hospital of Philadelphia, Philadelphia, Pennsylvania, USA.
J Heart Lung Transplant. 2018 Jun;37(6):749-754. doi: 10.1016/j.healun.2018.01.1296. Epub 2018 Jan 31.
The purpose of this study was to describe the prevalence, characteristics, and outcomes in pediatric patients with chromosomal anomalies (CA) undergoing orthotopic heart transplantation (OHT).
A query of the database of the Pediatric Health Information System, a large administrative and billing database of 43 tertiary children's hospitals, was performed for the Years 2004 to 2016. Pediatric patients who received OHT were analyzed based on presence and type of CA. CA analyzed included: Down syndrome (DS); Turner syndrome (TS)/gonadal dysgenesis; conditions due to anomaly of unspecified chromosome; autosomal deletion; microdeletion; and autosomal anomaly. Healthcare-associated charge analysis during hospitalization for OHT and survival after OHT were assessed.
A total of 3,080 hospitalizations were identified in which OHTs were performed. Of these OHTs, 64 (2.1%) were performed in patients with a concomitant diagnosis of CA. The presence of CA did not confer a higher risk of in-hospital mortality after OHT (odds ratio 1.2 [0.5 to 3.2], p = 0.651). Differences in in-hospital mortality between different types of CA, including DS and TS, did not reach statistical significance. Survival at 1-year post-OHT was similar in patients with CA compared to those without CA (p = 0.248). Length of stay after OHT was longer in patients with CA: 76 (interquartile range [IQR] 76 to 142 days vs 49 [IQR 21 to 98] days) (p < 0.001), respectively. Overall adjusted hospital charges were significantly higher in the CA group: $1.2 million (IQR $740,000 to $2.2 million) vs $792,000 (IQR $425,000 to $1.5 million] (p < 0.001), respectively.
CA is present in ~2% of pediatric patients undergoing OHT. The presence of CA was not associated with increased mortality in pediatric patients undergoing OHT. Limitations of this study include the small number of patients available for analysis and a likely highly selective cohort of patients with CA.
本研究旨在描述行原位心脏移植术（OHT）的染色体异常（CA）儿科患者的流行率、特征和结局。
对 2004 年至 2016 年的 43 家三级儿童医院的大型行政和计费数据库儿科健康信息系统进行数据库查询。根据 CA 的存在和类型对接受 OHT 的儿科患者进行分析。CA 分析包括：唐氏综合征（DS）；特纳综合征（TS）/性腺发育不全；不明染色体异常引起的疾病；常染色体缺失；微缺失；和常染色体异常。评估 OHT 住院期间的医疗保健相关费用分析和 OHT 后的存活情况。
共确定 3080 例接受 OHT 的住院患者。其中，64 例（2.1%）患者同时诊断为 CA。CA 的存在并不会增加 OHT 后院内死亡率的风险（优势比 1.2[0.5 至 3.2]，p=0.651）。不同类型 CA（包括 DS 和 TS）之间的院内死亡率差异无统计学意义。OHT 后 1 年的存活率在 CA 患者与无 CA 患者之间相似（p=0.248）。CA 患者 OHT 后住院时间较长：76（四分位距 [IQR]76 至 142 天）与 49（IQR21 至 98）天）（p<0.001）。CA 组的总体调整后医院费用明显较高：120 万美元（IQR74 万美元至 220 万美元）与 79.2 万美元（IQR42.5 万美元至 150 万美元）（p<0.001）。
CA 存在于约 2%接受 OHT 的儿科患者中。CA 的存在与接受 OHT 的儿科患者死亡率增加无关。本研究的局限性包括可用于分析的患者数量较少，以及 CA 患者可能存在高度选择性队列。
