PDF(Pubmed)
Abstract：
OBJECTIVE: To describe the newborn population with Patau (T13) and Edwards Syndrome (T18) with congenital heart diseases that stayed in the Intensive Care Unit (ICU) of a quaternary care hospital complex, regarding surgical and non-surgical medical procedures, palliative care, and outcomes.
METHODS: Descriptive case series conducted from January/2014 to December/2018 through analysis of records of patients with positive karyotype for T13 or T18 who stayed in the ICU of a quaternary hospital. Descriptive statistics analysis was applied.
RESULTS: 33 records of eligible patients were identified: 27 with T18 (82%), and 6 T13 (18%); 64% female and 36% male. Eight were preterm infants with gestational age between 30-36 weeks (24%), and only 4 among the 33 infants had a birth weight >2500 g (12%). Four patients underwent heart surgery and one of them died. Intrahospital mortality was 83% for T13, and 59% for T18. The majority had other malformations and underwent other surgical procedures. Palliative care was offered to 54% of the patients. The median hospitalization time for T18 and T13 was 29 days (range: 2-304) and 25 days (13-58), respectively.
CONCLUSIONS: Patients with T13 and T18 have high morbidity and mortality, and long hospital and ICU stays. Multicentric studies are needed to allow the analysis of important aspects for creating protocols that, seeking therapeutic proportionality, may bring better quality of life for patients and their families.
METHODS: Descriptive case series conducted from January/2014 to December/2018 through analysis of records of patients with positive karyotype for T13 or T18 who stayed in the ICU of a quaternary hospital. Descriptive statistics analysis was applied.
RESULTS: 33 records of eligible patients were identified: 27 with T18 (82%), and 6 T13 (18%); 64% female and 36% male. Eight were preterm infants with gestational age between 30-36 weeks (24%), and only 4 among the 33 infants had a birth weight >2500 g (12%). Four patients underwent heart surgery and one of them died. Intrahospital mortality was 83% for T13, and 59% for T18. The majority had other malformations and underwent other surgical procedures. Palliative care was offered to 54% of the patients. The median hospitalization time for T18 and T13 was 29 days (range: 2-304) and 25 days (13-58), respectively.
CONCLUSIONS: Patients with T13 and T18 have high morbidity and mortality, and long hospital and ICU stays. Multicentric studies are needed to allow the analysis of important aspects for creating protocols that, seeking therapeutic proportionality, may bring better quality of life for patients and their families.
摘要：
目的：描述患有Patau（T13）和Edwards综合征（T18）的先天性心脏病的新生儿，这些新生儿住在四级护理综合医院的重症监护病房（ICU）中，关于手术和非手术医疗程序，姑息治疗,和结果。
方法：描述性病例系列于2014年1月至2018年12月进行，通过分析T13或T18染色体核型阳性患者入住四级医院ICU的记录。采用描述性统计分析。
结果：确定了33例合格患者的记录：27例T18（82％），和6个T13（18％）；64％的女性和36％的男性。8名胎龄在30-36周之间的早产儿（24％），33名婴儿中只有4名出生体重>2500克(12%)。四名患者接受了心脏手术，其中一人死亡。T13的院内死亡率为83%，T18为59%。大多数患有其他畸形，并接受了其他外科手术。54％的患者接受了姑息治疗。T18和T13的中位住院时间为29天（范围：2-304）和25天（13-58），分别。
结论：T13和T18患者有较高的发病率和死亡率，长期住院和ICU住院。需要进行多中心研究，以分析创建协议的重要方面，寻求治疗相称性，可以为患者及其家人带来更好的生活质量。
方法：描述性病例系列于2014年1月至2018年12月进行，通过分析T13或T18染色体核型阳性患者入住四级医院ICU的记录。采用描述性统计分析。
结果：确定了33例合格患者的记录：27例T18（82％），和6个T13（18％）；64％的女性和36％的男性。8名胎龄在30-36周之间的早产儿（24％），33名婴儿中只有4名出生体重>2500克(12%)。四名患者接受了心脏手术，其中一人死亡。T13的院内死亡率为83%，T18为59%。大多数患有其他畸形，并接受了其他外科手术。54％的患者接受了姑息治疗。T18和T13的中位住院时间为29天（范围：2-304）和25天（13-58），分别。
结论：T13和T18患者有较高的发病率和死亡率，长期住院和ICU住院。需要进行多中心研究，以分析创建协议的重要方面，寻求治疗相称性，可以为患者及其家人带来更好的生活质量。
