Cuturilo Goran, Drakulic Danijela, Jovanovic Ida, Ilic Slobodan, Kalanj Jasna, Vulicevic Irena, Raus Misela, Skoric Dejan, Mijovic Marija, Medjo Biljana, Rsovac Snezana, Stevanovic Milena
Faculty of Medicine, University of Belgrade, Dr Subotica 8, 11000, Belgrade, Serbia.
Department of Medical Genetics, University Children's Hospital, Tirsova 10, Belgrade, Serbia.
Pediatr Cardiol. 2017 Dec;38(8):1680-1685. doi: 10.1007/s00246-017-1713-7. Epub 2017 Sep 22.
22q11.2 microdeletion is the most common microdeletion in humans. The purpose of this study was to evaluate postoperative outcome in children with 22q11.2 microdeletion who had undergone complete surgical correction of a congenital heart defect. The study included 34 patients who underwent complete correction of conotruncal heart defects. Of these, 17 patients diagnosed with 22q11.2 microdeletion represent the investigated group. Another 17 patients without 22q11.2 microdeletion represent the control group. Investigated and control groups differ significantly for total length of stay in the hospital (average 37.35 and 14.12 days, respectively); length of postoperative stay in the intensive care unit (average 10.82 and 6.76 days, respectively); sepsis (eight and two patients, respectively); administration of antibiotics (15 and seven patients, respectively); duration of antibiotic therapy (average 17.65 and 14.59 days, respectively); occurrence of hypocalcemia (16 and 0 patients, respectively); and initiation of peroral nutrition during the postoperative course (average 10.29 and 3.88 days, respectively). No difference was found for duration of ventilatory support (average 6.12 and 4.24 days, respectively), administration of total parenteral nutrition, and postoperative mortality rate. The study results suggest that genotype of 22q11.2 microdeletion affects postoperative outcome after cardiac surgery. Possible targets for intervention in postoperative intensive care management are prevention and treatment of systemic infections, monitoring, and treatment of hypocalcemias, rational administration of antibiotics and careful planning of nutrition. Consequently, this could shorten patients' intensive care stay and overall duration of hospitalization.
22q11.2微缺失是人类最常见的微缺失。本研究的目的是评估接受先天性心脏病完全手术矫正的22q11.2微缺失患儿的术后结局。该研究纳入了34例接受圆锥动脉干心脏缺陷完全矫正的患者。其中,17例被诊断为22q11.2微缺失的患者为研究组。另外17例无22q11.2微缺失的患者为对照组。研究组和对照组在住院总时长(分别为平均37.35天和14.12天)、重症监护病房术后住院时长(分别为平均10.82天和6.76天)、败血症(分别为8例和2例)、抗生素使用(分别为15例和7例)、抗生素治疗时长(分别为平均17.65天和14.59天)、低钙血症的发生(分别为16例和0例)以及术后病程中经口营养的开始时间(分别为平均10.29天和3.88天)方面存在显著差异。在通气支持时长(分别为平均6.12天和4.24天)、全胃肠外营养的使用以及术后死亡率方面未发现差异。研究结果表明,22q11.2微缺失的基因型会影响心脏手术后的术后结局。术后重症监护管理中可能的干预靶点是全身感染的预防和治疗、低钙血症的监测和治疗、抗生素的合理使用以及营养的精心规划。因此,这可以缩短患者的重症监护住院时间和总体住院时长。