Di Maria Michael V, Barrett Cindy, Rafferty Carey, Wolfe Kelly, Kelly Sarah L, Liptzin Deborah R, Brigham Dania, Younoszai Adel
Heart Institute, Section of Pediatric Cardiology, Department of Pediatrics, University of Colorado School of Medicine, Children's Hospital Colorado, Aurora, CO.
Breathing Institute, Section of Pulmonary Medicine, Department of Pediatrics, University of Colorado School of Medicine, Children's Hospital Colorado, Aurora, CO.
Congenit Heart Dis. 2019 Jul;14(4):590-599. doi: 10.1111/chd.12769. Epub 2019 Apr 8.
Children with single ventricle (SV) heart disease who undergo Fontan operation are at risk for developing multiorgan dysfunction. Although survival has improved, significant comorbidities involving multiple organ systems may develop, requiring evaluation and management by many subspecialists. Using data from an internal survey, we documented high care variability for our Fontan population. We then developed a multidisciplinary clinic, designed and implemented a clinical care pathway to decrease variability of patient assessment.
After creating a multidisciplinary team and a clinical care pathway, we initiated a multidisciplinary clinic (MDC) where patients could see multiple subspecialists during a single encounter. We then monitored our effectiveness by retrospective chart review to determine care pathway adherence (process measure) and incidence of new diagnoses of end-organ injury (outcome measure) as well interventions implemented. Adherence was analyzed using statistical process control (SPC) charts.
Eighty-six patients were seen in the MDC from January 2016 to September 2017. The proportion of patients with appropriate testing increased, related to strong care pathway adherence. A significant amount of novel pathology was diagnosed in all evaluated organ systems, both Fontan-associated comorbidities and general pediatric diagnoses. Subsequent interventions included cardiac catheterization n = 21 (31%) with more than half of these patients undergoing intervention n = 17 (20%), and liver biopsy n = 9 (10%). Additionally, 58 patients (67%) were referred to a neuropsychologist based on perceived clinical need, with n = 34 (40%) undergoing a neuropsychological evaluation.
Children who have undergone Fontan palliation are at risk for developing cardiac and noncardiac comorbidities. Use and adherence to an institutional care pathway resulted in the diagnosis of significant novel pathology and subsequently provided opportunity for intervention.
接受Fontan手术的单心室（SV）心脏病患儿有发生多器官功能障碍的风险。尽管生存率有所提高，但可能会出现涉及多个器官系统的严重合并症，需要许多专科医生进行评估和管理。利用内部调查的数据，我们记录了Fontan患者群体护理的高度变异性。然后，我们设立了一个多学科诊所，设计并实施了一条临床护理路径，以减少患者评估的变异性。
在组建了多学科团队并制定了临床护理路径后，我们开设了一个多学科诊所（MDC），患者可以在一次就诊中见到多个专科医生。然后，我们通过回顾性病历审查来监测我们的有效性，以确定护理路径的依从性（过程指标）、终末器官损伤新诊断的发生率（结果指标）以及实施的干预措施。使用统计过程控制（SPC）图表分析依从性。
2016年1月至2017年9月，MDC共诊治了86例患者。由于对护理路径的高度依从，进行适当检查的患者比例有所增加。在所有评估的器官系统中都诊断出了大量新的病理情况，包括与Fontan相关的合并症和一般儿科诊断。随后的干预措施包括心脏导管插入术n = 21例（31%），其中一半以上的患者接受了干预n = 17例（20%），以及肝活检n = 9例（10%）。此外，58例患者（67%）根据临床需求被转诊至神经心理学家处，其中n = 34例（40%）接受了神经心理学评估。
接受Fontan姑息治疗的儿童有发生心脏和非心脏合并症的风险。使用并遵循机构护理路径导致了大量新病理情况的诊断，并随后提供了干预机会。
