Open Access

ARTICLE

Initiating a Fontan multidisciplinary clinic: Decreasing care variability, improving surveillance, and subsequent treatment of Fontan survivors

Michael V. Di Maria¹, Cindy Barrett¹, Carey Rafferty¹, Kelly Wolfe¹, Sarah L. Kelly¹, Deborah R. Liptzin², Dania Brigham³, Adel Younoszai¹

1 Heart Institute, Section of Pediatric Cardiology, Department of Pediatrics, University of Colorado School of Medicine, Children’s Hospital Colorado, Aurora, CO
2 Breathing Institute, Section of Pulmonary Medicine, Department of Pediatrics, University of Colorado School of Medicine, Children’s Hospital Colorado, Aurora, CO
3 Digestive Health Institute, Section of Pediatric Gastroenterology, Hepatology and Nutrition, Department of Pediatrics, University of Colorado School of Medicine, Children’s Hospital Colorado, Aurora, CO

* Corresponding Author: Michael V. Di Maria, Heart Institute, Section of Pediatric Cardiology, Department of Pediatrics, University of Colorado School of Medicine, Children’s Hospital Colorado, 13123 E. 16th Ave. Box 100, Aurora, Colorado 80045. Email:

Congenital Heart Disease 2019, 14(4), 590-599. https://doi.org/10.1111/chd.12769

Abstract

Background: 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.
Methods: 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.
Results: 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.
Conclusions: 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.

---

Keywords

---