Relationships between Physiologic and Neuropsychologic Functioning after Fontan.

OBJECTIVE: To investigate potential relationships between neuropsychologic functioning and cardiac, gastroenterologic/hepatologic, and pulmonary complications in the single ventricle heart disease (SVHD) post-Fontan population. STUDY DESIGN: Following the initiation of a Fontan Multidisciplinary Clinic, patients with SVHD were evaluated systematically according to a clinical care pathway, and data from multiple subspecialty evaluations were collected prospectively from 2016 to 2019. Biomarkers of cardiology, pulmonary, and hepatology/gastroenterology functioning were abstracted, along with neuropsychologic testing results. Bivariate correlations and regression analyses examined cross-sectional relationships between physiologic predictors and neuropsychologic outcomes. RESULTS: The sample included a cohort of 68 youth with SVHD age 3-19 years, after Fontan palliation. Sleep-disordered breathing was related to poorer visual-motor integration skills (r = -0.33; P < .05) and marginally related to poorer executive functioning (r = -0.33; P = .05). Lower arterial blood oxygen content was related to poorer executive functioning (r = .45; P < .05). Greater atrioventricular valve regurgitation was related to lower parent-rated adaptive functioning (ρ = -0.34; P < .01). These results were maintained in regression analyses controlling for history of stroke and/or seizures. CONCLUSIONS: We demonstrated associations between neuropsychologic functioning and potentially modifiable aspects of physiologic functioning in a prospectively evaluated cohort of patients with SVHD with Fontan physiology. Our findings emphasize the importance of multidisciplinary screening and care after a Fontan procedure and suggest avenues for intervention that may improve patient outcomes and quality of life.

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

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.

Echocardiographic Measurements of Right Ventricular Mechanics in Infants with Bronchopulmonary Dysplasia at 36 Weeks Postmenstrual Age.

OBJECTIVE: To test the hypothesis that specific echocardiographic measurements of right ventricular (RV) mechanics at 36 weeks postmenstrual age (PMA) are associated with the severity of bronchopulmonary dysplasia (BPD). STUDY DESIGN: A subset of 93 preterm infants (born between 27 and 29 weeks of gestation) was selected retrospectively from a prospectively enrolled cohort. BPD was defined using the National Institutes of Health workshop definition, with modifications for oxygen reduction testing and altitude. The cohort was divided into no-BPD and BPD groups using previously published methodology for analyses. Echocardiographic measurements of RV function (ie, tricuspid annular plane systolic excursion, fractional area of change, systolic-to-diastolic ratio, tissue Doppler myocardial performance index, and RV strain), RV remodeling/morphology (end-systolic left ventricular eccentricity index), and RV afterload (pulmonary artery acceleration time measure) were evaluated at 36 weeks PMA. Multivariable logistic regression determined associations between RV measurements and BPD severity. RESULTS: Compared with the no-BPD cohort, the BPD group had lower birth weight z-scores (P = .04) and trended toward a male predominance (P = .08). After adjusting for birth weight z-score, gestational age, and sex, there were no between-group differences in echocardiographic measurements except for the eccentricity index (scaled OR [0.1-unit increase], 1.49; 95% CI, 1.13-2.12; P = .01). CONCLUSIONS: Among conventional and emerging echocardiographic measurements of RV mechanics, eccentricity index was the sole variable independently associated with BPD severity in this study. The eccentricity index may be a useful echocardiographic measurement for characterizing RV mechanics in patients with BPD at 36 weeks PMA.

Pulmonary Screening in Subjects after the Fontan Procedure.

OBJECTIVES: To review the pulmonary findings of the first 51 patients who presented to our interdisciplinary single-ventricle clinic after undergoing the Fontan procedure. STUDY DESIGN: We performed an Institutional Review Board-approved retrospective review of 51 patients evaluated following the Fontan procedure. Evaluation included history, physical examination, pulmonary function testing, and 6-minute walk. Descriptive statistics were used to describe the population and testing data. RESULTS: Sixty-one percent of the patients had a pulmonary concern raised during the visit. Three patients had plastic bronchitis. Abnormal lung function testing was present in 46% of patients. Two-thirds (66%) of the patients had significant desaturation during the 6-minute walk test. Patients who underwent a fenestrated Fontan procedure and those who underwent unfenestrated Fontan were compared in terms of saturation and 6-minute walk test results. Sleep concerns were present in 45% of the patients. CONCLUSIONS: Pulmonary morbidities are common in patients after Fontan surgery and include plastic bronchitis, abnormal lung function, desaturations with walking, and sleep concerns. Abnormal lung function and obstructive sleep apnea may stress the Fontan circuit and may have implications for cognitive and emotional functioning. A pulmonologist involved in the care of patients after Fontan surgery can assist in screening for comorbidities and recommend interventions.