Evaluation and Management of the Child and Adult With Fontan Circulation: A Scientific Statement From the American Heart Association.

It has been 50 years since Francis Fontan pioneered the operation that today bears his name. Initially designed for patients with tricuspid atresia, this procedure is now offered for a vast array of congenital cardiac lesions when a circulation with 2 ventricles cannot be achieved. As a result of technical advances and improvements in patient selection and perioperative management, survival has steadily increased, and it is estimated that patients operated on today may hope for a 30-year survival of >80%. Up to 70 000 patients may be alive worldwide today with Fontan circulation, and this population is expected to double in the next 20 years. In the absence of a subpulmonary ventricle, Fontan circulation is characterized by chronically elevated systemic venous pressures and decreased cardiac output. The addition of this acquired abnormal circulation to innate abnormalities associated with single-ventricle congenital heart disease exposes these patients to a variety of complications. Circulatory failure, ventricular dysfunction, atrioventricular valve regurgitation, arrhythmia, protein-losing enteropathy, and plastic bronchitis are potential complications of the Fontan circulation. Abnormalities in body composition, bone structure, and growth have been detected. Liver fibrosis and renal dysfunction are common and may progress over time. Cognitive, neuropsychological, and behavioral deficits are highly prevalent. As a testimony to the success of the current strategy of care, the proportion of adults with Fontan circulation is increasing. Healthcare providers are ill-prepared to tackle these challenges, as well as specific needs such as contraception and pregnancy in female patients. The role of therapies such as cardiovascular drugs to prevent and treat complications, heart transplantation, and mechanical circulatory support remains undetermined. There is a clear need for consensus on how best to follow up patients with Fontan circulation and to treat their complications. This American Heart Association statement summarizes the current state of knowledge on the Fontan circulation and its consequences. A proposed surveillance testing toolkit provides recommendations for a range of acceptable approaches to follow-up care for the patient with Fontan circulation. Gaps in knowledge and areas for future focus of investigation are highlighted, with the objective of laying the groundwork for creating a normal quality and duration of life for these unique individuals.

Flow profile characteristics in Fontan circulation are associated with the single ventricle dilation and function: principal component analysis study.

The Fontan circulation is characterized as a nonpulsatile flow propagation without a pressure-generating ventricle. However, flow through the Fontan circulation still exhibits oscillatory waves as a result of pressure changes generated by the systemic single ventricle. Identification of discrete flow patterns through the Fontan circuit may be important to understand single ventricle performance. Ninety-seven patients with Fontan circulation underwent phase-contrast MRI of the right pulmonary artery, yielding subject-specific flow waveforms. Principal component (PC) analysis was performed on preprocessed flow waveforms. Principal components were then correlated with standard MRI indices of function, volume, and aortopulmonary collateral flow. The first principal component (PC) described systolic versus diastolic-dominant flow through the Fontan circulation, accounting for 31.3% of the variance in all waveforms. The first PC correlated with end-diastolic volume (R = 0.34, P = 0.001), and end-systolic volume (R = 0.30, P = 0.003), cardiac index (R = 0.51, P < 0.001), and the amount of aortopulmonary collateral flow (R = 0.25, P = 0.027)-lower ventricular volumes and a smaller volume of collateral flow-were associated with diastolic-dominant cavopulmonary flow. The second PC accounted for 19.5% of variance and described late diastolic acceleration versus deceleration and correlated with ejection fraction-diastolic deceleration was associated with higher ejection fraction. Principal components describing the diastolic flow variations in pulmonary arteries are related to the single ventricle function and volumes. Particularly, diastolic-dominant flow without late acceleration appears to be related to preserved ventricular volume and function, respectively.NEW & NOTEWORTHY The exact physiological significance of flow oscillations of phasic and temporal flow variations in Fontan circulation is unknown. With the use of principal component analysis, we discovered that flow variations in the right pulmonary artery of Fontan patients are related to the single ventricle function and volumes. Particularly, diastolic-dominant flow without late acceleration appears to be related to more ideal ventricular volume and systolic function, respectively.

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.

Early Pulmonary Vascular Disease in Preterm Infants Is Associated with Late Respiratory Outcomes in Childhood.

RATIONALE: Early pulmonary vascular disease (PVD) after preterm birth is associated with a high risk for developing bronchopulmonary dysplasia (BPD), but its relationship with late respiratory outcomes during early childhood remains uncertain. OBJECTIVES: To determine whether PVD at 7 days after preterm birth is associated with late respiratory disease (LRD) during early childhood. METHODS: This was a prospective study of preterm infants born before 34 weeks postmenstrual age (PMA). Echocardiograms were performed at 7 days and 36 weeks PMA. Prenatal and early postnatal factors and postdischarge follow-up survey data obtained at 6, 12, 18, and 24 months of age were analyzed in logistic regression models to identify early risk factors for LRD, defined as a physician diagnosis of asthma, reactive airways disease, BPD exacerbation, bronchiolitis, or pneumonia, or a respiratory-related hospitalization during follow-up. MEASUREMENTS AND MAIN RESULTS: Of the 221 subjects (median, 27 wk PMA; interquartile range, 25-28 and 920 g; interquartile range, 770-1090 g) completing follow-up, 61% met LRD criteria. Gestational diabetes and both mechanical ventilator support and PVD at 7 days were associated with LRD. The combination of PVD and mechanical ventilator support at 7 days was among the strongest prognosticators of LRD (odds ratio, 8.1; confidence interval, 3.1-21.9; P < 0.001). Modeled prenatal and early postnatal factors accurately informed LRD (area under the curve, 0.764). Adding BPD status at 36 weeks PMA to the model did not change the accuracy (area under the curve, 0.771). CONCLUSIONS: Early echocardiographic evidence of PVD after preterm birth in combination with other perinatal factors is a strong risk factor for LRD, suggesting that early PVD may contribute to the pathobiology of BPD.

The Fontan outcomes network: first steps towards building a lifespan registry for individuals with Fontan circulation in the United States.

The Fontan Outcomes Network was created to improve outcomes for children and adults with single ventricle CHD living with Fontan circulation. The network mission is to optimise longevity and quality of life by improving physical health, neurodevelopmental outcomes, resilience, and emotional health for these individuals and their families. This manuscript describes the systematic design of this new learning health network, including the initial steps in development of a national, lifespan registry, and pilot testing of data collection forms at 10 congenital heart centres.

Surveillance Testing and Preventive Care After Fontan Operation: A Multi-Institutional Survey.

More children with single ventricle heart disease are surviving after Fontan surgery. This circulation has pervasive effects on multiple organ systems and has unique modes of failure. Many centers have created multidisciplinary programs to care for these patients. Our aim was to survey such programs to better understand current approaches to care. We hypothesized that significant variability in surveillance testing strategy would be present. Eleven academic institutions with established Fontan care programs performing a combined estimated 300 Fontan surgeries per year, with a total population of 1500-2000 Fontan patients, were surveyed using a REDCap survey regarding surveillance testing and basic practice philosophies. Fontan care programs were structured both as consultative services (64%) and as the primary clinical team (9%). Electrocardiograms (73%) and echocardiograms (64%) were most commonly obtained annually. Serum studies, including complete blood count (73%), complete metabolic panel (73%), and Brain-type natriuretic peptide (54%), were most commonly obtained annually. Hepatic testing consisted of liver ultrasound in most centers, obtained biennially (45%) or > every 2 years (45%). Liver biopsy was not routinely recommended (54%). Neurodevelopmental outcomes were assessed at most institutions (54%), with a median frequency of every 3-4 years. There is considerable variability in the surveillance testing regimen and management strategy after a Fontan procedure at surveyed programs. There is an urgent need for surveillance guidelines to reduce variability, define quality metrics, streamline collaborative practice, and prospective research to better understand the complex adaptations of the body to Fontan physiology.

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.

Data-Driven Quality Improvement Project to Increase the Value of the Congenital Echocardiographic Report.

Echocardiography is the primary diagnostic modality for congenital heart disease patients. The written report is used to communicate with the care team and organization is often divided into the body with detailed findings and the conclusions with important findings summarized. Strategies to increase workflow efficiency include batch writing of reports after performance of multiple echocardiograms and the use of report templates which may contribute to discrepancies within report leading to potential downstream medical errors. The aim of this project was to measure the rate of inconsistencies in the echocardiogram reports and through an iterative series of process improvement decrease this rate while maintaining sonographer efficiency and diagnostic accuracy. The discrepancy rate, diagnostic error rate, and sonographer productivity were collected one-year prior and during the iterative quality improvement process. The primary outcome and discrepancies in reports were determined by two reviewers: an experienced pediatric echocardiographic cardiologist and a senior sonographer. Minor discrepancies were defined as contradictions between the body and the conclusion of the report that were unlikely to affect patient care. Major discrepancies were defined as discrepancies between the body and the conclusion that had significant potential to affect patient care. Sonographer productivity was measured as studies per sonographer per month. Our primary intervention was to initiate a quarterly QI meeting and to decrease the batch writing of preliminary echocardiogram reports. No major discrepancies were identified pre- or post-intervention. The minor discrepancies decreased from 40.7 to 6%. Sonographer productivity was not significantly changed with a slight increase from 100 studies/sonographer/month during the baseline to 101 studies/sonographer/month during the intervention. There was no change in major or minor diagnostic error rate. Our quality improvement intervention increased the value of our reports by significantly decreasing minor discrepancies without negatively impacting sonographer productivity or diagnostic accuracy.

Worsening in Longitudinal Strain and Strain Rate Anticipates Development of Pediatric Transplant Coronary Artery Vasculopathy as Soon as One Year Following Transplant.

Transplant coronary artery vasculopathy (TCAV) following orthotopic heart transplantation (OHT) continues to be the primary reason for late graft failure in children. The current gold standard of diagnosis of TCAV is coronary angiography with or without intravascular ultrasound. This study investigates the longitudinal use of speckle-tracking echocardiographic strain imaging as an early non-invasive marker to screen for development of TCAV. Echocardiograms from patients who underwent OHT between 2006 and 2010 at Children's Hospital Colorado (n = 50) were retrospectively assessed. Studies were evaluated at baseline (within a month of transplant), then at each annual clinical follow-up for peak longitudinal (LS) and circumferential (CS) strain, systolic strain rate, and diastolic strain rate using Siemens Velocity Vector Imaging software. Comparisons were made between subjects who did and did not develop TCAV. Mean time to TCAV diagnosis following OHT was 3.2 years (range 1-5.1 years). One year after transplant, significant differences were seen between groups in LS (non-TCAV mean -19.6%, TCAV mean -17.3%, p = 0.03) and longitudinal strain rate (non-TCAV mean -1.7%/s, TCAV mean -1.4%/s, p = 0.04). These differences persisted in subsequent years. Differences in LS preceded the catheterization-based diagnosis of TCAV in pediatric heart recipients and were noted as early as one year post transplant. Additionally, within-subject LS changes may have utility as a non-invasive screening tool to predict those patients at increased risk for development of TCAV.

Reliability of Echocardiographic Indicators of Pulmonary Vascular Disease in Preterm Infants at Risk for Bronchopulmonary Dysplasia.

OBJECTIVES: To determine the assessment and inter-rater reliability of echocardiographic evaluations of pulmonary vascular disease (PVD) in preterm infants at risk for bronchopulmonary dysplasia. STUDY DESIGN: We prospectively studied echocardiograms from preterm infants (birthweights 500-1250 g) at 7 days of age and 36 weeks postmenstrual age (PMA). Echocardiograms were assessed by both a cardiologist on clinical service and a single research cardiologist. Interpretations were reviewed for inclusion of determinants of PVD and assessed for inter-rater reliability using the Prevalence Adjusted Bias Adjusted Kappa Score (PABAK). RESULTS: One hundred eighty and 188 matching research and clinical echocardiogram reports were available for the 7-day and 36-week PMA studies. At least one of the specific qualitative measures of PVD was missing from 54% of the clinical reports. PVD was diagnosed at 7 days in 31% and 20% of research and clinical interpretations, respectively (PABAK score of 0.54). At 36 weeks, PH was diagnosed in 15.6% and 17.8% of research and clinical interpretations, respectively (PABAK score of 0.80). CONCLUSIONS: Although all qualitative variables of PVD are not consistently provided in echocardiogram reports, the inter-rater reliability of cardiologists evaluating measures of PVD revealed strong agreement, especially at 36 weeks PMA. We speculate that establishment of a protocol for echocardiographic evaluation may improve the identification of PVD in preterm infants.

Early pulmonary vascular disease in preterm infants at risk for bronchopulmonary dysplasia.

RATIONALE: Pulmonary hypertension (PH) is associated with poor outcomes among preterm infants with bronchopulmonary dysplasia (BPD), but whether early signs of pulmonary vascular disease are associated with the subsequent development of BPD or PH at 36 weeks post-menstrual age (PMA) is unknown. OBJECTIVES: To prospectively evaluate the relationship of early echocardiogram signs of pulmonary vascular disease in preterm infants to the subsequent development of BPD and late PH (at 36 wk PMA). METHODS: Prospectively enrolled preterm infants with birthweights 500-1,250 g underwent echocardiogram evaluations at 7 days of age (early) and 36 weeks PMA (late). Clinical and echocardiographic data were analyzed to identify early risk factors for BPD and late PH. MEASUREMENTS AND MAIN RESULTS: A total of 277 preterm infants completed echocardiogram and BPD assessments at 36 weeks PMA. The median gestational age at birth and birthweight of the infants were 27 weeks and 909 g, respectively. Early PH was identified in 42% of infants, and 14% were diagnosed with late PH. Early PH was a risk factor for increased BPD severity (relative risk, 1.12; 95% confidence interval, 1.03-1.23) and late PH (relative risk, 2.85; 95% confidence interval, 1.28-6.33). Infants with late PH had greater duration of oxygen therapy and increased mortality in the first year of life (P < 0.05). CONCLUSIONS: Early pulmonary vascular disease is associated with the development of BPD and with late PH in preterm infants. Echocardiograms at 7 days of age may be a useful tool to identify infants at high risk for BPD and PH.

Longitudinal Strain and Strain Rate Abnormalities Precede Invasive Diagnosis of Transplant Coronary Artery Vasculopathy in Pediatric Cardiac Transplant Patients.

Transplant coronary artery vasculopathy (TCAV) is the primary cause of late graft loss in pediatric heart transplant recipients. TCAV is diagnosed using angiography or intravascular ultrasound; however, noninvasive methods remain elusive. We sought to define patterns of myocardial mechanics in patients with TCAV and to determine whether this can detect TCAV before invasive methods. In this retrospective study, we queried our heart transplant database to identify all recipients with TCAV since 2006 (n = 41). Echoes were reviewed from the last normal catheterization and at TCAV diagnosis, and from time-matched transplant controls (n = 33) without TCAV. Peak global circumferential and longitudinal strain and systolic and diastolic strain rate (SSR and DSR) of the left ventricle were derived using velocity vector imaging. T tests were used to compare both groups longitudinally and between groups at both time points. Longitudinal strain, SSR, and DSR were diminished in the TCAV group compared to the transplant control group at both time points. No differences were found across time points in either group. Retrospective modeling using a longitudinal strain cutoff of 15 % on echoes 2 years prior to TCAV diagnosis predicted development or exclusion of TCAV with sensitivity of 53 %, specificity of 89 % with an area under the curve of 0.8. Decreases in longitudinal strain measurements demonstrate that alterations in myocardial mechanics occur in patients with TCAV at least 2 years prior to invasive diagnosis. These early changes may be due to microvascular disease. This modality could aid in earlier treatment and intervention for this challenging problem .

Acute ischaemic heart block in hypoplastic left heart syndrome.

In hypoplastic left heart syndrome, thrombosis of the native ascending aorta is rare and often fatal; there are no previously reported cases presenting with acute heart block. We review a case of native ascending aorta thrombosis in a 2-year-old boy with hypoplastic left heart syndrome, presenting with acute heart block. This case highlights the benefit of multi-modality imaging in complex cases.

Strain and Strain Rate Measured on Echocardiogram 1-3 Weeks after Starting Treatment Is Worse in Acute Dilated Cardiomyopathy Pediatric Patients with Poor Outcomes at One Year.

BACKGROUND: Predicting later outcome around time of diagnosis of acute dilated cardiomyopathy (DCM) is difficult. We hypothesized that strain and strain rate on initial and follow-up echoes were worse in patients with acute DCM from all causes with poor one-year outcomes. METHODS: This was a retrospective study including all patients with DCM aged 0-18 years with left ventricle dilation, low ejection fraction, or low fractional shortening on initial echo. Longitudinal and circumferential strain and systolic and diastolic strain rate were measured on echo at presentation, 1-3 weeks after presentation, and at 1 year. Patients were separated into "Stable" (survivors) and "Progressive" (referred for transplant or died) outcome groups, and results were analyzed to determine whether strain or strain rate at each echo was worse in the "Stable" group compared with the "Progressive" group. RESULTS: The patient population included patients with DCM from idiopathic causes, myocarditis, iron deficiency anemia, lupus, chemotherapy, and LV noncompaction. Longitudinal and circumferential strain and systolic strain rate were significantly better in the "Stable" (n = 7) compared with the "Progressive" (n = 8) outcome group on 1- to 3-week echo. Longitudinal strain more negative than -10% had 87% specificity and 100% sensitivity for predicting "stable" outcome (AUC 0.98), while circumferential strain more negative than -8% had 60% specificity and 100% sensitivity (AUC 0.83). CONCLUSIONS: Longitudinal and circumferential strain and systolic strain rate measured 1-3 weeks after starting therapy are worse in acute dilated cardiomyopathy patients with poor one-year outcomes. Further studies with less heterogeneity and more study subjects are needed.

Three-dimensional echocardiography enhances diagnostic accuracy of supramitral ring.

Real time three-dimensional echocardiography (RT3DE) allows for accurate morphological characterization of congenital heart disease and complements two-dimensional (2D) echocardiography. Accurate evaluation of the mitral valve and supramitral ring prior to surgery aids the surgeon in successful resection of the ring and repair of associated mitral valve abnormalities. We report a case of congenital mitral valve stenosis from a supramitral ring in a young child using real time three-dimensional transesophageal echocardiography.

Left ventricular strain and strain rates are decreased in children with normal fractional shortening after exposure to anthracycline chemotherapy.

BACKGROUND: Anthracycline chemotherapeutic agents carry the well-recognised risk of cardiotoxicity. Previous methods to evaluate cardiac function are useful, but have significant limitations. We sought to determine the left ventricular strain and strain rate of paediatric cancer patients with normal fractional shortening treated with anthracyclines using the latest ultrasound feature-tracking technology. PATIENTS AND METHODS: Echocardiograms on cancer patients before anthracycline exposure and following completion of treatment were retrospectively analysed using Velocity Vector Imaging software in the circumferential and longitudinal planes. The same analysis was performed on matched controls. Only patients with a fractional shortening ≥28% were included. RESULTS: In all, 71 patients were identified with an age at diagnosis of 10.5 ± 4.7 years. The time from diagnosis to follow-up was 3.9 ± 4.0 years and the cumulative anthracycline dose was 356 ± 106 mg/m². Following anthracycline exposure, paediatric cancer patients had a higher heart rate and a lower longitudinal strain, longitudinal diastolic strain rate, circumferential strain, and circumferential systolic and diastolic strain rate when compared with controls. Diastolic strain rate showed the greatest percent difference following anthracycline exposure versus controls. CONCLUSION: Despite having a normal fractional shortening, children exposed to anthracyclines have subclinical derangement of their left ventricular deformation as measured by decreases in strain and strain rate in both the circumferential and longitudinal axis. In particular, there was a profound decrease in diastolic strain rate following anthracycline exposure compared with controls. Whether the decline of strain or strain rate can predict future risk of developing cardiomyopathy requires further investigation.

Longitudinal myocardial deformation is selectively decreased after pediatric cardiac transplantation: a comparison of children 1 year after transplantation with normal subjects using velocity vector imaging.

The transplanted heart experiences numerous hemodynamic changes during and after cardiac transplantation. This study sought to evaluate the left ventricular myocardial mechanics in the pediatric heart transplant population using Velocity Vector Imaging (VVI). This study retrospectively evaluated 28 heart transplant recipients by echocardiography 12 months after transplantation. Echocardiograms from 28 age- and gender-matched subjects were used as a control group. Peak global longitudinal and circumferential left ventricular strain, systolic strain rate, and diastolic strain rate were obtained. Student's t tests were used to assess differences between the two groups (defined as p ≤ 0.05). The peak global left ventricular longitudinal strain was lower in the transplant group (17.21%) than in the control group (22.14%). The transplant and control groups did not differ significantly in terms of their peak global circumferential strain (20.28% vs. 20.79%, respectively). Similar results were observed for longitudinal and circumferential systolic and diastolic strain rates. The transplant patients showed statistically significant reductions in all peak global longitudinal measures compared with those of the control subjects. Circumferential myocardial deformation appears to be preserved in transplant recipients. This could suggest evidence of ischemia given the known myocardial fiber arrangement of longitudinal fibers toward the endocardial surface, which is also more distal in the coronary arterioles.

Patients with Fontan circulation have abnormal aortic wave propagation patterns: A wave intensity analysis study.

BACKGROUND: Elevated systemic afterload in patients with Fontan circulation may lead to impaired single ventricular function. Wave intensity analysis (WIA) enables evaluation of compression and expansion waves traveling through vasculature. We aimed to investigate the unfavorable wave propagation causing excessive afterload may be an important contributor to the overall single ventricle function and to the limited functional capacity in this patient population. METHODS: Patients with hypoplastic left heart syndrome (HLHS) (n = 25), single left ventricle (SLV) (n = 24), and normal controls (n = 10) underwent phase-contrast MRI based WIA analysis evaluated in the ascending aorta. Forward compression wave (FCW) representing dP/dt, backward compression wave (BCW) reflecting vascular stiffness, and forward decompression wave (FDW) representing LV relaxation were recorded and indexed to each other. RESULTS: FCW was lowest in HLHS patients (1098 mm5/s), and higher in the SLV group (1457 mm5/s), and controls (6457 mm5/s) (P < 0.001). BCW/FCW was increased in HLHS (0.22) and SLV (0.14) groups compared to controls (0.08) (P = 0.003). Peak VO2 correlated with FCW (R = 0.50, P = 0.015), stroke volume (R = 0.72, P < 0.001), and cardiac output (R = 0.44, P = 0.034). CONCLUSIONS: Patients with HLHS and SLV have unfavorable aortic WIA patterns with increased BCW/FCW ratio indicating increased systemic afterload due to retrograde compression waves. Reduced FCW and systolic MRI indices correlated with peak VO2 suggesting that abnormal systolic wave propagation may play a role in exercise intolerance for Fontan patients.

Patient and Family Experience in a Multidisciplinary Clinic for Children With Single-Ventricle Heart Disease.

Children with single-ventricle heart disease (SVHD) are at risk for morbidity across multiple organ systems. A single-ventricle multidisciplinary clinic (SVMDC) may address complex health-care needs by providing access to, and coordination among, pediatric subspecialties. However, the patient and family experience of multidisciplinary care for SVHD remains unexplored. We e-mailed a 26-question survey to families after an SVMDC visit, which included evaluation with subspecialists from cardiology, pulmonology, gastroenterology, neuropsychology, and pediatric psychology, as well as social activities during clinic. Responses were anonymized to protect privacy, and data were analyzed quantitatively and qualitatively. Over 3 years, 22% (27/122) of families completed the survey. Overall, families' experiences were positive, with 100% reporting that they would recommend the SVMDC to others. Qualitative themes emerged regarding logistics, multidisciplinary care, key takeaways from clinic, and connection-making with other families. A multidisciplinary clinic demonstrated overall acceptability and perceived benefit to families of children with SVHD. Considerations for mixed experiences regarding financial commitment and connection-making among parents are discussed, as are the benefits of the synergy achieved through multidisciplinary care.