Anomalous right ventricular muscle bands obstructing a large apical muscular ventricular septal defect: From fetal to post-natal three-dimensional assessment.

Anomalous right ventricle muscle bands and apical ventricular septal defect are two anomalies sometimes associated. We report a fetal diagnosis of a large apical ventricular septal defect, right intraventricular obstruction caused by anomalous muscle bands; consequently, the high right intraventricular pressure resulted in a right-to-left bulging of ventricular septum and moderate tricuspid regurgitation. Postnatal echocardiogram confirmed the fetal diagnosis and defined accurately the right ventricular anatomy through the three-dimensional echocardiographic assessment.

Acometimento miocárdico na Doença de Chagas: uma perspectiva a partir da avaliação pela ressonância magnética cardiovascular / Myocardial involvement in Chagas Disease: from the perspective of cardiovascular magnetic ressonance assessment

A doença de Chagas representa um importante problema de saúde pública, sobretudo nos países endêmicos da América Latina. Dentre suas apresentações clínicas, a cardiomiopatia crônica é a mais frequente. De patogênese multifatorial, o acometimento miocárdico pode levar à insuficiência cardíaca, a eventos tromboembólicos, a arritmias e à morte súbita. Nesse contexto, a ressonância magnética cardiovascular é um excelente método não invasivo para a investigação do dano miocárdico e a compreensão dos mecanismos e consequências relacionados às essas lesões. Com elevada resolução espacial e capacidade de caracterização tecidual, a ressonância magnética cardiovascular proporciona análise morfofuncional altamente confiável e possibilita a identificação de marcadores de risco de eventos adversos em pacientes com doença de Chagas, sendo de grande utilidade para o diagnóstico e o acompanhamento desses indivíduos na rotina clínica. (AU)

Chagas disease represents an important public health problem, especially in endemic countries in Latin America. Chronic cardiomyopathy is its most frequent clinical presentation. Myocardial involvement has a multifactorial pathogenesis and can lead to heart failure, thromboembolic events, arrhythmias, and sudden death. In this context, cardiovascular magnetic resonance imaging (CMR) is an excellent noninvasive method for investigating myocardial damage and understanding the mechanisms and consequences of these injuries. CMR has high spatial resolution and tissue characterization capacity, enabling a highly reliable morphofunctional analysis and the identification of risk markers for adverse events in patients with Chagas disease. This exam is very useful for the diagnosis and follow-up of these patients in the routine clinical setting. (AU)

The assessment of the fetal heart function using two-dimensional speckle tracking with a high frame rate.

BACKGROUND: Two-dimensional (2D) speckle tracking echography (STE) is a new diagnostic modality that allows for the assessment of myocardial deformation. The present study assessed the feasibility of fetal 2D-STE at a high frame rate and determined the reference values for left ventricular (LV)- and right ventricular (RV)- global longitudinal strain (GLS). METHODS: In this prospective study, 109 fetuses with gestational ages ranging from 18 to 38 weeks underwent 2D echocardiography between August 2018 and December 2019. All recordings were performed using the Aplio i800 (CANON Medical Systems Corporation, Tochigi, Japan) and a convex probe (4 MHz) for fetuses. RESULTS: Longitudinal peak systolic strain measurements were obtained in 98 of 109 healthy fetuses (90%). All ultrasound videos were recorded with a median frame rate of 172 (range, 100-274) frames/s. The LV-GLS was almost constant, regardless of the number of weeks since the second trimester (r = -0.0087, p = ns). The RV-GLS decreased significantly with gestational age (r = 0.39, p < 0.01). CONCLUSIONS: STE may be useful for quantifying the systolic myocardial function in a fetus.

Simultaneous Volumetric and Functional Assessment of the Right Ventricle in Hypoplastic Left Heart Syndrome After Fontan Palliation, Utilizing 3-Dimensional Speckle-Tracking Echocardiography.

BACKGROUND: Right ventricular (RV) volumetric and functional assessments are both crucial for the management of patients with hypoplastic left heart syndrome (HLHS). 3-dimensional echocardiography (3DE) for volume measurements and 2D speckle-tracking echocardiography (2D-STE) for strain analysis are performed separately. 3D-STE is capable of evaluating those parameters simultaneously and providing principal strain (PS), unifying the concepts of myofiber orientation and contraction into a single, maximal contractile direction. However, the application of 3D-STE to HLHS has not been studied and so became the aim of our study.Methods and Results:64 HLHS patients after Fontan palliation underwent 3D-STE analysis measuring RV end-diastolic volume index (EDVi), ejection fraction (EF), global PS (GPS), global circumferential strain (GCS), and global longitudinal strain (GLS). Volume measurements were compared between 3D-STE and 3DE, and strains were compared between 3D- and 2D-STE. EDVi and EF showed strong correlations between 3D-STE and 3DE (r=0.93 and 0.87, respectively). GCS and GLS showed moderate correlations between 3D- and 2D-STE (r=0.65 and 0.61, respectively). GPS showed highest magnitude and excellent correlation with EF (r=-0.95). CONCLUSIONS: Simultaneous volumetric and functional assessment by 3D-STE was a useful method in this HLHS cohort. PS is a promising parameter for evaluating the RV function of HLHS, which could be useful during longitudinal follow-up.

Parenting stress trajectories during infancy in infants with congenital heart disease: Comparison of single-ventricle and biventricular heart physiology.

OBJECTIVE: Parents of infants with congenital heart disease (CHD) experience increased parenting stress levels, potentially interfering with parenting practices and bear adverse family outcomes. Condition severity has been linked to parenting stress. The current study aimed to explore parenting stress trajectories over infancy in parents of infants with complex CHD, and to compare them by post-operative cardiac physiology. DESIGN: Data from a larger prospective cohort study was analyzed using longitudinal mixed-effects regression modeling. SETTING: Cardiac intensive care unit and outpatient clinic of a 480-bed children's hospital in the American North-Atlantic region. PARTICIPANTS: Parents of infants with complex CHD (n = 90). MEASURES: Parenting stress was measured via the parenting stress index-long form over four time points during infancy. RESULTS: Parents of infants with a single-ventricle heart experienced a decrease in total stress over time. Parents of infants with a biventricular heart experienced a decrease in attachment-related stress, and an increase in stress related to infant temperament over time. Parenting stress trajectories over time significantly differed between groups on infant temperamental subscales. CONCLUSIONS: Findings highlight stressful and potentially risky periods for parents of infants with complex CHD, and introduce additional illness-related and psychosocial/familial aspects to the parenting stress concept. Early intervention may promote parental adaptive coping and productive parenting practices in this population.

Outcome following heart transplant assessment in adults with congenital heart disease.

OBJECTIVES: Adults with congenital heart disease (ACHD) are a growing group with end-stage heart failure. We aim to describe the outcomes of ACHD patients undergoing assessment for orthotopic heart transplant (OHT). METHODS: Case notes of consecutive ACHD patients (>16 years) assessed for OHT between 2000 and 2016 at our centre were reviewed. Decision and outcome were reported as of 2017. Data were analysed in three groups: systemic left ventricle (LV), systemic right ventricle (RV) and single ventricle (SV). RESULTS: 196 patients were assessed (31.8 years, 27% LV, 29% RV, 44% SV). 89 (45%) patients were listed for OHT and 67 (34%) were transplanted. 41 (21%) were unsuitable or too high risk and 36 (18%) were too well for listing. Conventional surgery was undertaken in 13 (7%) and ventricular assist device in 17 (9%) with 7 (4%) bridged to candidacy. Survival from assessment was 84.2% at 1 year and 69.7% at 5 years, with no difference between groups. Patients who were considered unsuitable for OHT (HR 11.199, p<0.001) and listed (HR 3.792, p=0.030) were more likely to die than those who were considered too well. Assessments increased over the study period. CONCLUSIONS: The number of ACHD patients assessed for OHT is increasing. A third are transplanted with a small number receiving conventional surgery. Those who are unsuitable have a poor prognosis.

Comparison of inhospital outcomes of pediatric heart transplantation between single ventricle congenital heart disease and cardiomyopathy.

Data investigating the impact of household income and other factors on SV patient status-post-Fontan palliation after heart transplantation are lacking. We aim to evaluate factors affecting outcomes after OHT in this population. The PHIS database was interrogated for either SV or myocarditis/primary CM who were 4 years or older who underwent a single OHT. There were 1599 patients with a median age of 13.2 years (IQR: 9.3-16.1). Total hospital costs were significantly higher in the SV group ($408 000 vs $294 000, P < 0.0001), but as median household income increased, the risk of inhospital mortality, post-transplant LOS, and LOS-adjusted total hospital costs all decreased. The risk of inhospital mortality increased 6.5% per 1 year of age increase at the time of transplant. Patients in the SV group had significantly more diagnoses than those in the CM group (21 vs 15, P < 0.0001) and had longer total hospital LOSs as a result of longer post-transplant courses (25 days vs 15, P < 0.0001). Increased median household income and younger age are associated with decreased resource utilization and improved inhospital mortality in SV CHD patients who undergo OHT. In conclusion, earlier consideration of OHT in this population, coupled with improved selection criteria, may increase survival in this population.

Echocardiographic assessment of single-ventricle diastolic function and its correlation to short-term outcomes after the Fontan operation.

BACKGROUND/HYPOTHESIS/OBJECTIVES: Postoperative complications after the Fontan operation for single ventricle heart disease are common and include persistent pleural drainage and prolonged length of hospital stay (LOS). Diastolic ventricular dysfunction may increase risk for postoperative complications by raising central venous pressures. We sought to determine the relationship between preoperative echocardiographic measurements of diastolic function, including myocardial deformation imaging, on (a) preoperative invasive catheterization measurements and (b) postoperative outcomes after the Fontan procedure. DESIGN/METHODS: All patients that underwent Fontan procedure from 2011 to 2017 were included. Echocardiograms performed within 6 months prior to Fontan operation were evaluated. Measurements of ventricular global and diastolic strain and strain rate were performed offline with TomTec speckle tracking software. Other diastolic function measurements included atrioventricular valve inflow and annular tissue Doppler imaging. Diastolic function measurements were correlated with pre-Fontan catheterization measurements and postoperative Fontan outcomes using Spearman's rho. Multivariable logistic regression for a prolonged LOS (>75%ile for postoperative LOS) was performed to adjust for preoperative risk factors. RESULTS: A total of 141 patients were included in the study. Majority had single morphologic right ventricle (58.9%). Median age at time of Fontan was 3.4 years (IQR 2.9-4). Median hospital LOS was 9 days (IQR 7-11). Circumferential diastolic strain rate weakly correlated with LOS (rho = -0.21, P = .01). There was no correlation between any other diastolic strain measurements and pre-Fontan end-diastolic pressure or postoperative LOS. In multivariable analysis, E/E' was the only echo measurement that predicted prolonged hospital LOS (OR 1.4, 95%CI: 1.1-1.8, P = .003). CONCLUSION: Preoperative diastolic strain measurements did not have a strong association with postoperative Fontan outcomes. Increased E/E' ratio, however, did predict greater LOS after Fontan procedure, and may be useful in preoperative risk stratification. Future studies are needed to further assess the utility of diastolic strain imaging in the single-ventricle population.

Pulmonary hypertension in patients with a subaortic right ventricle: prevalence, impact and management.

OBJECTIVE: This study sought to determine the prevalence, predictors, prognostic relevance and evolution of pulmonary hypertension (PH) (mean pulmonary artery pressure ≥25 mm Hg) in adult patients with a subaortic right ventricle (RV) in a biventricular circulation (2V-RV). METHODS: We analysed retrospective data from patients with 2V-RV undergoing cardiac catheterisation in our centre between 2000 and 2018. Echocardiographic assessment of subpulmonary ventricular pressures (left ventricular systolic pressure (LVSP)), age and B-type natriuretic peptide (BNP) were assessed as PH screening tools. Kaplan-Meier curves examined time to a composite outcome of death, transplant or ventricular assist device (VAD). Data from repeat catheterisations were analysed to evaluate PH changes over time, including the effects of therapy. RESULTS: A total of 141 patients (median age 39 (IQR 33-45) years, 68% men) underwent 191 cardiac catheterisations. At baseline, 55% had PH (isolated postcapillary 24%, combined precapillary and postcapillary 26% and precapillary 5%). BNP (area under the curve 0.80; 95% CI 0.72 to 0.88; p<0.0001), but not age at catheterisation or echocardiographic estimates of LVSP were associated with the presence of PH. The absence of PH and BNP <100 pg/mL discriminated a subgroup at very low risk during short-term (2.5 (1.3-3.9) years) follow-up (p<0.0001). Diuretics, milrinone and VAD improved haemodynamics over time. CONCLUSION: PH is prevalent in patients with 2V-RV even when asymptomatic. It is difficult to identify by echocardiography and most importantly, is strongly associated with adverse outcomes. PH affects prognosis and transplant options for this patient group and yet is often amenable to treatment. Awareness of these results ought to lower the threshold for invasive haemodynamic assessment and may change the management of failing patients with 2V-RV.

Gated SPECT myocardial perfusion imaging with cadmium-zinc-telluride detectors allows real-time assessment of dobutamine-stress-induced wall motion abnormalities.

BACKGROUND: Left ventricular (LV) ejection fraction (EF) during high dobutamine stress (HD) by real-time gated-SPECT myocardial perfusion imaging (MPI) on a cadmium-zinc-telluride (CZT) gamma camera was validated versus cardiac magnetic resonance imaging (CMR). METHODS AND RESULTS: After injecting 99mTc-tetrofosmin (320 MBq) in 50 patients (mean age 64 +/- 11 years), EF at rest and post-stress as well as relevant changes in EF at HD (ΔEF ≥ 5%) were assessed. CZT and CMR rest EF values yielded an excellent correlation and agreement (r = 0.96; P < 0.001; Bland-Altman limits of agreement (BA): + 0 to 14.8%). HD EF acquisition was feasible using CZT and correlated better to HD CMR EF than did post-stress CZT EF (r = 0.85 vs 0.76, respectively, all P < 0.001). Agreement in ΔEF detection between HD CMR and immediate post-stress CZT (reflecting standard acquisition using conventional SPECT camera unable to scan during stress) was 45%, while this increased to 85% with real-time HD CZT scan. CONCLUSION: Real-time ultrafast dobutamine gated-SPECT MPI with a CZT device is feasible and provides accurate measurements of HD LV performance.

Assessment of global and regional strain left ventricular in patients with preserved ejection fraction after Fontan operation using a tissue tracking technique.

To evaluate the use of the tissue tracking (TT) technique to measure myocardial strain left ventricular in post-Fontan children with preserved ejection fraction (pEF). Nineteen (male/female, 10/9) patients with univentricular hearts after completion of the Fontan circulation (post-Fontan group) and 19 age- and gender-matched healthy children (control group) were retrospectively enrolled. Cardiovascular magnetic resonance (CMR) imaging was conducted on a 1.5-T MRI scanner. Global and regional strains of the left ventricle in post-Fontan patients (EF > 55%) and controls were obtained using CMR-TT software. The Mann-Whitney U test was used to compare parameters between the two groups. Correlation between EF and strain was investigated using Pearson correlation coefficients. The Bland-Altman method was used to identify the inter- and intra-observer agreement in measurement of global strain. Global longitudinal strain was lower in post-Fontan patients than in healthy controls (- 18.87 ± 4.61 vs. -19.72 ± 1.58; P = 0.54), though the difference was not statistically significant. Global circumferential strain and global radial strain were significantly lower in post-Fontan patients than in healthy controls (- 14.55 ± 3.79 vs. -19.91 ± 1.97; P < 0.001; and 29.62 ± 8.41 vs. 36.85 ± 5.95; P = 0.01; respectively). The regional circumferential strain (RCS) decrease was marked in regional segments compare with post-Fontan patients and controls (basal, - 11.81 ± 2.98 vs. - 16.21 ± 2.72, P < 0.001; mid, - 15.05 ± 3.31 vs. - 20.17 ± 2.28, P = 0.005; apical, - 16.86 ± 3.09 vs. - 23.37 ± 2.62, P < 0.001). All circumferential and longitudinal parameters had an inter-observer ICC of ≥ 0.85, but this coefficient was lower for radial parameters. CMR-TT appears to be a feasible technique for identification of early myocardial dysfunction in post-Fontan with pEF.

Longitudinal Assessment of Outcome From Prenatal Diagnosis Through Fontan Operation for Over 500 Fetuses With Single Ventricle-Type Congenital Heart Disease: The Philadelphia Fetus-to-Fontan Cohort Study.

Background Prenatal diagnosis of single ventricle-type congenital heart disease is associated with improved clinical courses. Prenatal counseling allows for optimal delivery preparations and opportunity for prenatal intervention. Expectant parents frequently ask what the likelihood of survival through staged palliation is and the factors that influence outcome. Our goal was specifically to quantify peri- and postnatal outcomes in this population. Methods and Results We identified all patients with a prenatal diagnosis of single ventricle-type congenital heart disease presenting between July 2004 and December 2011 at our institution. Maternal data, fetal characteristics, and data from the postnatal clinical course were collected for each patient. Kaplan-Meier curves and multivariate analysis with logistic regression were used to evaluate variables associated with decreased transplant-free survival. Five hundred two patients were identified, consisting of 381 (76%) right ventricle- and 121 left ventricle-dominant lesions. After prenatal diagnosis, 42 patients did not follow up at our center; 79 (16%) chose termination of pregnancy, and 11 had intrauterine demise with 370 (74%) surviving to birth. Twenty-two (6%) underwent palliative care at birth. Among 348 surviving to birth with intention to treat, 234 (67%) survived to at least 6 months post-Fontan palliation. Presence of fetal hydrops, right ventricle dominance, presence of extracardiac anomalies, and low birthweight were significantly associated with decreased transplant-free survival. Conclusions In patients with a prenatal diagnosis of single ventricle-type congenital heart disease and intention to treat, 67% survive transplant-free to at least 6 months beyond Fontan operation. An additional 5% survive to 4 years of age without transplant or Fontan completion. Fetuses with right ventricle-dominant lesions, extracardiac anomalies, hydrops, or low birthweights have decreased transplant-free survival.

Neighborhood Socioeconomic Status and Outcomes Following the Norwood Procedure: An Analysis of the Pediatric Heart Network Single Ventricle Reconstruction Trial Public Data Set.

BACKGROUND: Children with single ventricle heart disease require frequent interventions and follow-up. Low socioeconomic status (SES) may limit access to high-quality care and place these children at risk for poor long-term outcomes. METHODS AND RESULTS: Data from the SVR (Pediatric Heart Network Single Ventricle Reconstruction Trial Public Use) data set were used to examine the relationship of US neighborhood SES with 30-day and 1-year mortality or cardiac transplantation and length of stay among neonates undergoing the Norwood procedure (n=525). Crude rates of death or transplantation at 1 year after Norwood were highest for patients living in neighborhoods with low SES (lowest tertile 37.0% versus middle tertile 31.0% versus highest tertile 23.6%, P=0.024). After adjustment for patient demographics, birth characteristics, and anatomy, patients in the highest SES tertile had significantly lower risk of death or transplant than patients in the lowest SES tertile (hazard ratio 0.62, 95% confidence interval, 0.40, 0.96). When SES was examined continuously, the hazard of 1-year death or transplant decreased steadily with increasing neighborhood SES. Hazard ratios for 30-day transplant-free survival and 1-year transplant-free survival were similar in magnitude. There were no significant differences in length of stay following the Norwood procedure by SES. CONCLUSIONS: Low neighborhood SES is associated with worse 1-year transplant-free survival after the Norwood procedure, suggesting that socioeconomic and environmental factors may be important determinants of outcome in critical congenital heart disease. Future studies should investigate aspects of SES and environment amenable to intervention. CLINICAL TRIAL REGISTRATION: URL:http://www.clinicaltrials.gov> http://www.clinicaltrials.gov. Unique identifier: NCT00115934.

Hospital Inpatient Costs for Single Ventricle Patients Surviving the Fontan Procedure.

We estimated the inpatient resource use for a Fontan patient from birth to adulthood and explored factors that might induce cost differences (2014 US dollar). Inpatient costing records from 4 hospitals with greatest numbers of Fontan patients in Australia and New Zealand were linked with the Fontan registry database. Inpatient records between July 1995 and September 2014 for 420 Fontan patients were linked, and the most frequent primary diagnoses were hypoplastic left heart syndrome (20.7%), tricuspid atresia (19.7%), and double inlet left ventricle (17.1%). The mean hospital cost for a Fontan patient from birth to 18 years of age was estimated to be $390,601 (95% confidence interval [CI] $264,703 to $516,499), corresponding to 164 (95% CI 98 to 231) inpatient days. The cost incurred from birth through to Fontan completion (the staged procedures period) was $219,482 (95% CI $202,410 to $236,553) and the cost thereafter over 15 years was $146,820 (95% CI $44,409 to $249,231), corresponding to 82 (95% CI 72 to 92) and 65 (95% CI 18 to 112) inpatient days, respectively. Costs were higher in male and hypoplastic left heart syndrome patients in the staged procedures period (p <0.001). Having fenestration was associated with higher costs in the staged procedures period (p <0.001) and lower cost after Fontan over 15 years (p = 0.66). In conclusion, patients with single ventricle congenital heart disease continue to demand considerable inpatient resources after the staged procedures period. Over 40% of the pediatric hospital costs for Fontan patients were estimated to occur after the last planned surgery.

A multi-institutional study of factors affecting resource utilisation following the Fontan operation.

The few studies evaluating data on resource utilisation following the Fontan operation specifically are outdated. We sought to evaluate resource utilisation and factors associated with increased resource use after the Fontan operation in a contemporary, large, multi-institutional cohort. This retrospective cohort study of children who had the Fontan between January, 2004 and June, 2013 used the Pediatric Health Information Systems Database. Generalised linear regression analyses evaluated factors associated with resource use. Of 2187 Fontan patients included in the study, 62% were males. The median age at Fontan was 3.2 years (inter-quartile range (IQR): 2.6-3.8). The median length of stay following the Fontan was 9 days (IQR: 7-14). The median costs and charges in 2012 dollars for the Fontan operation were $93,900 (IQR: $67,800-$136,100) and $156,000 (IQR: $112,080-$225,607), respectively. Postoperative Fontan mortality (30 days) was 1% (n=21). Factors associated with increased resource utilisation included baseline and demographic factors such as region, race, and renal anomaly, factors at the bidirectional Glenn such as seizures, valvuloplasty, and surgical volume, number of admissions between the bidirectional Glenn and the Fontan, and factors at the Fontan such as surgical volume and age at Fontan. The most strongly associated factors for both increased Fontan length of stay and increased Fontan charges were number of bidirectional Glenn to Fontan admissions (p<0.001) and Fontan surgical volume per year (p<0.001). As patient characteristics and healthcare-related delivery variables accounted for most of the factors predicting increased resource utilisation, changes should target healthcare delivery factors to reduce costs in this resource-intensive population.

National In-Hospital Outcomes of Pregnancy in Women With Single Ventricle Congenital Heart Disease.

Most patients with single ventricle (SV) congenital heart disease are expected to survive to adulthood. Women with SV are often counseled against pregnancy; however, data on pregnancies in these women are lacking. We sought to evaluate in-hospital outcomes of pregnancy in women with SV. We used nationally representative data from the 1998 to 2012 National Inpatient Sample to identify women ≥18 years of age admitted to the hospital with International Classification of Diseases-9th Revision codes for an intrauterine pregnancy and a diagnosis of hypoplastic left heart syndrome, tricuspid atresia, or common ventricle. A matched comparison group without a diagnosis of congenital heart disease or pulmonary hypertension was identified from the database. National estimates of hospitalizations were calculated. Length of stay, hospital charges, and complications were analyzed and compared between groups. Charge data were adjusted to 2012 dollars. There were 282 admissions of pregnant women with SV (69% with deliveries) and 1,405 admissions in the control group (88% with deliveries). Vaginal delivery was more common in SV (74% vs 71%, p <0.001). Length of stay (4.1 ± 0.91 vs 2.8 ± 0.18 days, p <0.001) and charges ($30,787 ± 8,109 vs $15,536 ± 1,006, p <0.0001) were higher in the SV group. Complications occurred in most SV admissions and were more common in the SV group than in the control group. No deaths occurred. Cardiovascular complications occurred in 25% of pregnancy-related hospitalizations, although in-hospital pregnancy-related death is rare. Vaginal delivery is common in these patients. These data suggest that pregnancy and vaginal delivery can be tolerated in women with SV, although the risk for a cardiovascular event is significantly higher than in the general population.

Clinical Application and Multidisciplinary Assessment of Three Dimensional Printing in Double Outlet Right Ventricle With Remote Ventricular Septal Defect.

BACKGROUND: Double outlet right ventricle (DORV) with two well-developed ventricles and with a remote ventricular septal defect (VSD) may present a therapeutic challenge. Echocardiographic imaging of such complex cases does not always provide all of the information required to decide on an operative approach (biventricular or univentricular) and to design an intracardiac baffle to direct left ventricular outflow through the VSD and to the aorta for biventricular repair. A three dimensional (3D) printed model of the heart based upon data derived from computed tomography (CT) or magnetic resonance imaging (MRI) may contribute to a more complete appreciation of the intracardiac anatomy. METHODS: From April to September 2015, six consecutive patients with DORV and remote VSD underwent CT/MRI scans. Data sets from these studies were used to generate life-size 3D models using a 3D printer. We compared the assessment of 3D printed heart model findings with information obtained from echocardiography, CT, or cardiac MRI and with details of the surgeon's intraoperative direct observations when available. Quantification of the information provided by the 3D model was achieved using a unique scale that was created for the purpose of this study. The accuracy and utility of information derived preoperatively from the models were assessed. RESULTS: Six data sets from six patients were analyzed. Five data sets could be successfully used to create sandstone models using 3D printing. The five patients ranged from 7 months to 11 years of age and weighed 6.7 to 26 kg. The spatial orientation of the heart in the thorax, the relationships of the great arteries and the semilunar valves, the size and location of the VSD were well appreciated in all models, as were the anticipated dimensions and orientation of a surgically planned interventricular baffle. Three of the five patients underwent successful biventricular repair. CONCLUSION: The 3D printed models scored higher than conventional imaging, with respect to most aspects of the surface spatial orientation and intracardiac anatomy. The models are a useful adjunct in preoperative assessment of complex DORV. The unique scale helps quantify the advantages and limitations of the 3D heart models.

Assessment of Kidney Function in Survivors Following Fontan Palliation.

OBJECTIVE: The Fontan operation is a palliative procedure for congenital single ventricle heart disease. Long-term kidney function in this cohort is not well-known. Our aim was to evaluate renal function in long-term survivors post-Fontan palliation, and we hypothesize that this cohort will have a higher prevalence of chronic kidney disease (CKD) compared to controls. DESIGN: We performed a retrospective cohort study of 68 subjects evaluated through the Single Ventricle Survivorship Program at the Children's Hospital of Philadelphia between July 2010 and December 2014 compared to 70 healthy children similar in age and sex. Primary outcome was CKD, defined as estimated glomerular filtration rate (eGFR) <90 mL/min/1.73 m2 using creatinine and cystatin C-based estimating equations. Secondary outcomes included proteinuria and elevated intact parathyroid hormone. RESULTS: The Fontan cohort included 68 subjects with median age 13 years (IQR 9.0, 17.3) who were median 11.1 years (IQR 6.5, 15.7) post-Fontan palliation. This cohort was compared to 70 healthy individuals (median age 15.5 years (IQR 12.5, 18.3). Although median eGFRs were comparable: 102.6 vs. 101.9 mL/min/1.73 m2 (P = .89) in Fontan vs. healthy subjects <18 years of age (Full CKiD equation), and 128.5 vs. 129.7 mL/min/1.73 m2 (P = .56) in Fontan vs. healthy subjects ≥18 years of age (CKD-EPI creatinine and cystatin formula); 10% of Fontan subjects had an eGFR<90 mL/min/1.73 m2 . Median intact parathyroid hormone level was higher at 59.4 pg/mL (IQR 43.0, 83.1) in the Fontan group compared to 23.4 pg/mL (IQR 16.7, 30.0) in controls (P ≤ .001). Proteinuria was present in 10% of the Fontan group compared to 4.7% in controls (P = .27). CONCLUSION: Ten percent of long-term survivors post-Fontan palliation had eGFR <90 ml/min/1.73 m2 , and higher median parathyroid hormone levels compared to controls. Taken together, these measures may indicate early kidney disease. Future studies will focus on longitudinal assessment of kidney function and evaluation of risk factors for CKD post-Fontan palliation.