RESUMEN

BACKGROUND: Despite a rigorous screening process, including cardiac catheterization, a subset of patients with a single right ventricle (SRV) demonstrates suboptimal short-term outcomes after the Fontan operation. The goal of this study was to perform a comprehensive assessment of diastolic function in pre-Fontan patients with an SRV using invasive reference-standard measures and determine their associations with post-Fontan outcomes. METHODS AND RESULTS: Children aged 2 to 6 years with SRV physiology undergoing pre-Fontan heart catheterization were recruited prospectively. Patients were divided into those who had an optimal or suboptimal outcome. A suboptimal outcome was defined as length of stay ≥14 days or heart transplant/cardiac death in first year after Fontan. Patients underwent pressure-volume loop analysis using reference-standard methods. The measure of ventricular stiffness, ß, was obtained via preload reduction. Cardiac magnetic resonance imaging for extracellular volume and serum draws for matrix metalloproteinase activity were performed. Of 19 patients with an SRV, 9 (47%) had a suboptimal outcome. Mean age was 4.2±0.7 years. Patients with suboptimal outcomes had lower ventricular stiffness (0.021 [0.009-0.049] versus 0.090 [0.031-0.118] mL-1; P=0.02), lower extracellular volume (25% [28%-32%] versus 31% [28%-33%]; P=0.02), and lower matrix metalloproteinase-2 (90 [79-104] versus 108 [79-128] ng/mL; P=0.01) compared with patients with optimal outcomes. The only invasive measure that had an association with suboptimal outcome was ß (P=0.038). CONCLUSIONS: Patients with an SRV with suboptimal outcome after the Fontan operation had lower ventricular stiffness and evidence of maladaptive extracellular matrix metabolism compared with patients with optimal outcome. This appears to be a novel phenotype that may have important clinical implications and requires further study.

Asunto(s)

Procedimiento de Fontan , Ventrículos Cardíacos , Fenotipo , Humanos , Procedimiento de Fontan/efectos adversos , Preescolar , Masculino , Femenino , Niño , Ventrículos Cardíacos/fisiopatología , Ventrículos Cardíacos/diagnóstico por imagen , Ventrículos Cardíacos/anomalías , Estudios Prospectivos , Resultado del Tratamiento , Cateterismo Cardíaco , Función Ventricular Derecha/fisiología , Trasplante de Corazón , Metaloproteinasa 2 de la Matriz/sangre , Corazón Univentricular/cirugía , Corazón Univentricular/fisiopatología , Corazón Univentricular/diagnóstico por imagen , Cardiopatías Congénitas/cirugía , Cardiopatías Congénitas/fisiopatología , Factores de Tiempo

RESUMEN

Patients with functionally univentricular hearts are usually palliated surgically. There have been several reports of successful attempts to complete the Fontan procedure without surgery. The pathways created at the time of the preconditioning were largely reminiscent of the lateral tunnel Fontan. However, this approach is still confidentially limited to a small number of centers. In 2013, we designed a circuit that mimics the actual surgical technique of extracardiac total cavopulmonary connection to allow for transcatheter completion in an animal study. A polytetrafluoroethylene conduit was connected between the pulmonary artery and the inferior vena cava (IVC). The superior anastomosis was occluded to avoid flow between IVC and superior vena cava (SVC). The conduit was connected to the right atrium (RA) and a large fenestration was created to allow free flow from the IVC to the RA. Extrapolating our approach, a center reported the successful transcatheter completion of an extracardiac Fontan in a 6-year-old child. However, this technique is not directly transposable to our population of patients who require preconditioning in infancy. We report here an innovative extension of this technique that may allow preparing patients in infancy, ideally at the time of the Glenn in the future, to receive an extracardiac Fontan at 2 years/11 kg without additional surgery.

Asunto(s)

Cateterismo Cardíaco , Procedimiento de Fontan , Cardiopatías Congénitas , Procedimiento de Fontan/efectos adversos , Cateterismo Cardíaco/instrumentación , Humanos , Cardiopatías Congénitas/cirugía , Cardiopatías Congénitas/fisiopatología , Cardiopatías Congénitas/diagnóstico por imagen , Arteria Pulmonar/cirugía , Arteria Pulmonar/fisiopatología , Arteria Pulmonar/diagnóstico por imagen , Vena Cava Inferior/diagnóstico por imagen , Vena Cava Inferior/cirugía , Vena Cava Inferior/fisiopatología , Politetrafluoroetileno , Corazón Univentricular/cirugía , Corazón Univentricular/fisiopatología , Corazón Univentricular/diagnóstico por imagen , Diseño de Prótesis , Resultado del Tratamiento , Hemodinámica , Animales , Prótesis Vascular

RESUMEN

Although the terms "single ventricle" and "univentricular heart" are frequently used to describe a variety of complex congenital heart defects, in fact, nearly all hearts have two ventricles, although one of the two may be too small to be functional. A better term for these hearts would therefore be "functional single ventricle."

Asunto(s)

Atresia Tricúspide , Corazón Univentricular , Humanos , Ventrículos Cardíacos/fisiopatología , Ventrículos Cardíacos/anomalías , Atresia Tricúspide/cirugía , Atresia Tricúspide/fisiopatología , Atresia Tricúspide/diagnóstico por imagen , Corazón Univentricular/cirugía , Corazón Univentricular/fisiopatología , Corazón Univentricular/diagnóstico por imagen , Corazón Univentricular/terapia , Corazón Univentricular/genética

RESUMEN

Background and aims of the study A functionally single ventricle (FSV) refers to a group of congenital heart defects that are not amenable for biventricular correction. The Fontan operation is utilized as surgical treatment for most of FSV patients. The evaluation of FSV function is extremely difficult due to its unique pathophysiology. This study aimed to explore the efficacy of speckle tracking echocardiography (STE) parameters measured at rest and during exercise for comprehensive assessment of univentricular heart. METHODS: We enrolled 37 patients with a functionally single ventricle after the Fontan operation, hospitalized in the Department of Congenital Heart Defects between years 2019 and 2021.The echocardiographic stress tests were performed in the Echocardiography Laboratory of the Congenital Heart Defects Department. The study was conducted on a bicycle ergometer in a semi-recumbent position. The parameters obtained by speckle tracking echocardiography (STE): the longitudinal strain of the FSV free wall (Ɛ) and the longitudinal strain of myocardial layers: subendocardial, medial and subepicardial were analyzed. A transmural longitudinal strain gradient (TG) was calculated as the difference between longitudinal deformation of the subendocardial and subepicardial layers. Current results of cardiac magnetic resonance imaging (CMR) and cardio-pulmonary test (CPET) were also incorporated. Demographic data, past interventions, pharmacological treatment and comorbidities were extracted from medical records. RESULTS: Ɛ at rest and during exercise were not related to the parameters of physical capacity obtained on CPET nor to the CMR results. The transmural strain gradient was dependent on physical performance parameter- peak oxygen uptake- and related to the FSV ejection fraction calculated by magnetic resonance imaging. CONCLUSIONS: The transmural strain gradient and FSV free wall strain are readily measurable and suitable for evaluating single ventricle function. The TG is positively correlated with peak oxygen uptake during the cardiopulmonary test and with the ejection fraction derived from cardiac magnetic resonance imaging. The applicability of these findings in patients undergoing the Fontan procedure warrants further exploration.

Asunto(s)

Ecocardiografía , Procedimiento de Fontan , Humanos , Procedimiento de Fontan/métodos , Masculino , Femenino , Ecocardiografía/métodos , Adolescente , Ventrículos Cardíacos/diagnóstico por imagen , Ventrículos Cardíacos/fisiopatología , Ventrículos Cardíacos/anomalías , Adulto , Corazón Univentricular/cirugía , Corazón Univentricular/diagnóstico por imagen , Cardiopatías Congénitas/cirugía , Cardiopatías Congénitas/diagnóstico por imagen , Niño , Adulto Joven , Prueba de Esfuerzo/métodos , Cuidados Paliativos/métodos

RESUMEN

BACKGROUND: Hybrid stage I palliation (HS1P) is an alternative approach for initial palliation in hypoplastic left heart syndrome (HLHS) patients. Unlike surgical stage I palliation where atrial septectomy is routinely performed, atrial septal intervention (ASI) during HS1P is variable. In this study, we described our experience with ASI in single ventricle (SV) patients who underwent HS1P and identified factors associated with need for ASI after HS1P. METHODS: Data were retrospectively collected for all HLHS patients who underwent HS1P at our center over the past 12 years. We evaluated ASIs performed during the HS1P (intra-HS1P ASI) and ASIs performed during the period from HS1P to the subsequent surgical stage, either interval Norwood stage I or comprehensive stage II (post-HS1P ASI). Patient factors and procedural data were compared to identify factors associated with undergoing post-HS1P ASI and the impact of ASI on patient outcomes was evaluated. RESULTS: Of 50 SV patients included, 23 (46%) underwent intra-HS1P ASI and 26 (52%) underwent post-HS1P ASI. Need for post-HS1P ASI was lower among patients who had an intra-HS1P ASI as compared to those who did not (30% vs. 70%; p = 0.005). There were no significant differences in short or Midterm outcomes between patients who underwent intra-HS1P ASI or post-HS1P ASI and their counterparts. CONCLUSIONS: ASI is common both during and after HS1P but is generally well tolerated and type of ASI does not significantly impact overall patient outcomes. Our findings suggest that the current approach of individualizing management of ASI in the HS1P population is effective and safe.

Asunto(s)

Cateterismo Cardíaco , Síndrome del Corazón Izquierdo Hipoplásico , Procedimientos de Norwood , Cuidados Paliativos , Humanos , Síndrome del Corazón Izquierdo Hipoplásico/cirugía , Síndrome del Corazón Izquierdo Hipoplásico/fisiopatología , Estudios Retrospectivos , Resultado del Tratamiento , Femenino , Masculino , Factores de Tiempo , Factores de Riesgo , Procedimientos de Norwood/efectos adversos , Recién Nacido , Cateterismo Cardíaco/efectos adversos , Cateterismo Cardíaco/instrumentación , Tabique Interatrial/diagnóstico por imagen , Tabique Interatrial/fisiopatología , Tabique Interatrial/cirugía , Lactante , Corazón Univentricular/cirugía , Corazón Univentricular/fisiopatología , Corazón Univentricular/diagnóstico por imagen

RESUMEN

OBJECTIVE: The study objective was to analyze survival and incidence of Fontan completion of patients with single-ventricle and concomitant unbalanced atrioventricular septal defect. METHODS: Data from 4 Dutch and 3 Belgian institutional databases were retrospectively collected. A total of 151 patients with single-ventricle atrioventricular septal defect were selected; 36 patients underwent an atrioventricular valve procedure (valve surgery group). End points were survival, incidence of Fontan completion, and freedom from atrioventricular valve reoperation. RESULTS: Median follow-up was 13.4 years. Cumulative survival was 71.2%, 70%, and 68.5% at 10, 15, and 20 years, respectively. An atrioventricular valve procedure was not a risk factor for mortality. Patients with moderate-severe or severe atrioventricular valve regurgitation at echocardiographic follow-up had a significantly worse 15-year survival (58.3%) compared with patients with no or mild regurgitation (89.2%) and patients with moderate regurgitation (88.6%) (P = .033). Cumulative incidence of Fontan completion was 56.5%, 71%, and 77.6% at 5, 10, and 15 years, respectively. An atrioventricular valve procedure was not associated with the incidence of Fontan completion. In the valve surgery group, freedom from atrioventricular valve reoperation was 85.7% at 1 year and 52.6% at 5 years. CONCLUSIONS: The long-term survival and incidence of Fontan completion in our study were better than previously described for patients with single-ventricle atrioventricular septal defect. A concomitant atrioventricular valve procedure did not increase the mortality rate or decrease the incidence of Fontan completion, whereas patients with moderate-severe or severe valve regurgitation at follow-up had a worse survival. Therefore, in patients with single-ventricle atrioventricular septal defect when atrioventricular valve regurgitation exceeds a moderate degree, the atrioventricular valve should be repaired.

Asunto(s)

Procedimientos Quirúrgicos Cardíacos , Defectos de los Tabiques Cardíacos/cirugía , Corazón Univentricular/cirugía , Bélgica/epidemiología , Procedimientos Quirúrgicos Cardíacos/efectos adversos , Procedimientos Quirúrgicos Cardíacos/mortalidad , Anuloplastia de la Válvula Cardíaca , Bases de Datos Factuales , Femenino , Procedimiento de Fontan , Defectos de los Tabiques Cardíacos/diagnóstico por imagen , Defectos de los Tabiques Cardíacos/mortalidad , Defectos de los Tabiques Cardíacos/fisiopatología , Mortalidad Hospitalaria , Humanos , Incidencia , Masculino , Países Bajos/epidemiología , Reoperación , Estudios Retrospectivos , Medición de Riesgo , Factores de Riesgo , Factores de Tiempo , Resultado del Tratamiento , Corazón Univentricular/diagnóstico por imagen , Corazón Univentricular/mortalidad , Corazón Univentricular/fisiopatología

RESUMEN

OBJECTIVES: The gold standard to obtain pressure-volume relations (PVR) of the heart, the conductance technology (PVRCond), is rarely used in children. PVR can also be obtained by 3D-echocardiography volume data combined with simultaneously measured pressure data by a mini pressure-wire (PVR3DE). We sought to investigate the feasibility of both methods in patients with univentricular hearts and to compare them, including hemodynamic changes. METHODS: We studied 19 patients (age 2-29 years). PVR3DE and PVRCond were assessed under baseline conditions and stimulation with dobutamine. RESULTS: Obtaining PVR3DE was successful in all patients. Obtaining PVRCond was possible in 15 patients during baseline (79%) and in 12 patients under dobutamine (63%). Both methods showed that end-systolic elastance (Ees) and arterial elastance (Ea) increased under dobutamine and that Tau showed a statistically significant decrease. Intraclass correlation (95% confidence interval) showed moderate to good agreement between methods: Ees: 0.873 (0.711-0.945), Ea: 0.709 (0.336-0.873), Tau: 0.867 (0.697-0.942). Bland-Altman analyses showed an acceptable bias with wider limits of agreement: Ees: 1.63 mmHg/ml (-3.83-7.08 mmHg/ml), Ea: 0.53 mmHg/ml (-5.23-6.28 mmHg/ml), Tau: -0,76 ms (-10.73-9.21 ms). CONCLUSION: Changes of PVR-specific parameters under dobutamine stimulation were reflected in the same way by both methods. However, the absolute values for these parameters could vary between methods and, therefore, methods are not interchangeable. Obtaining PVR3DE in a single ventricle was easier, faster and more successful than PVRCond. PVR3DE provides a promising and needed alternative to the conductance technology for the assessment of cardiac function in univentricular hearts.

Asunto(s)

Presión Sanguínea , Ecocardiografía Tridimensional , Corazón Univentricular/diagnóstico por imagen , Corazón Univentricular/fisiopatología , Adolescente , Adulto , Niño , Preescolar , Femenino , Humanos , Masculino , Adulto Joven

RESUMEN

OBJECTIVE: While the surgical stages of single ventricle (SV) palliation serve to separate pulmonary venous and systemic venous return, and to volume-unload the SV, staged palliation also results in transition from parallel to series circulation, increasing total vascular resistance. How this transition affects pressure loading of the SV is as yet unreported. METHODS: We performed a retrospective chart review of Stage I, II, and III cardiac catheterization (CC) and echocardiographic data from 2001-2017 in all SV pts, with focus on systemic, pulmonary, and total vascular resistance (SVR, PVR, TVR respectively). Longitudinal analyses were performed with log-transformed variables. Effects of SVR-lowering medications were analyzed using Wilcoxon rank-sum testing. RESULTS: There were 372 total patients who underwent CC at a Stage I (median age of 4.4 months, n=310), Stage II (median age 2.7 years, n = 244), and Stage III (median age 7.3 years, n = 113). Total volume loading decreases with progression to Stage III (P< 0.001). While PVR gradually increases from Stage II to Stage III, and SVR increases from Stage I to Stage III, TVR dramatically increases with progress towards series circulation. TVR was not affected by use of systemic vasodilator therapy. TVR, PVR, SVR, and CI did not correlate with indices of SV function at Stage III. CONCLUSIONS: TVR steadily increases with an increasing contribution from SVR over progressive stages. TVR was not affected by systemic vasodilator agents. TVR did not correlate with echo-based indices of SV function. Further studies are needed to see if modulating TVR can improve exercise tolerance and outcomes.

Asunto(s)

Enfermedades Asintomáticas/terapia , Procedimientos Quirúrgicos Cardíacos , Corazón Univentricular , Resistencia Vascular/fisiología , Circulación Sanguínea , Procedimientos Quirúrgicos Cardíacos/efectos adversos , Procedimientos Quirúrgicos Cardíacos/métodos , Procedimientos Quirúrgicos Cardíacos/estadística & datos numéricos , Niño , Preescolar , Progresión de la Enfermedad , Ecocardiografía/métodos , Femenino , Humanos , Lactante , Estudios Longitudinales , Masculino , Evaluación de Procesos y Resultados en Atención de Salud , Cuidados Paliativos/métodos , Estudios Retrospectivos , Tiempo , Corazón Univentricular/diagnóstico por imagen , Corazón Univentricular/fisiopatología , Corazón Univentricular/cirugía , Vasodilatadores/uso terapéutico , Función Ventricular

RESUMEN

Single ventricle hearts palliated with the Fontan operation present complications later in life as a result of increased venous pressures and abnormal ventricle function. Wave intensity analysis uses measurements of blood velocity and pressure to represent arterial hemodynamics as summations of energy waves. This methodology could potentially be a useful tool in assessment of Fontan patients. The clinical value of wave intensity parameters was utilized to evaluate the functional performance of the single ventricle in Fontan patients. A retrospective analysis of invasive hemodynamic data was retrospectively obtained from routine cardiac catheterization of patients with Fontan circulation (n = 20) and comparison to those with biventricular circulation (n = 10) who presented to the catheterization laboratory for closure of small patent ductus arteriosus (PDAs). Wave intensity analysis and wave energy flux was calculated using aortic pressure waveforms and echocardiography aortic Doppler measurements as previously described. Significant differences were seen in the peak forward compression wave (p = 0.013), early systolic energy flux (p = 0.005) and the systolic and diastolic ratio (p = 0.006) in Fontan patients versus controls. Within the Fontan group, there was a positive correlation (0.54, p = 0.02) between the wave speed and pulmonary vascular resistance. Early systolic energy flux was a potential individual indicator of a Fontan patients heart failure classification (AUC = 0.71). Wave intensity analysis could be a useful tool in screening Fontan patients and predicting clinical outcomes and Fontan failure. Future prospective analyses of Fontan hemodynamics and WIA are needed.

Asunto(s)

Presión Arterial , Ecocardiografía Doppler/métodos , Procedimiento de Fontan/efectos adversos , Corazón Univentricular/cirugía , Función Ventricular , Adolescente , Cateterismo Cardíaco/métodos , Niño , Preescolar , Diástole , Conducto Arterioso Permeable/terapia , Femenino , Procedimiento de Fontan/métodos , Insuficiencia Cardíaca/diagnóstico , Insuficiencia Cardíaca/etiología , Ventrículos Cardíacos/diagnóstico por imagen , Ventrículos Cardíacos/fisiopatología , Hemodinámica , Humanos , Lactante , Masculino , Análisis de la Onda del Pulso/métodos , Estudios Retrospectivos , Corazón Univentricular/diagnóstico por imagen , Corazón Univentricular/fisiopatología , Adulto Joven

RESUMEN

We developed models of pulmonary artery (PA) size in Fontan patients as a function of age and body surface area (BSA) using linear regression and breakpoint analyses based on data from 43 Fontan patients divided into two groups: the extracardiac conduit (ECC) group (n = 24) and the non-ECC group (n = 19). Model predictions were compared against those of a non-Fontan control group (n = 18) and published literature. We observed strong positive correlations of the mean PA diameter with BSA (r = 0.9, p < 0.05) and age (r = 0.88, p < 0.05) in the ECC group. The absolute percentage differences between our BSA and age model predictions against published literature were less than 16% and 20%, respectively. Predicted PA size for Fontan patients was consistently smaller than the control group. These models may serve as useful references for clinicians and be utilized to construct 3D anatomic models that correspond to patient body size or age.

Asunto(s)

Procedimiento de Fontan , Angiografía por Resonancia Magnética , Modelación Específica para el Paciente , Arteria Pulmonar/diagnóstico por imagen , Arteria Pulmonar/cirugía , Corazón Univentricular/cirugía , Adolescente , Adulto , Factores de Edad , Superficie Corporal , Niño , Preescolar , Femenino , Procedimiento de Fontan/efectos adversos , Hemodinámica , Humanos , Masculino , Modelos Anatómicos , Modelos Cardiovasculares , Valor Predictivo de las Pruebas , Arteria Pulmonar/fisiopatología , Circulación Pulmonar , Estudios Retrospectivos , Resultado del Tratamiento , Corazón Univentricular/diagnóstico por imagen , Corazón Univentricular/fisiopatología , Adulto Joven

RESUMEN

Speckle-tracking echocardiography has been used to assess atrial function. This analysis is feasible in univentricular hearts. The aim of this study was to characterize the relationship between atrial strain and functional capacity in the Fontan circulation. Apical four-chamber echocardiographic loops of 39 Fontan patients were reviewed. The dominant atrium was assessed for active (εact), conduit (εcon), and reservoir (εres) strain and εact/εres ratio. Cardiopulmonary exercise test was performed on the same day and oxygen uptake (VO2) at ventilatory threshold (VT) and peak VO2 were chosen as the dependent variables. Statistical analysis was performed using SPSS® version 23. Unpaired t test was used for binomial and continuous variable correlation; single and multivariable linear regression were used for continuous variable correlation. Statistical significance was defined as p value < 0.05. VO2 at VT as a percentage of predicted VO2 was 36.8% (SD 10.7). Peak VO2 was 64.7% (SD 18.9) of the predicted value. In univariate analysis, both were associated with age, atrioventricular regurgitation, ejection fraction, εres, εcon, and εact/εres. In multivariate regression, higher VO2 at VT and peak VO2 were associated with younger age (p = 0.003 and p = 0.001, respectively) and higher εcon (p = 0.026 and p = 0.020). Evaluation of heart function is difficult in the Fontan circulation, hindered by complex ventricular morphology and lack of normative data. VO2 provides a good surrogate. Atrial strain parameters are compromised in these patients and associated with VO2. Therefore, whenever possible, atrial strain should be measured as it may provide a new method of risk stratification.

Asunto(s)

Función Atrial , Procedimiento de Fontan/métodos , Corazón Univentricular/cirugía , Adolescente , Niño , Ecocardiografía/métodos , Prueba de Esfuerzo , Femenino , Atrios Cardíacos/diagnóstico por imagen , Humanos , Masculino , Análisis Multivariante , Consumo de Oxígeno , Corazón Univentricular/diagnóstico por imagen , Corazón Univentricular/fisiopatología

RESUMEN

Background We investigated serial serum levels of GDF-15 (growth differentiation factor 15) in Fontan patients and their relation to outcome. Methods and Results In this single-center prospective study of consecutive Fontan patients, serial serum GDF-15 measurement and clinical assessment was done at baseline (n=81) and after 2 years (n=51). The association between GDF-15 and the combined end point of all-cause mortality, heart transplant listing, and Fontan-related hospitalization was investigated. Median age at baseline was 21 years (interquartile range: 15-28 years). Median GDF-15 serum levels at baseline were 552 pg/mL (interquartile range: 453-729 pg/mL). GDF-15 serum levels correlated positively with age, age at Fontan initiation, New York Heart Association class, and serum levels of NT-proBNP (N-terminal pro-B-type natriuretic peptide) and É£GT (γ-glutamyltransferase) and negatively with exercise capacity. During a median follow-up of 4.8 years (interquartile range: 3.3-5.5 years), the combined end point occurred in 30 patients (37%). Multivariate Cox regression showed that patients with the highest baseline GDF-15 (n=20, defined as the upper quartile) had a higher risk of hospitalization or death than the lowest 3 quartiles (hazard ratio [HR], 2.76; 95% CI, 1.27-6.00; P=0.011). After 2 years of follow-up, patients in whom serum level of GDF-15 increased to >70 pg/mL (n=13) had a higher risk of hospitalization or death than the lowest 3 quartiles (HR, 2.69; 95% CI, 1.03-6.99; P=0.043). Conclusions In Fontan patients, elevated serum levels of GDF-15 are associated with worse functional status and predict Fontan-related events. Furthermore, serial measurements showed that an increase in GDF-15 serum level was associated with increased risk for adverse outcome.

Asunto(s)

Procedimiento de Fontan/efectos adversos , Factor 15 de Diferenciación de Crecimiento/sangre , Readmisión del Paciente , Corazón Univentricular/cirugía , Adolescente , Adulto , Biomarcadores/sangre , Niño , Femenino , Procedimiento de Fontan/mortalidad , Estado Funcional , Trasplante de Corazón , Humanos , Masculino , Valor Predictivo de las Pruebas , Estudios Prospectivos , Medición de Riesgo , Factores de Riesgo , Factores de Tiempo , Resultado del Tratamiento , Corazón Univentricular/sangre , Corazón Univentricular/diagnóstico por imagen , Corazón Univentricular/mortalidad , Regulación hacia Arriba , Adulto Joven

RESUMEN

Tricuspid and pulmonary atresia with single ventricle physiology and major aortopulmonary collateral arteries (MAPCAs) is a complex cyanotic congenital heart disease with heterogeneous pulmonary artery morphology and arborization. The complex anatomy and physiology, coupled with a dearth of existing literature, pose imitable challenges to treatment. Although the exact surgical algorithm is still unclear, the goal is a well-developed, low-resistance pulmonary vascular bed. A precise understanding of the blood supply to each lung is a requisite for successful surgery, and a multimodality and multidisciplinary approach is compulsory. Herein, we describe a case of tricuspid and pulmonary atresia with single ventricle, MAPCAs and aortopulmonary collateral arteries.

Asunto(s)

Aorta/fisiopatología , Circulación Colateral , Arteria Pulmonar/fisiopatología , Atresia Pulmonar/fisiopatología , Circulación Pulmonar , Atresia Tricúspide/fisiopatología , Corazón Univentricular/fisiopatología , Adulto , Aorta/diagnóstico por imagen , Aorta/cirugía , Humanos , Masculino , Arteria Pulmonar/diagnóstico por imagen , Arteria Pulmonar/cirugía , Atresia Pulmonar/complicaciones , Atresia Pulmonar/diagnóstico por imagen , Atresia Pulmonar/cirugía , Resultado del Tratamiento , Atresia Tricúspide/complicaciones , Atresia Tricúspide/diagnóstico por imagen , Atresia Tricúspide/cirugía , Corazón Univentricular/complicaciones , Corazón Univentricular/diagnóstico por imagen , Corazón Univentricular/cirugía

RESUMEN

The Fontan procedure is often the only definitive palliative surgical option for patients with a variety of complex CHD sharing in common, a single, dominant ventricle. In recent decades, imaging and therapeutic improvement have played a crucial role in those patients in whom many complications can hamper their life. After 50 years from the first procedure, heart transplantation remains the only definitive treatment for those with a failing Fontan circulation.

Asunto(s)

Procedimiento de Fontan , Ventrículos Cardíacos/cirugía , Corazón Univentricular/cirugía , Difusión de Innovaciones , Procedimiento de Fontan/efectos adversos , Procedimiento de Fontan/historia , Trasplante de Corazón , Ventrículos Cardíacos/anomalías , Ventrículos Cardíacos/diagnóstico por imagen , Ventrículos Cardíacos/fisiopatología , Hemodinámica , Historia del Siglo XX , Historia del Siglo XXI , Humanos , Recuperación de la Función , Factores de Riesgo , Insuficiencia del Tratamiento , Corazón Univentricular/diagnóstico por imagen , Corazón Univentricular/historia , Corazón Univentricular/fisiopatología , Función Ventricular

RESUMEN

An 18-year-old male with an unknown medical history presented with palpitations and paroxysmal episodes of shortness of breath over a 7-year period. Transthoracic echocardiography displayed a univentricle with an ejection fraction of 45%. The patient refused treatment and was lost to follow-up.

Asunto(s)

Ecocardiografía/métodos , Corazón Univentricular , Adolescente , Aleteo Atrial/diagnóstico , Aleteo Atrial/etiología , Disnea/diagnóstico , Disnea/etiología , Electrocardiografía/métodos , Humanos , Perdida de Seguimiento , Masculino , Volumen Sistólico , Corazón Univentricular/diagnóstico por imagen , Corazón Univentricular/fisiopatología

RESUMEN

OBJECTIVE: To develop a risk score to predict mortality or transplant in the interstage period. BACKGROUND: The "interstage" period between the stage 1 and stage 2 palliation is a time of high morbidity and mortality for infants with single-ventricle congenital heart disease. METHODS: This was an analysis of patients with single-ventricle congenital heart disease requiring arch reconstruction who were enrolled in the National Pediatric Cardiology Quality Improvement Collaborative registry from 2008 to 2015. The primary composite endpoint was interstage mortality or transplant. Multivariable logistic regression and classification and regression tree analysis were performed on two-thirds of the patients ("learning cohort") to build a risk score for the composite endpoint, that was validated in the remaining patients ("validation cohort"). RESULTS: In the 2128 patients analyzed in the registry, the overall event rate was 9% (153 [7%] deaths, 42 [2%] transplants). In the learning cohort, factors independently associated with the composite endpoint were (1) type of Norwood; (2) postoperative ECMO; (3) discharge with Opiates; (4) No Digoxin at discharge; (5) postoperative Arch obstruction, (6) moderate-to-severe Tricuspid regurgitation without an oxygen requirement, and (7) Extra Oxygen required at discharge in patients with moderate-to-severe tricuspid regurgitation. This model was used to create a weighted risk score ("NEONATE" score; 0-76 points), with >75% accuracy in the learning and validation cohorts. In the validation cohort, the event rate in patients with a score >17 was nearly three times those with a score ≤17. CONCLUSIONS: We introduce a risk score that can be used post-stage 1 palliation to predict freedom from interstage mortality or transplant.

Asunto(s)

Procedimiento de Blalock-Taussing/efectos adversos , Reglas de Decisión Clínica , Trasplante de Corazón , Procedimientos de Norwood/efectos adversos , Cuidados Paliativos , Corazón Univentricular/cirugía , Procedimiento de Blalock-Taussing/mortalidad , Causas de Muerte , Femenino , Humanos , Lactante , Masculino , Procedimientos de Norwood/mortalidad , Valor Predictivo de las Pruebas , Sistema de Registros , Reproducibilidad de los Resultados , Medición de Riesgo , Factores de Riesgo , Factores de Tiempo , Resultado del Tratamiento , Estados Unidos , Corazón Univentricular/diagnóstico por imagen , Corazón Univentricular/mortalidad , Corazón Univentricular/fisiopatología

RESUMEN

OBJECTIVES: This study aimed to develop non-invasive machine learning classifiers for predicting post-Glenn shunt patients with low and high risks of a mean pulmonary arterial pressure (mPAP) > 15 mmHg based on preoperative cardiac computed tomography (CT). METHODS: This retrospective study included 96 patients with functional single ventricle who underwent a bidirectional Glenn procedure between November 1, 2009, and July, 31, 2017. All patients underwent post-procedure CT, followed by cardiac catheterization. Overall, 23 morphologic parameters were manually extracted from cardiac CT images for each patient. The Mann-Whitney U or chi-square test was applied to select the most significant predictors. Six machine learning algorithms including logistic regression, Naive Bayes, random forest (RF), linear discriminant analysis, support vector machine, and K-nearest neighbor were used for modeling. These algorithms were independently trained on 100 train-validation random splits with a 3:1 ratio. Their average performance was evaluated by area under the curve (AUC), accuracy, sensitivity, and specificity. RESULTS: Seven CT morphologic parameters were selected for modeling. RF obtained the best performance, with mean AUC of 0.840 (confidence interval [CI] 0.832-0.850) and 0.787 (95% CI 0.780-0.794); sensitivity of 0.815 (95% CI 0.797-0.833) and 0.778 (95% CI 0.767-0.788), specificity of 0.766 (95% CI 0.748-0.785) and 0.746 (95% CI 0.735-0.757); and accuracy of 0.782 (95% CI 0.771-0.793) and 0.756 (95% CI 0.748-0.764) in the training and validation cohorts, respectively. CONCLUSIONS: The CT-based RF model demonstrates a good performance in the prediction of mPAP, which may reduce the need for right heart catheterization in post-Glenn shunt patients with suspected mPAP > 15 mmHg. KEY POINTS: • Twenty-three candidate descriptors were manually extracted from cardiac computed tomography images, and seven of them were selected for subsequent modeling. • The random forest model presents the best predictive performance for pulmonary pressure among all methods. • The computed tomography-based machine learning model could predict post-Glenn shunt pulmonary pressure non-invasively.

Asunto(s)

Presión Sanguínea , Procedimiento de Fontan , Cardiopatías Congénitas/diagnóstico por imagen , Cardiopatías Congénitas/cirugía , Arteria Pulmonar/diagnóstico por imagen , Máquina de Vectores de Soporte , Adolescente , Algoritmos , Teorema de Bayes , Cateterismo Cardíaco , Niño , Preescolar , Análisis Discriminante , Ventrículo Derecho con Doble Salida/diagnóstico por imagen , Ventrículo Derecho con Doble Salida/cirugía , Femenino , Defectos de los Tabiques Cardíacos/diagnóstico por imagen , Defectos de los Tabiques Cardíacos/cirugía , Humanos , Lactante , Modelos Logísticos , Pulmón , Aprendizaje Automático , Masculino , Pronóstico , Atresia Pulmonar/diagnóstico por imagen , Atresia Pulmonar/cirugía , Estudios Retrospectivos , Tomografía Computarizada por Rayos X/métodos , Transposición de los Grandes Vasos/diagnóstico por imagen , Transposición de los Grandes Vasos/cirugía , Atresia Tricúspide/diagnóstico por imagen , Atresia Tricúspide/cirugía , Corazón Univentricular/diagnóstico por imagen , Corazón Univentricular/cirugía , Adulto Joven

Asunto(s)

Procedimiento de Fontan/tendencias , Corazón Univentricular/cirugía , Animales , Difusión de Innovaciones , Procedimiento de Fontan/efectos adversos , Procedimiento de Fontan/historia , Procedimiento de Fontan/mortalidad , Predicción , Hemodinámica , Historia del Siglo XX , Historia del Siglo XXI , Humanos , Complicaciones Posoperatorias/mortalidad , Complicaciones Posoperatorias/terapia , Recuperación de la Función , Factores de Riesgo , Resultado del Tratamiento , Corazón Univentricular/diagnóstico por imagen , Corazón Univentricular/mortalidad , Corazón Univentricular/fisiopatología

RESUMEN

BACKGROUND: Published palliation outcomes of infants with functional single ventricle (SV) and common atrioventricular septal defect (AVSD) are poor due to associated cardiac and extracardiac anomalies and development of atrioventricular valve (AVV) regurgitation. We report current palliation results. METHODS: From 2002 to 2012, 80 infants with functional SV with AVSD underwent multistage palliation. Competing-risks analyses modeled events after first-stage surgery and Glenn (death/transplantation vs next palliation surgery) and examined factors associated with survival and AVV intervention. RESULTS: Sixty-eight (80%) patients received neonatal palliation: modified Blalock-Taussig shunt (n = 33, 41%), Norwood (n = 20, 25%), and pulmonary artery band (n = 15, 19%), whereas 12 (15%) received primary Glenn. On competing-risks analysis, one-year following first-stage surgery, 29% of patients had died or received transplantation and 62% had undergone Glenn. Five years following Glenn, 9% of patients had died or received transplantation and 68% had undergone Fontan. Overall eight-year survival was 64% and was lower in patients with genetic syndromes (53% vs 82%), patients requiring concomitant total anomalous pulmonary venous connection repair (53% vs 69%), and those requiring neonatal palliation (48% vs 100%). Factors associated with mortality were unplanned reoperation (hazard ratio [HR]: 3.7 [1.7-8.0], P = .001) and extracorporeal membrane oxygenation use (HR: 7.1 [3.0-16.6], P < .001). Initial AVV regurgitation ≥ moderate was associated with AVV intervention (HR: 6.2 [2.4-16.1], P = .002) with eight-year freedom from death or AVV intervention of 25% in those patients. CONCLUSIONS: Patients with SV with AVSD are a distinct group and commonly have associated cardiac and extracardiac malformations that complicate care and affect survival. The development of AVV regurgitation requiring intervention is common but does not affect survival.

Asunto(s)

Procedimiento de Blalock-Taussing , Defectos de los Tabiques Cardíacos/cirugía , Procedimientos de Norwood , Cuidados Paliativos/métodos , Corazón Univentricular/cirugía , Procedimiento de Blalock-Taussing/métodos , Ecocardiografía , Oxigenación por Membrana Extracorpórea , Femenino , Procedimiento de Fontan , Defectos de los Tabiques Cardíacos/diagnóstico por imagen , Defectos de los Tabiques Cardíacos/mortalidad , Humanos , Lactante , Recién Nacido , Masculino , Procedimientos de Norwood/métodos , Modelos de Riesgos Proporcionales , Arteria Pulmonar/cirugía , Reoperación , Estudios Retrospectivos , Medición de Riesgo , Factores de Riesgo , Análisis de Supervivencia , Resultado del Tratamiento , Corazón Univentricular/diagnóstico por imagen , Corazón Univentricular/mortalidad

RESUMEN

A 5-month-old girl with single ventricle, interrupted inferior vena cava and polysplenia syndrome palliated with bilateral Blalock-Taussig shunts developed severe cyanosis despite apparently increased pulmonary blood flow. Angiography revealed diffuse pulmonary arteriolar capillary dilatation and early pulmonary venous filling, suggesting the presence of pulmonary arteriovenous malformations. Abdominal angiography at 6 months demonstrated a large extrahepatic portosystemic shunt, which was percutaneously closed with a vascular plug. Kawashima operation was performed 2 weeks after portosystemic shunt closure. Although cyanosis improved temporarily, the patient suffered from deteriorating cyanosis at 9 months of age and underwent Fontan completion. Thereafter, her oxygen saturation gradually improved to 95% over the course of 3 weeks. Both the congenital extrahepatic portosystemic shunt and Kawashima operation contributed to the development of pulmonary arteriovenous malformations.

Asunto(s)

Malformaciones Arteriovenosas/complicaciones , Malformaciones Arteriovenosas/diagnóstico por imagen , Síndrome de Heterotaxia/complicaciones , Síndrome de Heterotaxia/diagnóstico por imagen , Corazón Univentricular/complicaciones , Corazón Univentricular/diagnóstico por imagen , Malformaciones Arteriovenosas/cirugía , Angiografía por Tomografía Computarizada/métodos , Femenino , Síndrome de Heterotaxia/cirugía , Humanos , Lactante , Pulmón/irrigación sanguínea , Pulmón/diagnóstico por imagen , Pulmón/cirugía