Poor Weight Recovery Between Stage 1 Palliation and Hospital Discharge for Infants with Single Ventricle Physiology: An Analysis of the National Pediatric Cardiology Quality Improvement Collaborative Phase II Dataset.

OBJECTIVE: To investigate change in weight-for-age z-scores (WAZ) and risk factors for impaired weight gain between stage 1 palliation (S1P) for single ventricle physiology and discharge. STUDY DESIGN: This was a secondary analysis of the National Pediatric Cardiology Quality Improvement Collaborative Phase II database. The primary outcome was change in WAZ between S1P and discharge. Risk factors were selected using multivariable mixed effects regression constructed by step-wise model selection, with adjustment for WAZ at S1P and a random effect for center. RESULTS: Of 730 infants who were discharged after S1P, WAZ decreased in 98.6% (-1.5 ± 0.7). WAZ at discharge was <-1 but >-2 (at risk) in 40% and <-2 (failure to thrive) in 35% of participants. Males, higher WAZ at S1P, non-S1P procedures (mostly noncardiac), increased length of stay, necrotizing enterocolitis, and angiotensin-converting enzyme inhibitor use at discharge were associated with a greater decrease in WAZ. Preoperative enteral feeding and respiratory medications were associated with a lesser decrease in the WAZ. CONCLUSIONS: Nearly all infants lose weight after S1P with little recovery by hospital discharge. At discharge, three-quarters of the infants in the cohort were at risk for impaired weight gain or had failure to thrive. Most risk factors associated with change in WAZ were unmodifiable or surrogates of disease severity. Novel interventions are needed to minimize the early catabolic effects and promote anabolic recovery after S1P.

Imposition of Fontan physiology: Effects on strain and global measures of ventricular function.

OBJECTIVE: We sought to evaluate contractile function in single-ventricle patients before and after imposition of Fontan physiology. METHODS: Single right ventricle (SRV; n = 38) and single left ventricle (SLV; n = 11) patients underwent cardiac magnetic resonance imaging pre and post Fontan operation. Global radial strain (GRS), global circumferential strain (GCS), and global longitudinal strain were measured along with ejection fraction (EF) and atrioventricular valve regurgitation (AVVR). RESULTS: Age at cardiac magnetic resonance imaging before the Fontan operation was 3.1 ± 1.3 years and after the Fontan procedure was 5.8 ± 2.7 years. There were no significant EF differences between SRV and SLV patients before and after the Fontan procedure, and EF did not deteriorate significantly after the Fontan operation. GRS was significantly lower for SRV patients than for SLV patients before (24.3% vs 32.1%; P = .048) and after (21.8% vs 29.7%; P = .045) the Fontan procedure. GRS and GCS of the SRV patients deteriorated significantly after the Fontan operation (GRS, P = .01; GCS, P = .009). Strains showed positive correlations before and after the Fontan operation with positive correlations among each strain. Within all patients, strains correlated positively with EF. Strains and EF negatively correlated with AVVR (GRS P = .03, r = -0.22; GCS P = .03, r = -0.23; EF P < .001, r = -0.37). CONCLUSIONS: Strains were lower for SRV than for SLV patients before and after the Fontan operation and deteriorated after the Fontan operation. Our study suggests that strain measures might detect ventricular deterioration earlier than EF. Because strains before and after the Fontan operation were positively correlated, and negatively correlated with AVVR, the early institution of myocardial protective therapy including AVVR management, especially for SRV patients, might have benefit.

Assessment of functional capacity among students with univentricular heart. / Evaluación de la capacidad funcional en escolares con fisiología de corazón univentricular.

A cross-sectional study was done in students with univentricular heart after undergoing total cavopulmonary connection (Fontan procedure) to establish their baseline functional capacity based on the six-minute walk test and identify potential determining factors. Thirty patients were included; their median age was 12 years old. Eighteen patients were males. The median distance walked was 551.3 meters, 84 % of the theoretical distance for a healthy pediatric population. Height, pre-test systolic blood pressure, and resting arterial oxygen saturation showed a significant association with the distance walked in the multiple linear regression model. No significant association was observed in the meters walked in terms of the following outcome measures: sex, nutritional status, baseline cardiological diagnosis, number of prior surgeries, and age at the time of Fontan procedure.

Se realizó un estudio transversal en escolares con corazón univentricular en estadio pos-bypass total de ventrículo derecho con el objetivo de determinar la capacidad funcional basal mediante el test de marcha en 6 minutos e identificar posibles factores determinantes. Participaron 30 pacientes con una mediana de edad de 12 años. Dieciocho pacientes fueron de sexo masculino. La mediana de distancia recorrida fue de 551,3 metros, un 84 % de la distancia teórica para población pediátrica sana. Las variables talla, presión arterial sistólica pretest y saturación arterial de oxígeno de reposo se asociaron significativamente con la distancia recorrida en el modelo de regresión lineal múltiple. No hubo asociación significativa en los metros caminados respecto de las variables sexo, estado nutricional, dignóstico cardiológico inicial, número de cirugías previas y edad al momento del bypass total de ventrículo derecho.

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.

Atrioventricular Valve Repair in Single Ventricle Physiology: Timing Matters.

OBJECTIVES: Atrioventricular valve (AVV) regurgitation in patients with single ventricle (SV) physiology severely impacts prognosis; the appropriate timing for surgical treatment is unknown. We sought to study the results of surgical treatment of AVV regurgitation in SV patients and evaluate risk factors for mortality. METHODS: Medical records of 81 consecutive patients with moderate or severe AAV regurgitation who were submitted to AVV repair or replacement during any stage of univentricular palliation between January 2013 and May 2017 were examined. We studied demographic data and perioperative factors looking for predictors that might have influenced the results. Binary logistic regression was used to assess the impact on postoperative ventricular dysfunction and mortality. RESULTS: Median age and weight were seven months (interquartile range [IQR]: 3-24) and 5.2 kg (IQR: 3.7-11.2), respectively. Seventy (86.4%) patients underwent AVV repair, and 11 (13.6%) patients underwent AVV replacement. There was an association between AVV repair effectiveness and timing of intervention (P = .004). Atrioventricular valve intervention at the time of initial surgical palliation was associated with more ineffective repairs (P = .001), while AVV replacement was more common between Glenn and Fontan procedures (P = .004). Overall 30-day mortality was 30.5% (25 patients). In-hospital mortality was 49.4%, and it was higher when AVV repair was performed concomitant with initial (stage 1) palliation (64.1% vs 35.7%; P = .01) and when an effective repair was not achieved (75% vs 41%; P = .008). Multivariable analysis identified timing concomitant with stage 1 palliation as an independent risk factor for mortality (P = .01); meanwhile, an effective repair was a protective factor against in-hospital mortality (P = .05). CONCLUSION: Univentricular physiology with AVV regurgitation is a high-risk group of patients. Surgery for AVV regurgitation at stage 1 palliation was associated with less effective repair and higher mortality in this initial experience. On the other hand, effective repair determined better outcomes, highlighting the importance of experience and the learning curve in the management of such patients.

Factors Associated with Delayed Transition to Oral Feeding in Infants with Single Ventricle Physiology.

OBJECTIVES: To describe the duration of time to achieve exclusive oral feeding in infants with single ventricle physiology and to identify risk factors associated with prolonged gastrostomy tube dependence. STUDY DESIGN: Single center, retrospective study of infants with single ventricle physiology. The primary outcome was duration of time required to achieve oral feeding. Transition periods were defined as exclusive oral feeding by Glenn palliation (early), by 1 year of age (mid), or after 1 year of age (late). RESULTS: Seventy-eight infants were analyzed; 46 (59%) were discharged to home with a gastrostomy tube after the initial hospitalization. Overall, 39 infants (50%) achieved early transition, 14 (18%) mid, and 18 (23%) late. The group who achieved early transition had a higher percentage of preoperative oral feeding (P < .01), greater weight-for-age z score at initial discharge (P = .03), shorter initial intensive care unit duration (P < .01), shorter initial hospital length of stay (P < .01), and greater weight-for-age z score at Glenn admission (P = .02). No preoperative oral feeding (OR = 0.12, P = .02) and greater number of cardiac medications at initial discharge (OR = 3.8, P = .03) were associated with failure to achieve early transition. No preoperative oral feeding (OR = 0.09, P = .01) and longer initial intensive care unit duration (OR = 1.1, P = .03) were associated with failure to achieve mid transition. CONCLUSION: Preoperative oral feeding may potentially be a modifiable factor to help improve early transition to oral feeding.

Outcomes of the Conversion of the Fontan-Kreutzer Operation to a Total Cavopulmonary Connection for the Failing Univentricular Circulation.

BACKGROUND: The Fontan-Kreutzer procedure (FK) was widely performed in the past, but in the long-term generated many complications resulting in univentricular circulation failure. The conversion to total cavopulmonary connection (TCPC) is one of the options for treatment. OBJECTIVE: To evaluate the results of conversion from FK to TCPC. METHODS: A retrospective review of medical records for patients who underwent the conversion of FK to TCPC in the period of 1985 to 2016. Significance p < 0,05. RESULTS: Fontan-type operations were performed in 420 patients during this period: TCPC was performed in 320, lateral tunnel technique in 82, and FK in 18. Ten cases from the FK group were elected to conversion to TCPC. All patients submitted to Fontan Conversion were included in this study. In nine patients the indication was due to uncontrolled arrhythmia and in one, due to protein-losing enteropathy. Death was observed in the first two cases. The average intensive care unit (ICU) length of stay (LOS) was 13 days, and the average hospital LOS was 37 days. A functional class by New York Heart Association (NYHA) improvement was observed in 80% of the patients in NYHA I or II. Fifty-seven percent of conversions due to arrhythmias had improvement of arrhythmias; four cases are cured. CONCLUSIONS: The conversion is a complex procedure and requires an experienced tertiary hospital to be performed. The conversion has improved the NYHA functional class despite an unsatisfactory resolution of the arrhythmia.

Outcomes of the Conversion of the Fontan-Kreutzer Operation to a Total Cavopulmonary Connection for the Failing Univentricular Circulation / Resultados da Conversão da Cirurgia de Fontan-Kreutzer para Cavo-Pulmonar Total Devido Falência da Circulação Univentricular

Abstract Background: The Fontan-Kreutzer procedure (FK) was widely performed in the past, but in the long-term generated many complications resulting in univentricular circulation failure. The conversion to total cavopulmonary connection (TCPC) is one of the options for treatment. Objective: To evaluate the results of conversion from FK to TCPC. Methods: A retrospective review of medical records for patients who underwent the conversion of FK to TCPC in the period of 1985 to 2016. Significance p < 0,05. Results: Fontan-type operations were performed in 420 patients during this period: TCPC was performed in 320, lateral tunnel technique in 82, and FK in 18. Ten cases from the FK group were elected to conversion to TCPC. All patients submitted to Fontan Conversion were included in this study. In nine patients the indication was due to uncontrolled arrhythmia and in one, due to protein-losing enteropathy. Death was observed in the first two cases. The average intensive care unit (ICU) length of stay (LOS) was 13 days, and the average hospital LOS was 37 days. A functional class by New York Heart Association (NYHA) improvement was observed in 80% of the patients in NYHA I or II. Fifty-seven percent of conversions due to arrhythmias had improvement of arrhythmias; four cases are cured. Conclusions: The conversion is a complex procedure and requires an experienced tertiary hospital to be performed. The conversion has improved the NYHA functional class despite an unsatisfactory resolution of the arrhythmia.

Resumo Fundamento: O procedimento de Fontan-Kreutzer (FK) foi amplamente realizado no passado, mas a longo prazo gerou muitas complicações, resultando em falha na circulação univentricular. A conversão para conexão cavopulmonar total (CCPT) é uma das opções de tratamento. Objetivo: Avaliar os resultados da conversão de FK para CCPT. Métodos: Revisão retrospectiva de prontuários de pacientes submetidos à conversão de FK para CCPT no período de 1985 a 2016. Significância p < 0,05. Resultados: Operações do tipo Fontan foram realizadas em 420 pacientes durante este período: CCPT foi realizada em 320, técnica de túnel lateral em 82 e FK em 18. Dez casos do grupo FK foram eleitos para conversão em CCPT. Todos os pacientes submetidos à conversão de Fontan foram incluídos neste estudo. Em nove pacientes, a indicação deveu-se a arritmia não controlada e em um devido à enteropatia perdedora de proteínas. A morte foi observada nos dois primeiros casos. O tempo médio de internação na unidade de terapia intensiva (UTI) foi de 13 dias e o tempo médio de internação hospitalar foi de 37 dias. Uma classe funcional pela melhora da New York Heart Association (NYHA) foi observada em 80% dos pacientes em NYHA I ou II. Cinquenta e sete por cento das conversões devido a arritmias tiveram melhora das arritmias; quatro casos foram curados. Conclusões: A conversão é um procedimento complexo e requer que um hospital terciário experiente seja realizado. A conversão melhorou a classe funcional da NYHA, apesar de uma resolução insatisfatória da arritmia.