Uso de vasopresina en el postoperatorio de cirugía de Fontan: inferencias sobre morbimortalidad / Vasopressin use in postoperative Fontan surgery: implications for morbidity and mortality

Introducción: Se ha postulado que el uso de vasopresina tendría efectos beneficiosos en el postoperatorio de cirugía cardiovascular. Objetivo: Evaluar la respuesta a la vasopresina en el postoperatorio (POP) de cirugía de Fontan de nuestra población. Métodos: Estudio de casos y controles anidados en una cohorte retrospectiva. Se incluyeron pacientes con cirugía de Fontan entre 2014 y 2019. Se registraron variables demográficas, datos del cateterismo pre-Fontan, días de asistencia respiratoria mecánica (ARM), necesidad de inotrópicos, diuréticos, diálisis, dieta hipograsa, octreotide, sildenafil y nutrición parenteral total (NPT); balance de fluidos al primer y segundo día POP, necesidad de cateterismo en el POP, días de permanencia de tubo pleural, días de internación, necesidad de reinternación y mortalidad. Se compararon los grupos con y sin vasopresina utilizando la prueba de Mann- Whitney-Wilcoxon test. Se consideró significativa una p < 0.05. Resultados: Del total analizado, 35 pacientes recibieron vasopresina. En el grupo control fueron 58 pacientes con características similares de gravedad sin vasopresina. No se encontraron diferencias en la evolución postoperatoria entre ambos grupos. El grupo con vasopresina recibió en mayor proporción dieta hipograsa. Conclusiones: En nuestra serie el uso de vasopresina no marcó diferencias significativas en términos de morbimortalidad con relación al grupo control (AU)

Introduction: The use of vasopressin has been suggested to have beneficial effects in the postoperative period after cardiovascular surgery. Objective: To evaluate the response to vasopressin in the postoperative period (POP) of Fontan surgery in our population. Methods: Nested case-control study in a retrospective cohort. Patients who underwent Fontan surgery between 2014 and 2019 were included. Demographic variables, pre-Fontan catheterization data, days of mechanical ventilation (MRA), need for inotropics, diuretics, dialysis, low-fat diet, octreotide, sildenafil and total parenteral nutrition (TPN); fluid balance at first and second day POP, need for catheterization at POP, duration of chest tube drainage, days of hospitalization, need for readmission, and mortality were recorded. Groups with and without vasopressin were compared using the Mann-Whitney- Wilcoxon test. A p < 0.05 was considered significant. Results: Of all patients analyzed, 35 received vasopressin. The control group consisted of 58 patients with similar severity characteristics who did not receive vasopressin. No differences were found in the postoperative outcome between the two groups. The vasopressin group received a higher proportion of low-fat diet. Conclusions: In our series the use of vasopressin did not show significant differences in terms of morbidity and mortality compared to the control group (AU)

Early and mid-term results of Fontan operation versus anatomic correction for congenitally corrected transposition of the great arteries: A retrospective cohort study in a single center / 中国胸心血管外科临床杂志

@#Objective     To compare the early and mid-term results between Fontan operation and anatomic correction for congenitally corrected transposition of the great arteries (ccTGA). Methods     The clinical data of 53 patients with ccTGA who underwent anatomic correction and Fontan operation from January 2009 to September 2021 in our hospital were reviewed, including 41 males and 12 females with a mean age of 55.02 (3-168) months. They were divided into an anatomic correction group (16 patients) and a Fontan operation group (37 patients) according to the operation. The hospitalization mortality, survival rate, postoperative complications, and free rate from re-intervention between the two groups were compared. Another 180 healthy children were recruited as a control group, and 14 children were matched with the propensity score matching method as a Fontan control group. The results of cardiopulmonary exercise testing (CPET) between the Fontan operation group and the Fontan control group were compared. Results     There were 2 (12.5%) early deaths and 3 (18.8%) early re-intervention in the anatomic correction group, while 1 death and 2 re-intervention in the Fontan operation group. In addition, there were 9 patients (56.3%) in the anatomic correction group and 6 (16.2%) patients in the Fontan operation group suffering from arrhythmia after operation, respectively. Compared with the anatomic correction group, cardiopulmonary bypass time, aortic cross-clamping time, intubation time and ICU stay were significantly shortened in the Fontan operation group (P<0.05). CPET results showed that, percent predicted max VO2 in the Fontan operation group was lower than that in the Fontan control group (0.84±0.11 vs. 0.99±0.12, P<0.05). The patients were followed up for 0.5-126.0 months. Two patients were lost in the Fontan operation group. There was no death and 1 re-intervention in the anatomic correction group, while no death or re-intervention in the Fontan operation group. The 1-year, 5-year and 10-year transplant-free survival rate of the anatomic correction group and the Fontan operation group was 87.5%, 87.5%, 87.5% and 97.3%, 97.3%, 97.3%, respectively (P>0.05). The 48 patients were classified as grade Ⅰ-Ⅱ in cardiac function in the last follow-up. Conclusion     There is no statistical difference in the transplant-free survival rate between the anatomic correction and the Fontan operation group. The postoperative complications in the Fontan operation group are decreased than those in the anatomic correction group. The Fontan operation is also a good choice, even though the patients with ccTGA meet the condition of the procedure of anatomic correction.

A Case Report, Successful Treatment for Extracardiac Conduit Thrombus in Patient after Fontan Completion / 日本心臓血管外科学会雑誌

There are no clear guidelines on thromboprophylaxis in patients following Fontan surgery. In addition, most reports on thromboprophylaxis refer to systemic thromboembolism. Therefore, there are few reports on thromboprophylaxis for extracardiac conduits. We experienced a case of thrombus in extracardiac conduit leading to liver damage after the Fontan completion. The patient was an 11-year-old girl, who underwent the Fontan procedure at 3 years of age. She was on aspirin for thromboprophylaxis. Eight years postoperatively, cardiac catheterization demonstrated severe conduit stenosis, and chronic liver damage was seen at that time. The patient successfully underwent conduit replacement. Subsequently, ascites disappeared and platelet count improved. Conduit stenosis after Fontan completion is a rare but serious complication, therefore we should always keep it in mind and aspire to early detection.

Fontan-associated liver disease current status and transplantation consideration / 中华肝脏病杂志

Fontan-associated liver disease (FALD) is one of the main complications after the Fontan procedure, manifesting mostly as liver fibrosis and even cirrhosis, with a high incidence rate and a lack of typical clinical symptoms that seriously affect patient prognosis. The specific cause is unknown, although it is considered to be associated with long-term elevated central venous pressure, impaired hepatic artery blood flow, and other relevant factors. The absence of association between laboratory tests, imaging data, and the severity of liver fibrosis makes clinical diagnosis and monitoring difficult. A liver biopsy is the gold standard for diagnosing and staging liver fibrosis. The most important risk factor for FALD is time following the Fontan procedure; therefore, it is recommended to do a liver biopsy 10 years after the Fontan procedure and to be cautious for the presence of hepatocellular carcinoma. Combined heart-liver transplantation is a recommended choice with favorable outcomes for patients with Fontan circulatory failure and severe hepatic fibrosis.

Cardiovascular Risk Factors in Children and Adolescents with Fontan Circulation

Abstract Background: Long-term outcomes of patients with Fontan circulation are uncertain regarding the prevalence and role of risk factors (RFs) such as increased body mass index (BMI), arterial hypertension, and hypercholesterolemia. Objectives: To describe the prevalence of RFs in patients with univentricular heart, with variable follow-up times. Methods: This mixed cohort study was performed with 66 patients, who underwent blood count, fasting blood glucose, C-reactive protein (CRP), and lipid profile tests; systolic/diastolic blood pressure (SBP/DBP) measurements; and anthropometric and sociodemographic data collection. Cardiovascular RFs among first-degree relatives and physical activity habits were also assessed. Prevalence was described using proportions, with a 95% confidence interval. Continuous variables (height, weight, age, SBP, DBP) were described as means and standard deviations (m±SD). Associations between RFs were assessed using chi-squared or Fisher's exact tests. Spearman's correlation was used for analyzing CRP and the presence of 2 or more RFs. The Shapiro-Wilk test was used to check for data normality. Statistical significance considered p<0.05. Results: In our population, 19.7% were overweight, mean SBP was 89.44±37.4, and mean DBP was 60.0±26.08. The most prevalent diseases in the interviewees' families were systemic arterial hypertension (30.3%), obesity (16.7%), and 2 or more cardiovascular RFs among first-degree relatives (13.8%). We observed a trend towards significance between the presence of 2 familial RFs and overweight, as well as a risk profile for cardiovascular disease. There was an association between the BMI percentile, the presence of 2 or more RFs (p<0.05), and CRP (p<0.01). Conclusions: Overweight is common in patients with univentricular heart, being related to more than 2 cardiovascular RFs among first-degree relatives; physical inactivity and changes in lipid profiles are also frequent.

Papel de la ecocardiografía speckle-tracking (rastreo de marcas) y la resonancia magnética cardíaca-feature tracking en la circulación de Fontan. Diferencias entre los ventrículos derecho e izquierdo / Speckle-tracking echocardiography and MCR-feature tracking in Fontan circulation. Differences between right and left ventricles

Resumen Introducción: En los pacientes con DCPT, la disfunción ventricular es inevitable, y más temprana en VU derechos. La deformación miocárdica por STE y RMC-FT parece promisoria. Objetivo: Analizar la función ventricular mediante STE y RMC-FT en pacientes con DCPT, en comparación con RMC convencional según la morfología del VU y la posible implicación en su diagnóstico temprano. Método: Se recogieron medidas del strain longitudinal y circunferencial por STE y RMC-FT, volúmenes ventriculares y FE por RMC en 64 pacientes con DCPT. Resultados: La morfología ventricular no se relacionó con disfunción por RMC. Los VU derechos tuvieron valores por STE y RMC-FT disminuidos respecto de los VU izquierdos, con FE similares. Existe correlación entre STE y RMC-FT, no equivalentes, con buena factibilidad y reproducibilidad. Conclusiones: La RMC-FT y el STE son técnicas útiles en el diagnóstico temprano y la vigilancia de la función ventricular en VU derechos con FE preservada.

Abstract Introduction: In patients with TCPC, the development of ventricular dysfunction is inevitable and is more precocious in SRVs. Myocardial deformation by STE and CMR-FT is promising. Objective: To analize ventricular function in patients with TCPC using STE and CMR-FT compared with conventional cMRI, depending on SV morphology, to determine their role in early diagnosis of ventricular dysfunction. Method: Sixty-four patients with TCPC were included. Longitudinal and circumferential strain by STE and CMR-FT and ventricular volume and EF were obtained. Results: Dysfunction analyzed by cMRI showed no association with ventricular morphology. SRVs had lower values in STE and CMR-FT compared with SLVs, with similar EF. While not equivalent, correlation was observed between the STE and the CMR-FT values, demonstrating good feasibility and reproducibility. Conclusion: The strain data in CMR-FT and STE could be useful for diagnosis and monitoring of ventricular function and as markers of early SRV dysfunction with preserved EF.

Clinical efficacy and safety of Treprostinil in the treatment of children with early decompensation after the Fontan procedure / 中华实用儿科临床杂志

Objective:To investigate the efficacy and safety of Treprostinil in the treatment of children with early decompensation after the Fontan procedure.Methods:A retrospectively analysis was performed on the clinical data of 16 children with early decompensation after the Fontan procedure treated with Treprostinil injection from December 2017 to June 2020 at Fuwai Central China Cardiovascular Hospital.A total of 16 patients were included, including 5 boys (31.2%) and 11 girls (68.8%). The age was (4.6 ±1.2) years, the weight was (16.0±2.1) kg.The changes of central venous pressure (CVP), heart rate (HR), systolic blood pressure (SBP), central venous oxygen saturation (ScvO 2), lactic acid (Lac), oxygenation index and B-type natriuretic peptide (BNP) were recorded at the infusion of Treprostinil and 3 hours, 24 hours, 48 hours and 72 hours after the infusion.The short-term efficacy of Treprostinil was observed[mortality, mechanical ventilation time, and length of intensive care unit (ICU) stay]; paired t-test was used to analyze the above indexes at different time points.The adverse reactions during the administration were also recorded. Results:Of the 16 children, the median mechanical ventilation time was 9 (5, 22) h, and the median ICU stay time was 2 (1, 12)days.After 72 hours of drug administration, CVP, Lac, BNP and HR decreased: CVP decreased from(16±5) mmHg (1 mmHg=0.133 kPa) to (11±2) mmHg ( P<0.001), Lac decreased from(6.8±3.2) mmol/L to (3.2±1.2) mmol/L ( P=0.002), BNP decreased from(980±223) ng/L to (250±120) ng/L( P<0.001), HR decreased from(150±20) times/min to (125±16) times/min( P=0.002); SBP, ScvO 2 and oxygenation index increased: SBP increased from(83±10) mmHg to (98±12) mmHg( P<0.001), ScvO 2 increased from 0.53±0.13 to 0.65±0.11 ( P=0.003), oxygenation index increased from (200±72) mmHg to (298±13) mmHg ( P<0.001), and the differences were statistically significant(all P< 0.05). One case died (6.3%), 2 cases (12.5%) had transient blood pressure drop and 1 case (6.3%) had nausea and vomiting.Besides, no other treatment-related complications were observed. Conclusions:As for children with early decompensation after the Fontan procedure, the intravenous application of Treprostinil can reduce pulmonary artery pressure rapidly, effectively improve circulatory status and oxygenation and ultimately improve the prognosis.

Echocardiographic and Ultrasonographic Parameters Associated with Protein-losing Enteropathy in Patients with Fontan Physiology: a Systematic Review with Meta-analysis

Abstract Background: Fontan circulation can be associated with significant morbidity, especially Protein-Losing Enteropathy (PLE). Echocardiographic parameters can provide valuable diagnostic information about a patient's risk of developing PLE after Fontan surgery. Objectives: To describe echocardiographic/ultrasonographic parameters associated with PLE in patients after Fontan surgery through a systematic review with meta-analysis. Methods: A literature search was performed in electronic databases to identify relevant studies about echocardiographic parameters and PLE prediction in children after Fontan surgery. The search terms used were: "echocardiography", "ultrasonography", "Fontan," and "protein-losing enteropathy". A p < 0.05 was considered statistically significant. Results: A total of 653 abstracts were obtained from electronic databases and bibliographic references. From these, six articles met criteria to be included in the qualitative analysis and three in the quantitative (meta-analysis). The resistance index in the superior mesenteric artery was described in three studies, and the quantitative analysis showed statistical significance (p < 0.001). Other echocardiographic and ultrasonographic parameters were also described, albeit in single studies not allowing a meta-analysis. Conclusion: This systematic review with meta-analysis identified echocardiographic and ultrasonographic parameters related to PLE in patients with Fontan physiology. Vascular ultrasonography seems to play a prominent role in this aspect, but additional studies are needed to increase the degree of evidence.

Oxigenación de músculos respiratorios y locomotores durante el test cardiopulmonar en pacientes con circulación de fontan: serie de casos / Patients with fontan: oxygen consumption in respiratory and Locomotor muscles during excersie testing

Resumen: Antecedentes: Los pacientes con circulación de Fontan (PCF) presentan limitación cardíaca durante el esfuerzo máximo lo que repercute en menor capacidad de ejercicio (VO2-peak). La rehabilitación cardiovascular (RC) revierte este desacondicionamiento, al aumentar el gasto cardíaco y diferencia arteriovenosa de oxígeno, aspectos evaluados con monitorización invasiva y gases exhalados. La valoración no invasiva de la saturación muscular de oxígeno (SmO2) es un método de reciente aplicación para evaluar la limitación muscular al ejercicio. En PCF esta limitación puede atribuirse a la mayor acción de músculos respiratorios (cambios ventilatorios) y/o locomotores (carga periférica). Objetivo: Evaluar el trabajo de músculos respiratorios y locomotores durante el ejercicio físico máximo e incremental mediante los cambios en la SmO2. Métodos: A seis PCF (5 hombres; 13.8±2.9 años; 158±9cm; 49.8±13.3 kg) se les valoró el VO2-peak (23.0±4.5mL·kg-1·min-1) mediante ciclo-ergoespirometría sincrónicamente con SmO2 en músculos respiratorios (SmO2-m.Intercostales) y locomotores (SmO2-m.Vastus-Laterallis) mediante espectroscopía cercana al rango infrarrojo durante el test cardiopulmonar. Resultados: SmO2-m.Intercostales disminuyó desde el 60% del VO2-peak (p<0.05), mientras que SmO2-m.Vastus-Laterallis no cambió. La ventilación pulmonar (VE) aumentó progresivamente, siendo significativo a partir del 60% VO2-peak (p<0.05). La mayor desoxigenación de SmO2-m.Intercostales (∆SmO2) se asoció con los máximos cambios en ventilación pulmonar (∆VE) en ejercicio (rho=0.80; p=0.05). Conclusiones: Durante un protocolo de esfuerzo, los pacientes con circulación de Fontan presentan mayor trabajo muscular respiratorio que locomotor. Los cambios en la ventilación pulmonar se asocian a mayor extracción de oxígeno en la musculatura respiratoria, reforzando la necesidad de incorporar el entrenamiento respiratorio en la rehabilitación cardiovascular.

Abstract: Background: During a maximum incremental exercise patients with Fontan circulation (PFC) show cardiac limitation reducing aerobic exercise capacity (VO2-peak). Cardiovascular rehabilitation (CR) reverses this deconditioning by increasing cardiac output and arteriovenous oxygen difference, aspects that can be evaluated by invasive methods and analyzing the exhaled gases. Non-invasive assessment of muscle oxygen saturation (SmO2) is a novel method for recording local oxygen levels. By this technology, it is possible to evaluate the muscle limitation to exercise. In PFC, that limitation could be attributed to higher contractions of respiratory (ventilatory changes) and/ or locomotor muscles (peripheral load). Objective: To evaluate in PFC the changes at SmO2 of respiratory and locomotor muscles during a maximum and incremental exercise protocol (cardiopulmonary test, VO2-peak). Methods: Six PFC (5 men; 13.8±2.9 years; 158±9 cm; 49.8±13.3 kg) were assessed during the VO2peak test (23.0±4.5mL·kg-1·min-1) by cyclo-ergospirometry synchronously with SmO2 at respiratory (SmO2-m.Intercostales) and locomotor (SmO2-m. Vastus-Laterallis) muscles by Near-Infrared Spectroscopy (NIRS). Results: SmO2-m.Intercostales decreased from 60% of VO2-peak (p<0.05), while SmO2-m.Vastus-Laterallis did not change. Minute ventilation (VE) increased progressively, showing changes to rest at 60% of VO2-peak (p<0.05). The higher deoxygenation of SmO2-m.Intercostales (∆SmO2) correlated to maximum changes of lung ventilation (∆VE) (rho=0.80; p=0.05). Conclusions: During an incremental and maximum exercise protocol, patients with Fontan circulation have more work at respiratory than locomotor muscles. Changes in VE are direct associated with greater extraction of oxygen at respiratory muscles, reinforcing the incorporation of respiratory muscle training in cardiovascular rehabilitation.

Prognosis and risk factors for mild to moderate or moderate atrioventricular valve regurgitation after Fontan operation / 中国胸心血管外科临床杂志

@#Objective    To investigate the prognosis and risk factors of mild to moderate or moderate atrioventricular valve regurgitation (AVVR) after Fontan operation. Methods    A total of 34 patients with mild to moderate or moderate AVVR who accepted Fontan operation and atrioventricular valve (AVV) repair between 2004 and 2018 in our center were selected as an AVV repair group. The patients in the same period were matched as a control group by the ratio of 1 : 1-2. Finally 99 patients were included into this study, including 64 males and 35 females, with an average age of 63.4±36.3 months and weight of 17.3±6.7 kg. Grades of AVVR decreased more than 1 was defined as significant improvement. Endpoints of the study were death, Fontan takedown, AVV replacement. Risk factors including Fontan procedures, AVV repair procedures, cardiac anatomy were analyzed. Results    Patients were followed up for 1.5 (0.3-4.0) years. Overall mortality was 15.2%. Most (82.4%) of AVV repair group accepted single AVV repair procedure while partial annuloplasty was the most common (52.9%). With the extension of follow-up, the degree of AVVR in the whole group showed a gradually increasing trend (r=0.352, P=0.000). Mild to moderate AVVR improved spontaneously after Fontan operation, while moderate AVVR did not. AVV repair could improve the degree of AVVR after moderate regurgitation, without increasing the surgical mortality, and regurgitation significantly decreased in 8.8% patients. AVV repair was not effective for mild to moderate AVVR and would increase surgical mortality. Conclusion    AVV function shows a gradual downward trend after Fontan operation. AVV repair is effective for moderate AVVR, does not increase mortality, but the degree of improvement is limited. AVV repair is not effective for mild to moderate AVVR and increases surgical mortality.

Study on modeling, simulation, and sensorless feedback control algorithm of the cavopulmonary assist device based on the subpulmonary ventricular exclusion / 生物医学工程学杂志

The subpulmonary ventricular exclusion (Fontan) could effectively improve the living quality for the children patients with a functional single ventricle in clinical. However, postoperative Fontan circulation failure can easily occur, causing obvious limitations while clinically implementing Fontan. The cavopulmonary assist devices (CPAD) is currently an effective means to solve such limitations. Therefore, in this paper the

Hemodynamic Study on Non-Newtonian Properties of Fontan Procedure / 医用生物力学

Objective To investigate the effects of non-Newtonian properties on hemodynamic characteristics of Fontan procedure in computational simulation. Methods A Fontan vascular model was reconstructed based on patient-specific medical images. The boundary conditions were set according to velocity profiles measured by real-time echocardiography. The Newtonian model and two non-Newtonian (Casson and Carreau) models were applied to analyze the hemodynamic parameters, including flow ratio (FR), energy loss (EL), wall shear stress (WSS) as well as non-Newtonian importance factor, so as to compare flow differences between the Newtonian model and non-Newtonian models. Results The rheological models had small effects on the FR. The EL of non-Newtonian models was higher than that of the Newtonian model, and the EL of Casson model was the highest. Flow recirculation and flow disturbance accompanied with low WSS were observed in inferior vena cava (IVC). At low inflow velocity, flow disturbance in IVC of Newtonian model was more obvious. The calculations of non-Newtonian importance factor suggested significant non-Newtonian viscosity in IVC. Conclusions Non-Newtonian viscosity is significant in IVC where low velocity and flow recirculation are observed. Non-Newtonian properties of the blood should be considered in patient-specific hemodynamic simulation of Fontan procedure.

Hepatopatía crónica asociada a Fontan: a propósito de dos casos y revisión de la literatura / Fontan-associated liver disease: report of two cases and literature review

La técnica quirúrgica de Fontan consiste en transmitir el flujo de la vena cava inferior a través de una anastomosis cavopulmonar para evitar que se mezcle la sangre oxigenada con la no oxigenada, en caso de atresia tricuspídea asociada a ventrículo hipoplásico. Las alteraciones fisiológicas que produce esta nueva circulación tienen varias consecuencias a nivel cardiaco y extracardiaco, incluido el hígado, con múltiples descripciones de hepatopatía crónica de origen vascular, e incluso de carcinoma hepatocelular en hígado cirrótico a edades tempranas. Con el objetivo de conocer un poco más sobre las afectaciones hepáticas que produce este procedimiento, se describen dos casos clínicos y se realiza una revisión de la literatura. A pesar de que la hepatopatía asociada a Fontan es una entidad poco frecuente, los hepatólogos deben tenerla presente y aprender a reconocerla, ya que un adecuado seguimiento puede ser la diferencia al momento de seleccionar de manera eficaz, aquellos pacientes que se beneficiarían de un trasplante cardiaco único o combinado de hígado y corazón, además de permitir la detección de manera oportuna de complicaciones tan devastadoras como un carcinoma hepatocelular en estadios avanzados.

Fontan surgery diverts the flow of the inferior vena cava through a cavopulmonary anastomosis, to avoid mixing the oxygenated and non-oxygenated blood, in the presence of tricuspid atresia associated with hypoplastic ventricle. The physiological changes produced by this new circulation have several consequences at cardiac and extracardiac level, including the liver, with multiple descriptions of chronic liver disease of vascular origin, and even hepatocellular carcinoma in cirrhotic liver at an early age. With the aim of a better understanding of liver complications caused by this procedure, two clinical cases are described and a literature review is presented. Despite the fact that Fontan-associated liver disease is a rare entity, hepatologists must keep it in mind and learn how to recognize it, since an adequate follow-up can make the difference when effectively selecting those patients who would benefit from a single or combined liver and/or heart transplant, as well as allowing the early detection of devastating complications such as advanced stage hepatocellular carcinoma.

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

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

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

A simple predictive factor for mortality in fontan surgery: serum hypo-osmolality

Abstract Objective: Close follow-up is important after the Fontan procedure, which is a palliative surgical method for a single ventricle. In this period, serum osmolality is an important parameter with the advantages of easy to obtain and poor outcome prediction. Methods: Patients who had undergone Fontan operation between May 2011 and February 2017 were retrospectively evaluated. Patients were divided into three groups based on their serum osmolality values: hypoosmolar (Group 1), isosmolar (Group 2), and hyperosmolar (Group 3). Demographics, clinical information and postoperative data of the groups were compared. Results: Forty-three patients had undergone extracardiac Fontan operation in the study period. There were 8, 19 and 16 patients in Groups 1, 2 and 3, respectively. Among the three groups, postoperative intubation and length of hospital stay, prolonged pleural effusion, need for inotropic support and mortality were statistically significantly higher in Group 1. Conclusion: After the Fontan procedure, one of the determinants of cardiac output might be affected by serum osmolality. Decreased serum osmolality might be associated with poor prognosis after Fontan procedure. Serum osmolality monitoring may be beneficial to improve postoperative outcomes in these patients.

Anesthetic management for non-cardiac surgery in a patient with Fontan palliation: case report / Manejo anestésico para cirugía no cardiaca en paciente con paliación de Fontán: Reporte de caso

Abstract Survival of patients with Fontan palliation has improved significantly over the years and it constitutes a condition frequently found nowadays in the perioperative setting for non-cardiac surgery. A history of this disease condition implies complex physiologic and multiorgan considerations for the anesthetist who may need to resort to special measures in order to maintain homeostasis and avoid complications. In this paper we present the case of a patient with a history of Fontan, and describe successful anesthetic management during laparoscopic surgery.

Resumen En los últimos años la sobrevida de pacientes con paliación de Fontán ha aumentado significativamente, y hoy es una condición frecuente en el escenario perioperatorio para cirugía no cardiaca. Este antecedente supone complejas consideraciones fisiológicas y multiorgánicas para el anestesiólogo, quien puede requerir medidas especiales para mantener la homeostasia y evitar complicaciones. En este artículo presentamos el caso de un paciente con antecedente de Fontán y describimos el manejo anestésico exitoso en cirugía laparoscópica.

An unusual cause of edema in a child with congenital heart disease post Fontan procedure

Protein Losing Enteropathy Post Fontan procedure. Protein Losing Enteropathy (PLE) is an uncommon cause of edema in children with congenital heart disease. Protein-Losing Enteropathy may be defined as excessive loss of proteins across the intestinal mucosa and is due to either a primary gastrointestinal abnormality or secondary to cardiac disease. Protein-losing enteropathy (PLE) is a rare complication of the Fontan palliation for functional single-ventricle. Although PLE occurs in about 3.5% of patients post-Fontan, it confers marked morbidity and high mortality within 5 years of diagnosis. The pathogenesis of Fontan-related PLE is not completely understood, and it is unclear why it develops in some patients post-Fontan and not others. We describe a child with Double Inlet Right Ventricle who had undergone Fontan procedure, and presented to us with generalised oedema. The child had hypoproteinaemia, the common causes for which were ruled out and was diagnosed as Protein Losing Enteropathy (PLE) related to his surgical intervention. Though, not frequently encountered it should be kept in mind as one of the causes of anasarca.