Effects of intravenous treprostinil in high-risk patients after total cavo-pulmonary connection / 中国胸心血管外科临床杂志

@#Objective     To explore the effects of intravenous treprostinil in different doses on the hemodynamics and postoperative outcomes after high-risk total cavo-pulmonary connection (TCPC). Methods    From 2018 to 2021, among 189 patients who underwent TCPC in the Department of Pediatric Cardiac Surgery of Fuwai Hospital, 26 high-risk patients who received the intravenous treprostinil therapy were retrospectively analyzed. There were 12 males and 14 females, with an age of 4 (3, 6) years and a weight of 17.6±6.2 kg. The patients were divided into two groups: a high-dose group [15 patients, maintaining dose>10 ng/(kg·min)] and a low-dose group [11 patients, maintaining dose≤ 10 ng/(kg·min)]. The hemodynamics before treprostinil using and during the first 24 hours after reaching the maintaining dose of treprostinil, and postoperative outcomes of the two groups were investigated. Results    The incidence of heterotaxia was higher in the high-dose group (66.7% vs. 18.2%, P=0.021). During the observation period, the mean pulmonary artery pressure decreased from 11.9±3.6 mm Hg to 11.0±3.3 mm Hg in the low-dose group (P=0.013), and from 12.9±4.7 mm Hg to 10.2±3.4 mm Hg in the high-dose group (P=0.001). The decreasing effect in the high-dose group was better than that in the low-dose group (P=0.010). There was no statistical difference in the postoperative outcomes between the two groups (P>0.05). In terms of side effects, patients needed temporarily increased dosage of vasoactive drugs to maintain stable blood pressure during 6-12 h after treprostinil therapy in the high-dose group. Conclusion    In patients after high-risk TCPC, intravenous high-dose treprostinil has a better therapeutic effect on reducing pulmonary artery pressure. However, it should be noted that increased dosage of vasoactive agents may be required to maintain blood pressure stability in patients with high-dose treprostinil.

Surgical management of total cavopulmonary connection procedure in functional single ventricle with separate hepatic venous drainage / 中国胸心血管外科临床杂志

@#Objective    To investigate the management of hepatic vein (HV) in patients with functional single ventricle (FSV) and separate hepatic venous drainage (SHVD) undergoing total cavopulmonary connection (TCPC) and evaluate this kind of surgery. Methods    The clinical data of 32 consecutive patients with SHVD who underwent modified TCPC operation from August 2005 to January 2017 in our center were retrospectively analyzed. There were 25 males and 7 females with an average age of 2-19 (8.0±5.0) years and body weight of 11-66 (25.4±15.8) kg. Results    There were 20 patients with heterotaxy syndrome and 12 patients with other types of FSV. SHVD was diagnosed preoperatively in 27 patients, among whom 20 patients were connected by intra-extracardiac Gore-Tex conduit, and the other 7 patients were connected by extracardiac Gore-Tex conduit. Because of the missed diagnosis of SHVD, the other 5 patients showed severe decrease of blood oxygen saturation in the early postoperative period and underwent re-operation soon. The postoperative blood oxygen saturation was 92.0% (90.0%, 96.0%), central venous pressure was 10-23 (15.5±3.5) mm Hg, mechanical ventilation assisted time was 16.0 (7.5, 24.0) h, and ICU stay time was 3.0 (2.0, 5.5) d. There were 3 early and 1 late deaths. Conclusion    Intra-extracardiac conduit is an effective and feasible modified TCPC operation for patients with FSV and SHVD, while the surgical details need to be formulated in combination with individual anatomical structure. Preoperative missed diagnosis of SHVD must be avoided. Otherwise, after TCPC, a large amount of stealing blood from HV with low circulation pressure into atrium would lead to unacceptable hypoxemia.

Glenn surgery: a safe procedure in the path of univentricular correction / Cirugía de Glenn: un procedimiento seguro en el camino de la corrección univentricular

Abstract Background: This study describes 35 years of experience in a tertiary care level hospital that treats cardiac patients with univentricular heart physiology who underwent Glenn surgery. Methods: The study consisted of a retrospective analysis of patients who underwent Glenn surgery, including variables related to pre-operative, intra-operative, and post-operative morbidity and mortality. Results: From 1980 to 2015, 204 Glenn surgeries were performed. The most common heart disease was tricuspid atresia IB (19.2%). In 48.1% of the cases, the procedure was performed with antegrade flow. A bilateral Glenn procedure was performed in 12.5% of the cases and 10.3% were carried out without using a cardiopulmonary bypass pump. Reported complications included infections, bleeding, arrhythmias, chylothorax, neurological alterations, and pleural effusion. The mortality rate was 2.9% Conclusions: Glenn surgery is a palliative surgery with good results. It significantly improves patient quality of life over a long period until a total cavopulmonary shunt is performed. The complications observed are few, and the mortality rate is low. Therefore, it is a safe surgery that should be used for univentricular congenital heart disease.

Resumen Introducción: El objetivo de este trabajo fue describir la experiencia de 35 años en un hospital de tercer nivel con pacientes cardiópatas con fisiología univentricular que fueron sometidos a cirugía de Glenn. Métodos: Se presenta un análisis retrospectivo de los pacientes sometidos a cirugía de Glenn. Se incluyeron variables relacionadas con la morbilidad y la mortalidad preoperatorias, transoperatorias y posoperatorias. Resultados: Desde 1980 hasta 2015 se realizaron 204 cirugías de Glenn para la corrección de cardiopatías. De ellas, la más frecuente fue la atresia tricuspídea IB (19.2%); en el 48.1% se realizó Glenn con flujo anterógrado, en el 12.5% Glenn bilateral y el 10.3% de las cirugías fueron sin apoyo de bomba de circulación extracorpórea. Las complicaciones reportadas fueron infecciones, sangrado, arritmias, quilotórax, alteraciones neurológicas y derrame pleural. La mortalidad fue del 2.9%. Conclusiones: La cirugía de Glenn es un procedimiento paliativo que se ha utilizado con buenos resultados. Mejora en forma importante la calidad de vida del paciente durante un largo periodo mientras que se realiza la derivación cavopulmonar total. Presenta pocas complicaciones y muy baja mortalidad, por lo que es una cirugía segura que debe ser utilizada para cardiopatías congénitas univentriculares.

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

Evaluation of methods for monitoring transpulmonary gradien after total cavopulmonary connection surgery / 中国小儿急救医学

Objective:To explore the reliability of estimated transpulmonary gradient(TPG)by comparing the measured TPG with the estimated TPG in echocardiography.Methods:The cardiothoracic surgery database of Shanghai Children′s Medical Center was reviewed.Children with hemodynamic monitoring and ultrasound findings who underwent total cavopulmonary connection between January 2015 and December 2018 were included.TPG was calculated separately according to the formula.Intraclass correlation efficient was used for consistency test.Results:Finally, 27 patients were selected, including 16 males and 11 females with age(4.0±1.6)years old, weight(15.2±3.3)kg and height(99.1±11.2)cm.There were nine cases (33.3%) of right ventricular double outlet and seven cases (25.9%) of pulmonary atresia.For hemodynamic blood monitoring, TPG was 5-16(10.1±3)mmHg, and its echocardiography parameters were estimated as 5.8-20.3(11±3.3)mmHg.The ICC value was 0.117 which was less than 0.4( P=0.277). Conclusion:TPG estimated by total cavopulmonary connection pipe window during perioperative period is inaccurate and higher than actual value, so invasive hemodynamic monitoring is still recommended during perioperative period.

Prognosis of acute kidney injury after total cavopulmonary connection: A retrospective cohort study / 中国胸心血管外科临床杂志

@#Objective    To investigate the prevalence, severity and consequences of acute kidney injury (AKI) in the patients who underwent total cavopulmonary connection (TCPC). Methods    The clinical data of TCPC patients in our center from January 1, 2010 to December 31, 2014 were collected and retrospectively analyzed. The patients with renal replacement therapy, missing serum creatinine data before operation or combined with valve procedures were excluded. We identified whether AKI was associated with hospital length of stay, ICU duration, mechanical ventilation duration, hospital acquired infection, and early mortality by univariable and multivariable analyses. Results    A total of 163 patients were included. AKI occurred in 57% of patients (n=93), mild AKI in 26.4% (n=43), moderate AKI in 12.3% (n=20) and severe AKI in 18.4% (n=30). Compared with the no AKI group, the AKI group had higher hospital acquired infection rate (15.1% vs. 0.0%, P<0.001). AKI was independently associated with hospital length of stay (median, 10 d, 95%CI  3.9-16.0,  P=0.001), ICU duration (median, 103.9, 95%CI 48.6-159.2, P<0.001) , but not associated with mechanical ventilation duration (median, 8 h vs. 7 h, P=0.529). Conclusion    Postoperative AKI in the patients undergoing TCPC is common. AKI is associated with higher hospital acquired infection rate, longer hospital length of stay and ICU duration, but not associated with mechanical ventilation duration.

Prognosis of acute kidney injury after total cavopulmonary connection: A retrospective cohort study / 中国胸心血管外科临床杂志

@#Objective    To investigate the prevalence, severity and consequences of acute kidney injury (AKI) in the patients who underwent total cavopulmonary connection (TCPC). Methods    The clinical data of TCPC patients in our center from January 1, 2010 to December 31, 2014 were collected and retrospectively analyzed. The patients with renal replacement therapy, missing serum creatinine data before operation or combined with valve procedures were excluded. We identified whether AKI was associated with hospital length of stay, ICU duration, mechanical ventilation duration, hospital acquired infection, and early mortality by univariable and multivariable analyses. Results    A total of 163 patients were included. AKI occurred in 57% of patients (n=93), mild AKI in 26.4% (n=43), moderate AKI in 12.3% (n=20) and severe AKI in 18.4% (n=30). Compared with the no AKI group, the AKI group had higher hospital acquired infection rate (15.1% vs. 0.0%, P<0.001). AKI was independently associated with hospital length of stay (median, 10 d, 95%CI  3.9-16.0,  P=0.001), ICU duration (median, 103.9, 95%CI 48.6-159.2, P<0.001) , but not associated with mechanical ventilation duration (median, 8 h vs. 7 h, P=0.529). Conclusion    Postoperative AKI in the patients undergoing TCPC is common. AKI is associated with higher hospital acquired infection rate, longer hospital length of stay and ICU duration, but not associated with mechanical ventilation duration.

Surgical management of early Fontan failure:Fontan takedown / 中华胸心血管外科杂志

To analyze the outcomes of early Fontan failure after Fontan takedown. Methods A retrospective study of early Fontan Failure(EFF) children undergoing Fontan takedown from November 2013 to December 2017 was performed. Fontan takedown was defined as takedown back to an intermediate circulation, superior cavopulmonary connection. Fontan takedown was performed in 14 patients. There were 9 boys and 5 girls. Children were on average aged(4. 3 ± 1. 4) years when they had Fontan procedure. The mean weight was(14. 3 ±2. 7)kg. Operative procedure was extra-cardiac Fontan in 5 children, 9 had intra-cardiac Fontan. Fenestration was used in 11/14 patients. The outcomes were summarized with statistics, and risk factors for mortality after Fontan takedown were identified. Results The mortality after Fontan takedown was 4/ 14(28. 6％). In two patients(14. 3％), ECMO was followed after takedown, one of them died after two days. The time of ICU stay and hospital stay was relatively long(17. 0 ±11. 2)days and(33. 8 ±19. 4)days. The interval time between the Fontan procedure and the takedown operation is the risk fact after Fontan takedown. Conclusion Fontan takedown can be used as an effective management for the early Fontan failure, still with a high risk of mortality. Early diagnosis and takedown is recommended for EFF.

Nutritional status and risk factors for malnutrition in children with single ventricle physiology after total cavopulmonary connection / 上海交通大学学报（医学版）

Objective: To analyse nutritional status of children underwent total cavopulmonary connection (TCPC) also called Fontan procedure, furthermore to investigate risk factors related to malnutrition including underweight, stunting, and thinness. Methods: Clinical data of 314 children underwent Fontan procedure in Shanghai Children's Medical Center from Jan. 2012 to Dec. 2015 were reviewed. WHO Z score was used to evaluate nutritional status of the children underwent Fontan procedure at the time of long-term follow-up. According to the nutritional status, children were divided into underweight and no-underweight groups, stunting and no-stunting groups, thinness and no-thinness groups. Univariate and multivariate Logistic regression analysis were used to detect risk factors for malnutrition in the children after Fontan procedure. Results: In the follow-up survey after Fontan procedure, the proportion of underweight, stunted and thin children were 11.9%, 17.8%, and 16.9%, respectively. Multivariate Logistic regression analysis showed that preoperative malnutrition, worse cardiac function, moderate or severe atrioventricular valve regurgitation and long time use of diuretics were the risk factors for underweight in the children after Fontan procedure (P=0.008, P=0.000, P=0.025, P=0.001); preoperative malnutrition, elevated mean pulmonary artery pressure, worse cardiac function, moderate or severe atrioventricular valve regurgitation and long time use of diuretics were the risk factors for stunting in the children after Fontan procedure (P=0.001, P=0.046, P=0.000, P=0.019, P=0.004); preoperative malnutrition, elevated mean pulmonary artery pressure, worse cardiac function, moderate or severe atrioventricular valve regurgitation and long time use of diuretics were the risk factors for thinness in the children after Fontan procedure (P=0.019, P=0.018, P=0.015, P=0.026, P=0.000). Conclusion: Malnutrition is still remained during follow-up period in the children with single ventricle physiology after Fontan procedure. Preoperative malnutrition, worse cardiac function, elevated pulmonary artery pressure, long time use of diuretics and moderate or severe atrioventricular valve regurgitation may be associated with malnutrition after Fontan procedure.

Modified intra/extracardiac conduit total cavopulmonary connection for the treatment of complex congenital heart disease / 中国胸心血管外科临床杂志

@#Objective     To investigate the surgery experience of modified intra/extracardiac conduit total cavopulmonary connection (TCPC). Methods     We retrospectively analyzed clinical data of 47 patients of complex congenital heart disease undergoing intra/extracardiac conduit total cavopulmonary connection in our hospital between January 2008 and December 2015. There were 29 males and 18 females with a median age of 7 years (range 4 to 9 years) and median body weight of 22 kg (range 14 to 38 kg). The heart echocardiography and cardiac imaging confirmed diagnosis suitable for TCPC surgery. Results     There was no early death in the whole group. The mean pulmonary arterial pressure was 16 (12–20) mm Hg and the ventilation time was 14 (7–97) h. The main complications were intractable pleural effusion in 7 patients, low cardiac output syndrome in 3 patients, repeated supraventricular tachycardia in 1 patient. All the patients recovered after treatment. At the end of discharge, the percutaneous oxygen saturation was 85%–96% (92.6%±3.3%). The echocardiography showed the conduit pressure was 0–2 mm Hg. Patients were followed up for 1 to 7 years. Three patients were lost. One patient had intestinal nutrition loss, receving repeated pleural effusion, the treatment was ineffective, died after 4 years. Four patients of repeated pleural effusion improved after treatment. One patient repeated attacks supraventricular tachycardia within 1 year, controlled by amiodaronum, already stopped about 28 months. No recurrence occurred. All survivors were in New York Heart Association (NYHA) functional class Ⅰ or Ⅱ, with good activity tolerance. Conclusion     The modified intra/extracardiac conduit TCPC combines the advantages of both the lateral tunnel and the extracardiac conduit. The operation is simple, used in the treatment of complex congenital heart disease. The short-term and mid-term results are encouraging.

Exercise capacity and quality of life after total cavopulmonary connection at adults / 中华胸心血管外科杂志

Objective The aim of this study was to evaluate the clinical and phychology profile after total cavopulmonary connection procedure and to identify the significant determinants of quality of life.Methods Data from 21 patients underwent total cavopulmonary connection procedure from January 2008 to December 2015 were retrospectively analyzed.Patients under went echocardiography aud cardiopulmonary exercise testing.Used ST-36 questionnaire to perceived health status.The NT-ProBNP levels was test.Results 21 patients aged between 19 to 36 years(mean 26.6 years) were enrolled.The mean follow-up time was(44.2 ± 29.9) months.The mean EF was (59.4 ± 6.2) ％,and the peak oxygen uptake was (19.9 ± 3.6) ml · kg-1 · min-1,perceut predict value was(52.5 ± 8.9) ％.SF-36 score was well.Independent risk for impaired exercise capacity was interval time from surgery.Conclusion The exercise capacity of total cavopulmonary connection patients was impaired.Identify more predict factors of the quality of these patients need further study.

Impact of rhBNP infusion on early postoperative recovery after total cavo-pulmonary connection surgery / 中华胸心血管外科杂志

Objective To compare the effects of rhBNP on the discharge time and pleural effusion in children with total cavo-pulmonary connection, and to provide a more reasonable method for the clinical treatment of postoperative children. Meth-ods Retrospective analysis of Jan 2016 to Jan 2017 during the hospital 40 cases of complex congenital heart disease in children with total cavo-pulmonary connection clinical data, of which 5 cases due to postoperative thrombosis or postoperative venous pressure was too high and had a second Fontan surgery, the patients excluded from the inclusion criteria. 9 cases of children re-turned to the ward after surgery, such as cardiac, diuretic and other conventional treatment based on the application of unequal dose of rhBNP(3-11 days) for the rhBNP group, 26 cases of conventional treatment of children with conventional treatment group. By comparing the early postoperative survival rate, the number of days of hospital stay and the retention time of the tho-racic drainage between the two groups. Results There were no significant differences in the time of cardiopulmonary bypass, postoperative ventilator use time, ICU time and positive inotropic drug scores in all the two groups. All the patients underwent preoperative examination with total cavo-pulmonary connection were alive and healthy discharge. The median hospital stay was 18 days in the rhBNP group(11-33 days, mean 19. 2 days), and the median length of hospital stay was 28 days in the routine treatment group(9 to 95 days, mean 34. 4 days). The difference of hospitalization days between the two groups was statistically significant(P=0. 038). In the retention time of the thoracic drainage tube, the median thoracic drainage tube retention time was 14 days(9-27 days, mean 15. 6 days) in the rhBNP group and 23 days in the conventional treatment group(7-91 days, mean 30. 9(P=0. 046). All the patients had no adverse effects such as excessive fluid load, intractable hypotension and liver or kidney function injury. Conclusion RhBNP can be used safely in pediatric cardiac surgery. Compared with the convention-al treatment group, rhBNP has advantages in the early discharge time and the time of thoracic drainage tube removal in children with total cavo-pulmonary connection.

Exercise capacity and quality of life after total cavopulmonary connection at adults / 中华胸心血管外科杂志

Objective The aim of this study was to evaluate the clinical and phychology profile after total cavopulmonary connection procedure and to identify the significant determinants of quality of life.Methods Data from 21 patients underwent total cavopulmonary connection procedure from January 2008 to December 2015 were retrospectively analyzed.Patients under went echocardiography aud cardiopulmonary exercise testing.Used ST-36 questionnaire to perceived health status.The NT-ProBNP levels was test.Results 21 patients aged between 19 to 36 years(mean 26.6 years) were enrolled.The mean follow-up time was(44.2 ± 29.9) months.The mean EF was (59.4 ± 6.2) ％,and the peak oxygen uptake was (19.9 ± 3.6) ml · kg-1 · min-1,perceut predict value was(52.5 ± 8.9) ％.SF-36 score was well.Independent risk for impaired exercise capacity was interval time from surgery.Conclusion The exercise capacity of total cavopulmonary connection patients was impaired.Identify more predict factors of the quality of these patients need further study.

Echocardiographic Assessment of Pulmonary Arteries Pulsatility Index in Fontan Circulation

BACKGROUND: Late complications after Fontan procedure may be due to the absence of pump and pulsatile pulmonary blood flow in this type of palliation. Our aim was to quantify the degree of pulsation by echocardiographic method in patients with extracardiac total cavopulmonary connection (ECTCPC) in comparison with biventricular circulation and few cases of pulsatile Fontan. METHODS: In a case series study, pulsatility index (PI) derived by echocardiographic method were compared between 20 patients with ECTCPC, 6 patients with pulsatile Fontan and 18 normal individual aged 4 to 20 years old. All patients were in New York Heart Association class of I and there was no report of complication. RESULTS: In patients with ECTCPC pulmonary artery branches Doppler flow study showed lower peak and mean velocities compared to the pulsatile Fontan and normal groups. ECTCPC patients had PI of 0.59 +/- 0.14 and 0.59 +/- 0.09 for right and left pulmonary arteries (RPA and LPA) respectively. PI was higher in patients with preserved antegrade flow (RPA PI = 0.94 +/- 0.26, LPA PI = 0.98 +/- 0.27) and in normal individuals (RPA PI = 1.59 +/- 0.12, LPA PI = 1.64 +/- 0.17) for both branches (p = 0.000). CONCLUSION: Using a Doppler derived index for pulsatility, patients with ECTCPC had the least pulsation. The pulmonary artery flow pattern in patients with preserved antegrade flow showed higher pulsatility indices in both branches. Normal individuals had the greatest pulsatility index.

Risk factors associated with prolonged recovery after the total cavopulmonary connection / 中华胸心血管外科杂志

Objective To study the risk factors of prolonged postoperative recovery after the total cavopulmonary connection(TCPC) in the current era.Methods Data on all patients admitted to the cardiac intensive care unit (CICU) after a TCPC between January 2013 and March 2014 were retrospectively analyzed.We excluded all patients who died and required TCPC takedown.The study cohort was further divided into a prolonged recovery group that included patients with 75％ ile for duration of mechanical ventilation or pleural drainage,and a standard recovery group which included all other patients.A multivariable logistic regression model was used to compare demographic,anatomic,and physiological variables between the prolonged and standard recovery groups.Then,the cohort was separated into a high volume resuscitation group and a low volume resuscitation based on the 75％ ile for volume resuscitation(ml/kg) administered on the first three days after the TCPC.Results Totally 118 TCPC operations were performed.Of the study population (n =118),the median age was 3.8 years (3.1 to 4.8 years) and median weight was 14.8 kg(13.3 to 17.1 kg).The most common diagnosis was double outlet of right ventricle (n =47,39.8％).The extracardiac conduit fenestrated TCPC was the most common surgery(n =79,66.9％).Within the study population,43 (39.8％) patients met criteria for prolonged recovery.Univariate risk factors for prolonged recovery included higher preoperative mPAP(P =0.022),atrioventricular valve regurgitation (P =0.000),longer total bypass time (P =0.044),higher postoperative central venous pressure (P =0.000),AST (P =0.001),ALT (P =0.010),NT-proBNP (P =0.000),SaO2 (P =0.012),I n-otropic score (P =0.001),higher incidence of arrhythmia (P =0.000),low cardiac output syndrome (P =0.000),need for peritoneal dialysis (P =0.000),and requirement for greater volume resuscitation during the 72 postoperative hours(75％ for the entire group,P =0.000).In a multivariable Logistic model,need for greater volume resuscitation (OR 10.860,95 ％ CI 2.681,43.987) and the higher postoperative central venous pressure (OR 1.446,95 ％ C I 1.113,1.879) were the only two independent risk factors for prolonged outcome after the TCPC.Conclusion The need for high volume expansion and higher central venous pressure were the risk factors of mediate prolonged recovery.

Transvascular Implantation of an Implantable Cardioverter-Defibrillator in a Patient Who has Undergone One-and-a-Half Ventricle Repair

Implantable cardioverter-defibrillator (ICD) therapy is acknowledged as a valid treatment method for the effective prevention of sudden cardiac death, which is a major cause of mortality in adult congenital heart disease patients. But ICD implantation by the conventional transvascular approach is not always possible in patients who have undergone palliative surgery due to congenital and structural heart disease. Here, we report a case in which an ICD was transvascularly implanted in an arrhythmogenic right ventricular cardiomyopathy patient who had undergone a one-and-a-half ventricle repair.

Enteropatía perdedora de proteínas en pacientes con corazón univentricular / Protein-losing enteropathy found in patients with univentricular heart

INTRODU: la enteropatía perdedora de proteínas puede aparecer en la evolución de los pacientes con corazón univentricular que sobreviven a la derivación cavopulmonar total. Una vez que se diagnostica, la mortalidad es alta. OBJETIVO: identificar los posibles factores de riesgo de esta complicación. MÉTODOS: se realizó un estudio de cohorte prospectivo de la evolución en 74 pacientes con derivación cavopulmonar total, intervenidos en el Cardiocentro Pediátrico "William Soler", desde enero de 1992 hasta enero de 2011. RESULTADOS: el tiempo promedio de evolución fue de 8 años. Sufrió enteropatía perdedora de proteínas 8,1 % de los pacientes. Se presentó con mayor frecuencia en los operados con la técnica intratrial, en los operados con más de 6 años de edad, y en quienes sufrieron derrames pleurales persistentes en el posoperatorio inmediato. Se encontró relación significativa entre la enteropatía y la disfunción ventricular posoperatoria, con RR= 11,45 (IC: 95 %: 2,37 a 55,16). El análisis multivariado identificó a la disfunción ventricular como factor de riesgo. CONCLUSIÓN: la detección de disfunción ventricular en la evolución del paciente con derivación cavopulmonar debe orientar el tratamiento, en aras de evitar la aparición de enteropatía perdedora de proteínas.

INTRODUCTION: protein-losing enteropathy may occur in the progression of patients with univentricular heart, who survived total cavopulmonary shunt. Once diagnosed, the mortality rate of the condition is high. ONJECTIVE: to identify the possible risk factor of this complication. METHODS: a prospective cohort study of the progression of 74 patients with total cavopulmonary shunt was conducted from January 1992 through January 2011. They had been operated on at "William Soler" pediatric cardiac center. RESULTS: the average time of progression was 8 years. In this group, 8.1 % of patients suffered protein-losing enteropathy that was more frequently seen in patients operated on by the intraatrial technique, aged over 6 years and in those suffering persistent pleural effusion in the immediate postoperative period. Significant statistical relation was found between enteropathy and postoperative ventricular dysfunction with RR= 11.45 (CI: 95 %: 2.37 to 55.16). The multivariate analysis showed that the ventricular dysfunction was a risk factor. CONCLUSIONS: Detection of the ventricular dysfunction in the progression of a patient with cavopulmonary shunt should guide the treatment to avoid occurrence of protein-losing enteropathy.

Modified extracardiac Fontan operation with direct total cavopulmonary connection / 中华胸心血管外科杂志

Objective In order to optimize the Fontan circulation,a technique for direct total cavopulmonary connection was devised.To evaluate its surgical feasibility as well as surgical outcomes,our clinical experience was retrospectively reviewed.Methods From August 2005 to March 2012,23 consecutive patients underwent modified extracardiac Fontan operation with direct total cavopulmonary connection.Clinical profile of the patients,and procedural variables were examined and analyzed.Results All patients had adequately developed main and branch pulmonary arteries.Inferior caval vein was contralateral to the pulmonary trunk main pulmonary artery in 7 cases,ipsilateral in 8,and others in 8.There was 1 hospital death.The other 22 patients remained hemodynamically stable postoperatively.Prolonged effusions (n =13,62％) was a challenging problem.No obvious stenosis was found at the direct cavopulmonary anastomosis.Conclusion we are convinced that a direct total cavopulmonary connection is feasible in select subset of patients.This modified Fontan procedure retains the advantage of extracardiac connections together with the avoidance of prosthetic materials.

Morbilidad y mortalidad posoperatoria en niños con corazón univentricular según diagnóstico morfológico / Postoperative mortality and morbidity found in children with univentricular heart according to the morphological diagnosis

Introducción: la atresia tricuspídea es la cardiopatía congénita cianótica que motiva mayor indicación de cirugía univentricular. El tratamiento quirúrgico consiste en separar las circulaciones sistémica y pulmonar a través de una derivación cavopulmonar total (proceder de Fontan). Objetivo: comparar la evolución posoperatoria del proceder de Fontan entre enero de 1990 y diciembre de 2010 en relación con el diagnóstico morfológico. Métodos: se ordenaron en 2 grupos de estudio (AT: atresia tricuspídea y No AT: con diagnóstico diferente a atresia tricuspídea). Las variables cualitativas y cuantitativas se analizaron a través de las pruebas chi cuadrado y t de student para un nivel de significación £ 0,05. Resultados: presentaron atresia tricuspídea 31 pacientes y 61 no atresia tricuspídea. En el posoperatorio 68 pacientes presentaron complicaciones, 23 con atresia tricúspidea (74,2 por ciento) y 45 con diagnóstico diferente a atresia tricuspídea (73,7 por ciento). Treinta pacientes presentaron efusiones pleurales persistentes durante el posoperatorio, 11 (35,5 por ciento) y 19 (31,4 por ciento) para la atresia tricúspidea y con diagnóstico diferente a atresia tricuspídea respectivamente. Fallecieron 18 enfermos, sin diferencia significativa entre ambos grupos. Durante el seguimiento, la causa de morbilidad más frecuentes fue por capacidad funcional disminuida de forma moderada o grave, en 13 pacientes con atresia tricúspidea (44,8 `por ciento) y en 20 (44,4 por ciento) en el otro grupo (p= 1). Conclusiones: el proceder de Fontan es una opción quirúrgica que permite la supervivencia de los pacientes con corazón univentricular asociada a morbilidad posoperatoria a largo plazo. El diagnóstico morfológico de atresia tricuspídea no se relaciona con la morbilidad y mortalidad

Introduction: tricuspid atresia is the cyanotic congenital heart disease that more frequently involves the indication of univentricular heart surgery. The surgical treatment consists of separating the systemic circulation from the pulmonary one through total cavopulmonary shunt (Fontan procedure). Objective: to compare the evolution of Fontan procedure postoperatively from January 1990 to December 2010 with the morphological diagnosis. Methods: the children were divided into 2 study groups (Triscupid atresia and Triscupid atresia-free with diagnosis different from that of tricuspid atresia). Chi square and Student's tests helped to analyze qualitative and quantitative variables for significance level £ 0.05. Results: tricuspid atresia was observed in 31 patients and 61 tricuspid atresia-free. In the postoperative period, 68 patients suffered complications, 23 with tricuspid atresia (74.2 percent) and 45 diagnosed with a disease other than tricuspid atresia (73.7 percent). Thirty patients had persistent pleural effusions during the postoperative period, 11 (35.5 percent) and 19 (31.4) in the tricuspid atresia group and in the tricuspid atresia-free group respectively. Eighteen patients died and no significant association was found in neither of the groups. During the follow-up, the most frequent cause of morbidity was reduced functional capacity either moderate or severe in 13 patients with tricuspid atresia (44.8 percent) and in 20 children (44.4 percent)from the other group (p= 1). Conclusions: Fontan procedure is a surgical option that allows the survival of patients with univentricular heart associated to long-term postoperative morbidity. The morphological diagnosis of tricuspid atresia is not related to morbility and mortality

Advantages and disadvantages of bidirectional cavopulmonary shunt with additional pulmonary blood flow : a theoretical analysis / 中华胸心血管外科杂志

Objective The bidirectional cavopulmonary shunt (BCPS) is a major step in the staged palliation of functionally univentricular heart defects.Whether to preserve of additional pulmonary blood flow (APBF) has been a highly controversial issue.The purpose is to mathematical model and set out to determine the significa advantages and disadvantages of bidirectional cavopulmonary shunt with additional pulmonary blood flow:a theoretical analysis nce of APBF and the appropriate APBF ratio.Methods We used models of the univentricular circulation after the bidirectional cavopulmonary anastomosis with additional pulmonary blood and to computational analyze the impact of APBF on oxygen delivery,APBF flow on the CVP and SaO2.Results The influence of APBF depends on the ratio of superior vena cava flow to inferior vena cava flow ( QSVC/QIVC ).For QSVC/QIVC ＞ 0.3,APBF may be associated with decreased oxygen delivery.For QSVC/QIVC ＜ 0.3,appropriate APBF may be associated with increased oxygen delivery.A linear relationship exists between the increase of APBF and CVP,and the slope was depended on the value of pulmonary vascular resistance.Estimating APBF from CVP measurements may be a feasible method.A nonlinear relationship between the increase of APBF and oxygen saturation,and estimating APBF from SaO2 measurements may result in errors.BCPS and appropriate APBF may optimal the oxygen delivery with the increase of age and the decrease in QSVC/QIVC.For patients who accepted BCPS without APBF,there is a decreasing tendency of oxygen delivery with the increase of age and the decrease in QSVC/QIVC.For patients who suffered pulmonary arterivenous malformation,there is a more obvious decrease in oxygen delivery.ConclusionFor patients under age who has normal pulmonary vascularbed ( that is,QSVC/QVC ＞ 0.3),elimination of additional pulmonary blood flow can improve the oxygen delivery under a given cardiac output.For patients who with hypoplastic pulmonary vascular or in older patients under age,APBF is necessary to improve oxygen delivery.For patients who have to accept BCPS as the final procedure,preserving of APBF is suggested.