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Objective: The aim of this study was to evaluate outcome measures between our standard multidose cardioplegia protocol and a del Nido cardioplegia protocol in congenital heart surgery patients. Methods: Retrospective single-center study including 250 consecutive patients that received del Nido cardioplegia (DN group) with a mandatory reperfusion period of 30% of cross clamp time and 250 patients that received a modified St. Thomas’ solution (ST group). Groups were matched by age, weight, gender, and Risk Adjustment for Congenital Heart Surgery (RACHS-1) scores. Preoperative hematocrit and oxygen saturation were also recorded. Outcomes analyzed were the vasoactive inotropic score (VIS), lactate, ventilation time, ventricular dysfunction with low cardiac output syndrome (LCOS), intensive care unit (ICU) length of stay (LOS), hospital LOS, bypass and aortic cross-clamp times, and in-hospital mortality. Results: Both groups were comparable demographically. Statistically significant differences (p ⩽ 0.05) were noted for cardiac dysfunction with LCOS, hematocrit at end of surgery (p = 0.0038), VIS on ICU admission and at end of surgery (p = 0.0111), and ICU LOS (p = 0.00118) with patients in the DN group having more desirable values for those parameters. Other outcome measures did not reach statistical significance. Conclusion: In our congenital cardiac surgery population, del Nido cardioplegia strategy was associated with less ventricular dysfunction with LCOS, a lower VIS and decreased ICU LOS compared with patients that received our standard myocardial protection using a modified St. Thomas’ solution. Despite the limitation of this study, including its retrospective nature and cohort size, these data supported our transition to incorporate del Nido cardioplegia solution with a mandatory reperfusion period as the preferred myocardial protection method in our program.

Pediatric end-stage heart disease is surgically managed by heart transplantation. A major complication of primary transplantation (PTx) is coronary allograft vasculopathy (CAV), a form of accelerated atherosclerosis. Retransplantation (RTx) has been the management of CAV; however, there is limited comprehensive literature on this subject. Here we report 25 years of single-center experience in managing CAV with RTx and place it in the context of recent studies.A retrospective cohort study was undertaken on patients who underwent PTx 18 years old and subsequent RTx due to CAV at the Heart Institute (InCor) University of São Paulo Medical School between 1992 and 2018. The maintenance immunosuppression protocol was double immunosuppression. For both PTx and RTx, quantitative and qualitative analyses were conducted for transplantation indication, donor/recipient demographics, post-transplant survival, rejection, infection, and immunosuppression.Between 1992 and 2018, 200 children underwent heart transplantation. Ten re-transplantations were performed, for which 7 (70%) were for CAV. Ages at RTx ranged from 11.5 to 29.3 years (19.1 ± 5.68 years; median 18.2 years). The mean time between PTx and RTx was 12.9 ± 3.4 years (median 13.4 years). The Kaplan-Meier survival rate at 1 month, 3 years, and 5 years was 85.7%, 71.5%, and 47.6%, respectively.Cardiac RTx can be a management option for CAV in patients who have undergone PTx in childhood with double immunosuppression therapy.

Objective: This study aimed to evaluate Ebstein’s anomaly surgical correction and its early and long-term outcomes. Methods: A retrospective analysis of 62 consecutive patients who underwent surgical repair of Ebstein’s anomaly in our institution from January 2000 to July 2016. The following long-term outcomes were evaluated: survival, reoperations, tricuspid regurgitation, and postoperative right ventricular dysfunction. Results: Valve repair was performed in 46 (74.2%) patients - 12 of them using the Da Silva cone reconstruction; tricuspid valve replacement was performed in 11 (17.7%) patients; univentricular palliation in one (1.6%) patient; and the one and a half ventricle repair in four (6.5%) patients. The patients’ mean age at the time of surgery was 20.5±14.9 years, and 46.8% of them were male. The mean follow-up time was 8.8±6 years. The 30-day mortality rate was 8.06% and the one and 10-year survival rates were 91.9% both. Eleven (17.7%) of the 62 patients required late reoperation due to tricuspid regurgitation, in an average time of 7.1±4.9 years after the first procedure. Conclusion: In our experience, the long-term results of the surgical treatment of Ebstein's anomaly demonstrate an acceptable survival rate and a low incidence of reinterventions.

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.

Introduction: Donor shortage and organ allocation is the main problem in pediatric heart transplant. Mechanical circulatory support is known to increase waiting list survival, but it is not routinely used in pediatric programs in Latin America. Methods: All patients listed for heart transplant and supported by a mechanical circulatory support between January 2012 and March 2016 were included in this retrospective single-center study. The endpoints were mechanical circulatory support time, complications, heart transplant survival and discharge from the hospital. Results: Twenty-nine patients from our waiting list were assessed. Twelve (45%) patients were initially supported by extracorporeal membrane oxygenation (ECMO) and a centrifugal pump was implanted in 17 (55%) patients. Five patients initially supported by ECMO were bridged to another device. One was bridged to a centrifugal pump and four were bridged to Berlin Heart Excor®. Among the 29 supported patients, 18 (62%) managed to have a heart transplant. Thirty-day survival period after heart transplant was 56% (10 patients). Median support duration was 12 days (interquartile range [IQR] 4 - 26 days) per run and the waiting time for heart transplant was 9.5 days (IQR 2.5-25 days). Acute kidney injury was identified as a mortality predictor (OR=22.6 [CI=1.04-494.6]; P=0.04). Conclusion: Mechanical circulatory support was able to bridge most INTERMACS 1 and 2 pediatric patients to transplant with an acceptable complication rate. Acute renal failure increased mortality after mechanical circulatory support in our experience.

Background: The Fontan operation (FO) has evolved and many centers have demonstrated improved results relative to those from earlier eras. We report a single-institution experience over three decades, describing the outcomes and evaluating risk factors. Methods: Successive patients undergoing primary FO were divided into era I (1984-1994), era II (1995-2004), and era III (2005-2014). Clinical and operative notes were reviewed for demographic, anatomic, and procedure details. End points included early and late mortality and a composite of death, heart transplantation (HTX), or Fontan takedown. Results: A total of 420 patients underwent 18 atriopulmonary connections, 82 lateral tunnels (LT), and 320 extracardiac conduit (EC) Fontan procedures. Forty-six (11%) patients died; early and late mortality were 7.9% and 3.1%, respectively. Eight (1.9%) patients underwent HTX, 11 (2.6%) underwent Fontan conversion to EC, and 1 (0.2%) takedown of EC to bidirectional Glenn shunt. Prevalence of concomitant valve surgery ( P < .001) and pulmonary artery reconstruction ( P < .001) differed over the eras. Preoperative valve regurgitation was associated with likelihood of early mortality (odds ratio [OR] = 3.5, P = .002). Embolic events (OR = 1.9, P = .047), preoperative valve regurgitation (OR = 2.3, P = .029), diagnosis of unbalanced atrioventricular canal defect (OR = 1.14, P = .03), and concomitant valve replacement (OR = 6.9, P = .001) during the FO were associated with increased risk of the composite end point (death, HTX, or takedown). Conclusion: Technical modifications did not result in improved results across eras, due in part to more liberal indications for surgery in the recent years. Valve regurgitation, unbalanced atrioventricular canal, embolic events, or concomitant valve replacement were associated with FO failure.

Background: Heart transplantation is considered the gold standard therapy for the advanced heart failure, but donor shortage, especially in pediatric patients, is the main limitation for this procedure, so most sick patients die while waiting for the procedure. Objective: To evaluate the use of short-term circulatory support as a bridge to transplantation in end-stage cardiomyopathy. Methods: Retrospective clinical study. Between January 2011 and December 2013, 40 patients with cardiomyopathy were admitted in our Pediatric Intensive Care Unit, with a mean age of 4.5 years. Twenty patients evolved during hospitalization with clinical deterioration and were classified as Intermacs 1 and 2. One patient died within 24 hours and 19 could be stabilized and were listed. They were divided into 2 groups: A, clinical support alone and B, implantation of short-term circulatory support as bridge to transplantation additionally to clinical therapy. Results: We used short-term mechanical circulatory support as a bridge to transplantation in 9. In group A (n=10), eight died waiting and 2 patients (20%) were transplanted, but none was discharged. In group B (n=9), 6 patients (66.7%) were transplanted and three were discharged.The mean support time was 21,8 days (6 to 984h). The mean transplant waiting list time was 33,8 days. Renal failure and sepsis were the main complication and causeof death in group A while neurologic complications were more prevalent en group B. Conclusion: Mechanical circulatory support increases survival on the pediatric heart transplantation waiting list in patients classified as Intermacs 1 and 2.

Introduction: Post-cardiotomy myocardial dysfunction requiring mechanical circulatory support occurs in about 0.5% of cases. In our environment, the use of extracorporeal membrane oxygenation has been increasing in recent years. Objective: To evaluate the impact of investment in professional training and improvement of equipment in the rate of weaning from extracorporeal membrane oxygenation and survival. Methods: A retrospective study. Fifty-six pediatric and/or congenital heart patients underwent post-cardiotomy extracorporeal membrane oxygenation at our institution between November 1999 and July 2014. We divided this period into two phases: phase I, 36 cases (before the structuring of the extracorporeal membrane oxygenation program) and phase II, 20 cases (after the extracorporeal membrane oxygenation program implementation) with investment in training and equipment). Were considered as primary outcomes: extracorporeal membrane oxygenation weaning and survival to hospital discharge. The results in both phases were compared using Chi-square test. To identify the impact of the different variables we used binary logistic regression analysis. Results: Groups were comparable. In phase I, 9 patients (25%) were weaned from extracorporeal membrane oxygenation, but only 2 (5.5%) were discharged. In phase II, extracorporeal membrane oxygenation was used in 20 patients, weaning was possible in 17 (85%), with 9 (45%) hospital discharges (P

Background:Transposition of the great arteries (TGA) is the most common cyanotic cardiopathy, with an incidence ranging between 0.2 and 0.4 per 1000 live births. Many patients not treated in the first few months of life may progress with severe pulmonary vascular disease. Treatment of these patients may include palliative surgery to redirect the flow at the atrial level.Objective:Report our institutional experience with the palliative Senning procedure in children diagnosed with TGA and double outlet right ventricle with severe pulmonary vascular disease, and to evaluate the early and late clinical progression of the palliative Senning procedure.Methods:Retrospective study based on the evaluation of medical records in the period of 1991 to 2014. Only patients without an indication for definitive surgical treatment of the cardiopathy due to elevated pulmonary pressure were included.Results:After one year of follow-up there was a mean increase in arterial oxygen saturation from 62.1% to 92.5% and a mean decrease in hematocrit from 49.4% to 36.3%. Lung histological analysis was feasible in 16 patients. In 8 patients, pulmonary biopsy grades 3 and 4 were evidenced.Conclusion:The palliative Senning procedure improved arterial oxygen saturation, reduced polycythemia, and provided a better quality of life for patients with TGA with ventricular septal defect, severe pulmonary hypertension, and poor prognosis. Fundamento:A transposição das grandes artérias (TGA) é a cardiopatia cianogênica mais comum, com incidência variando de 0,2 a 0,4 por 1000 nascidos vivos. Muitos dos pacientes não tratados nos primeiros meses de vida podem evoluir com doença vascular pulmonar grave, e um método terapêutico para o tratamento desses pacientes pode ser realizado por meio de uma cirurgia paliativa para redirecionamento do fluxo em nível atrial.Objetivo:Relatar a experiência institucional com a cirurgia de Senning paliativo em crianças com diagnóstico de TGA e dupla via de saída do ventrículo direito com doença vascular pulmonar severa. Avaliar, também, a evolução clínica precoce e tardia da operação de Senning paliativo.Métodos:Estudo retrospectivo, baseado na avaliação de prontuários no período de 1991 a 2014. Somente pacientes sem indicação para o tratamento cirúrgico definitivo da cardiopatia em razão de elevadas pressões pulmonares.Resultados:Evidenciou-se após um ano de evolução um aumento médio da saturação arterial de oxigênio de 62,1% para 92,5% e uma redução média do hematócrito de 49,4% para 36,3%. A análise da histologia pulmonar foi possível em 16 pacientes. Em oito pacientes foi evidenciada lesão pulmonar grau 3 e 4.Conclusão:A cirurgia de Senning paliativo melhorou a saturação de oxigênio arterial, reduziu a policitemia e proporcionou uma melhora da qualidade de vida em pacientes com TGA com comunicação interventricular, os quais possuíam hipertensão pulmonar severa com prognóstico fechado.

Alterações estruturais e funcionais estão presentes na circulação pulmonar em pacientes com comunicações cardíacas congênitas. A inflamação tem um papel primordial no remodelamento vascular pulmonar que acompanha esta condição. Todo este processo tem implicação na indicação cirúrgica e no padrão hemodinâmico pulmonar no período pós-operatório imediato e tardio. Informações sobre a relação entre hemodinâmica pulmonar pós-operatória, inflamação e desfechos de curto, médio e longo prazo, em pacientes pediátricos submetidos a cirurgia, são escassas. Desta forma, este estudo teve por objetivo investigar em pacientes pediátricos, no contexto do tratamento cirúrgico, possíveis relações entre hemodinâmica pulmonar, elementos de resposta inflamatória e eventos clínicos no período pós-operatório. Além disso, identificar possíveis preditores de pressão arterial pulmonar persistentemente alterada após a alta hospitalar. MÉTODOS: Estudo do tipo prospectivo, de coorte, com observações de caráter longitudinal, envolvendo a comparação entre grupos hemodinâmicos. A coleta de dados ocorreu entre os anos de 2016 e 2018. O protocolo do estudo foi aprovado pela Comissão de Ética para Análise de Projetos de Pesquisa (CAPPesq 4324/15/151) e teve o suporte financeiro da Fundação de Amparo a Pesquisa do Estado de São Paulo (FAPESP Processo nº 2016/21587-5). Foram incluídas 40 crianças. A idade variou entre quatro e 35 meses (mediana de 11 meses), sendo 63% com síndrome de Down, todas submetidas ao tratamento cirúrgico de correção do defeito do septo átrio ventricular (60%) ou comunicação interventricular (40%). A coleta dos dados clínicos, cirúrgicos, laboratoriais e hemodinâmicos ocorreu durante o período de internação. A ecocardiografia transtorácica foi realizada seis meses após a correção cirúrgica para determinação não invasiva da pressão arterial pulmonar. A resposta inflamatória foi avaliada pela análise de 36 marcadores séricos específicos, na condição basal, quatro e 24 horas após o término da circulação extracorpórea, além de elementos celulares e humorais pesquisados habitualmente na rotina assistencial. No período pós-operatório, os pacientes ficaram monitorizados com cateter em artéria pulmonar e pressão arterial sistêmica invasiva por 72 horas. A razão entre as pressões médias arteriais pulmonar e sistêmica foi mensurada no período pós-operatório e a média dos quatro primeiros valores (PMAP/PMASPOI) com medidas obtidas a cada duas horas na unidade de cuidados intensivos, foi usada para categorizar os pacientes em dois grupos: PMAP/PMASPOI < 0,40 (22 pacientes) e PMAP/PMASPOI > 0,40 (18 pacientes). Eventos Cardiorrespiratórios e Hemodinâmicos Relevantes (ECRHR) foram definidos antes do início do projeto como uma conjunção de alterações cardiocirculatórias e respiratórias importantes que tivessem início com o aumento da pressão pulmonar. RESULTADOS: A situação hemodinâmica pulmonar observada nas primeiras seis horas após a cirurgia (PMAP/PMASPOI) correlacionou com os dados obtidos no centro cirúrgico (p < 0,001) e antecipou a curva pressórica registrada nos 2,5 dias subsequentes (p < 0,001). Na comparação entre grupos, cinco marcadores inflamatórios se mostraram elevados no grupo PMAP/PMASPOI > 0,40 comparativamente ao grupo PMAP/PMASPOI < 0,40: G-CSF (p=0,040), IL1-RA (p = 0,020), IL-6 (p=0,003), IL-21(p=0,047) e volume plaquetário médio (p=0,018). A condição PMAP/PMASPOI > 0,40 e o nível sérico elevado da proteína MIF no período pós-operatório imediato mostraram-se preditores de ECRHR (hazard ratio e IC 95%, respectivamente, 5,07 (1,10 - 23,45), p=0,038, e 3,29 (1,38 7,88), p < 0,001). Os ECRHR ocorreram em 27,5% dos pacientes e influenciaram o tempo de ventilação mecânica (p < 0,001). A condição PMAP/PMASPOI > 0,40 foi preditora de pressão sistólica arterial pulmonar elevada (> 30 mmHg, pela ecocardiografia) seis meses após a cirurgia (hazard ratio 7,50 (1,72 32,80), p=0,007). CONCLUSÕES: O padrão de comportamento hemodinâmico pulmonar foi identificado no início do período pós-operatório e correlacionou-se com parâmetros hemodinâmicos do centro cirúrgico ao final da circulação extracorpórea. PMAP/PMASPOI > 0,40 correlacionou-se com níveis circulantes de marcadores inflamatórios mensurados tanto no período pré como no pós-operatório imediato: IL-6, IL1-RA, G-CSF, IL-21 e volume plaquetário médio. O comportamento hemodinâmico inicial e o nível da proteína MIF na quarta hora correlacionam-se com Eventos Cardiorrespiratórios Hemodinâmicos Relevantes. A evolução da pressão arterial pulmonar seis meses após o tratamento cirúrgico pode ser prevista a partir do comportamento hemodinâmico pulmonar no período pós-operatório imediato Structural and functional changes are present in the pulmonary circulation in patients with congenital cardiac communications. Inflammation, which comes alongside with this condition, plays a major role in pulmonary vascular remodeling. This process has implications for the surgical indication and for the pulmonary hemodynamic performance, both in the immediate and late postoperative period. There is currently little information on the correlation among postoperative pulmonary hemodynamics, inflammation, and outcomes in pediatric patients undergoing surgery. Thus, this study aimed at investigating relationships among pulmonary hemodynamics, elements of inflammatory response, and clinical events in the postoperative period in pediatric patients undergoing surgical treatment. Furthermore, this study also aimed at identifying possible predictors of late persistent changes in pulmonary arterial pressure after hospital discharge. METHODS: Prospective, cohort study with longitudinal observations involving comparison between hemodynamic groups. Data were collected from November 2016 to October 2018. The study protocol was approved by the Ethics Committee for Analysis of Research Projects (CAPPesq 4324/15/151) with the financial support of the Fundação de Amparo à Pesquisa do Estado de São Paulo (FAPESP - Process nº 2016 / 21587-5). Forty children were included. The age ranged from four to 35 months (median 11 months), 63% of whom had Down syndrome. They underwent surgical treatment to correct either the atrioventricular septal defect (60%) or the ventricular septal defect (40%). The collection of clinical, surgical, laboratory, and hemodynamic data occurred during the hospitalization period. Transthoracic echocardiography was performed six months after surgical correction for noninvasive determination of pulmonary arterial pressure. The inflammatory response was assessed at baseline (before surgical procedure) and 4 hours and 24 hours after the end of cardiopulmonary bypass - by the analysis of 36 specific serum markers. In addition, cellular and humoral elements were also investigated as part of the hospital routine. Postoperatively, pulmonary and systemic pressure were monitored with invasive indwelling catheters for 72 hours. The pulmonary/systemic mean arterial pressure ratio was also obtained. The mean of the first four values (PMAP/PMASPOI), with measurements obtained every two hours in the ICU, was used to categorize the patients into two groups: PMAP/PMASPOI < 0.40 (22 patients) and PMAP/PMASPOI > 0.40 (18 patients). The relevant cardiorespiratory and hemodynamic events were defined prior to the study as a composite of major cardiocirculatory and respiratory disturbances. RESULTS: The pulmonary hemodynamic performance observed in the first six hours after surgery (PMAP/PMASPOI) was correlated with data obtained in the operating room (p < 0.001) and anticipated the pressure curve recorded in the 2.5 days (p 0.40: G-CSF (p=0.040), IL1-RA (p=0.020), IL-6 (p=0.003), IL-21 (p=0.047) and mean platelet volume (p=0.018). The PMAP/PMASPOI > 0.40 condition and the elevated serum level of the MIF protein in the immediate postoperative period were shown as predictors of relevant cardiorespiratory and hemodynamic events (hazard ratio with 95% CI, respectively, 5.07 (1.10 - 23.45), p=0.038, and 3.29 (1.38 - 7.88), p < 0.001). Besides, relevant cardiorespiratory and hemodynamic events influenced the time of mechanical ventilation (p < 0.001). The PMAP/PMASPOI > 0.40 was a predictor of elevated pulmonary arterial systolic pressure (> 30 mmHg, by echocardiography) six months after surgery (hazard ratio 7.50 (1.72 - 32.80), p=0.007). CONCLUSIONS: The pattern of pulmonary hemodynamic performance was identified at the beginning of the postoperative period and correlated with hemodynamic parameters in the operating room at the end of cardiopulmonary bypass. PMAP/PMASPOI > 0.40 was correlated with circulating levels of inflammatory markers measured both before surgery and in the immediate postoperative period as IL-6, IL1-RA, G-CSF, IL-21, and mean platelet volume. The initial postoperative hemodynamic performance and the level of the MIF protein four hours postoperatively were correlated with relevant hemodynamic and cardiorespiratory events. The evolution of pulmonary arterial pressure six months after surgery can be predicted by taking into consideration the pulmonary hemodynamic performance in the immediate postoperative period