Myocardial tissue expression of mRNA and preoperative neutrophil-lymphocyte ratio in children undergoing congenital heart surgery.

Background: The neutrophil-lymphocyte ratio (NLR) is an easily accessible and inexpensive biomarker that has been shown to predict morbidity and mortality in congenital cardiac surgery. However, its regulatory mechanism remains unclear. This study aims to compare and correlate the tumor necrosis factor alpha (TNF-α), interleukin (IL)-1ß, IL-6, and IL-10 messenger RNAs (mRNAs) with the NLR in patients with tetralogy of Fallot (ToF) and ventricular septal defect (VSD). Methods: A prospective translational study was conducted on 10 children with ToF and 10 with VSD, aged between 1 and 24 months. The NLR was calculated from the blood count taken 24 hours before surgery. The expression of these mRNAs was analyzed in the myocardial tissue of the right atrium prior to cardiopulmonary bypass. Results: Patients with ToF exhibited a higher NLR [ToF 0.46 (interquartile range; IQR) 0.90; VSD 0.28 (IQR 0.17); P=0.02], longer mechanical ventilation time [ToF 24 h (IQR 93); VSD 5.5 h (IQR 8); P<0.001], increased use of vasoactive drugs [ToF 2 days (IQR 1.75); VSD 0 (IQR 1); P=0.01], and longer ICU [ToF 5.5 (IQR 1); VSD 2 (IQR 0.75); P=0.02] and hospital length of stays [ToF 18 days (IQR 17.5); VSD 8.5 days (IQR 2.5); P<0.001]. A negative correlation was found between NLR and oxygen saturation (SaO2) (r=-0.44; P=0.002). In terms of mRNA expression, the ToF group showed a lower expression of IL-10 mRNA (P=0.03). A positive correlation was observed between IL-10-mRNA and SaO2 (r=0.40; P=0.07), and a negative correlation with NLR (r=-0.27; P=0.14). Conclusions: Patients with ToF demonstrated a higher preoperative NLR and lower IL-10 mRNA expression by what appears to be a pro-inflammatory phenotype of cyanotic patients.

Neutrophil-Lymphocyte Ratio in Congenital Heart Surgery: What Is Known and What Is New?

Operative mortality after repair of congenital heart disease has improved dramatically over the past few decades. Nevertheless, there is always room for the additional mitigation of complications and mortality. Being able to anticipate adverse outcomes is clearly important, especially when using low-cost and easily accessible resources. The neutrophil-lymphocyte ratio (NLR) is defined as the ratio of the absolute neutrophil to lymphocyte count, which can be easily measured using a regular white blood cell count. Recently, preoperative NLR has been shown to be a predictor of outcomes in patients undergoing congenital heart surgery. Although it presented promising results, there are still many gaps to be filled like the normal value for children, the ideal cutoff value to predict adverse outcomes, the wide variation and its correlation with other biomarkers, and if it is a modifiable risk factor. The aim of this review is to understand the prognostic value of preoperative NLR as a biomarker predictor of outcomes in patients undergoing congenital heart surgery based on previous clinical studies and to propose future directions in order to solve the above-mentioned questions.

COVID-19 pandemic implications in paediatric and congenital heart surgery in Brazil.

INTRODUCTION: The Coronavirus Disease 2019 (COVID-19) pandemic negatively impacted global healthcare. Consequences in Pediatric and Congenital Heart Surgery programmes and mortality of congenital heart patients infected with severe acute respiratory syndrome coronavirus-2 (SARS-Cov-2) is still to be determined. OBJECTIVE: To study the COVID-19 pandemic implications in Brazilian Pediatric and Congenital Heart Surgery programmes. METHODS: We conducted a national online survey covering all states that perform Pediatric and Congenital Heart Surgery from 10 November to 22 November, 2020, using a Google forms questionnaire. We formulated questions related to impact on surgical volume, case-mix, and mortality. Then we asked about short-term post-operative COVID-19 infection and outcomes. RESULTS: We received responses from 46 centres representing all states where there were a Pediatric and Congenital Heart Surgery programme and all high-volume centres across the country. All but one centre experienced a significant decrease in surgical volume, and 23.9% of the responders revealed less than one-quarter of volume decrement. On the other hand, in over 70% of the centres, there was a significant surgical volume reduction. In addition to this, there was a shift in case-mix in 41 centres (89.1%) towards more complex cases. More than one-third of the responders revealed increased mortality in 2020 compared to previous years, and 43.5% of the programmes (20 centres) had at least one patient contaminated by SARS-Cov-2, accounting for 48 patients. Mortality in post-operative infected patients was 45.8% (22 patients). CONCLUSIONS: In general, Brazilian Pediatric and Congenital Heart Surgery programmes were severely affected by decreased surgical volume, unbalanced case-mix towards more complex cases, and increased mortality. Almost half of the programmes related post-operative COVID-19 contamination with high mortality.

Initial experience with del Nido cardioplegia solution at a Pediatric and Congenital Cardiac Surgery Program in Brazil.

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.

Translation and Validation of the Boston Technical Performance Score in a Developing Country.

INTRODUCTION: The Technical Performance Score (TPS) was developed and subsequently refined at the Boston Children's Hospital. Our objective was to translate and validate its application in a developing country. METHODS: The score was translated into the Portuguese language and approved by the TPS authors. Subsequently, we studied 1,030 surgeries from June 2018 to October 2020. TPS could not be assigned in 58 surgeries, and these were excluded. Surgical risk score was evaluated using Risk Adjustment in Congenital Heart Surgery (or RACHS-1). The impact of TPS on outcomes was studied using multivariable linear and logistic regression adjusting for important perioperative covariates. RESULTS: Median age and weight were 2.2 (interquartile range [IQR] = 0.5-13) years and 10.8 (IQR = 5.6-40) kilograms, respectively. In-hospital mortality was 6.58% (n=64), and postoperative complications occurred in 19.7% (n=192) of the cases. TPS was categorized as 1 in 359 cases (37%), 2 in 464 (47.7%), and 3 in 149 (15.3%). Multivariable analysis identified TPS class 3 as a predictor of longer hospital stay (coefficient: 6.6; standard error: 2.2; P=0.003), higher number of complications (odds ratio [OR]: 1.84; 95% confidence interval [CI]: 1.1-3; P=0.01), and higher mortality (OR: 3.2; 95% CI: 1.4-7; P=0.004). CONCLUSION: TPS translated into the Portuguese language was validated and showed to be able to predict higher mortality, complication rate, and prolonged postoperative hospital stay in a high-volume Latin-American congenital heart surgery program. TPS is generalizable and can be used as an outcome assessment tool in resource diverse settings.

Preoperative Neutrophil-Lymphocyte Ratio Can Predict Outcomes for Patients Undergoing Tetralogy of Fallot Repair

Abstract Introduction: Elevated neutrophil-lymphocyte ratio (NLR) has been associated with poorer outcomes in cyanotic patients undergoing single ventricle palliation. Little is known about this biomarker on patients with tetralogy of Fallot (TOF), the most common cyanotic congenital heart disease. Our objective is to study the impact of preoperative NLR on outcomes of TOF patients undergoing total repair. Methods: This retrospective study included 116 consecutive patients between January 2014 and December 2018. Preoperative NLR was measured from the last complete blood count test before the surgery. Using the cutoff value of 0.80, according to the receiver-operating characteristic (ROC) curve, the sample was divided into two groups (NLR < 0.80 and ≥ 0.80). The primary endpoint was hospital length of stay (LOS). Results: ROC curves showed that higher preoperative NLR was associated with longer hospital LOS, with an area under the curve of 0.801±0.040 (95% confidence interval 0.722 - 0.879; P<0.001). High preoperative NLR was also associated with long intensive care unit (ICU) LOS (P=0.035). Preoperative NLR predicted longer hospital LOS with a sensitivity of 63% and a specificity of 81.4%. Conclusion: Higher preoperative NLR was associated with long ICU and hospital LOS in patients undergoing TOF repair.

Translation and Validation of the Boston Technical Performance Score in a Developing Country

Abstract Introduction: The Technical Performance Score (TPS) was developed and subsequently refined at the Boston Children's Hospital. Our objective was to translate and validate its application in a developing country. Methods: The score was translated into the Portuguese language and approved by the TPS authors. Subsequently, we studied 1,030 surgeries from June 2018 to October 2020. TPS could not be assigned in 58 surgeries, and these were excluded. Surgical risk score was evaluated using Risk Adjustment in Congenital Heart Surgery (or RACHS-1). The impact of TPS on outcomes was studied using multivariable linear and logistic regression adjusting for important perioperative covariates. Results: Median age and weight were 2.2 (interquartile range [IQR] = 0.5-13) years and 10.8 (IQR = 5.6-40) kilograms, respectively. In-hospital mortality was 6.58% (n=64), and postoperative complications occurred in 19.7% (n=192) of the cases. TPS was categorized as 1 in 359 cases (37%), 2 in 464 (47.7%), and 3 in 149 (15.3%). Multivariable analysis identified TPS class 3 as a predictor of longer hospital stay (coefficient: 6.6; standard error: 2.2; P=0.003), higher number of complications (odds ratio [OR]: 1.84; 95% confidence interval [CI]: 1.1-3; P=0.01), and higher mortality (OR: 3.2; 95% CI: 1.4-7; P=0.004). Conclusion: TPS translated into the Portuguese language was validated and showed to be able to predict higher mortality, complication rate, and prolonged postoperative hospital stay in a high-volume Latin-American congenital heart surgery program. TPS is generalizable and can be used as an outcome assessment tool in resource diverse settings.

Impact of COVID-19 Pandemic in a Pediatric and Congenital Cardiovascular Surgery Program in Brazil.

INTRODUCTION: The coronavirus disease 2019 (COVID-19) has negatively impacted healthcare services worldwide. We hypothesized that the pandemic would affect our case mix and mortality. Our objective was to study this impact. METHODS: We retrospectively studied all patients who underwent congenital heart surgeries from March 21st to August 21st in 2019 and 2020 using the institutional electronic database. We compared demographic data, preoperative and postoperative length of stay (LOS), risk stratification using Risk Adjustment for Congenital Heart Surgery (RACHS) classification and outcomes in both periods. RESULTS: We observed a 66.7% decrease in our surgical volume (285 × 95 patients). Patients operated in the pre-pandemic period were older (911.3 [174.8 - 5953.8] days-old) compared to the pandemic period (275 days-old; P<0.05). When the case mix was compared between periods, the percentage of neonatal surgery was increased in the pandemic era (8% × 21.1%; P<0.05), and the number of RACHS 1-2 surgeries decreased significantly (60.7 × 27.4%; P<0.05). Preoperative LOS was increased in the pandemic period (1.2 × 7 days; P=0.001). There was no significant increment in mortality (P=0.1). Two patients tested positive for COVID-19 in the postoperative period and both died. CONCLUSION: Our program observed a sudden decrease in surgical volume and a consequent increase in surgical complexity. There was a non-significant increment in mortality.

Preoperative Neutrophil-Lymphocyte Ratio Can Predict Outcomes for Patients Undergoing Tetralogy of Fallot Repair.

INTRODUCTION: Elevated neutrophil-lymphocyte ratio (NLR) has been associated with poorer outcomes in cyanotic patients undergoing single ventricle palliation. Little is known about this biomarker on patients with tetralogy of Fallot (TOF), the most common cyanotic congenital heart disease. Our objective is to study the impact of preoperative NLR on outcomes of TOF patients undergoing total repair. METHODS: This retrospective study included 116 consecutive patients between January 2014 and December 2018. Preoperative NLR was measured from the last complete blood count test before the surgery. Using the cutoff value of 0.80, according to the receiver-operating characteristic (ROC) curve, the sample was divided into two groups (NLR < 0.80 and ≥ 0.80). The primary endpoint was hospital length of stay (LOS). RESULTS: ROC curves showed that higher preoperative NLR was associated with longer hospital LOS, with an area under the curve of 0.801±0.040 (95% confidence interval 0.722 - 0.879; P<0.001). High preoperative NLR was also associated with long intensive care unit (ICU) LOS (P=0.035). Preoperative NLR predicted longer hospital LOS with a sensitivity of 63% and a specificity of 81.4%. CONCLUSION: Higher preoperative NLR was associated with long ICU and hospital LOS in patients undergoing TOF repair.

The role of the neutrophil-lymphocyte ratio for pre-operative risk stratification of acute kidney injury after tetralogy of Fallot repair.

INTRODUCTION: Acute kidney injury is a risk factor for chronic kidney disease and mortality after congenital heart surgery under cardiopulmonary bypass. The neutrophil-lymphocyte ratio is an inexpensive and easy to measure biomarker for predicting outcomes in children with congenital heart disease undergoing surgical correction. OBJECTIVE: To identify children at high risk of acute kidney injury after tetralogy of Fallot repair using the neutrophil-lymphocyte ratio. METHODS: This single-centre retrospective analysis included consecutive patients aged < 18 years who underwent tetralogy of Fallot repair between January 2014 and December 2018. The pre-operative neutrophil-lymphocyte ratio was measured using the last pre-operative complete blood count test. We used the Acute Kidney Injury Network definition. RESULTS: A total of 116 patients were included, of whom 39 (33.6%) presented with acute kidney injury: 20 (51.3%) had grade I acute kidney injury, nine had grade II acute kidney injury (23.1%), and 10 (25.6%) had grade III acute kidney injury. A high pre-operative neutrophil-lymphocyte ratio was associated with grade III acute kidney injury in the post-operative period (p = 0.04). Patients with acute kidney injury had longer mechanical ventilation time (p = 0.023), intensive care unit stay (p < 0.001), and hospital length of stay (p = 0.002). CONCLUSION: Our results suggest that the pre-operative neutrophil-lymphocyte ratio can be used to identify patients at risk of developing grade III acute kidney injury after tetralogy of Fallot repair.

Anomalous aortic origin of the left main coronary artery associated with arterial compression-Case report.

BACKGROUND: Anomalous aortic origin of coronary artery is a rare finding, with varied presentation and symptomatology. Increasingly recognized by cardiac imaging, when found it raises questions about the appropriate approach and management. CASE PRESENTATION: We present a case of an 11-year-old female who presented with episodes of shortness of breath, angina, and syncope during exercise. Further investigation demonstrated episodes of nonsustained ventricular tachycardia on Holter and coronary angiotomography revealed that the left coronary artery had an anomalous origin from the right cusp with initial short intramural segment and significant external compression in its initial course between the aorta and the pulmonary artery. The patient was submitted to surgical correction with dissection of left coronary artery posterior to the pulmonary artery, coronary arteriotomy, roof ampliation with the autologous pericardium, and creation of neo-ostium in aorta. The patient had a satisfactory postoperative recovery, was discharged on the fifth-day post-op, and remains asymptomatic after 14 months of follow-up. Herein we present surgical video and postoperative echo and computed tomography scan.

Atrioventricular Valve Repair in Single Ventricle Physiology: Timing Matters.

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

Prognostic value of the preoperative neutrophil-lymphocyte ratio in patients undergoing the bidirectional Glenn procedure.

INTRODUCTION: The neutrophil-lymphocyte ratio (NLR) has been associated with worse outcomes in patients undergoing coronary artery bypass graft surgery. Little is known about this association in the pediatric population who require surgery for congenital heart defects, especially in patients with a single ventricle (SV). OBJECTIVE: To analyze the association of the preoperative NLR with outcomes in patients undergoing the bidirectional Glenn procedure. METHODS: This study involved a retrospective cohort analysis of 141 consecutive patients with SV undergoing the bidirectional Glenn procedure between January 2011 and December 2017 in two centers. The preoperative NLR was included in the last hemogram test before surgery. According to the NLR level, the patients were divided into group I (NLR < 1), group II (NLR between 1 and 2), and group III (NLR > 2). The primary endpoint was total hospital length of stay (LOS), and secondary endpoints were mechanical ventilation (MV) time, intensive care unit (ICU) LOS, ventricular dysfunction, complications, and middle-term mortality. RESULTS: The average follow-up duration was 48 months. There were 61, 47, and 33 patients in groups I, II, and III, respectively. Patients in group III exhibited an increased risk of prolonged total hospital LOS (P = .00). An increase in MV time (P = .03) and ICU LOS (P = .02) was also observed in this group, and these patients experienced greater mortality in 24 months following the surgery (P = .03). There was no association between the NLR and ventricular dysfunction (P = .26) and complications (P = .46). CONCLUSION: A high preoperative NLR was associated with worse outcomes in patients with SV physiology undergoing the bidirectional Glenn procedure.

Single Ventricle Palliation in a Developing Sub-Saharan African Country: What Should be Improved?

INTRODUCTION: Single ventricle physiology management is challenging, especially in low-income countries. OBJECTIVE: To report the palliation outcomes of single ventricle patients in a developing African country. METHODS: We retrospectively studied 83 consecutive patients subjected to single ventricle palliation in a single center between March 2011 and December 2017. Preoperative data, surgical factors, postoperative results, and survival outcomes were analyzed. The patients were divided by palliation stage: I (pulmonary artery banding [PAB] or Blalock-Taussig shunt [BTS]), II (Glenn procedure), or III (Fontan procedure). RESULTS: Of the 83 patients who underwent palliation (stages I-III), 38 deaths were observed (31 after stage I, six after stage II, and one after stage III) for an overall mortality of 45.7%. The main causes of operative mortality were multiple organ dysfunction due to sepsis, shunt occlusion, and cardiogenic shock. Twenty-eight survivors were lost to follow-up (22 after stage I, six after stage II). Thirteen stage II survivors are still waiting for stage III. The mean follow-up was 366 ± 369 days. Five-year survival was 28.4 % for PAB and 30.1% for BTS, while that for stage II and III was 49.8% and 57.1%, respectively. Age (hazard ratio, 0.61; 95% confidence interval: 0.47-0.7; P = .000) and weight at surgery (hazard ratio, 0.45; 95% confidence interval: 0.31-0.64; P = .002) impacted survival. CONCLUSION: A high-mortality rate was observed in this initial experience, mainly in stage I patients. A large number of patients were lost to follow-up. A task force to improve outcomes is urgently required.

Rational Use of Mechanical Circulatory Support as a Bridge to Pediatric and Congenital Heart Transplantation.

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.

Rational use of mechanical circulatory support as a bridge to pediatric and congenital heart transplantation

Abstract 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.