Approach to red blood cell transfusions in post-operative congenital heart disease surgery patients: when to stop?

BACKGROUND: The best transfusion approach for CHD surgery is controversial. Studies suggest two strategies: liberal (haemoglobin ≤ 9.5 g/dL) and restrictive (waiting for transfusion until haemoglobin ≤ 7.0 g/dL if the patient is stable). Here we compare liberal and restrictive transfusion in post-operative CHD patients in a cardiac intensive care unit. METHODS: Retrospective analysis was conducted on CHD patients who received liberal transfusion (2019-2021, n=53) and restrictive transfusion (2021-2022, n=43). RESULTS: The two groups were similar in terms of age, gender, Paediatric Risk of Mortality-3 score, Paediatric Logistic Organ Dysfunction-2 score, Risk Adjustment for Congenital Heart Surgery-1 score, cardiopulmonary bypass time, vasoactive inotropic score, total fluid balance, mechanical ventilation duration, length of cardiac intensive care unit stay, and mortality. The liberal transfusion group had a higher pre-operative haemoglobin level than the restrictive group (p < 0.05), with no differences in pre-operative anaemia. Regarding the minimum and maximum post-operative haemoglobin levels during a cardiac intensive care unit stay, the liberal group had higher haemoglobin levels in both cases (p<0.01 and p=0.019, respectively). The number of red blood cell transfusions received by the liberal group was higher than that of the restrictive group (p < 0.001). There were no differences between the two groups regarding lactate levels at the time of and after red blood cell transfusion. The incidence of bleeding, re-operation, acute kidney injury, dialysis, sepsis, and systemic inflammatory response syndrome was similar. CONCLUSIONS: Restrictive transfusion may be preferable over liberal transfusion. Achieving similar outcomes with restrictive transfusions may provide promising evidence for future studies.

Effect of cardiopulmonary bypass on thiol/disulfide homeostasis in congenital heart surgery.

Background: This study aims to investigate whether thiol/disulfide homeostasis parameters measurements could be used as a new biomarker to predict the pre- and post-cardiopulmonary bypass oxidative status of pediatric patients undergoing congenital heart surgery. Methods: A total of 40 children with congenital heart disease (17 males, 23 females; mean age: 39.6±40.0 months; range, 2 to 216 months) who underwent open-heart surgery were included. The control group consisted of 40 age- and sex-matched healthy children (18 males, 22 females; mean age: 42.8±46.6 months; range, 12 to 156 months). The patients with congenital heart disease were divided into two groups as cyanotic patients (n=18) and acyanotic patients (n=22). Thiol/disulfide parameters were compared among the cyanotic, acyanotic congenital heart disease patients, and control group preoperatively (pre-CPB). The effects of cardiopulmonary bypass on thiol/disulfide parameters, pre-CBP, immediately after cardiopulmonary bypass (post-CPB0), and 24 h after cardiopulmonary bypass (post-CPB24) were investigated. Results: The mean native and total thiol levels in the cyanotic patients were significantly lower than those in the acyanotic patients and control group (p<0.0001). The cyanotic group exhibited higher disulfide levels than the acyanotic group (p<0.01). The mean native thiol and total thiol levels significantly decreased in the post-CPB0 (p<0.0001). The mean disulfide levels significantly increased in the post-CPB0 than the pre-CPB values (p<0.001). Post-CPB24 native and total thiol levels were elevated compared to post-CPB0 (p<0.0001). The mean disulfide levels significantly increased in the post-CPB24 period than the post-CPB0 values (p<0.001). The survivor patients responded better to oxidative stress than non-survivor patients. Conclusion: Thiol/disulfide measurement is a promising biomarker in determining the pre- and post-cardiopulmonary bypass oxidative status of pediatric patients undergoing congenital heart surgery. The interpretation of thiol/disulfide levels, pre- and postoperatively, may be used in predicting mortality and outcomes of these patients earlier.

Predictors of extubation in the operating room after pediatric cardiac surgery: A single-center retrospective study.

Background: In this study, we aimed to evaluate the outcomes of our on-table extubation strategy in patients with congenital heart disease. Methods: Between April 2021 and November 2022, a total of 114 pediatric patients (58 males, 56 females; median age: 25.3 months; range, 57.5 to 4.4 months) who were operated for congenital heart diseases were retrospectively analyzed. The patients were evaluated according to the Society of Thoracic Surgeons-European Association for Cardio-Thoracic Surgery (STS-EACTS) scoring system. Perioperative patient data were analyzed and correlated with the extubation status. Results: Overall, 56% of the patients were extubated in the operating room. There was an association between fluid balance per unit body surface area, longer cardiopulmonary bypass and cross-clamp times and on-table extubation. Lactate value prior to extubation, STS-EACTS mortality category, estimated mortality, and estimated morbidity were statistically significant with regards to the achievability of extubation. Multivariate analysis revealed lactate value prior to extubation and estimated postoperative length of hospital stay to be significant factors affecting on-table extubation. There was a significant correlation between decreased length of intensive care unit and hospital stay and on-table extubation. Conclusion: The outcomes of our on-table extubation strategy for patients with congenital heart disease reveal the feasibility of this approach. Higher lactate and fluid balance/body surface area levels, longer cross-clamp and cardiopulmonary bypass durations, increased surgical complexity are indicators of a failure to perform on-table extubation. This strategy is also associated with shorter intensive care unit and hospital length of stays as an additional clinical benefit.

Comparison of high flow oxygen therapy versus noninvasive mechanical ventilation for successful weaning from invasive ventilation in children: An observational study.

Post-extubation respiratory failure is associated with a poor prognosis due to increased ventilator-associated pneumonia, and longer length of stay in the ICU and hospital. In this study, we aimed to evaluate the efficacy of high-flow nasal cannula (HFNC) and noninvasive mechanical ventilation (NIMV) on extubation success in children. A total of 48 patients, aged between 1 month and 18 years, who were weaned to either NIMV or HFNC were included. Patients who had tracheostomy or were not weaned and underwent unplanned extubation were excluded. Age, gender, anthropometric parameters, Pediatric Risk of Mortality and Pediatric Logistic Organ Dysfunction scores, oxygenation index, mechanical ventilation length of stay (LOS), HFNC/NIMV LOS, Modified Downes-Silverman score (MDS), and venous blood gas parameters, pediatric intensive care unit (PICU) LOS were recorded. 24 patients were extubated to NIMV, and 24 patients to HFNC. HFNC LOS and NIMV LOS were similar (P = .621). The failure rates at the 48th hour of HFNC and NIMV were 33% (n = 8), and 33% respectively (n = 8) (P = 1.0). PICU LOS and mortality rate was also similar (P = .06, P = .312 respectively). MDS decreased significantly in both groups (P < .001, P = .02 respectively). Changes in blood gas parameters and MDS within the first 48-hour of device application were similar between the 2 groups. HFNC is not inferior to NIMV in patients with extubation difficulty or those expected to have such difficulty in terms of treatment success, PICU LOS, and mortality. Therefore, HFNC appears to be a weaning technique alternative to NIMV after extubation.

Macrophage Activation Syndrome in a Child with Juvenile Idiopathic Arthritis Secondary to SARS-CoV-2.

Severe acute respiratory syndrome coronavirus-2 (SARS-CoV-2) has caused a pandemic affecting many countries and millions of people. Physicians have encountered some rare and challenging cases related to SARS-CoV-2, a novel virus with still many unknowns. In order to share our experience of a such clinical picture, we present here a child with SARS-CoV-2-induced macrophage activation syndrome in the setting of juvenile idiopathic arthritis.

Continuous Renal Replacement Therapy for Treatment of Severe Attacks of Inborn Errors of Metabolism.

Severe metabolic crises in children with inborn errors of metabolism can result in mortality or severe morbidities where continuous renal replacement therapy (CRRT) can be lifesaving . Clinical data, the pediatric risk of mortality (PRISM) scores calculated in the first 24 hours, and pediatric logistic organ dysfunction (PELOD) scores calculated in the last 24 hours before CRRT, were studied . Overall, CRRT was successful in restoring metabolic balance in 72% of patients. PELOD scores before CRRT were lower in survivors ( p = 0.02). Despite numerous comorbid factors, CRRT can be used effectively in management of metabolic crises. Early intervention with this therapy before occurrence of complications must be targeted.

A Novel Treatment Strategy for Severe Guillain-Barré Syndrome: Zipper Method.

OBJECTIVE: Intravenous immunoglobulin and plasma exchange are proven treatments for Guillain-Barré syndrome. Despite these treatments, the prognosis for severe Guillain-Barré syndrome is still not satisfactory. This article seeks for a logical timing for plasma exchange-intravenous immunoglobulin synergy, which may improve outcome in severe Guillain-Barré syndrome requiring mechanical ventilation. STUDY DESIGN: This study is an open-label study. Nine pediatric severe Guillain-Barré syndrome patients requiring mechanical ventilation were treated with novel treatment strategy named as "zipper method." In this method, following diagnosis of Guillain-Barré syndrome, plasma exchange was started immediately. In the first session of plasma exchange, one and a half volume of patients' plasma was removed by using 5% albumin as replacement solution. At the end of the plasma exchange session, 0.4 g/kg intravenous immunoglobulin infusion was started immediately. Second plasma exchange session was applied with one volume change after 24 hours from the end of the intravenous immunoglobulin infusion. Each plasma exchange session was followed by intravenous immunoglobulin infusions. This plasma exchange-intravenous immunoglobulin cycle was repeated for 5 times. RESULTS: Among the 9 patients, the mean mechanical ventilation duration was 7 (5-14) days and the mean hospital stay was 18 (10-30) days. Medical Research Council sum score was increased in all patients, especially after the third session. All patients survived and all patients were able to walk unaided on the 28th day of admission. CONCLUSION: The zipper method as a novel treatment modality seems to reduce mortality, speed up weaning from mechanical ventilation, and shorten hospital stay, with excellent outcome in severe Guillain-Barré syndrome patients, who require intensive care. This technique stands as a promising immunomodulation strategy for various scenarios.

A Report of 7-Year Experience on Pediatric Continuous Renal Replacement Therapy.

BACKGROUND: Continuous renal replacement therapies (CRRTs) either as continuous venovenous hemofiltration (CVVH) or hemodiafiltration (CVVHD) are used frequently in critically ill children. Many clinical variables and technical issues are known to affect the result. The factors that could be modified to increase the survival of renal replacement are sought. As a contribution, we present the data on 104 patients who underwent CRRT within a 7-year period. MATERIALS AND METHOD: A total of 104 patients admitted between 2009 and 2016 were included in the study. The demographic information, admittance pediatric risk of mortality (PRISM) scores, indication for CRRT, presence of fluid overload, CRRT modality, durations of CRRT, and pediatric intensive care unit (PICU) stay were compared between survivors and nonsurvivors. RESULTS: The overall rate of survival was 51%. Patients with fluid overload had significantly increased rate of death, CRRT duration, and PICU stay. Multiorgan dysfunction syndrome as the indication for CRRT was significantly related to decreased survival when compared to acute renal failure and acute attacks of metabolic diseases. The CRRT modality was not different between survivors and nonsurvivors. Standardized mortality ratio of the group was calculated to be 0.8. CONCLUSION: The CRRT in critically ill patients is successful in achieving fluid removal and correction of metabolic imbalances caused by organ failures or attacks of inborn errors of metabolism. It has a positive effect on expected mortality in high-risk PICU patients. To affect the outcome, follow-up should be focused on starting therapy in early stages of fluid overload. Prospective studies defining relative importance of risk factors causing mortality can assist in building up guidelines to affect the outcome.

Reversible Dilated Cardiomyopathy Due to Combination of Vitamin D-Deficient Rickets and Primary Hypomagnesemia in an 11-Month-Old Infant.

Vitamin D-deficient rickets is still an important and common health problem in developing countries. Since calcium is an essential ion for cardiac muscle contraction, calcium deficiency caused by rickets can cause secondary dilated cardiomyopathy. This situation can be exacerbated by coexisting hypomagnesemia. Here, we report a case of dilated cardiomyopathy due to hypocalcemia induced by vitamin D-deficient rickets and accompanying primary hypomagnesemia in an infant whose cardiomyopathy was successfully treated by replacement of calcium, vitamin D, and magnesium. In addition to genetic causes, viral infections, and idiopathic factors, metabolic abnormalities are important etiologic factors in pathogenesis of dilated cardiomyopathy and since they are treatable, prompt diagnosis of these disorders is crucial.

Early initiated feeding versus early reached target enteral nutrition in critically ill children: An observational study in paediatric intensive care units in Turkey.

AIM: Although early enteral nutrition (EN) is strongly associated with lower mortality in critically ill children, there is no consensus on the definition of early EN. The aim of this study was to evaluate our current practice supplying EN and to identify factors that affect both the initiation of feeding within 24 h after paediatric intensive care unit (PICU) admission and the adequate supply of EN in the first 48 h after PICU admission in critically ill children. METHODS: We conducted a prospective, multicentre, observational study in nine PICUs in Turkey. Any kind of tube feeding commenced within 24 h of PICU admission was considered early initiated feeding (EIF). Patients who received more than 25% of the estimated energy requirement via enteral feeding within 48 h of PICU admission were considered to have early reached target EN (ERTEN). RESULTS: Feeding was initiated in 47.4% of patients within 24 h after PICU admission. In many patients, initiation of feeding seems to have been delayed without an evidence-based reason. ERTEN was achieved in 43 (45.3%) of 95 patients. Patients with EIF were significantly more likely to reach ERTEN. ERTEN was an independent significant predictor of mortality (P < 0.001), along with reached target enteral caloric intake on day 2 associated with decreased mortality. CONCLUSIONS: There is a substantial variability among clinicians' perceptions regarding indications for delay to initiate enteral feeding in critically ill children, especially after the first 6 h of PICU admission. ERTEN, but not EIF, is associated with a significantly lower mortality rate in critically ill children.

Continuous Renal Replacement Therapy Applications on Extracorporeal Membrane Oxygenation Circuit.

BACKGROUND AND AIMS: Continuous venovenous hemofiltration or hemodiafiltration is used frequently in pediatric patients, but experience of continuous renal replacement therapy (CRRT) application on extracorporeal membrane oxygenation (ECMO) circuit is still limited. Among several methods used for applying CRRT on ECMO patients, we aim to share our experience on inclusion of a CRRT device in the ECMO circuit which we believe is easier and safer to apply. MATERIALS AND METHODS: The data were collected on demographics, outcomes, and details of the treatment of ECMO patients who had CRRT. During the study period of 3 years, venous cannula of ECMO circuit before pump was used for CRRT access for both the filter inlet and outlet of CRRT machine to minimize the thromboembolic complications. The common indication for CRRT was fluid overload. RESULTS: CRRT was used in 3.68% of a total number of patients admitted and 43% of patients on ECMO. The patients have undergone renal replacement therapy for periods of time ranging between 24 h and 25 days (260 h mean). The survival rate of this group of patients with multiorgan failure was 33%. Renal recovery occurred in all of the survivors. Complications such as electrolyte imbalance, hypothermia, and bradykinin syndrome were easily managed. CONCLUSIONS: Adding a CRRT device on ECMO circuit is a safe and effective technique. The major advantages of this technique are easy to access, applying CRRT without extra anticoagulation process, preventing potential hemodynamic disturbances, and increased clearance of solutes and fluid overload using larger hemofilter.

Acute kidney injury in congenital cardiac surgery: Pediatric risk-injury-failure-loss-end-stage renal disease and Acute Kidney Injury Network.

BACKGROUND: Acute kidney injury (AKI) is associated with an increased risk of mortality, especially in pediatric intensive care units. The aim of this study was to determine the risk factors of AKI in children undergoing cardiac surgery for congenital heart disease and to compare two different classification systems: pediatric risk-injury-failure-loss-end-stage renal disease (pRIFLE) and Acute Kidney Injury Network (AKIN). METHODS: We retrospectively analyzed 145 patients undergoing pediatric congenital heart surgery who were between 1 month and 18 years of years, and treated at a cardiovascular surgery department from January 2009 to October 2011. RESULTS: One hundred and thirty-seven patients (mean age, 36.6 ± 43.3 months) were enrolled: 84 (61.3%) developed AKI according to the pRIFLE criteria (25.5%, risk; 20.4%, injury; 15.3%, failure); and 65 patients (47.4%) developed AKI according to the AKIN criteria (15.3%, stage I; 18.2%, stage II; and 13.9%, stage III). Children younger than 11 months were more likely to develop AKI (P < 0.005). Longer cardiopulmonary bypass time was associated with an increased risk of AKI (P < 0.05). pRIFLE identified AKI more frequently than AKIN (P < 0.0005). pRIFLE may help in the early identification of patient at risk for AKI and seems to be more sensitive in pediatric patients (P < 0.05). Any degree of AKI in both classifications was associated with increased mortality (pRIFLE: OR, 15.1; AKIN: OR, 11.2; P = 0.007). CONCLUSION: pRIFLE identified AKI more frequently than the AKIN criteria. pRIFLE identified patients at risk for AKI earlier, and was more sensitive in pediatric patients. Any degree of AKI in both classifications was associated with increased mortality. Both sets of criteria had the same association with mortality.

Severe hyponatremia and repeated intestinal resections for intestinal dysmotility mimicking congenital aganglionic megacolon due to delay in the diagnosis of congenital hypothyroidism.

Congenital hypothyroidism (CH) may present with nonspecific signs and symptoms, though, majority of infants can be asymptomatic. Therefore, understimation and delay in diagnosis may result in severe complications. A 5-month-old female admitted to our clinic with the history of repeated surgical operations due to the diagnosis of congenital aganglionic megacolon. Investigations performed in our clinic revealed the diagnosis of congenital (primary) hypothyroidism due to thyroid agenesis. Histopathologic evaluation of previously resected colon sample revealed normal ganglionic cell included colon. During follow-up she developed severe hyponatremia with a plasma sodium level of 106 mEq/L. Eunatremia was maintained following achievement of euthyroid state. In conclusion, since presenting symptoms can be variable and nonspecific, hypotyhroidism should be kept in mind in the differential diagnosis of patients with persistent abdominal distention mimicking aganglionic megacolon and severe hyponatremia of unknown origin.

Familial Mediterranean fever in small children in Turkey.

OBJECTIVES: Familial Mediterranean fever (FMF) is an autosomal recessive disease, characterised by recurrent, self limited attacks of fever with serositis. The aim of our study was to describe the demographic, clinical and genetic features of FMF patients who had early disease onset and to compare them with late onset patients. Our second aim was to investigate the factors associated with delay in diagnosis. METHODS: The study group consisted of recently diagnosed FMF patients who came to routine follow-up visits between January and July 2009. Patients were divided into two groups according to age of disease onset (Group I: ≤ 3 years of age; Group II: >3 years of age). In the second part, patients were analysed according to the duration of delay in diagnosis. RESULTS: There were 83 patients in group I and 73 patients in Group II. Median delay in diagnosis was 4 years in Group I and 2 years in Group II (p<0.001). The presence of M694V mutation was more frequent in Group I (81%) as compared to Group II (65%), (p=0.034). Mean attack Hb was lower (p<0.01) and mean attack leukocyte count was higher (p=0.017) in Group I. Final colchicine dosages were higher in Group I as compared to Group II. There was a statistically significant negative correlation between the age at disease onset and period of delay in diagnosis (p<0.001). CONCLUSIONS: This study suggests that FMF patients with early disease onset have more severe disease. Moreover, the smaller the age of disease onset, the more likely their diagnoses are delayed.