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After the arterial switch operation (ASO) for transposition of the great arteries (TGA), many patients have an impaired exercise tolerance. Exercise tolerance is determined with cardiopulmonary exercise testing by peak oxygen uptake (VO 2peak ). Unlike VO 2peak , the oxygen uptake efficiency slope (OUES) does not require a maximal effort for interpretation. The value of OUES has not been assessed in a large group of patients after ASO. The purpose of this study was to determine OUES and VO 2peak , evaluate its interrelationship and assess whether exercise tolerance is related to ventricular function after ASO. A cardiopulmonary exercise testing, assessment of physical activity score and transthoracic echocardiography (fractional shortening and left/right ventricular global longitudinal peak strain) were performed to 48 patients after ASO. Median age at follow-up after ASO was 16.0 (IQR 13.0-18.0) years. Shortening fraction was normal (36 ± 6%). Left and right global longitudinal peak strain were reduced: 15.1 ± 2.4% and 19.5 ± 4.5%. This group of patients showed lower values for all cardiopulmonary exercise testing parameters compared to the reference values: mean VO 2peak % 75% (95% CI 72-77) and mean OUES% 82(95% CI 77-87); without significant differences between subtypes of TGA. A strong-to-excellent correlation between the VO 2peak and OUES was found (absolute values: R = 0.90, p < 0.001; normalized values: R = 0.79, p < 0.001). No correlation was found between cardiopulmonary exercise testing results and left ventricle function parameters. In conclusion, OUES and VO 2peak were lower in patients after ASO compared to reference values but are strongly correlated, making OUES a valuable tool to use in this patient group when maximal effort is not achievable.

Surgical repair of Tetralogy of Fallot (ToF) is usually performed in the first months of life with low early postoperative mortality. During long-term follow-up, however, both right (RV) and left ventricular (LV) performances may deteriorate. Tissue Doppler imaging (TDI) and speckle tracking echocardiography (ST) can unmask a diminished RV and LV performance. The objective of the current study was to assess the cardiac performance before and shortly after corrective surgery in ToF patients using conventional, TDI and ST echocardiographic techniques. Thirty-six ToF patients after surgery were included. Transthoracic echocardiography including TDI and ST techniques was performed preoperatively and at hospital discharge after surgery (10 days to 4 weeks after surgery). Median age at surgery was 7.5 months [5.5-10.9]. Regarding the LV systolic function there was a significant decrease in interventricular septum (IVS) S' at discharge as compared to preoperatively (pre IVS S' = 5.4 ± 1.4; post IVS S' = 3.9 ± 1.2; p < 0.001) and in global longitudinal peak strain (GLS) (pre = - 18.3 ± 3.4; post = - 14.2 ± 4.1; p = 0.003); but not in the fractional shortening (FS). Both conventional and TDI parameters showed a decrease in diastolic function at discharge. Tricuspid Annular Plane Systolic Excursion and RV S' were significantly lower before discharge. When assessing the RV diastolic performance, only the TDI demonstrated a RV impairment. There was a negative correlation between age at surgery and postoperative LV GLS (R = - 0.41, p = 0.031). There seems to be an impairment in left and right ventricle performance at discharge after ToF corrective surgery compared to preoperatively. This is better determined with TDI and ST strain imaging than with conventional echocardiography.

Pericardial effusion (PE) after pediatric cardiac surgery is common. Because of the lack of a uniform classification of the presence and severity of PE, we evaluated PE altering clinical management: clinically relevant PE. Risk factors for clinically relevant PE were studied. After cardiac surgery, children were followed until 1 month after surgery. Preoperative variables were studied in the complete cohort. Perioperative and postoperative variables were studied in a case-control manner. Patients with and without clinically relevant PE were matched on age, gender, and diagnosis severity in a 1:1 ratio. Multivariate analysis was conducted using important preoperative variables from the complete cohort combined with perioperative and postoperative variables from the case-control data. 1241 surgical episodes in 1031 patients were included. Clinically relevant PE developed in 136 episodes (11.0%). Multivariate correlation with the outcome was present for age, BSA (adjusted odds ratio: 1.6, 95% CI 0.9-2.8), right-sided heart defect (adjusted odds ratio: 1.3, 95% CI 0.9-1.9), history of previous operation (adjusted odds ratio: 0.5, 95% CI 0.3-0.7), cardiopulmonary bypass use (adjusted odds ratio: 2.1, 95% CI 0.9-4.5), duration of CPAP postoperatively, and an inotropic score (adjusted odds ratio: 1.01, 95% CI 0.998-1.03). In this large patient cohort, 11.0% of postoperative periods of pediatric cardiac surgery were complicated by PE requiring alteration of treatment. Secondly, we newly identified cardiopulmonary bypass use and right-sided heart defects as risk factors for clinically relevant PE and confirmed previously described risk factors: age, CPAP duration, BSA, and inotropic score and a previously described risk reductor: history of previous operation.

Objective: To investigate the safety and effects of a restrictive red blood cell (RBC) transfusion strategy in pediatric cardiac surgery patients., Design: Randomized controlled trial., Setting: Pediatric ICU in an academic tertiary care center, Leiden University Medical Center, Leiden, The Netherlands., Patients: One hundred seven patients with non-cyanotic congenital heart defects between 6 weeks and 6 years of age. One hundred three patients underwent corrective surgery on cardiopulmonary bypass., Interventions: Prior to surgery patients were randomly assigned to one of two groups with specific RBC transfusion thresholds: Hb 10.8 g/dl (6.8 mmol/l) and Hb 8.0 g/dl (5.0 mmol/l)., Measurements: Length of stay in hospital (primary outcome), length of stay in PICU, duration of ventilation (secondary outcome), incidence of adverse events and complications related to randomization (intention to treat analysis)., Results: In the restrictive transfusion group, mean volume of transfused RBC was 186 (±70) ml per patient and in the liberal transfusion group 258 (±87) ml per patient, (95% CI 40.6-104.6), p < 0.001. Length of hospital stay was shorter in patients with a restrictive RBC transfusion strategy: median 8 (IQR 7-11) vs. 9 (IQR 7-14) days, p = 0.047. All other outcome measures and incidence of adverse effects were equal in both RBC transfusion groups. Cost of blood products for the liberal transfusion group was 438.35 (±203.39) vs. 316.27 (±189.96) euros (95% CI 46.61-197.51) per patient in the restrictive transfusion group, p = 0.002., Conclusions: For patients with a non-cyanotic congenital heart defect undergoing elective cardiac surgery, a restrictive RBC transfusion policy (threshold of Hb 8.0 g/dl) during the entire perioperative period is safe, leads to a shorter hospital stay and is less expensive.

Cardiac surgery with cardiopulmonary bypass is associated with the development of a systemic inflammatory response, which can lead to myocardial damage. However, knowledge concerning the time course of ventricular performance deterioration and restoration after correction of a congenital heart defect (CHD) in pediatric patients is sparse. Therefore, the authors perioperatively quantified left ventricular (LV) and right ventricular (RV) performance using echocardiography. Their study included 141 patients (ages 0-18 years) undergoing CHD correction and 40 control subjects. The study assessed LV systolic performance (fractional shortening) and diastolic performance (mitral Doppler flow) in combination with RV systolic performance [tricuspid annular plane systolic excursion (TAPSE)] and diastolic performance (tricuspid Doppler flow). Additionally, systolic (S') and diastolic (E', A', E/E') tissue Doppler imaging (TDI) measurements were obtained at the LV lateral wall, the interventricular septum, and the RV free wall. Echocardiographic studies were performed preoperatively, 1 day postoperatively, and at hospital discharge after 9 ± 5 days. Although all LV echocardiographic measurements showed a deterioration 1 day after surgery, only LV TDI measurements were impaired in patients at discharge versus control subjects (S': 5.7 ± 2.0 vs 7.1 ± 2.7 cm/s; E': 9.8 ± 3.9 vs 13.7 ± 5.1 cm/s; E/E': 12.2 ± 6.4 vs 8.8 ± 4.3; p < 0.05). In the RV, TAPSE and RV TDI velocities also were impaired in patients at discharge versus control subjects (TAPSE: 9 ± 3 vs 17 ± 5 mm; S': 5.2 ± 1.7 vs 11.4 ± 3.4 cm/s; E': 7.3 ± 2.5 vs 16.3 ± 5.2 cm/s; E/E': 12.5 ± 6.8 vs 4.8 ± 1.9; p < 0.05). Furthermore, longer aortic cross-clamp times were associated with more impaired postoperative LV and RV performance (p < 0.05). In conclusion, both systolic and diastolic biventricular performances were impaired shortly after CHD correction. This impairment was detected only by TDI parameters and TAPSE. Furthermore, a longer-lasting negative influence of cardiopulmonary bypass on myocardial performance was suggested.

Purpose: We conducted a retrospective cohort study in an academic tertiary care center to characterize ventilator-associated pneumonia (VAP) in pediatric patients after cardiac surgery in The Netherlands., Methods: All patients following cardiac surgery and mechanically ventilated for ≥24 h were included. The primary outcome was development of VAP. Secondary outcomes were duration of mechanical ventilation and length of ICU stay., Results: A total of 125 patients were enrolled. Their mean age was 16.5 months. The rate of VAP was 17.1/1,000 mechanical ventilation days. Frequently found organisms were Haemophilus influenzae, Moraxella catarrhalis, Staphylococcus aureus and Pseudomonas aeruginosa. Patients with VAP had longer duration of ventilation and longer ICU stay. Risk factors associated with the development of VAP were a PRISM III score of ≥10 and transfusion of fresh frozen plasma., Conclusion: The mean VAP rate in this population is higher than that reported in general pediatric ICU populations. Children with VAP had a prolonged need for mechanical ventilation and a longer ICU stay.

An aortopulmonary window (APW) is a communication between the ascending aorta and the pulmonary trunk in the presence of two separate semilunar valves. In order to increase our understanding about the surgical management of this rare lesion and its long-term results, we describe our experience over a 37-year period. Between 1968 and 2005, 18 patients were diagnosed with APW. Seventeen underwent surgical correction. Age at operation ranged from 22 days to 22 years (median, 0.20 years). Follow-up ranged from 2 weeks to 28.6 years (median, 11.0 years). Surgical closure was achieved using a single patch in 7 patients (41.2%) double patch in 4 (23.5%), primary closure in 3 (17.6%), clip in 2 (11.8%), and ligation in 1 (5.9%). Complex APW was present in 8 patients (44.4%). One patient was treated nonsurgically. There were no early or late deaths after surgery. Both primary closure and patch closure gave excellent long-term results. Sporadic postoperative complications were only associated with complex lesions. One patient who was treated conservatively died (of pulmonary hypertension) 21 years after diagnosis. Repair of APW is ideally performed in the first months of life, before irreversible PHT has developed. Various surgical repair techniques in this series of patients gave excellent short-term and long-term results, without significant hemodynamic sequelae.

The objective of this study was to evaluate the different causes of extubation failure and the consequent mortality rates in a pediatric population after cardiac surgery. We studied 184 consecutive patients with a median age of 9 months (range, 0-165). In 158 patients, extubation was successful (group A). Nine patients were reintubated for upper airway obstruction and finally extubated successfully (group B). Seventeen patients were reintubated for cardiorespiratory failure, finally leading to death in 11 of 17 patients (65%) (group C). Group B patients were younger and had a longer intubation period compared to group A patients. Group C patients had more reoperations (30% vs 4% in group A patients, p < 0.001), a lower PaO2 on admission at the intensive care unit as well as just prior to extubation, a lower base deficit before extubation, and needed more inotropic support during their stay in the intensive care unit. We conclude that extubation failure after pediatric cardiac surgery due to cardiorespiratory failure is a bad prognostic sign. Patients with high inotropic support and a low PaO2 prior to extubation are especially at risk and probably need careful evaluation before final extubation.

Objective: Pediatric cardiac surgery is associated with a temporary rise in cardiac troponin T (cTnT) during the postoperative period. We examined whether dexamethasone given before cardiopulmonary bypass has myocardial protective effects as assessed by the postoperative production of cTnT., Design and Setting: Prospective randomized interventional study in the pediatric intensive care unit in a university hospital., Interventions: Patients were randomly allocated to act as controls or receive a single dose of dexamethasone (1 mg/kg) during induction of anesthesia., Measurements and Results: cTnT was measured four times postoperatively: immediately after admission to the pediatric intensive care unit (PICU) and 8, 15, and 24 h thereafter. The two groups had similar mean cTnT concentrations on PICU admission: those receiving dexamethasone 1.85 ng/ml (1.55-2.15) and those not receiving it 2 ng/ml (95% confidence interval 1.56-2.51). Concentrations of cTnT 8 h after admission to the PICU differed significantly after 8 h: 1.99 ng/ml (1.53-2.45) in those receiving dexamethasone and 3.08 ng/ml (2.46-3.69) in those not receiving it. After subgroup statistical analysis differences between the two groups remained significant only at 8 h, not those after 15 or 24 h., Conclusions: The use of dexamethasone (1 mg/kg) before cardiopulmonary bypass is associated with a brief but significant reduction in postoperative cTnT production. The clinical significance of this effect is unclear.

The aim of the study was to investigate renal function and renal replacement therapy after cardiopulmonary bypass surgery in children. Patient characteristics (sex, age, diagnosis), operation type, and death were listed. The study was performed retrospectively using serum creatinine level before, and peak values after, cardiopulmonary bypass surgery for assessment of renal function. Of the children on renal replacement therapy, indication, efficacy, and complications were recorded. In a 5-year period, 1075 children had cardiopulmonary bypass surgery at the Department of Cardiothoracic Surgery at Leiden University Medical Center and Academic Medical Center of Amsterdam. One-hundred eighty (17%) patients developed acute renal insufficiency. Twenty-five (2.3%) patients required renal replacement therapy. Peritoneal dialysis is a safe and effective treatment for children after cardiopulmonary bypass surgery. However, 15 (60%) of 25 children on renal replacement therapy died of nonrenal causes. In 9 out of 10 surviving children, renal function was normal at time of discharge from hospital. Acute renal insufficiency is a frequent complication after open-heart surgery, although renal replacement therapy was infrequently necessary. Peritoneal dialysis is a safe and effective therapeutic measure for children after cardiac bypass surgery.

Unlabelled: In a 2 year period seven patients who presented with stridor, without respiratory compromise, and three patients without obstructive symptoms were prospectively selected, and underwent MRI. In eight patients with a vascular ring and a pulmonary sling, MRI delineated the vascular abnormality and normal great vessels were found in two patients., Conclusion: MRI successfully delineates the great vessels and demonstrates the presence of a vascular ring and pulmonary sling.