Tetralogy Of Fallot In Children Research Articles

Assessment of Health-Related Quality of Life in Children with Totally Corrected Tetralogy of Fallot in Sulaymaniyah, Iraq

ABSTRACT Purpose This study examined the health-related quality of life among children who have undergone surgery for tetralogy of Fallot (TOF) compared to healthy children. Design Cross-sectional study Methods The study was carried out at Children’s Heart Hospital and Primary Healthcare Centers in Sulaymaniyah, Iraq, between July 1, 2022, and November 1, 2022. The study involved 400 participants, comprised of 200 parents of healthy children and 200 parents with children with repaired TOF. Data collection involved utilizing a 5-point Likert Scale questionnaire administered through direct face-to-face interviews with the parents of the children. Findings Children with surgically repaired TOF had markedly lower mean scores (P ≤ 0.001) in the overall dimension of QoL (Mean rank = 137) when compared to healthy children (Mean rank = 263). Furthermore, it was observed that children with repaired TOF had significantly lower mean scores (P < 0.001) across all subdimensions of QoL compared to their healthy counterparts. In the subject of children with repaired TOF, the impact of maternal education (illiterate and lower education) on overall QoL was found to be highly significant (P < 0.001). In addition, children with repaired TOF from lower socioeconomic status (SES) families had worse quality of life than those from medium and high SES families, with a p-value <0.001. Conclusions Repaired TOF children exhibited notable deficiencies across all dimensions of QoL compared to healthy children. Moreover, SES emerged as a significant determinant influencing the QoL outcomes of repaired TOF children. Clinical evidence Despite undergoing corrective surgery for TOF, the QoL in children with repaired TOF continues to be lower than that of their healthy counterparts. Notably, the economic and educational status of the family significantly impacts the way these children perceive and experience their QoL. This finding underscores the critical significance of factoring in socioeconomic elements when addressing the well-being of this particular group of children.

Effectiveness of Dexmedetomidine as Myocardial Protector in Children With Classic Tetralogy of Fallot Having Corrective Surgery: A Randomized Controlled Trial

ObjectivesEfficacy of dexmedetomidine as cardioprotective agent in Indonesian children undergoing classic tetralogy of Fallot (TOF) repair with cardiopulmonary bypass (CPB). DesignA prospective, parallel trial utilizing block randomization along with double-blinded preparation of treatment agents by other parties. SettingNational Cardiovascular Center Harapan Kita, Indonesia. Participants66 children with classic TOF scheduled for corrective surgery. No children were excluded. All patients had fulfilled criteria for analysis. Interventions0.5 mcg/kg bolus of dexmedetomidine was added in CPB priming solution, followed by 0.25 mcg/kg/hour maintenance during bypass. Placebo group used normal saline. Follow-ups were up to 30 days. Measurement and Main ResultsTroponin I was lower in the dexmedetomidine (Dex) group at 6 hours (30.48± 19.33 vs. 42.73± 27.16, p = 0.039) and 24 hours after CPB (8.89± 5.42 vs. 14.04± 11.17, p = 0.02). Within similar timeframe, Dexmedetomidine successfully lowered IL-6 (p = 0.03; p = 0.035, respectively). Lactate was lower in the Dex group at 1, 6, and 24 hours after CPB (p = <0.01; p = 0.048; p = 0.035, respectively). Dexmedetomidine increased cardiac output and index from 6 hours after bypass, but vice versa in systemic vascular resistance. Reduction of vasoactive inotropic score was seen during ICU monitoring in the Dex group (p = 0.049). Nevertheless, dexmedetomidine did not significantly affect length of ventilation (p = 0.313), ICU stay (p = 0.087), and mortality (p >0.99). ConclusionsDexmedetomidine during CPB is an effective cardioprotective agent in TOF children having surgery. Postoperative mortality was comparable across groups.

Development and validation of a predictive model for low cardiac output syndrome after surgical repair of tetralogy of Fallot.

Low cardiac output syndrome (LCOS) remains a serious postoperative complication for children with tetralogy of Fallot (TOF), which often leads to increased morbidity and mortality. Early identification of LCOS and timely management are critical for better outcomes. This study aimed to develop a prediction model incorporating pre- and intraoperative characteristics for LCOS within 24 hours after surgical repair of TOF in children. The training dataset consisted of patients with TOF who underwent surgical repair in 2021, while the validation dataset consisted of patients in 2022. The univariable and multivariable logistic regression analyses were performed to recognize the risk factors of postoperative LCOS and a predictive model was established based on multivariable logistic regression analysis in the training dataset. Model predictive power was assessed using the area under the receiver operating characteristic curve (AUC). The calibration of the nomogram was evaluated and the Hosmer-Lemeshow test was used to assess the good fit. Decision curve analysis (DCA) was used to estimate the net benefits of the prediction model at different threshold probabilities. In the multivariable logistic analysis, peripheral oxygen saturation, mean blood pressure, and central venous pressure were independent risk factors for postoperative LCOS. The AUC of the predictive model for postoperative LCOS was 0.84 (95% CI: 0.77-0.91) and 0.80 (95% CI: 0.70-0.90) in the training and validation datasets, respectively. The calibration curve for the probability of LCOS showed good agreement between the prediction by nomogram and actual observation both in the training and validation datasets. The Hosmer-Lemeshow test yielded nonsignificant statistics both in the training and validation datasets (P=0.69 and 0.54, respectively), indicating a good fit. The DCA revealed that more net benefits would be obtained by using the nomogram to predict LCOS than that achieved in either the treat-all-patient scheme or the treat-none scheme both in the training and validation datasets. This study is the first to incorporate pre- and intraoperative characteristics to develop a predictive model for LCOS after surgical repair of TOF in children. This model showed good discrimination, good fit and clinical benefits.

Relationship Between Cleft Lip and Palate Followed by Osteogenesis Imperfecta Accompanied by Tetralogy of Fallot in Children with a Maternal History of Catatonic Schizophrenia with Valproic Acid Therapy and Diabetes Mellitus

Researchers believe that most cases of cleft lip and cleft palate are caused by genetic and environmental factors. Nevertheless, the exact cause was not found. Nevertheless there are allegations that the condition of the cleft lip and palate has something to do with osteogenesis imperfecta with the heterozygous mutation COL1A2 and the formation of triple helix DNA accompanied by Tetralogy of Fallot in children with a maternal history of catatonic schizophrenia followed by the use of valproic acid and type II diabetes mellitus. Then, it is also found in babies with cleft lip and sky conditions usually accompanied by other congenital anomaly, but not always. This then further gives rise to speculation that there are links between the above variables and the cleft lip and palate. Therefore. Knowing the Relationship Between Cleft Lip and Palate Events with osteogenesis imperfecta with heterozygous mutation COL1A2 and triple helix DNA formation accompanied by Tetralogy of Fallot in children with a maternal history of catatonic schizophrenia followed by the use of valproic acid and type II diabetes mellitus. This research is a Systematic Review using the Preferred Reporting Items for Systematic Reviews and Meta-analyses method or commonly called PRISMA, this method is carried out systematically by following the correct research stages or protocols. The source was taken from the Google Scholar site with journals published in 2017-2023 and then screened the results of 1,870 journals. Journal clustering was carried out and the number of Q1 indexed journals was obtained in 4 journals, Q3 in 1 journal, and Q4 in 1 journal so that there were 6 journals extracted. The entire journal does not directly discuss the relationship between the research variables, but only provides an overview of the increased risk of celestial cleft conditions in babies if the expectant mother has type 2 diabetes mellitus conditions, osteogenesis imperfect, and the relationship of the two health conditions to the possibility of birth of the baby with the Tetralogy of Fallot condition, as well as the relationship of the baby to the condition of the cleft lip and palate and tetralogy of fallot

Letter to the editor regarding article by Dehghan et al.: "Biventricular strain and strain rate impairment shortly after surgical repair of tetralogy of Fallot in children: A case-control study".

Letter to the editor regarding article by Dehghan et al.: "Biventricular strain and strain rate impairment shortly after surgical repair of tetralogy of Fallot in children: A case-control study".

Biventricular strain and strain rate impairment shortly after surgical repair of tetralogy of Fallot in children: A case-control study.

BackgroundEarly biventricular dysfunction in repaired tetralogy of Fallot (TOF) children may lead to poor clinical outcomes. We aimed to assess biventricular function in TOF children before and after surgery by speckle tracking echocardiography (STE) and compare them with the controls.MethodsTwenty repaired TOF children and 20 normal children as controls were assessed by STE. Tricuspid annular plane systolic excursion (TAPSE), left ventricular ejection fraction (LVEF), biventricular strain, and strain rate were compared before and after surgery and between TOF children and controls.ResultsPostoperative LVEF (p = 0.001), strain (p = 0.001), and strain rate (p = 0.001) for left ventricle improved significantly compared to preoperative phase. However, postoperative left ventricular strain (p = 0.05) and strain rate (p = 0.01) in TOF children were significantly impaired compared to controls. Postoperative LVEF was correlated inversely with postoperative strain rate (r = −0.40, p = 0.04). Postoperative TAPSE (p = 0.001), strain (p = 0.001), and strain rate (p = 0.001) for right ventricle significantly worsened when compared with the preoperative phase. Moreover, postoperative TAPSE (p = 0.001), strain (p = 0.001), and strain rate (p = 0.01) were significantly impaired compared to controls. Postoperative right ventricular strain rate was correlated significantly with the weight of children (r = 0.48, p = 0.02), and postoperative left ventricular strain showed significant correlations with aortic clamp time (r = 0.44, p = 0.04) and with ICU stay (r = −0.46, p = 0.04).ConclusionDespite normal LVEF, TOF children exhibit impaired left ventricular strain and strain rate after surgery. TAPSE, strain, and strain rate for the right ventricle worsen after surgical repair. STE‐driven strain can be used to detect early ventricular dysfunction and the associated prognostic implications.

The Effect of Abnormal Regional Homogeneity and Spontaneous Low-Frequency Brain Activity on Lower Cognitive Ability: A Cross-Sectional Study on Postoperative Children With Tetralogy of Fallot.

Despite intracardiac malformation correction, children with Tetralogy of Fallot (TOF) may still suffer from brain injury. This cross-sectional study was primarily designed to determine the relationship between blood oxygenation level-dependent (BOLD) signal changes after surgery and cognition in school-aged children with TOF. To evaluate the differences between TOF children (n = 9) and healthy children (n = 9), resting-state functional magnetic resonance imaging (rs-fMRI) and the Wechsler Intelligence Scale for Children–Chinese revised edition (WISC-CR) were conducted in this study. The results showed that TOF children had a lower full-scale intelligence quotient (FSIQ, 95.444 ± 5.354, p = 0.022) and verbal intelligence quotient (VIQ, 92.444 ± 4.708, p = 0.003) than healthy children (FSIQ = 118.500 ± 4.330;VIQ = 124.250 ± 4.404), and that significant differences in regional homogeneity (ReHo) and amplitude of low-frequency fluctuation (ALFF) existed between the two groups. Besides, VIQ had significantly positive correlations with the decreased ALFF value of the middle inferior occipital gyrus (MIOG, beta = 0.908, p = 0.012) after fully adjusting for all covariates. In addition, elevated ReHo values of the left and right precuneus were positively related to ALFF in the MIOG. This study revealed that brain injury substantially influences neural activity and cognition in postoperative TOF children, providing direct evidence of an association between BOLD signal changes and the VIQ and prompting further attention to language development in TOF children.

Echocardiographic Assessment of Right Ventricular Systolic Function in Postoperative Tetralogy of Fallot Patients with Special Emphasis on Right Ventricular-Global Longitudinal Strain

Background: Limitations of echocardiography have long been known for the assessment of right ventricular (RV) systolic function in postoperative tetralogy of Fallot (TOF) patients. In this study, we evaluated the role of RV-global longitudinal strain (GLS) for the assessment of RV systolic function. Materials and Methods: It was a single institution prospective observational study of ninety postoperative TOF patients. Detailed 2-dimensional echocardiography along with RV-GLS using speckle tracing imaging was done at baseline and after 1 year. The children were divided into three groups based on duration since total correction, i.e., group A (6 months to 5 years), group B (6–10 years), and group C (&gt;10 years) to assess the differences in RV systolic function. Furthermore, correlation of RV-GLS with RV-fractional area change (FAC), tricuspid annular plane systolic excursion, and tricuspid valve tissue Doppler imaging s' velocity was done. Results: In group A patients, a statistically significant increase in RV systolic function was seen over a period of 1 year. However, in group B and C patients, no significant change was seen. There was a strong positive correlation of RV-GLS only with RV-FAC during the first follow-up (r = 0.41, P &lt; 0.01) and second follow-up periods (r = 0.67, P &lt; 0.01). Conclusion: RV-GLS has a strong positive correlation with RV-FAC, and it detects preclinical regional myocardial dysfunction even when the RV-FAC is normal and thus must be included in the evaluation of postoperative TOF children.

Association of perfusate oxygenation with cardiovascular disorder in tetralogy of fallot children: A nested case-control study.

Little is known regarding whether hyperoxic reoxygenation was associated with higher risk of cardiovascular disorder following tetralogy of Fallot repair. We performed a nested case-control study among patients aged 1 month-18 years undergoing complete repair of tetralogy of Fallot in 2012-2018. We measured the highest perfusate oxygenation (PpO2) during aortic occlusion in 107 cardiovascular disorder cases and in 321 controls matched 1:3 to the cases on date of surgery, sex, and area of residence. We analyzed the association between PpO2 and outcome using multivariable conditional logistic regression adjusted for covariates. We further identified and integrated the risk covariates to build prediction nomograms. Cases had higher percentage of exposure to PpO2 > 200 mmHg (86.0% vs. 76.1%, p = .019) than controls. Patients with PpO2 > 200 mmHg had an increased risk of cardiovascular disorder compared to those with PpO2 ≤ 200 mmHg (odd ratio [OR] = 2.075, 95% confidence interval [CI] = 1.035, 4.158, p = .039) adjusted for matching, clinical and procedural covariates. Categorical PpO2, lower body mass index, lower SpO2, untreated minor aortopulmonary collateral arteries, high immediately postoperative central venous pressure, and longer cardiopulmonary bypass time were independent risk factors for cardiovascular disorder (all p < .05). Combining PpO2 nomogram slightly improved discrimination compared with covariate-based nomogram alone for training cohort (area under receiver operating characteristic curve [AUC] = 0.768 vs. 0.761) and for internal validation (AUC = 0.759 vs. 0.753). Our findings suggest association exists between high PpO2 during aortic occlusion and cardiovascular disorder risk, and nomogram integrating clinical and procedural factors may be useful in management of patients with tetralogy of Fallot.

Contemporary early results of late repair of tetralogy of Fallot in children: a single-centre case series.

To describe the early results of complete repair of tetralogy of Fallot (TOF) in children older than one year of age. We identified all patients older than one year of age who underwent TOF repair between 2007 and 2017. Data collected included type of surgical repair, survival at 30 days after surgery, reintervention within 30 days of surgery, length of stay in the intensive care unit (ICU), length of stay in hospital, and degree of residual right ventricular outflow tract (RVOT) stenosis and pulmonary insufficiency 30 days after surgery. 125 children (median age 4.4 years, interquartile range [IQR] 3&ndash;5.7) underwent complete repair of TOF, with pulmonary valve preservation in 66%, a transannular patch in 19%, and a valved right ventricular-to-pulmonary artery conduit in 15%. All patients survived. There were twelve (9.6%) reinterventions. Median ICU length of stay was 7 days and median hospital length of stay was 12 days. Significant residual RVOT stenosis was present in 7.2% and significant pulmonary insufficiency in 19.2%. Patients with a transannular patch had a significantly higher proportion of significant residual RVOT stenosis and significant pulmonary insufficiency compared with the other types of surgical repair. Compared with the existing literature on complete late repair of TOF in children, this study showed good early results with no mortality, similar rates of reintervention and valve-preserving surgery, higher ICU and hospital length of stay, higher incidence of significant residual RVOT stenosis, and lower incidence of significant pulmonary insufficiency. Repair with a transannular patch was a risk factor for significant residual RVOT stenosis and significant pulmonary insufficiency.

Comparison of semi-automated versus manual quantitative right ventricular assessment in tetralogy of Fallot.

Tetralogy of Fallot is a congenital heart defect diagnosed in infancy. Assessment of right ventricular size and function is important for evaluation of patients with tetralogy of Fallot, but these quantitative measures are challenging by echocardiography. This study evaluates a semi-automated software (EchoInsight®, Epsilon Imaging) by comparing its measures to manual measures in children with tetralogy of Fallot. Echocardiographic measurements were performed using manual techniques and semi-automated software. Right ventricular measurements included end-diastolic and end-systolic area, fractional area change, chamber dimensions, and tricuspid annular plane systolic excursion. Reliability, correlation, and agreement between manual and semi-automated measures were assessed. Echocardiograms for 46 patients were analysed. Intra- and inter-observer reliabilities for semi-automated measures were good with intraclass correlation coefficients all over 0.95 and 0.85, respectively. There was high correlation between manual and semi-automated methods for areas and dimensions (r = 0.91-0.98). Tricuspid annular plane systolic excursion measures and fractional area change also correlated, albeit less strongly. The semi-automated measurements of end-systolic and end-diastolic area were a 20 and 47% higher than manual methods, respectively.The semi-automated method yielded a relative 52% lower fractional area change compared to the manual method. The semi-automated software generates quantitative right ventricular measures in children with tetralogy of Fallot with good reliability and good correlation with manual methods for all measures, but with significant difference between manual and semi-automated techniques for area and functional measures. The specific right ventricular geometry in tetralogy of Fallot children may be why, compared to normal anatomy, greater differences were observed between the two techniques.

Gastrostomy and Tracheostomy After Complete Repair of Tetralogy of Fallot in Children With 22q11.2 Deletion Syndrome.

Caring for a child with gastrostomy and/or tracheostomy can cause measurable parental stress. It is generally known that children with 22q11.2 deletion syndrome are at greater risk of requiring gastrostomy or tracheostomy after heart surgery, although the magnitude of that risk after complete repair of tetralogy of Fallot has not been described. We sought to determine the degree to which 22q11.2 deletion is associated with postoperative gastrostomy and/or tracheostomy after repair of tetralogy of Fallot. Retrospective cohort study. Pediatric Health Information System. Children undergoing complete repair of tetralogy of Fallot (ventricular septal defect closure and relief of right ventricular outflow tract obstruction) from 2003 to 2016. Patients were excluded if they had pulmonary atresia, other congenital heart defects, and/or genetic diagnoses other than 22q11.2 deletion. Two groups were formed on the basis of 22q11.2 deletion status. Outcomes were postoperative tracheostomy and postoperative gastrostomy. Bivariate analysis and Kaplan-Meier analysis at 150 days postoperatively were performed. There were 4,800 patients, of which 317 (7%) had a code for 22q11.2 deletion. There were no significant differences between groups for age at surgery or sex. Patients with 22q11.2 deletion had significantly higher rates of gastrostomy (18% vs 5%; p < 0.001) and higher rates of tracheostomy (7% vs 1%; p < 0.001); there was no difference for mortality. Kaplan-Meier analyses also showed higher rates of gastrostomy (p = 0.024) and tracheostomy (p = 0.037). The present study establishes rates of postoperative gastrostomy and tracheostomy in children with 22q11.2 deletion after complete repair of tetralogy of Fallot. These data are useful to clinicians for providing families with preoperative counseling.

Late correction of tetralogy of Fallot in children.

To report our experience of late correction after infancy in patients with tetralogy of Fallot (ToF). Observational single-centre retrospective analysis of the surgical techniques and perioperative development of patients from developing countries undergoing total surgical correction of ToF after infancy, between 1 November 2011 and 30 November 2016. Variables are presented as numbers with percentages or as mean &plusmn; standard deviation. Due to the setting of the humanitarian programme, clinical and echocardiographic follow-up procedures could be conducted for only one month postoperatively. Twenty-five children (mean age: 70.8 &plusmn; 42 months, range 23-163; 44% female) underwent total surgical correction of ToF. Two patients (0.8%) initially received a Blalock-Taussig shunt and underwent subsequent correction 24 and 108 months later, respectively. Preoperative mean right ventricular/pulmonary artery (RV/PA) gradient was 84 &plusmn; 32 mm Hg, with a Nakata index of 164 &plusmn; 71 mm2/m2. Major aortopulmonary collateral arteries (MAPCAs) were observed in eight children (32%), six (26%) of whom underwent transcatheter closure before surgery. 24 children (96%) underwent a valve-sparing pulmonary valve repair and one patient received a transannular patch (TAP). There were no cases which saw major adverse cardiac and cerebrovascular events (MACCE). Mean duration of mechanical ventilation was 28 &plusmn; 19.6 hours (range 7-76). Pre-discharge echocardiography demonstrated a mean RV/PA gradient of 25 &plusmn; 5.7 mm Hg, with left ventricular ejection fraction &gt;60% in all cases. Overall length of hospital stay was 11.7 &plusmn; 4.5 days. There were no in-hospital mortality cases. Late surgical correction of ToF can be safely performed and produce highly satisfying early postoperative results comparable to those of classical &ldquo;timely&rdquo; correction. A valve-sparing technique can be applied in the majority of children.

Different predictivity of fluid responsiveness by pulse pressure variation in children after surgical repair of ventricular septal defect or tetralogy of Fallot.

Pulse pressure variation derived from the varied pulse contour method is based on heart-lung interaction during mechanical ventilation. It has been shown that pulse pressure variation is predictive of fluid responsiveness in children undergoing surgical repair of ventricular septal defect. Right ventricle compliance and pulmonary vascular capacitance in children with tetralogy of Fallot are underdeveloped as compared to those in ventricular septal defect. We hypothesized that the difference in the right ventricle-pulmonary circulation in the twogroups of children would affect the heart-lung interaction and therefore pulse pressure variation predictivity of fluid responsiveness following cardiac surgery. Infants undergoing complete repair of ventricular septal defect (n=38, 1.05±0.75years) and tetralogy of Fallot (n=36, 1.15±0.68years) clinically presenting with low cardiac output were enrolled. Fluid infusion with 5% albumin or fresh frozen plasma was administered. Pulse pressure variation was recorded using pressure recording analytical method along with cardiac index before and after fluid infusion. Patients were considered as responders to fluid loading when cardiac index increased ≥15%. Receiver operating characteristic curves analysis was used to assess the accuracy and cutoffs of pulse pressure variation to predict fluid responsiveness. The pulse pressure variation values before and after fluid infusion were lower in tetralogy of Fallot children than those in ventricular septal defect children (15.2±4.4% vs 19.3±4.4%, P<.001; 11.6±3.8 vs 15.4±4.3%, P<.001, respectively). In ventricular septal defect children, 27 were responders and 11 nonresponders. Receiver operating characteristic curve area was 0.89 (95% confidence interval, 0.77-1.01) and cutoff value 17.4% with a sensitivity of 0.89 and a specificity of 0.91. In tetralogy of Fallot children, 26 were responders and 10 were nonresponders. Receiver operating characteristic curve area was 0.79 (95% CI, 0.64-0.94) and cutoff value 13.4% with a sensitivity of 0.81 and a specificity of 0.80. Pulse pressure variation is predictive of fluid responsiveness in ventricular septal defect and tetralogy of Fallot patients following cardiac surgery.

Cardiac protective effects of remote ischaemic preconditioning in children undergoing tetralogy of fallot repair surgery: a randomized controlled trial.

AimsRemote ischaemic preconditioning (RIPC) by inducing brief ischaemia in distant tissues protects the heart against myocardial ischaemia-reperfusion injury (IRI) in children undergoing open-heart surgery, although its effectiveness in adults with comorbidities is controversial. The effectiveness and mechanism of RIPC with respect to myocardial IRI in children with tetralogy of Fallot (ToF), a severe cyanotic congenital cardiac disease, undergoing open heart surgery are unclear. We hypothesized that RIPC can confer cardioprotection in children undergoing ToF repair surgery.Methods and resultsOverall, 112 ToF children undergoing radical open cardiac surgery using cardiopulmonary bypass (CPB) were randomized to either a RIPC group (n = 55) or a control group (n = 57). The RIPC protocol consisted of three cycles of 5-min lower limb occlusion and 5-min reperfusion using a cuff-inflator. Serum inflammatory cytokines and cardiac injury markers were measured before surgery and after CPB. Right ventricle outflow tract (RVOT) tissues were collected during the surgery to assess hypoxia-inducible factor (Hif)-1α and other signalling proteins. Cardiac mitochondrial injury was assessed by electron microscopy. The primary results showed that the length of stay in the intensive care unit (ICU) was longer in the control group than in the RIPC group (52.30 ± 13.43 h vs. 47.55 ± 10.34 h, respectively, P = 0.039). Patients in the control group needed longer post-operative ventilation time compared to the RIPC group (35.02 ± 6.56 h vs. 31.96 ± 6.60 h, respectively, P = 0.016). The levels of post-operative serum troponin-T at 12 and 18 h, CK-MB at 24 h, as well as the serum h-FABP levels at 6 h, after CPB were significantly lower, which was coincident with significantly higher protein expression of cardiac Hif-1α, p-Akt, p-STAT3, p-STAT5, and p-eNOS and less vacuolization of mitochondria in the RIPC group compared to the control group.ConclusionIn ToF children undergoing open heart surgery, RIPC attenuates myocardial IRI and improves the short-term prognosis.

Clinical diagnosis of tetralogy of Fallot children with concurrent DiGeorge syndrome

Objective To explore the clinical diagnosis of tetralogy of Fallot(TOF) children with concurrent DiGeorge syndrome(DGS). Methods Retrospective analyses were conducted for the clinical characteristics of 715 TOF children with concurrent DGS at Henan Provincial People's Hospital and the Third Affiliated Hospital of Zhengzhou University from January of 2008 to October of 2014.Among them, there were 78 definite cases of thymic aplasia(DGS group), including 45 boys and 33 girls with an age range of(9.12±4.35) months and a body mass range of(7.28±2.34) kg.And the remainder was designated as non-DGS group(NDGS group), including 387 boys and 250 girls with an age range of(8.21±5.61) months and a body mass range of(8.19±3.47) kg.In DGS group, genetic screening uncovered 10 cases of chromosome 22q11.2 gene deletion.And based upon this result, DGS group was further divided into genetic and clinical diagnosis subgroups.The genetic diagnosis group had 10 cases, including 6 boys and 4 girls with an age range of(8.12±4.15) months and a body mass range of(6.28±2.74) kg, the clinical diagnosis group had 68 cases, including 39 boys and 29 girls with an age range of(8.19±4.37) months and a body mass range of(7.05±2.39) kg. Results No statistical difference existed in age, body mass or preoperative developmental status of pulmonary vasculature between DGS group and NDGS group(P>0.05). And the preoperative incidence of recurrent pneumonia was obviously higher in DGS group than that in NDGS group(P 0.05). And an inter-group comparison of T lymphocyte immunity defect had no statistical difference(P>0.05). The duration of on-machine and intensive care unit stay was markedly longer in DGS group than that in NDGS group(P 0.05). And an inter-group comparison of T lymphocyte immunity defect had no statistical difference(P>0.05). Conclusions With diverse clinical manifestations, DGS patients may have non-specific findings of cellular immunity defect, hypocalcemia or facial malformation.TOF children with concurrent thymic aplasia should raise an alert so that effective interventions may be adopted to boost their long-term quality of life. Key words: DiGeorge syndrome; Tetralogy of Fallot; Clinical features

Evaluation of left and right ventricular volume and systolic function in tetralogy of Fallot children by real-time three-dimensional echocardiography

Objective To investigate the characteristics of left and right ventricular volume and systolic function of tetralogy of Fallot(TOF) children by real-time three-dimensional echocardiography (RT-3DE). Methods Forty-five TOF children and 46 normal age-matched children were recruited in this study. Full volume imaging of left and right ventricle were obtained by RT-3DE and analyzed off-line by TomTec LV-Analysis and RV-Function. The measurements including: end-diastolic volume (LVEDV/RVEDV), end-systolic volume (LVESV/RVESV), ejection fraction (LVEF/RVEF), peak systolic volume rate (LVPSVR/RVPSVR), stoke volume (LVSV/RVSV), cardiac output (LVCO/RVCO). And the volumetric parameters were indexed by BSA(indexed EDV, indexed SV, indexed CO). The measurements were compared between TOF group and normal group. The left and right ventricular measurements were also compared with each other within group. Results Compared with control group indexed LVEDV reduced in TOF group(P=0.000), while indexed RVEDV increased(P=0.002). For systolic function indexes: LVEF, RVEF, LVPSVR and RVPSVR in TOF group were both lower than that in normal group(P 0.05) and indexed RVCO was increased (P=0.016). The volume and function comparision between left and right ventricle demonstrated: in normal group no difference were found in biventricular EDV, PSVR, SV, CO(P>0.05), except that LVEF was a litter higher than RVEF(P=0.000). In TOF group RVEDV, RVSV, RVCO were all greater than that of left ventricle(P<0.05), but RVEF was lower than LVEF(P=0.039). Conclusions In TOF group biventricular systolic function were both impaired and lost banlance between them. Under the volume and pressure overload the right ventricle improved pumping function by increasing heart rate. Key words: Echocardiography, real-time three-dimensional; Tetralogy of Fallot; Ventricular volume; Ventricular function

English

Tetralogy of Fallot (TOF) is a structural congenital heart defect with functional significance, occurring 1 in 3,300 live births. Several studies have reported mutations in essential transcription factors responsible for the development of TOF. NKX2.5 is a homeobox containing transcription factor, expresses at various stages of heart development. The rationale of this study was to estimate the frequency of NKX2.5 Arg25Cys mutation in TOF children from India. Using PCR-RFLP the frequency was estimated in TOF and controls. Clinical characteristics and maternal data from 84 unrelated TOF patients was collected and evaluated in comparison with 117 healthy individuals. TOF patients were tested for 22q11.2 micro deletion for the inclusion of sporadic cases. In 6% of TOF cases (n=84) a positive family history of congenital heart defects was revealed and 85.7% of children had various clinical symptoms. Significant reproductive history such as abortions, intrauterine and perinatal deaths were seen in 31% (n=26) of patient’s mothers. NKX2.5 Arg25Cys mutation was observed only in TOF children with a frequency of 5.9% TT (homozygous) and 10.7% CT (heterozygous) respectively. None of the controls showed the presence of mutation. Hence the present study concludes that the mutation Arg25Cys was segregated with disease pathology with reduced penetrance and variable expressivity.

Noninvasive Measurement of Cardiac Output During Exercise in Children with Tetralogy of Fallot

In patients with surgically repaired tetralogy of Fallot (TOF), reported peak oxygen consumption (VO(2)) is decreased compared with control subjects. The measurement of exercise cardiac output (CO) could be a useful adjunct for assessing cardiovascular fitness. There are few data assessing noninvasive CO, cardiac index (CI), and stroke volume (SV) during exercise for these patients. This study sought to measure noninvasive CI and SV during rest and exercise in children with repaired TOF. The authors compared 21 asymptomatic children with repaired TOF ages 11-17 years during rest and exercise and 42 gender- and age-matched healthy control children without structural heart disease. Using a Bruce exercise protocol, exercise data were measured noninvasively by a novel inert gas rebreathing technique including peak duration and heart rate, as well as VO(2), CO, CI, and SV measured at 90 % of peak predicted theoretical heart rate (90 % ppHR). Statistical correlation between peak VO(2) and CI was performed. At baseline, there was no statistically significant difference in any of the measures between the groups. At 90 % ppHR, there was an increase in CI during exercise of 140 % in the TOF children and 180 % in the control children. During exercise, SV changed minimally in the patient group, whereas it increased more than 30 % in the control children. At 90 % ppHR, the patient group showed an increase in VO(2) during exercise similar to that of their healthy peers. The patients had a significantly shorter peak exercise duration than normal control subjects. The patients had a lower CI during exercise because they were less able to increase SV. Therefore, at similar heart rates, patients who have had TOF repair must rely on increased peripheral muscle extraction, with a higher arteriovenous oxygen difference (SaO(2)-MvO(2)) during exercise, which may limit peak exercise capacity. In this cohort of TOF patients, noninvasive CI measurement was feasible, and correlation with VO(2) was good.

Chromosome 22q11.2 microdeletion and phenotype analysis of patients with non-syndromic tetralogy of Fallot

To investigate the frequency and clinical phenotypes of 22q11.2 microdeletion in patients with non-syndromic tetralogy of Fallot (TOF). Six-eight non-syndromic TOF patients (38 males and 30 females, aged 0-11 years) were selected and evaluated by history, physical examination and review of medical records. After informed consent was obtained, peripheral blood was drawn for genomic DNA extraction. Chromosome 22q11.2 microdeletion was screened by multiplex ligation-dependent probe amplification (MLPA). Suspected cases were confirmed with fluorescence in situ hybridization (FISH). Data was analyzed with SPSS 11.5 software. Phenotype-genotype correlations were assessed using Fisher's exact test. P values less than 0.05 on a 2-sided test were considered to be significant. Six-eight non-syndromic TOF children were screened for a 22q11.2 deletion, among which 59 (86.8%) presented pulmonary stenosis (PS) and 9 (13.2%) presented pulmonary atresia (PA). Seven patients (10.3%) were found to have carried a deletion. Among these, four had TOF-PS, three had TOF-PA. The frequency of 22q11.2 deletion in patients with TOF-PA (3/9, 33.3%) is much higher than that of TOF-PS (4/59, 6.80%) (P< 0.05). 22q11.2 microdeletion is present in approximately 10.3% of patients with non-syndromic TOF. The deletion tends to have a higher prevalence in patients with TOF-PA. 22q11.2 deletion should be screened in non-syndromic TOF children and genetic counselling may be provided.