Surgical Repair Of Tetralogy Of Fallot Research Articles

Assessment and Grading of Pulmonary Regurgitation Following Tetralogy of Fallot Surgical Repair by Cardiac Magnetic Resonance Imaging

Assessment and Grading of Pulmonary Regurgitation Following Tetralogy of Fallot Surgical Repair by Cardiac Magnetic Resonance Imaging

Evaluation of Common Complications Following Tetralogy of Fallot Surgical Repair by Magnetic Resonance Imaging

Evaluation of Common Complications Following Tetralogy of Fallot Surgical Repair by Magnetic Resonance Imaging

Management of tetralogy of Fallot in South Africa

This issue of the Journal contains 2 papers on the local management of tetralogy of Fallot (TOF). The first comprehensively and frankly reviews the surgical repair of TOF in a state hospital. The detailed text is well complemented by excellent tables and figures. Particularly noteworthy is the complete (100%) early postoperative studies of the severity of pulmonary regurgitation, right ventricular outflow gradients and trans-annular plane systolic excursion (TAPSE), by transthoracic echocardiogram. Under the circumstances, the surgery itself was satisfactory.

Administration of Milrinone Following Tetralogy of Fallot Repair Increases Postoperative Volume Administration Without Improving Cardiac Output.

Phosphodiesterase inhibitors are known to relieve symptoms in the setting of heart failure, although their effects in restrictive ventricular physiology have been poorly characterized. We explored the association between the use of milrinone and volume administration during the first 72 hours following surgical repair of tetralogy of Fallot (TOF). We reviewed all cases of primary surgical repair of TOF with pulmonary stenosis or atresia at Boston Children's Hospital between 2011 and 2020. To adjust for baseline differences between patients who did and did not receive milrinone, we matched patients with similar propensity scores in a 1:1 ratio (use of milrinone versus not). We then compared the need for volume administration during the first 72 hours postoperatively, vital signs, and measures of cardiac output between the matched cohorts. Additionally, in the group of patients receiving milrinone, linear regression modeling was used to explore the relationship between total dose of milrinone and total volume administration. Among 351 included patients, 134 received perioperative milrinone. A total of 212 patients (106 per group) were matched based on anatomic and surgical risks using a propensity score. After propensity matching, compared with nonmilrinone-treated patients, milrinone-treated patients were given postoperative volume more frequently (66% vs 52%; difference 14% [95% confidence interval, CI, 1%-27%]; P = .036). Milrinone-treated patients had a slower recovery of tachycardia during the first 12 hours (difference in slope 0.30 [95% CI, 0.14-0.47] beats per minute [BPM]/h; P < .001), and the intergroup difference peaked at 12 hours postoperatively (8 [95% CI, 5-12] BPM). Milrinone administration was not associated with improved cardiac output, including arteriovenous oxyhemoglobin saturation difference. In propensity-matched patients receiving milrinone, the total volume administered during the first 72 postoperative hours was significantly associated with the cumulative dose of postoperative milrinone ( r = 0.20; 95% CI, 0.01-0.38; P = .036). Based on the slope of the regression line, for every 1000 µg/kg of milrinone (equivalent to ~0.25 µg/kg/min for 72 hours) administered in the first 72 postoperative hours, an estimated 11.0 (95% CI, 0.6-21.4) mL/kg additional volume was administered. The use of milrinone within the first 72 hours following TOF repair is associated with more frequent administration of volume, a positive association between a higher total dose of postoperative milrinone and the amount of postoperative volume administered, a higher heart rate, and a lower blood pressure, but is not associated with improved cardiac output.

Outcomes of Surgical Repair of Tetralogy of Fallot: A Comparison Between the Adult and Pediatric Population.

Introduction Tetralogy of Fallot (TOF) is the most common cyanotic congenital heart disease. Early detection and timely treatment have provided successful repair of the anomaly in the developed world. However, in the developing world, there is still a burden of uncorrected TOF patients reaching adulthood. The goal of this study is to determine whether there is any difference in postoperative complications between adult and pediatric populationsfollowing surgical correction for TOF. Methods This study involved all those patients who received primary or secondary surgical repair for TOF in our facility between January 2017 and December 2020. The patients were split according to their age into the pediatric groupif they were under 18 years and the adult groupif they were 18 years or older. Patients with absent pulmonary valve or pulmonary atresia were not included in this study. Patients with large major aortopulmonary collateral arteries (MAPCA) were also excluded from this study. All patients underwent total correction through a median sternotomy approach. The ventricular septal defect was closed with a Bard knitted fiber patch. The right ventricular outflow tract (RVOT) was augmented by excising muscle bands or fibrous bands in the RVOT. If the annulus was smaller than the 3.5 z score, then a transannular patch was done using an autologous pericardium. The main pulmonary artery was augmented in every surgery using an autologous pericardial patch. All patients were shifted to the ICU on the ventilator and were extubated after fulfillment of the extubation criteria. Postoperative complications measured included re-opening, re-intubation, prolonged ventilation (>24 hours), and mortality within the index hospital admission. The clinical data of all patients were prospectively collected and analyzed using the chi-square test and t-test. A p-value of less than or equal to 0.05 was considered significant. Results The total number of patients was 134. This included 83 males (60.1%). A total of 114 patients who were aged below 18 years were included in the pediatric group, and 20 patients aged equal to or more than 18 years were included in the adult group. The mean average perfusion time in minutes in the adult group was 125.8 and in the pediatric group, it was 98.79. Similarly, the mean average of the cross-clamp time was also longer in the adult group at 89.55 minutes versus 69.63 minutes in the pediatric group. Overall, in the adult group, three (15%) patients had postoperative complications, while in the pediatric group, a total of 14 (11.9%) patients had postoperative complications (p = 0.001). However, there was no significant difference in the number of re-openings (8.5% vs. 10%; p = 0.8). The total mortality observed was 16 (11.59%). This included 14 (11.9%) in the pediatric group and two (10%) in the adult group. There was no significant difference between the two groups (p = 0.8). Conclusions Surgical repair of TOF can be performed in both adult and pediatric populations with acceptable outcomes. The mortality rate was found to be slightlygreater in the pediatric population compared to the adults. However, it can be seen that the number of postoperative complications is greater in adults. Further research is needed to optimize outcomes for both pediatric and adult patients with TOF.

Health related quality of life in adults after late repair of tetralogy of fallot: experience from a low-middle income country.

Without neonatal screening in low middle-income countries like Pakistan, Tetralogy of Fallot (TOF) is a congenital heart disease which frequently remains untreated beyond infancy. The purpose of this study is to determine and assess outcomes and health related quality of life (HRQOL) in patients who undergo complete repair of TOF as adults. 56 patients who underwent complete TOF repair after 16years of age were included. Patient data was collected via retrospective chart review, and a semi structured interview along with Short-Form 36 (SF-36) questionnaire were used to assess HRQOL. 66.1% of patients were male with the mean age at surgery of 22.3 ± 6.00. All patients had a post-operative NYHA Classification of I or II, 94.6% had an ejection fraction of ≥ 50% and 28.6% showed small residual lesions in follow-up echocardiograms. 32.1% of patients suffered post-operative morbidity. For the quantitative assessment using SF-36 scores, patients showed good scores of median 95 (65-100). A major cause of delay to treatment was lack of consensus between treatments offered by doctors in different parts of Pakistan. There was a pattern of 'inability to fit in' among patients who had had late TOF repair, despite self- reported improved HRQOL. Our results indicate that even with a delayed diagnosis, surgical repair of TOF produces good functional results. However, these patients face significant psychosocial issues. While early diagnosis remains the ultimate goal, patients undergoing late repair should be managed in more holistic manner with attention to psychological impact of the disease as well.

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.

Serial echocardiography assessment and clinical outcomes among pregnant women with Tetralogy of Fallot

Abstract Funding Acknowledgements Type of funding sources: None. Following advancements in intracardiac repair of Tetralogy of Fallot (TOF), a greater proportion of patients survive well beyond child-bearing age. Pulmonary regurgitation (PR) and subsequent progressive right ventricular dilatation occur frequently as an intrinsic complication of surgical repair of TOF. High-output states such as pregnancy may exacerbate these late complications. The advocation of pre-pregnancy pulmonary valve replacement to mitigate pregnancy-related cardiac burden has remained controversial. This study aims to delineate the outcomes of pregnancy among women with repaired TOF. 105 pregnant episodes among were identified from a cohort of 240 adult female patients with TOF between 1990 to 2021. Patients with echocardiographic studies performed within 1 year prior to and following delivery were included for cardiac functional analysis. A paired sample t-test was performed to compare echocardiographic parameters between pre-delivery and post-delivery periods. Linear regression was used to identify changes to identify significant changes in echocardiographic parameters among patients with a baseline of severe PR. Within all pregnant episodes (n = 105), 65 successful pregnancies, 16 spontaneous miscarriages, 21 termination of pregnancies and 3 ectopic pregnancies were recorded. The mean maternal age was 28.9 (±6.7) years with deliveries at 37.86 (30-41) gestational weeks. Cardiovascular events occurred in 19 pregnancies with 4 patients having gestational hypertension, 4 patients with pre-eclampsia toxemia, 7 patients with heart failure symptoms and 4 patients with arrhythmias. Other complications included 4 patients with gestational diabetes mellitus, 3 patients with impaired glucose tolerance, 2 patients with anemia, 3 patients with maternal thyroid disease and 1 patients with proteinuria. Echocardiographic studies demonstrated significant changes in left ventricular ejection fraction (LVEF) (Pre-delivery = 60.69 ±8.73; post-delivery = 59.39 ±9.36) (P = 0.007), left ventricular end diastolic volume (LVEDV) (Pre-delivery: 89.71mL ±18.22mL; Post-delivery: 80.96mL ±12.32mL; P = 0.007), left end systolic volume (LVESV) (Pre-delivery: 35.43 ±12.36; Post-delivery: 32.70 ± 8.83; P = 3.7x10-5) and right ventricular index of myocardial performance score (Pre-delivery: 0.34 ±0.12; Post-delivery: 0.33 ±0.12; P = 0.007). Patients with severe PR was found to have significantly worse right ventricular global longitudinal strain (RV GLS) (P = 0.029). 2 patients progressed to severe PR following delivery. Adult female patients with TOF can have viable pregnancies with acceptable mortality and morbidity. Deterioration in echocardiographic parameters were identified when comparing between pre-delivery and post-delivery studies. TOF patients should be closely monitored throughout and post-delivery for detection of deterioration of cardiac function and clinical symptoms.

Long-term Outcomes of Tetralogy of Fallot in the Kingdom of Bahrain.

Introduction:Tetralogy of Fallot (TOF) is the most common cyanotic congenital heart disease. Surgical correction has improved survival but re-intervention is often required.Objectives:The objective is to assess outcomes after surgical repair of TOF, long-term follow-up, and factors that influence these results.Materials and Methods:This is a retrospective study conducted in a tertiary care center. Records of patients diagnosed with TOF from 1992 to 2019 (37 years) were retrieved from a detailed database. Patients who underwent complete correction were grouped according to diagnosis, the technique utilized in surgical repair, need for staged repair, and syndromic association. Univariate actuarial and event-free survival analysis was performed. The endpoint for an event was death or re-intervention.Results:A total of 230 patients were diagnosed with TOF and 174 patients underwent complete surgical repair. At 40 years postoperatively, survival was 96%. Actuarial survival was independent of syndromic associations, anatomical diagnosis, type of surgery, or previous shunt. Event-free survival (EFS) survival was 8.12%. EFS was significantly worse for patients with pulmonary atresia (PA) (Hazard ratio, 4.1125; 95% confidence interval [CI], 1.2654–13.3657; P < 0.0001) and for those that required homograft/conduit. The median duration for EFS was 22.73 years, 19.58 years, and 9.12 years for transannular patch (TAP), pulmonary valve-sparing (PVS), and homograft group, respectively. The survival curve for the PVS group merged with that of TAP 20 years postoperatively. Similarly, it merged at 22 years for staged versus primary repair and at 22.73 years for syndromic versus nonsyndromic patients. A weak correlation was found between age at surgery and event-free duration (cc, 0.309; P < 0.0001). The need for TAP was not influenced by the previous palliation, χ2(1, n = 154) = 3.36, P = 0.0667, or with interval to complete correction after the shunt procedure (P = 0.9672).Conclusions:Total correction of TOF has low perioperative mortality and good long-term survival, but the need for re-interventions is high. This study demonstrated that patients requiring homograft/conduit and those with a diagnosis of PA had worse outcomes. Comparison between different surgical groups showed merging of survival curves in follow-up that signifies gradual loss of survival advantage over time.

Our experience of epidural anesthesia for cesarean section in pregnant women with corrected tetralogy of fallot: A case report

Tetralogy of Fallot (TOF) is the most commonly encountered cyanotic congenital cardiac disease in pregnancy. While most pregnancy cases after surgical repair of TOF have focused on cardiovascular and obstetric concerns, relatively few authors have focused on specific intraoperative and postoperative anesthetic management strategies. Determine the type of anesthesia in these patients is very difficult and current recommendations are based only on reported clinical experiences and pathophysiological concepts. In this case report, we aimed to share our epidural anesthesia experience for caesarean surgery in 37 weeks pregnant who underwent corrected TOF surgery.

The Effect of Geometric and Hemodynamic Parameters on Blood Flow Efficiency in Repaired Tetralogy of Fallot Patients.

Surgical repair of Tetralogy of Fallot (TOF) involves a series of steps to remove right ventricular outflow tract and pulmonary artery obstruction. However, the large degree of anatomic variability among preoperative TOF patients may impact the effectiveness of different repair strategies and, subsequently, different geometric modifications for different patients. This study investigates the relationships between geometric and hemodynamic parameters and mechanical energy efficiency for a patient-specific dataset of 16 postoperative TOF repairs, using morphometric and statistical shape analyses, as well as computational fluid dynamics simulations with physiologically-relevant inlet and outlet boundary conditions. Quantitatively, negative correlations were found between the right and left pulmonary artery centerline tract cumulative torsion and energy efficiency (r = -0.65, p = 0.01, for both). A positive correlation was also found for a statistical shape mode associated with skewing of the geometric sub-regions (r = 0.61, p = 0.01). Qualitatively, medium- and low-efficiency geometries exhibit disturbed flow and much more proximal vortex formation as compared to a high-efficiency geometry. Thus, it is recommended, as much as possible, to both relieve and avoid the introduction of torsion into the patient's anatomy during surgical repair of TOF.

Abstract 15118: Pulmonary Valve Preservation During Tetralogy of Fallot Repair is Associated With Enhanced Pulmonary Valve and Right Ventricular Function

Introduction: Many centers have recently adopted pulmonary valve (PV) preservation procedures to prevent the detrimental long-term effects of chronic pulmonary regurgitation after tetralogy of Fallot (ToF) repair. Hypothesis: Here, we investigated PV and right ventricular function after PV preservation surgical strategies compared to standard transannular patch (TAP) repair. Methods: All patients undergoing transatrial-transpulmonary repair for ToF with PV stenosis at our institution between January 2007 and May 2020 were reviewed retrospectively. Patients were divided into 2 main groups, according to the different techniques used: patients undergoing a PV preservation strategy and patients undergoing TAP repair. Results: Overall, 82 patients underwent a successful PV preservation strategy while 34 underwent a standard TAP repair. At index surgery, BSA (0.31±0.1 m 2 , P=0.3), age (4.8±0.3 months, P=0.5) and preoperative PV Z-score (-3.20±0.1, P=0.1) did not differ between groups. Five-year actuarial freedom from moderate/severe PV regurgitation was significantly higher in the PV preservation group compared to the TAP (61.3% [95% CI: 48-73%] vs 25.9% [95% CI: 12-43%], respectively; p=0.02). After adjusting for age, gender, BSA, and type of PV, the use of a TAP was still significantly associated with an increased risk for PV regurgitation at follow up (HR: 1.85, 95% CI: 1.09, 3.15; p=0.02). At a mean follow-up of 6.9±0.3 years, patients undergoing PV preservation showed an increased right ventricular fractional area change (46.9±0.8% vs 42.5±1.7%, P&lt;0.001) and tricuspid annular plane systolic excursion z-score (-3.36±0.3% vs -4.7±0.4%, P=0.005), while maintaining better PV competence in terms of pulmonary regurgitation index (87.9±1.2% vs 82.7±2.4%, P=0.02). Conclusions: Surgical repair of ToF with PV preservation provides excellent outcomes in terms of PV competence and right ventricular function and should be advocated whenever possible.

Evolution of ventricular function in children with permanent right ventricular pacing after tetralogy of Fallot repair: A midterm follow-up.

We aim to evaluate the midterm effect of chronic apical right ventricular (RV) pacing on right and left ventricular (LV) function using different modalities of echocardiography including conventional echocardiography, tissue Doppler imaging and two-dimension speckle tracking echocardiography. This case-control study enrolled 49 patients divided into two groups: a paced group and a nonpaced group. The paced group included 23 patients that underwent tetralogy of Fallot (TOF) repair and required permanent pacemaker insertion for postoperative complete heart block. The nonpaced group included 26 patients that had TOF repair at the same period. The median age for the paced and nonpaced groups was 6 and 8 years, respectively (P = .169). The follow-up duration after TOF surgical repair was 4 years for the paced patients and 5 years for nonpaced patients (P = .411). In the nonpaced group, the QRS duration increased and LV global longitudinal strain (GLS) decreased significantly with increasing duration after TOF repair, P value was .006 and .042, respectively. In the paced group, tricuspid annular systolic plane excursion (TAPSE) was significantly correlated with age (r = .578; P = .004) and duration following TOF correction (r = .724; P < .001). Chronic RV apical pacing in children after TOF repair was associated with better clinical status, preservation of RV systolic function, and prevention of progressive QRS prolongation. RV pacing was not associated with progressive deterioration of LV systolic function with increasing the time interval following TOF repair. Therefore, RV pacing can be beneficial in corrected TOF patients presenting with RV failure.

4D Flow versus Conventional 2D MRI for Measuring Pulmonary Flow after Tetralogy of Fallot Repair

After tetralogy of Fallot (TOF) repair, pulmonary regurgitation and right ventricular function must be monitored. Conventional (2D) cardiac magnetic resonance (CMR) is currently the clinical reference method for measuring pulmonary regurgitation. However, 4D CMR has been reported to provide a more comprehensive flow analysis than 2D CMR. We aimed to compare 4D CMR to 2D CMR for assessing pulmonary regurgitation and flow, as well as aortic flow, in children and adults after surgical repair of TOF. Retrospective analysis of patients with repaired TOF admitted for cardiac MRI with 4D flow acquisition from 2016 to 2018. Linear regression was used to assess correlations and Bland-Altman analyses were performed. The 60 included patients had a mean age of 18.2 ± 10.4 years (range, 2-54 years). Significant correlations between the two techniques were found for pulmonary regurgitant fraction (R 2 = 0.6642, P < 0.0001), net pulmonary flow (R 2 = 0.6782, P < 0.0001), forward pulmonary flow (R 2 = 0.6185, P < 0.0001), backward pulmonary flow (R 2 = 0.8192, P < 0.0001), and aortic valve flow (R 2 = 0.6494, P < 0.0001). The Bland-Altman analysis showed no significant bias, narrow limits of agreement, and few scattered points. The correlation between pulmonary and aortic flow was better with 4D CMR than with 2D CMR (R 2 = 0.8564, P < 0.0001 versus R 2 = 0.4393, P < 0,0001, respectively). Interobserver reliability was good. These results establish the feasibility and reliability of 4D CMR for assessing pulmonary flow in a large paediatric and adult population with repaired TOF. 4D CMR may be more reliable than 2D MRI for pulmonary flow assessment after TOF repair.

4D flow MRI versus conventional 2D for measuring pulmonary flow after Tetralogy of Fallot repair

BackgroundAfter tetralogy of Fallot (TOF) repair, pulmonary regurgitation and right ventricular function must be monitored. Conventional (2D) cardiac magnetic resonance (CMR) is currently the clinical reference method for measuring pulmonary regurgitation. However, 4DFlow CMR has been reported to provide a more comprehensive flow analysis than 2D CMR. We aimed to compare 4DFlow CMR to 2D CMR for assessing pulmonary regurgitation and flow, as well as aortic flow, in children and adults after surgical repair of TOF. MethodsRetrospective analysis of patients with repaired TOF admitted for cardiac MRI with 4DFlow acquisition from 2016 to 2018. Linear regression was used to assess correlations and Bland-Altman analyses were performed. ResultsThe 60 included patients had a mean age of 18.2 ± 10.4 years (range, 2–54 years).Significant correlations between the two techniques were found for pulmonary regurgitant fraction (R [2] = 0.6642, p < 0.0001), net pulmonary flow (R [2] = 0.6782, p < 0.0001), forward pulmonary flow (R [2] = 0.6185, p < 0.0001), backward pulmonary flow (R [2] = 0.8192, p < 0.0001), and aortic valve flow (R [2] = 0.6494, p < 0.0001). The Bland-Altman analysis showed no significant bias, narrow limits of agreement, and few scattered points. The correlation between pulmonary and aortic flow was better with 4DFlow CMR than with 2D CMR (R [2] = 0.8564, p < 0.0001 versus R [2] = 0.4393, p < 0,0001, respectively). Interobserver reliability was good. ConclusionThese results establish the feasibility and reliability of 4DFlow CMR for assessing pulmonary flow in a large paediatric and adult population with repaired TOF. 4DFlow CMR may be more reliable than 2D MRI for pulmonary flow assessment after TOF repair.

4D Flow versus Conventional 2D MRI for Measuring Pulmonary Flow after Tetralogy of Fallot Repair

After tetralogy of Fallot (TOF) repair, pulmonary regurgitation and right ventricular function must be monitored. Conventional (2D) cardiac magnetic resonance (CMR) is currently the clinical reference method for measuring pulmonary regurgitation. However, 4D CMR has been reported to provide a more comprehensive flow analysis than 2D CMR. We aimed to compare 4D CMR to 2D CMR for assessing pulmonary regurgitation and flow, as well as aortic flow, in children and adults after surgical repair of TOF. Retrospective analysis of patients with repaired TOF admitted for cardiac MRI with 4D flow acquisition from 2016 to 2018. Linear regression was used to assess correlations and Bland-Altman analyses were performed. The 60 included patients had a mean age of 18.2 ± 10.4 years (range, 2–54 years). Significant correlations between the two techniques were found for pulmonary regurgitant fraction (R 2 = 0.6642, P < 0.0001), net pulmonary flow (R 2 = 0.6782, P < 0.0001), forward pulmonary flow (R 2 = 0.6185, P < 0.0001), backward pulmonary flow (R 2 = 0.8192, P < 0.0001), and aortic valve flow (R 2 = 0.6494, P < 0.0001). The Bland-Altman analysis showed no significant bias, narrow limits of agreement, and few scattered points. The correlation between pulmonary and aortic flow was better with 4D CMR than with 2D CMR (R 2 = 0.8564, P < 0.0001 versus R 2 = 0.4393, P < 0,0001, respectively). Interobserver reliability was good. These results establish the feasibility and reliability of 4D CMR for assessing pulmonary flow in a large paediatric and adult population with repaired TOF. 4D CMR may be more reliable than 2D MRI for pulmonary flow assessment after TOF repair.

Histopathological evidence of aortopathy in newborns and infants with Tetralogy of Fallot at the time of the surgical repair.

The purpose of this study was to evaluate the microscopic structural abnormalities of the ascending aorta in infants with Tetralogy of Fallot (ToF) and compare them with aortic samples from control group of small children that died of other diseases. We aimed at identification of the specific histopathological changes associated with ToF and correlation of the severity of these changes with time to surgery and mean levels of saturation in the ToF group, and age at death in control group. The full-thickness ascending aortic wall sample was taken from 23 children with ToF at the time of surgical reconstruction (age spread 2 to 19 months) and evaluated by light microscopy. Corresponding samples were taken from 16 cadaverous cases of children with other diseases (0-76 months). The assessed morphological variables included elastic fiber fragmentation/loss, thinning and disorganisation, presence of laminar medial necrosis, intralamellar and translamellar mucoid extracellular matrix accumulations, smooth muscle cell disorganisation, presence of fibrosis, calcifications and neovascularisation and finally grade of overall medial degeneration. No difference was found between the two groups in the individual morphological variables. However, there was a difference in the distribution of the grades of the overall medial degeneration (P = .016). ToF group showed uniform mild degenerative changes, whereas control group harboured spectrum of changes ranging from normal to moderate. The presence of the given histopathological changes and their severity were associated neither with age at surgery or mean levels of saturation in ToF group, nor with the age at death in the control group. This study emphasizes the histopathological assessment of the bioptic samples of the ascending aorta during the surgical repair of ToF, since the patients demonstrating moderate or severe degenerative changes already in the early childhood may be in increased risk of the subsequent late complications.

Usefulness of Red Blood Cell Distribution Width in the Assessment of Hemodynamics After Tetralogy of Fallot Repair.

There are no reports on the effect of red blood cell distribution width (RDW) in surgical repair of tetralogy of Fallot (ToF). Methods and Results: A total of 50 patients who underwent cardiac catheterization after surgical repair of ToF were retrospectively assessed. RDW was positively correlated with the ratio of right ventricular pressure to left ventricular pressure (RVP/LVP; P<0.0001, r2=0.57). Patients with elevated RDW had a higher RVP/LVP than those with a normal RDW (P<0.0001). Also, elevated RDW was related to elevated central venous pressure (P<0.0001), decreased mixed venous oxygen saturation (P<0.0001), greater pulmonary stenosis (P=0.003) and severe pulmonary regurgitation on echocardiography (P<0.0001), a higher rate of residual ventricular septal defect leak (P=0.004) and higher reoperation rate (P=0.009). Of the 7 patients who underwent reoperation, 6 had decrease in RDW after reoperation (P=0.012). On multivariable regression analysis, RDW was the strongest indicator of higher RVP/LVP. For the first time, RDW has been shown to be a strong indicator for assessing the hemodynamics and risk of later reoperation after surgical repair of ToF.

Risk Factors and Outcome of Post Fallot Repair Junctional Ectopic Tachycardia (JET).

Junctional ectopic tachycardia (JET) is a common arrhythmia causing hemodynamic impairment following corrective cardiac surgery such as tetralogy of Fallot (TOF) repair. We report our experience with postoperative JET following surgical repair of TOF. The retrospective study was done from 2003 to 2012 with a total of 105 patients who underwent TOF repair. These patients' clinical and electrocardiographic data (pre-, intra-, and postoperative) were monitored to identify risk factors for the occurrence of JET and to evaluate the outcome of the affected patients. Incidence-Fourteen patients developed JET, with only four patients going directly from sinus rhythm to JET. In all others, either a transient atrioventricular (AV) block or a junctional rhythm preceded JET, mostly intraoperatively, showing a significant relation ( P = .010). Age-Patients with JET were of younger age ( P = .025) and had longer cardiopulmonary bypass ( P = .044) and aortic cross-clamping times ( P = .038). Increased cost and care-The occurrence of JET was associated with a longer stay in the intensive care unit (ICU) and a prolonged need for inotropic support and mechanical ventilation. Time to rate control correlated with length of ICU and hospital stay. All JET patients converted into sinus rhythm, one of them died shortly after cessation of JET and two patients subsequently developed a first-degree AV block. The occurrence of JET remains an important complication during the initial postoperative period by increasing mechanical ventilation time, the need for inotropic support, and prolonging the length of ICU and hospital stay. Risk factors are younger age, longer aortic cross-clamping/bypass times, and intraoperative arrhythmias.

4D flow CMR analysis in repaired tetralogy of Fallot: Where we are and where we are going

4D flow cardiovascular magnetic resonance (4D flow CMR) enable time-varying and multidirectional comprehensive retrospective analysis. However, this imaging modality must prove its relevance. After tetralogy of Fallot surgical repair, pulmonary valve function and right ventricle (RV) volumes are carefully followed. We investigate reliability of 4D flow CMR to measure these parameters compared to gold-standard 2D CMR. Twenty patients with rTOF and 5 healthy controls (mean age 29 ± 13 years, mean height 169 ± 7 cm, mean weight 66.2 ± 13.4 kg) underwent RV volumes and pulmonary regurgitation analysis by 2D CMR and 4D flow CMR using a clinical 1.5 T MRI scanner (GE; GE Healthcare, Milwaukee, WI, USA). Mean 4D flow acquisition time was 8.0 ± 1.8 min, whereas conventional 2D CMR was 39 ± 7.2 min ( P < 0.0001). Linear regression analysis shows a good correlation between 2D CMR and 4D flow CMR for RV stroke volume (SV) (r 2 = 0.89, P < 0.0001), end-diastolic RV volume (r 2 = 0.93, P < 0.0001), pulmonary regurgitation volume (r 2 = 0.94, P < 0.0001) and pulmonary regurgitation fraction (r 2 = 0.89, P < 0.0001). Mean bias [−1.96SD; +1.96SD] was 17.5 mL [4.75; 30.15] for RVSV, −8.4 mL [−17.04; 0.16] for end diastolic RV volume, 1.5 mL [−2.17; 5.23] for pulmonary regurgitation volume and 0.7% [−0.8; 2.2] for pulmonary regurgitation fraction by Bland–Altman analysis. Using 4D flow CMR, linear regression analysis showed a good correlation between RV SV and pulmonary valve systolic ejection volume (r 2 = 0.55, P < 0.0001), and between systemic and pulmonary flow (r 2 = 0.90, P < 0.0001). Mean bias was 22.7 mL [6.4; 39.0] for SV, and −0.06 mL [−0.18; 0.06] for systemic and pulmonary flow by Bland–Altman analysis. Vortex in the pulmonary artery trunk were observed in all cases of rTOF patients and limited analysis of pulmonary artery regurgitation flow. 4D flow imaging may be a clinically relevant alternative to 2D CMR in rTOF, providing faster and reliable data.