Corrected Tetralogy Of Fallot Research Articles

Comparison of Right Ventricular Outflow Tract Reconstruction Techniques on Mid-Term Pulmonic Valve Fate.

Introduction: The pulmonic valve-sparing technique (PVS) is an emerging approach of right ventricular outflow tract reconstruction in tetralogy of Fallot (TOF) correction aimed at reducing the incidence of pulmonic regurgitation (PR) and the need for subsequent reintervention. This study aims to compare the long-term occurrence of moderate to severe PR/stenosis (PR/PS) between three different approaches. Patients and Methods: We conducted a retrospective cohort study involving 173 patients who underwent TOF correction at Chiang Mai University hospital between January 2006 and December 2016. The patients were divided into three groups: transannular patch (TAP; n = 88, 50.9%), monocusp insertion (MCI; n = 40, 23.1%), and PVS (n = 45, 26%). The study assessed freedom from moderate to severe PR/PS. Results: The median overall follow-up time was 79.8 months (interquartile range: 50.7-115.5 months. The PVS exhibited larger PV Z-score (-2.6 ± 2.3 mm, P < .001), with predominantly tricuspid morphology (64.4%). The PVS had significantly shorter median ventilator time, intensive care unit stay, hospital stay, and longer median follow-up time. Postoperative moderate-severe PR was lower in the PVS group (P < .001), with no significant difference in PS (P = .356) and complications among the groups. Freedom from moderate-severe PR/PS was longer in the MCI group (2.8, 0.2-42.3 months vs 30.9, 0.2-50.9 months, respectively). Multivariable analysis showed TAP and MCI had a higher risk of developing moderate-severe PR (hazard ratio [HR] 2.51; 95% confidence interval [CI] 1.23-5.13 vs HR 1.41; 95%CI 0.59-3.38) but lower risk of moderate-severe PS (HR 0.14; 95%CI 0.02-0.9 vs HR 0.39; 95%CI 0.05-3.19). Conclusion: Pulmonic valve-sparing reconstruction showed promise in preventing late moderate-severe PR in patients with favorable PV anatomy. However, it should be noted that this technique is associated with a higher incidence of PS.

Long-Term Results Following Combined Repair of Atrioventricular Septal Defect with Tetralogy of Fallot.

Atrioventricular septal defect (AVSD) in association with tetralogy of Fallot (TOF) is a rare and complex congenital cardiac malformation. We report our institutional experience and outcomes following surgical correction over a 20-year period. Patients who underwent combined surgical AVSD and TOF correction between October 2001 and February 2020 were included for analysis. All patients underwent primary repair. The study data were prospectively collected and retrospectively analyzed. Primary outcomes were in-hospital mortality and long-term freedom from reoperation. During the study period, a total of 10 consecutive patients underwent combined surgical AVSD and TOF correction. Median age at operation was 307days (IQR 228-457) and median weight was 7.7kg (IQR 6.7-9.5). Down Syndrome was present in six of the patients. In-hospital mortality was 0%. One patient required re-exploration due to bleeding. Median follow-up was 11years (IQR 11months -16years). There was one case of reoperation due to significant residual ventricular septal defect after 2 months. None of the patients died during follow-up. Combined primary AVSD and TOF repair can be performed with low early mortality and morbidity, as well as a high long-term freedom from reoperation.

The Utility of Invengenx® Bovine Patch for Right Ventricular Outflow Tract (RVOT) Reconstruction and Augmentation in the Surgical Management of Tetralogy of Fallot (TOF): A Contemporary Study and Review of the Literature.

Background and objective Complex congenital heart diseases (CHDs), such as the tetralogy of Fallot (TOF), often warrant reconstruction and augmentation of the right ventricular outflow tract (RVOT). This procedure requires the use of both synthetic and natural materials. However, finding the ideal material for tissue implants can be challenging. Biological materials often face issues such as tissue degeneration, calcium deposition, antigenicity, rejection, shrinkage, and fibrosis. These issues can lead to complications such as stenosis and insufficiency, potentially requiring early reoperations. In light of this, this study aimed toinvestigate the effectiveness of the Invengenx® bovine patch for RVOT reconstruction and augmentation. Methods This was a retrospective observational studyconducted among eight children who underwent TOF correction cardiac surgery. Their demographic and clinical characteristics, intraoperative findings, and postoperative follow-up results at six months were collected from the hospital patient database. Results There were no deaths or complications in this study. We observed a significant reduction in the gradient across the pulmonary valve and the outflow tract at six months post-procedure. The analysis demonstrated that the Invengenx® bovine patch was successful and did not lead to any complications. Conclusions This study demonstrates the safety and efficacy of this engineered bovine pericardial patch (Invengenx®) as a cardiovascular substitute for surgical repair of both simple and more complex congenital cardiac defects.

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.

Association Between Preoperative Sarcopenia and Early Postoperative Outcomes in Pediatric Patients Undergoing Total Correction of Tetralogy of Fallot: A Retrospective Cohort Study

To identify the association between preoperative low muscle mass and early postoperative outcomes in pediatric patients undergoing total correction of tetralogy of Fallot (TOF). A retrospective cohort study. A single university hospital in Seoul, South Korea. Pediatric patients (≤3 years) who underwent total correction of TOF between May 2008 and February 2018. None. Cross-sectional areas of the pectoralis and erector spinae muscles were measured using preoperative chest computed tomography (CT) scans, and adjusted to body surface area to define muscle mass index. The patients were divided into sarcopenia, presarcopenia, and no sarcopenia groups based on cutoff values determined using the mean and SD of the muscle mass index in the third z-weight quintile. Of a total of 330 patients included in the final analysis, 13 were associated with the sarcopenia group, 57 in the presarcopenia group, and 260 in the no sarcopenia group. The sarcopenia group exhibited a higher incidence of major adverse events than the presarcopenia and no sarcopenia groups, respectively (38% v 25% v 18%; p=0.033). Logistic regression analyses revealed that only younger age at the time of surgery was significantly associated with major adverse events (odds ratio 0.82; 95% CI 0.72-0.94, p=0.003). The incidence of sarcopenia, as assessed by preoperative chest CT, was low in pediatric patients undergoing total correction of TOF, and preoperative sarcopenia did not predict early postoperative major adverse events.

Identification of rapid progression of right ventricular functional measures using three-dimensional cardiac computed tomography after total surgical correction of tetralogy of Fallot

ObjectiveTo identify subsets of patients with tetralogy of Fallot (TOF) after total surgical correction demonstrating the rapid progression of right ventricle (RV) functional measures using cardiac computed tomography (CT) ventricular volumetry. MethodsRapid or slow progression of RV functional measures was determined in 109 patients with TOF who underwent cardiac CT ventricular volumetry more than twice after total surgical correction. Patient age, body surface area, postoperative days, the time interval between the first and last cardiac CT examinations, and CT-based functional measures were evaluated using binary logistic regression to determine the predictors of the rapid progression. Receiver operating characteristic curve analysis was performed to evaluate diagnostic performance of the potential predictors. ResultsThe rapid progression of indexed RV end-systolic volume (ESV) (≥2.7 mL/m2/year) and indexed RV end-diastolic volume (≥0.9 mL/m2/year) could be predicted by RV ejection fraction (EF) at the last cardiac CT with an odds ratio of 1.340 (95 % confidence interval [CI], 1.122–1.600; p = 0.001) and age at the last cardiac CT with an odds ratio of 8.255 (95 % CI, 1.531–44.513; p = 0.014), respectively. RV EF at the last cardiac CT showed the highest diagnostic performance (area under the curve = 0.799; p < 0.002) for the rapid progression of indexed RV ESV. ConclusionCardiac CT ventricular volumetry can be used to identify patients demonstrating the rapid progression of RV functional measures after total surgical correction of TOF and follow-up imaging protocols can be individually optimized based on initial progression rate.

Chronic hypoxia prolongs postoperative mechanical ventilation and reduces the left atrial pressure threshold in children with tetralogy of Fallot.

Chronic hypoxia induces pulmonary microvascular endothelial dysfunction. The left atrial pressure (LAP) represents the hydrostatic pressure of pulmonary microcirculation. The conjunction of the LAP and any abnormal pulmonary microvascular endothelial barrier function will have an impact on pulmonary exudation, resulting in prolonged mechanical ventilation. This study aimed to investigate the tolerance threshold of the pulmonary microcirculation to LAP in children with tetralogy of Fallot (TOF) to avoid prolonged mechanical ventilation after surgery. This retrospective study included 297 Chinese patients who underwent TOF correction at Fuwai Hospital. Patients were categorized according to their preoperative oxygen saturation (SpO2) level. One-to-one propensity score matching (PSM) revealed a total of 126 participants in the SpO2 < 90% and SpO2 ≥ 90% groups. Between-group comparisons were conducted to verify the correlation between hypoxia and prolonged mechanical ventilation. A subgroup analysis was performed to reveal the significant role of postoperative LAP stewardship on prolonged mechanical ventilation. Failure to extubate within the first 48 h (23.81% vs. 9.52%, P = 0.031) and prolonged mechanical ventilation (26.98% vs. 11.11%, P = 0.023) were more commonly observed in children with preoperative SpO2 < 90%. The incidence of prolonged mechanical ventilation consistently increased with LAP in both the SpO2 < 90% and SpO2 ≥ 90% groups, although LAP was still within the normal range (6-12 mmHg). Children in chronic hypoxic conditions tolerated lower LAP well. The tolerance threshold for postoperative LAP in children diagnosed with TOF under chronic hypoxic conditions was identified as 7 mmHg. Children in a chronic hypoxic state may suffer from a high incidence of prolonged mechanical ventilation after surgical correction of TOF and may not tolerate higher postoperative LAP. To improve pulmonary prognosis, it is better to control and maintain the postoperative LAP at a lower state (≤7 mmHg) in children with chronic hypoxia.

CLINICAL CASE OF SIMULTANEOUS RADICAL TREATMENT OF TETRALOGY OF FALLOT WITH MAJOR AORTOPULMONARY COLLATERAL ARTERIES

Tetralogy of Fallot (TOF) combined with major aortopulmonary collateral arteries (MAPCA) is a severe congenital heart defect due to the combination of a triad of cardiac malformation withan additional vascular anomaly of the small circulatory system. To date, there is no single accepted standard in which sequence and according to which criteria radical surgical correction of combined anomalies is indicated. This paper describes a clinical case of simultaneous opentreatment of TOF and endovascular occlusion of the MAPCA in an 8-month old child. Based onthe evidence base of a large study, our patient belonged to the group where after TOF correction the therapeutic way of MAPCA treatment was used at first, which was ineffective in 38% and led to surgical methods of MAPCA occlusion. Choosing the way of simultaneous simultaneoussurgical treatment of two pathologies allowed us to minimize postoperative risks and achievegoodclinical results.

A natural systemic to pulmonary artery shunt.

Isolation of the left subclavian artery is a rare anomaly associated with right aortic arch. We report a case in which this subclavian artery was supplying the pulmonary circulation in a patient with tetralogy of Fallot (TOF) which effectively acted as a natural systemic to pulmonary artery shunt. The patient had good room air saturation and no features of vertebrobasilar insufficiency or left upper limb ischemia. She underwent a total correction of TOF and ligation of the aberrant left subclavian artery.

Pulmonary artery reconstruction and correction of tetralogy of Fallot combined with the absence of the mediastinal left pulmonary artery.

Tetralogy of Fallot (TOF) is the most common deformity combined with the unilateral absence of the mediastinal pulmonary artery (UAMPA), and its treatment strategy remains controversial. In this study, we analyzed the effect of bilateral pulmonary reconstruction in patients with TOF combined with UAMPA. This was a single-center, retrospective review of 1713 patients with TOF between January 2009 and November 2021. Overall, eight patients were diagnosed with TOF combined with UAMPA. Among them, seven underwent surgery: three underwent one-stage TOF correction with bilateral pulmonary artery reconstruction; three patients underwent bilateral pulmonary artery reconstruction, followed by two-stage TOF correction after several months; and one patient underwent two procedures of left pulmonary artery reconstruction, and the ventral septal defect remained open. The left pulmonary arteries were reconstructed with a Goretex conduit in three cases, direct anastomosis in two cases, and the modified autologous tissue extension technique in two cases. All seven patients survived during the postoperative follow-up and showed good cardiac function and normal oxygen saturation of >97%. During follow-up echocardiography, we noted that the left pulmonary arteries reconstructed with a Goretex conduit or direct anastomosis had thrombosis or stenosis. However, those reconstructed using the modified autologous tissue extension technique was unobstructed. In patients with TOF and UAMPA, if there is a pulmonary artery confluence in the affected hilum, it is feasible to implement bilateral pulmonary artery reconstruction for one-stage TOF correction. The use of the pulmonary artery extension technique and autologous tissue for bilateral pulmonary reconstruction could reduce the incidence of anastomotic stenosis.

Implantation of monocusp valve prolongs the duration of chest tube drainage in children with tetralogy of fallot after corrective surgery.

Right ventricular outflow tract obstruction relief is one of the major procedures during the total correction of tetralogy of Fallot (TOF). Pulmonary insufficiency (PI) is usually inevitable after a transannular incision with a patch repair is performed. Therefore, some surgeons advocate to place a monocusp valve within the transannular patch (TAP) in order to decrease the severity of the PI. However, the monocusp valve seemed not be very effective in some patients who underwent the complete TOF repair. Patients who had the classic form of TOF between January 2009 and January 2017 and underwent the corrective surgery with a TAP by the same cardiovascular surgeon were identified for further analysis. Clinical information including demographics at operation, perioperative data, and postoperative outcome were collected retrospectively and compared between the group with and without a monocusp valve. A total of 24 TOF cases were included in the final analysis, and 16 (66.7%) patients received a monocusp valve placement. The patients' characteristics before and during the surgery were similar between the two groups. The median duration of chest tube drainage after the total correction in the monocusp group was longer than those without the valve (p = 0.04). There was no difference in the immediate postoperative data, including the inflammation/infection status, the duration of mechanical ventilation, and the length of ICU and hospital stay. Implantation of a monocusp valve during the total TOF correction using a TAP did not bring benefit to improve the immediate postoperative outcomes, especially the duration of the pleural drainage. Further study with a prospective design and a larger number of cases is needed.

Incidence and Type of Right Ventricular Diastolic Dysfunction in Postoperative Tetralogy of Fallot Pediatric Patients

Objective: Assessment of right ventricular (RV) diastolic function in postoperative Tetralogy of Fallot (TOF) patients is not routinely done during intermediate follow-up. We have done this study to assess the incidence and type of RV diastolic dysfunction in these patients. Method: Ninety patients who were less than 18 years and underwent total correction for TOF were analyzed in this study. The incidence and type of RV diastolic dysfunction was measured and the effect of degree of pulmonary regurgitation on RV diastolic function was also seen. Results: The total number of patients with RV diastolic dysfunction was 46 (51.1%). Restrictive RV physiology was seen in 8 patients (8.8%) and the most common type of RV diastolic dysfunction seen was pseudonormal filling, seen in 23 (25.6%) patients. Conclusion: Subclinical RV diastolic dysfunction is present in almost half of the postoperative TOF patients of pediatric age group. The most prevalent form of diastolic dysfunction was pseudonormal filling during intermediate follow-up.

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.

Intracardiac Repair in Late Adolescent and Adult Tetralogy of Fallot - Early and Midterm Results from a Tertiary Care Centre.

IntroductionIn developing countries like India, it is common for late presentation of Tetralogy of Fallot (TOF) patients to a hospital as compared to that of developed countries. The objective of this study is to analyze the surgical outcome of TOF patients with age > 15 years.MethodsThis is a retrospective descriptive study of the surgical outcomes of 45 adult patients undergoing correction for TOF. Epidemiology, symptomology, and preoperative evaluation were performed.ResultsMost of the patients were male (33 [73%]). The median age was 21 years. A total of 42 (93.33%) patients had subaortic ventricular septal defect (VSD), while three (6.6%) patients presented with doubly committed VSD. The most common type of right ventricular outflow tract (RVOT) obstruction was combined infundibular and valvular types, accounting for 34 cases (75.5%). Six patients had infundibular RVOT obstruction, while three patients (6.6%) had predominantly valvular pulmonary stenosis. We performed trans-right atrial repair in 33 patients. Right atrium-pulmonary artery approach was used in five patients (11.1%). The most common postoperative complication was right bundle branch block, seen in 14 patients, with a mortality rate of 2% in the early postoperative period. We achieved excellent early and midterm survival results and significant improvement in functions and disease-free quality of life.ConclusionIntracardiac repair in adult TOF can be performed with low mortality, less residual RVOT obstruction, and need for revision of RVOT far less frequent by using the Jhajhria Infundibular Resection Adequacy Assessment technique (JIRAAT) to assess for adequacy of infundibular resection.

Rehabilitating Case of Tetralogy of Fallot Undergone Surgical Repair with Comprehensive Exercise Program: A Case Report

Tetralogy of Fallot (TOF) is one of the most common cyanotic congenital heart disorders (CHDs). The children with CHD lead relatively sedentary lifestyles, on account of the restriction imposed by treating doctors, parents, and society and by themselves due to fear. The disability related to CHD results in an additional adverse impact on quality of life and physical activity level due to deconditioning. The cardiovascular effects of deconditioning are a decrease in cardiac output, increase resting heart rate with activity, decreased blood pressure in upright positions (orthostatic hypotension), and decreased exercise capacity. These effects would be more pronounced and disabling in an already compromised heart. The operative procedure for the correction of TOF is not curative rather a palliative one. After the correction of the TOF defects, the patient has reduced activity level and exercise capacity. Cardiac rehabilitation including Interval and continuous exercise training modalities were proven to be safe and efficient in improving exercise capacity and functioning of the heart.

Molecular signaling pathways in right ventricular impairment of adult patients after tetralogy of Fallot repair.

Right ventricular impairment (RVI) secondary to altered hemodynamics contributes to morbidity and mortality in adult patients after tetralogy of Fallot (TOF) repair. The goal of this study was to describe signaling pathways contributing to right ventricular (RV) remodeling by analyzing over lifetime alterations of RV gene expression in affected patients. RV tissue was collected at the time of cardiac surgery in 13 patients with a diagnosis of TOF. RNA was isolated and whole transcriptome sequencing was performed. Gene profiles were compared between a group of 6 adults with signs of RVI undergoing right ventricle to pulmonary artery conduit surgery and a group of 7 infants, undergoing TOF correction. Definition of RVI in adult patients was based on clinical symptoms, evidence of RV hypertrophy, dilation, dysfunction or elevated pressure on echocardiographic, cardiovascular magnetic resonance, or catheterization evaluation. Median age was 34 years in RVI patients and 5 months in infants. Based on P adjusted value <0.01, RNA sequencing of RV specimens identified a total of 3,010 differentially expressed genes in adult patients with TOF and RVI as compared to infant patients with TOF. Gene Ontology and Kyoto Encyclopedia of Genes databases highlighted pathways involved in cellular metabolism, cell-cell communication, cell cycling and cellular contractility to be dysregulated in adults with corrected TOF and chronic RVI. RV transcriptome profiling in adult patients with RVI after TOF repair allows identification of signaling pathways, contributing to pathologic RV remodeling and helps in the discovery of biomarkers for disease progression and of new therapeutic targets.

COMPLETE REPAIR OF TETRALOGY OF FALLOT (TOF) ON BEATING HEART SURGERY WITHOUT AORTIC CROSS CLAMPING : REPORT OF A CASE

Introduction: Reperfusion injury is a well-known phenomenon that occurred in cardioplegic techniques with cardiopulmonary bypass. Therefore great effort is made to prevent reperfusion injury. Beating Heart Continuous Coronary Perfusion (BHCCP) surgery is one of the alternative techniques to improve an ischemic reperfusion injury in open-heart surgery, either in pediatric or in adult. This technique can be done in complete repair of tetralogy of Fallot (TOF). Purpose: To report our first experience in doing complete repair of TOF using beating heart technique without aortic cross-clamping Case Report: A five-year-old boy came on to ER on January 6th, 2012, with a history of cyanotic since birth. His echocardiography and catheterization concluded a tetralogy of Fallot with a McGoon ratio of 1.95 (the diameter of RPA and LPA were 9.14 mm and 8.79 mm, respectively). A complete repair of TOF was done using beating heart surgery without aortic cross-clamping technique. One month post-surgery evaluation, there weren't any neurological or motor disorders. Echocardiography results showed no residual pulmonary stenosis, but 0.2 mm residual VSD was found. Conclusion: This heart rate technique is an alternative method of myocardial protection in cardiac surgery. In several cardiac centres globally, this technique is relatively safe and can be used during surgery, especially in the total correction of TOF.

Fetal and maternal outcomes in patients with tetralogy of Fallot

Abstract Introduction Women with tetralogy of Fallot (TOF) have an increased risk of adverse cardiac and neonatal events during pregnancy. The aim was to assess fetal and maternal outcomes in patients (pts) with uncorrected and corrected TOF. Methods Retrospective analysis of cardiological and obstetric data in women with TOF followed at our institution. Pregnancy and neonatal outcomes were compared in patients (pts) submitted to corrective surgery vs pts with no previous intervention and in pts with or without cyanosis. Results 51 women (median age 27 years; median number of previous interventions of 2), 22% with cyanosis, who experienced 126 pregnancies resulting in 73 live births A previous corrective surgery was found in 78% (40 pts), while 4% (2 pts) had previous palliative surgery and 18% (9 pts) no previous procedure. We found that women that were not submitted to previous intervention had a significant higher incidence of preterm birth (56% vs 17.5%, p=0.029), spontaneous abortion (56% vs 10%, p=0.006) and stillbirth (22% vs 0%). No difference was found for obstetric complications between groups. Cyanotic pts also demonstrated higher incidence of low birth weight (LBW) (36% vs 7.5%, p=0.031), preterm birth (54% vs 17.5%, p=0.021), spontaneous abortion (55% vs 10%, p=0.004) and stillbirth (18% vs 0%). There were no cardiac complications during pregnancy nor evident deterioration on cardiac status. Congenital heart disease was reported in 2 infants (3% of live births). Conclusions Pregnancy is well tolerated in pts with TOF. In our population, even in uncorrected TOF and in pts with cyanosis, there were no cardiac complications during pregnancy, although those pts had worse fetal outcomes. An explanation for the low incidence of cardiac events may be less severe forms of the disease, allowing survival into adulthood without intervention. Funding Acknowledgement Type of funding sources: None.

Emergency Department Presentation and Outcome of Children With Cyanotic Congenital Heart Diseases.

ObjectiveThis study aimed to assess the outcome and see the spectrum of presenting symptoms of children with cyanotic congenital heart diseases (CHDs) admitted through the emergency department in Lady Reading Hospital (LRH) in Peshawar, Pakistan.Materials and methodsThis cross-sectional study included 104 children with cyanotic CHD admitted through the emergency department in the pediatric cardiology unit of LRH from February 2019 to January 2020. Diagnosis of cyanotic CHD was confirmed through two-dimensional echocardiography. Patients were treated according to standard protocols for their presenting symptoms. The data were analyzed using Statistical Package for the Social Sciences (SPSS), version 20.0 (IBM Corp., Armonk, NY) and frequencies were expressed as percentages.ResultsThe study included 65 male cases (62.5%) and 39 female cases (37.5%), and participant ages ranged from 10 days to 15 years (mean, 2.7 ± 3.4 years). Of 104 patients, 70 presented with cyanotic spells (84.5%), 53 with fever (10.7%), 28 with respiratory distress (26.5%), 11 with loose stools (10.5%), 10 with hemiplegia (9.6%), and eight with seizures (7.6%). Pneumonia was a precipitating factor in 13 patients (12.5%), infective endocarditis in 12 patients (11%), gastroenteritis in 11 (11%), brain abscess and cerebrovascular accidents in seven patients each (6% each), meningitis in six patients (6%), and tuberculous pericardial effusion in one patient (<1%). The primary CHD was tetralogy of Fallot (TOF) in 52 patients, tricuspid atresia in 14 patients, pulmonary atresia in 13 patients, double outlet right ventricle in 10 patients, transposition of great arteries (TGA) in seven patients, and total anomalous pulmonary venous return (TAPVR), truncus arteriosus, congenitally corrected TGA, and critical pulmonary stenosis in two patients each. Twenty-six patients received treatment of the acute problem and instructions for follow-up monitoring. Twelve patients died during their hospital stay, and three left against medical advice. Fifteen patients received patent ductus arteriosus stenting, and six received right ventricular outflow tract stenting. One patient received a Blalock-Taussig (BT) shunt stent, and one received a left pulmonary artery stent. Two patients received a balloon pulmonary valvotomy, and one received pericardial effusion drainage. Eleven patients received surgical correction of TOF, 11 received surgical correction for BT shunt, four received surgical correction for brain abscess drainage, and two received TAPVR repair.ConclusionTOF was the most common cyanotic heart disease in our study. Cyanotic spells or increased cyanosis was the most common presenting concern. Cyanotic CHDs present with variable extracardiac signs and symptoms in emergencies. Appropriate assessment, immediate management of the acute problem, and timely intervention or surgical management result in good outcomes.

Pulmonary valve replacement may not restore ventricular volume and functional status in patients with pulmonary regurgitation after late tetralogy of Fallot repair

Abstract OBJECTIVES Clinically, tetralogy of Fallot (TOF) patients who underwent repair late (older than 2 years) appears to have worse outcomes after pulmonary valve replacement than patients who underwent repair early. We proceeded to review the clinical features of late-repaired TOF patients who required pulmonary valve replacement. METHODS Fifty patients who underwent pulmonary valve replacement after TOF repair over the age of 2 years from 2000 to 2018 were retrospectively reviewed. Pre- and postoperative cardiac magnetic resonance imaging, cardiopulmonary exercise tests, and cardiac catheterization were analysed. RESULTS The median age of patients at the time of TOF repair and pulmonary valve replacement was 3.6 and 23.4 years, respectively. The median interval from TOF repair to pulmonary valve replacement was 20.5 years. Cardiac magnetic resonance imaging and cardiopulmonary exercise tests were performed at a median of 5.9 and 3.7 years after pulmonary valve replacement, respectively. Cardiac magnetic resonance revealed that there were significant changes in the indexed values of the right ventricle end-diastolic volume (164.7–106.9 ml/m2, P &amp;lt; 0.001), end-systolic volume (101.4–64.9 ml/m2, P &amp;lt; 0.001), stroke volume (66.8–48.0 ml/m2, P = 0.007) and cardiac output (5.1–3.6 l/m2, P = 0.040). Twenty-eight percentage of patients achieved normalization of the right ventricular volume after pulmonary valve replacement. In the exercise test, the maximum rate of oxygen consumption (72.5–69.5%) and oxygen pulse (95.0–83.0%) changed without statistical significance. CONCLUSIONS Although pulmonary valve replacement after late TOF correction improves right ventricular volume status, only a minority of patients achieve normalization of right ventricular end systolic volume and a normal functional status.