Corrected Tetralogy Of Fallot Research Articles

Surgical results of tetralogy of Fallot repair with pulmonary valve preservation techniques

The long-term complications arising from chronic pulmonary insufficiency after surgical correction of tetralogy of Fallot (TOF) are well documented. In our center, innovative techniques for pulmonary valve (PV) preservation have been adopted and developed for over 10 years. All patients with TOF and PV stenosis who underwent surgery at our center between January 2008 and December 2022 were included in this study. Patients were evaluated using echocardiography during follow-up, comparing those who underwent classic surgery with transannular patch (TAP) placement and those who underwent PV preservation techniques. Of the 134 patients, 99 (73.9%) were successfully treated using PV preservation techniques. At the time of surgery, the median age was 4.3 months (interquartile range [IQR] 3.4-5.9 months). The median preoperative PV z-score was -2.87 (IQR -3.61;-2.48). The median follow-up time from surgery was 6.6 years (IQR 3.5-8.4 years). During the median follow-up, the right ventricular area change fraction was 47% (IQR 43-50%) in patients treated with PV preservation and 43% (IQR 41-47%) in the TAP group (p=0.005). These data were associated with better PV function in patients treated with preservation. Specifically, insufficiency was mild in 12%, moderate in 35%, and severe in 53% of patients in the TAP group. Conversely, insufficiency was mild in 53%, moderate in 35%, and severe in 11% of patients treated with preservation (p<0.001). Annular integrity and PV function can be preserved during early TOF correction. PV preservation can prevent the long-term development of pulmonary insufficiency and right ventricular dysfunction.

Total Correction for Tetralogy of Fallot in Patients Weighing Over 10 kg: Experiences and Follow-Up Outcomes.

Background Although the recommended time for total correction of tetralogy of Fallot (TOF) is during infancy, sometimes TOF cases present to healthcare setups after pre-school age, with some cases presenting even beyond adolescence in developing countries. The objective of this study was to assess patients with TOF weighing 10 kg and above who underwent definitive corrective surgical techniques such as transannular patch (TAP), valve-sparing right ventricular outflow tract (RVOT) pericardial patch augmentation, non-ventriculotomy infundibular resection for postoperative complications, hospital stay, and right ventricular (RV) dysfunction in the immediate postoperative period and subsequent outpatient department follow-ups. Methodology This comprehensive, retrospective cohort study included single-center data collected between January 16, 2018, and January 15, 2024. The study included 63 patients diagnosed with TOF weighing 10 kg and above, ensuring a robust and representative sample. Results Of the 119 patients who underwent total correction for TOF, 63 met the study's inclusion criteria of TOF weighing above 10 kg. Of the 63 patients, 55.6% were males, and 44.4% were females.The mean weight of the study participants was 33.4 kg. The mean age was 15.9 years. Of the 63 patients, 39 underwent TAP surgery, 18 underwent RVOT patch augmentation, and six underwent total correction by non-ventriculotomy infundibular resection. There was a significant difference between the type of surgery groups and RV dysfunction, with the TAP group showing a higher incidence of RV dysfunction, indicating a potential risk factor associated with this technique. Conclusions Although TAP has significant immediate postoperative complications compared to other techniques, its long-term follow-up suggests that long-term survival and quality of life, as measured by major adverse cardiac events such as heart failure, arrhythmias, and reoperation rates, are commensurable in adulthood. This indicates that despite the initial challenges, TAP can provide satisfactory outcomes in the long run.

Modeling a Caring Approach towards Children with Fallot’s Tetralogy Repair: A Focus on the Child’s Profile

Children with Tetralogy of Fallot (TOF) continue to be on the rise in Africa. More research is needed to improve caring approach towards children with surgically corrected TOF. The consequences of surgical correction of TOF remains an important area of research interest as children with TOF repair run the high risk of increase mortality resulting from acute cardiopulmonary problems. Yet little or no research published focused on investigating which clinical or para-clinical profile of children going in for surgical correction could be the cause of morbidity or mortality amongst children with TOF repair. The aim of the study was to determine the association between the profiles of children visiting the Surgical Heart Center for consultation with Tetralogy of Fallot (TOF) on the healing process after surgical repair. It was a descriptive quantitative study in which 303 nurses were issued questionnaires. Data was collected using convenient sampling technique and analyzed using multiple corresponding analysis in SPSS software version 21.0. The results showed that the clinical symptoms, clinical history, para-clinical characteristics, and postoperative complications identified three groups of children operated on for TOF: children with unstable hemodynamics; children who usually have symptoms such as systolic thrill, oppression in chest, tachycardia, and heart murmur; and finally children with symptoms of failure to thrive and heart murmur.

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.

МНОГОЭТАПНОЕ ЛЕЧЕНИЕ ТЕТРАДЫ ФАЛЛО: ОТ ПАЛЛИАТИВНЫХ ВМЕШАТЕЛЬСТВ ДО ГИБРИДНОГО БИФУРКАЦИОННОГО Y-СТЕНТИРОВАНИЯ ЛЁГОЧНОЙ АРТЕРИИ И РАДИКАЛЬНОЙ КОРРЕКЦИИ (КЛИНИЧЕСКИЙ СЛУЧАЙ)

Primary radical correction of tetralogy of Fallot (ToF) is an optimal surgical option; however, in some conditions, in particular with concomitant extracardiac pathology and hypoplasia of the pulmonary artery (PA) branches, this intervention is not possible. Obstruction and/or hypoplasia of the PA is one of the risk factors for primary radical correction of this congenital heart defect (CHD). In world practice, there are several methods of palliative approach for PA growth with the possibility of further radical correction. However, in some cases, palliative approaches do not give the desired result. In this regard, we present a clinical case of a successful hybrid bifurcation Y-stenting of the PA and radical correction of ToF. Keywords: Tetralogy of Fallot, radical correction, branches of the pulmonary artery, hypoplasia, stenting of the branches of the pulmonary artery, bifurcation Y-stenting, clinical case.

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.