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Objective: Despite decades of experience, aspects of the management of tetralogy of Fallot with pulmonary stenosis (TOF) remain controversial. Practitioners must consider newer, evolving treatment strategies with limited data to guide decision making. Therefore, the TOF Clinical Practice Standards Committee was commissioned by the American Association for Thoracic Surgery to provide a framework on this topic, focused on timing and types of interventions, management of high-risk patients, technical considerations during interventions, and best practices for assessment of outcomes of the interventions. In addition, the group was tasked with identifying pertinent research questions for future investigations. It is recognized that variability in institutional experience could influence the application of this framework to clinical practice., Methods: The TOF Clinical Practice Standards Committee is a multinational, multidisciplinary group of cardiologists and surgeons with expertise in TOF. With the assistance of a medical librarian, a citation search in PubMed, Embase, Scopus, and Web of Science was performed using key words related to TOF and its management; the search was restricted to the English language and the year 2000 or later. Articles pertaining to pulmonary atresia, absent pulmonary valve, atrioventricular septal defects, and adult patients with TOF were excluded, as well as nonprimary sources such as review articles. This yielded nearly 20,000 results, of which 163 were included. Greater consideration was given to more recent studies, larger studies, and those using comparison groups with randomization or propensity score matching. Expert consensus statements with class of recommendation and level of evidence were developed using a modified Delphi method, requiring 80% of the member votes with 75% agreement on each statement., Results: In asymptomatic infants, complete surgical correction between age 3 and 6 months is reasonable to reduce the length of stay, rate of adverse events, and need for a transannular patch. In the majority of symptomatic neonates, both palliation and primary complete surgical correction are useful treatment options. It is reasonable to consider those with low birth weight or prematurity, small or discontinuous pulmonary arteries, chromosomal anomalies, other congenital anomalies, or other comorbidities such as intracranial hemorrhage, sepsis, or other end-organ compromise as high-risk patients. In these high-risk patients, palliation may be preferred; and, in patients with amenable anatomy, catheter-based procedures may prove favorable over surgical palliation., Conclusions: Ongoing research will provide further insight into the role of catheter-based interventions. For complete surgical correction, both transatrial and transventricular approaches are effective; however, the smallest possible ventriculotomy should be utilized. When possible, the pulmonary valve should be spared; and if unsalvageable, reconstruction can be considered. At the conclusion of the operation, adequate relief of the right ventricular outflow obstruction should be confirmed, and identification of a significant fixed anatomical obstruction should prompt further intervention. Given our current knowledge and the gaps identified, we propose several key questions to be answered by future research and potentially by a TOF registry: When to palliate or proceed with complete surgical correction, as well as the ideal type of palliation; the optimal surgical approach for complete repair for the best long-term preservation of right ventricular function; and the utility, efficacy, and durability of various pulmonary valve preservation and reconstruction techniques., (Copyright © 2022 The American Association for Thoracic Surgery. Published by Elsevier Inc. All rights reserved.)

Objectives: This study sought to compare the efficacy of ivabradine and amiodarone in the management of postoperative junctional ectopic tachycardia (JET) after cardiac surgery in children., Background: JET is a serious arrhythmia occurring in children after cardiac surgery and requires aggressive management. Amiodarone has been conventionally used in its treatment. Recent studies have reported the utility of ivabradine in this regard., Methods: In this open-label randomized controlled trial, 94 children (age ≤18 years) who developed postoperative JET were allocated to receive either amiodarone or ivabradine. The primary endpoint was restoration of normal sinus rhythm., Results: Sinus rhythm was achieved in 43 out of the 46 patients (93.5%) in the amiodarone group and 46 out of the 48 patients (95.8%) in the ivabradine group (mean difference of treatment effect: 2.3%; 95% confidence interval: -6.7% to 11.5%). The median (interquartile range) time taken to achieve sinus rhythm conversion was similar in both the groups: 21.5 (17-30.2) hours versus 22 (13.4-38.5) hours (p = 0.36)]. The time taken to rate control of JET was significantly less in the amiodarone group: median 7.0 (5.5-9.5) hours versus 8.0 (5.8-10.8) hours (p = 0.02)]. No drug-related adverse events were observed in the ivabradine group., Conclusions: Oral ivabradine is not inferior to intravenous amiodarone in converting postoperative JET to sinus rhythm. There was no difference in time taken to sinus rhythm conversion between the groups, although the rate control was earlier in patients who received amiodarone. Monotherapy with ivabradine may be considered as an alternative to amiodarone in the management of postoperative JET. (Comparison of Two Drugs, Ivabradine and Amiodarone, in the Management of Junctional Ectopic Tachycardia, an Abnormality in Cardiac Rhythm in Patients Under 18 years Who Undergo Cardiac Surgery: CTRI/2018/08/015182)., Competing Interests: Funding Support and Author Disclosures The authors have reported that they have no relationships relevant to the contents of this paper to disclose., (Copyright © 2021 American College of Cardiology Foundation. Published by Elsevier Inc. All rights reserved.)

Objectives: In this study, we aimed to determine the consequences of different amounts of leukocyte transfusion on the outcome of patients undergoing cardiac surgery., Design: This was a prospective, single-blinded cohort study conducted for 1 year from July 2018 to June 2019., Setting: The study setting was the Department of Transfusion Medicine, along with Cardiac Anaesthesia, Cardiac Surgery and Cardiac biochemistry departments in a tertiary care cardiac centre., Participants: A total of 150 patients undergoing cardiac surgery during the study period were divided into three groups (50 in each): Leukofiltered (LR), Buffy coat depleted (BCD) and Non-leukoreduced (NLR)., Intervention: The intervention was intra- and postoperative transfusion of packed red blood cells (PRBCs) having different amounts of leukocytes., Measurements and Main Results: Patient details about length of intensive care unit (ICU) and hospital stay, blood usage, inotropic drug duration, mechanical ventilation, urine output and infection were recorded from the patient data sheet, whereas patients were followed up for 30 days post-operation, and any mortality was noted. Haematological parameters and biochemical parameters for renal function test were analysed on pre- and post-surgical days 1, 3, 5 and 7, whereas on postoperative days 1 and 7, cytokine-like FAS ligands, Interleukin-10 (IL-10) and Interferon-γ (INF-γ) were tested. Patients in all three groups received an average of four, two and two units of packed red blood cells, platelets and fresh frozen plasma, respectively. There was a statistically significant (P < .05) rise in total leukocyte, neutrophil and lymphocyte count in all three groups from day 0 to day 3, but it reduced to preoperative level on day 5. There was shorter ICU and hospital stay in the LR group of patients (46 ± 19.9 hours and 7.5 ± 2.4 days) compared to NLR (52.1 ± 24.2 hours and 7.9 ± 4.1 days) and BCD (53.3 ± 26.7 hours and 8.8 ± 3.1 days) group of patients, but it was statistically non-significant. The duration of mechanical ventilation was significantly lesser in LR group patients (10.2 ± 6.2 hours) as compared to NLR group (14.7 ± 12.7 hours). On risk ratio calculation of developing postoperative kidney injury, the NLR group had 1.3 and 2.6 times more risk compared to the BCD and LR groups, respectively. On postoperative days 1 and 7, FAS-L levels significantly increased in all three group of patients, whereas IL-10 increased in the NLR and BCD groups and decreased in the LR group non-significantly. The INF-γ levels decreased on day 1 in the NLR and BCD groups but increased in the LR group, but it was inversed on day 7., Conclusion: Depletion of leukocytes decreased Transfusion Related Immunomodulation (TRIM) effects in patients undergoing cardiac surgery, but this also depends on the degree of leukoreduction. As found in our study, leukofiltration is more effective compared to buffy-coat depletion only., (© 2020 British Blood Transfusion Society.)

The coronavirus disease 2019 (COVID-19) pandemic currently gripping the globe is impacting the entire health care system with rapidly escalating morbidities and mortality. Although the infectious risk to the pediatric population appears low, the effects on children with congenital heart disease (CHD) remain poorly understood. The closure of congenital heart surgery programs worldwide to address the growing number of infected individuals could have an unintended impact on future health for COVID-19-negative patients with CHD. Pediatric and congenital heart surgeons, given their small numbers and close relationships, are uniquely positioned to collectively assess the impact of the pandemic on surgical practice and care of children with CHD. We present the results of an international survey sent to pediatric and congenital heart surgeons characterizing the early impact of COVID-19 on the care of patients with CHD.

Background: Various patch materials to close large atrial septal defects (ASDs) are autologous pericardium or a large of prosthetic patches which may be associated with infrequent but definite problems. We describe our experience with the use of the right atrial free wall patch over the last two decades., Methods and Results: Between July 1998 and December 2017, 157 patients (mean age 14.7 ± 13.9 years), underwent ASD closure using the right atrial free wall patch. Associated lesions were severe mitral regurgitation (n = 24), partial anomalous pulmonary venous drainage (n = 15), ASD closure occurring after myxoma excision (n = 12) total anomalous pulmonary venous drainage (n = 2) and tricuspid regurgitation (n = 2). Surgery was uneventful in all patients. All patients underwent serial electrocardiography and echocardiography. Follow-up in 140 of 155 survivors was 103.6 ± 0.6 months. One hundred forty of one hundred fifty-five survivors are in sinus rhythm and three have persistent atrial fibrillation. Twenty-four hours of Holter monitoring (n = 19) revealed normal sinus rhythm in all but three patients, with occasional atrial ectopics in one patient; four were lost to follow-op. Electrophysiological studies in seven consenting patients, 9 to 16 months following ASD closure showed normal atrial potentials from the site of the patch., Conclusions: The autologous right atrial free wall is a safe patch material for ASD closure. Its advantages are that it is autologous, endothelialized, probably viable, and carries a low risk of thromboembolism. Studies with a larger number of patients with longer follow are needed to further confirm these findings., (© 2020 Wiley Periodicals LLC.)

The commonly used technique to facilitate intracardiac exposure during transatrial repair of tetralogy of fallot involves considerable retraction of the tricuspid valve using retractors. We describe an alternative surgical technique in which it is possible to dispense away with the retractors. The advantages of such a technique are discussed., (© 2019 Wiley Periodicals, Inc.)

The World Database for Pediatric and Congenital Heart Surgery was created to provide a resource for centers to be able to perform complex outcomes analyses of children undergoing repair of a congenital heart defect. In just under two years, the World Society for Pediatric and Congenital Heart Surgery (WSPCHS) has amassed over 13,000 procedures from 55 centers into the database. This Proceedings of the 3rd World Database Symposium held at the 6th Scientific Meeting of the WSPCHS summarizes the presentations of international experts in the fields of outcomes analysis and care of children with congenital heart surgery.

Introduction: A number of guidelines are available for management of congenital heart diseases from infancy to adult life. However, these guidelines are for patients living in high-income countries. Separate guidelines, applicable to Indian children, are required when recommending an intervention for congenital heart diseases, as often these patients present late in the course of the disease and may have co-existing morbidities and malnutrition., Process: Guidelines emerged following expert deliberations at the National Consensus Meeting on Management of Congenital Heart Diseases in India, held on the 10th and 11th of August, 2018 at the All India Institute of Medical Sciences., Objectives: The aim of the study was to frame evidence-based guidelines for (i) indications and optimal timing of intervention in common congenital heart diseases and (ii) follow-up protocols for patients who have undergone cardiac surgery/catheter interventions for congenital heart diseases., Recommendations: Evidence-based recommendations are provided for indications and timing of intervention in common congenital heart diseases, including left-to-right shunts, obstructive lesions, and cyanotic congenital heart diseases. In addition, protocols for follow-up of postsurgical patients are also described., (Copyright © 2019 Cardiological Society of India. Published by Elsevier B.V. All rights reserved.)

A combination of ruptured sinus of Valsalva (RSOV) aneurysm with tetralogy of Fallot (TOF) is a very rare condition. We report a 32-year-old male who underwent repair of a RSOV aneurysm with TOF., (© 2018 Wiley Periodicals, Inc.)

Background: Decreases in serum total thyroxin and total triiodothyronine occurs after cardiopulmonary bypass, and is reflected as poor immediate outcome. We studied effects of oral thyroxin supplementation in infants who underwent open-heart surgery., Methods: In this prospective study, 100 patients were randomized into 2 groups: 50 in the thyroxin group (TH) and 50 in the placebo group (PL). Patients in the TH group received oral thyroxin (5 μg/kg) 12 hours before surgery and once daily for the remainder of their intensive care unit (ICU) stay. Data on intraoperative and postoperative variables were recorded. Cardiac index (CI) was measured. Perioperative serum thyroid hormone levels and serum interleukin-6 and tumor necrosis factor-α were measured. Secondary analysis was performed by dividing patients into simple and complex subcategories., Results: Results of the primary analysis indicated a higher CI in the TH compared with the PL. In the complex category, the mean duration of mechanical ventilation was 3.85 ± 0.93 and 4.66 ± 1.55 days in the TH and PL, respectively (P = .001). Mean ICU stay was 6.79 ± 2.26 and 8.33 ± 3.09 days (P = .03), and mean hospital stay was 15.70 ± 4.77 and 18.90 ± 4.48 days (P = .01) in the TH and PL, respectively. There were no significant differences between the TH and the PL in the simple category. CI was higher in the TH at all time points (P = .004). The average therapeutic intervention scoring system scores for the first 2 days were higher in the PL in the complex category., Conclusions: Oral thyroxin supplementation improves the CI and reduces the inotropic requirement. In addition, it reduces the duration of mechanical ventilation, ICU and hospital stay, and therapeutic intervention scoring system in infants after surgery for complex congenital heart defects., (Copyright © 2018 The American Association for Thoracic Surgery. Published by Elsevier Inc. All rights reserved.)

The case of an 8-year-old boy with an aortopulmonary window who underwent unidirectional valved patch closure of the window is described. The advantages of unidirectional valved patch closure in this setting are discussed.

Background: Postoperative arrhythmias are a known complication after cardiac surgical repairs for congenital heart disease., Methods: Data were reviewed pertaining to incidence, diagnosis, potential risk factors, and management of postoperative arrhythmias in 369 consecutive patients under 18 years of age, undergoing elective open heart surgery. All children were admitted to the intensive care unit and continuous electrocardiographic monitoring was performed. Patient factors such as Aristotle Basic Complexity Score, total surgical duration, hypotension, tachycardia, serum lactate level, and inotropic score were analyzed. Univariate analysis was done to assess associations between these factors and the occurrence of postoperative arrhythmias., Results: Twenty-five (6.7%) patients developed arrhythmias. Junctional ectopic tachycardia (JET) was the most common arrhythmia occurring in 15 (60%) patients, followed by supraventricular tachycardia in 3 (12%), ventricular premature contractions in 3 (12%), hemodynamically unstable ventricular tachycardia and fibrillation in 3 (12%), and atrial fibrillation in 1 (4%) patient. Different grades of heart block were noted in 13 patients. Aristotle score (P = .014), total surgical duration (P < .01), hypotension (P = .02), heart rate (beats per minute) (P = .001), serum lactate level (P = .04), and inotropic score (P = .02) in the early postoperative period were associated with arrhythmia occurrence. Surgeries for ventricular septal defect alone or in association with other diseases including tetralogy of Fallot (TOF) and transposition of the great arteries (TGA) were found to be associated with higher risk of arrhythmias., Conclusion: This study showed a low incidence of arrhythmias, JET being the commonest, seen more in TOF repair and these could be treated efficiently. Higher Aristotle score, longer surgical time, hypotension, tachycardia, high inotropic score, and high serum lactate levels were associated with the occurrence of arrhythmias postoperatively.

Objective: To determine effects of allopurinol administration on outcomes following intracardiac repair of tetralogy of Fallot (TOF)., Materials and Methods: Fifty patients undergoing TOF repair were randomized to 2 groups of 25 each: the allopurinol group (n = 25) and the placebo group (n = 25). Postoperatively, inotropic score, rhythm, duration of mechanical ventilation, cardiac output, intensive care unit (ICU) stay, and hospital stay were assessed. Plasma troponin-I, superoxide dismutase (SOD), interleukin (IL) 1-ß, IL-6, and malondialdehyde were measured serially., Results: Inotropic score was lower in the allopurinol compared with placebo group (11.04 ± 5.70 vs 17.50 ± 7.83; P = .02). Duration of ICU and hospital stay was lower in the allopurinol group. Plasma levels of SOD preoperative were (2.87 ± 1.21 U/mL vs 4.5 ± 2.08 U/mL; P = .012), immediately following release of crossclamp (2.32 ± 0.98 U/mL vs 5.32 ± 2.81 U/mL; P < .001), and after termination of CPB (2.18 ± 1.0.78 U/mL vs 3.44 ± 1.99 U/mL; P = .003) between the placebo versus allopurinol group, respectively. Postoperative levels of IL1-ß and IL-6 were lower in the allopurinol group. Malondialdehyde levels following CPB were lower in the allopurinol group (11.80 ± 2.94 pg/mL in the placebo vs 9.16 ± 3.02 g/mL in the allopurinol group; P < .001)., Conclusions: Allopurinol administration in patients undergoing intracardiac repair of TOF is associated with reduced inotropic scores, duration of mechanical ventilation, ICU stay, and hospital stay and favorable biochemical markers of inflammation. Further studies in multiple setups are needed before recommending it as a routine practice., (Copyright © 2017 The American Association for Thoracic Surgery. Published by Elsevier Inc. All rights reserved.)

Objectives: To study the anatomic and haemodynamic data and results of surgery in patients undergoing surgical repair of aortopulmonary window beyond infancy., Methods: Between July 2005 and December 2015, 23 patients, older than 1 year undergoing surgery for aortopulmonary window were analysed retrospectively. Postoperative clinical and echocardiography follow-up were performed., Results: Median age and weight at repair was 4 years (range 14 months-12 years) and 12 kg (range 3.5-22 kg), respectively. Fifteen patients had Richardson's Type I, 6 patients had Type II and 2 patients had Type III aortopulmonary window. Six patients had associated defects. Baseline mean systolic pulmonary artery pressure was 101 ± 14.9 mmHg (range 80-130, median 100 mmHg) and pulmonary vascular resistance index was 9.6 ± 5.9 (median 7.7 Wood units/m2, range 3.7-23.5 Wood units/m2). Patch repair of aortopulmonary window was performed using the sandwich method (transwindow) (n = 15), transaortic (n = 3) and transpulmonary artery (n = 2) approaches; 2 patients underwent double ligation and 1 underwent division and suturing. Two patients underwent valved patch closure of aortopulmonary window and 1 patient underwent valved patch closure of associated ventricular septal defect. There were 2 in-hospital deaths: one due to intractable pulmonary hypertension and the other due to low cardiac output. Mean follow-up was 36 months (range 2-119 months). Eighteen patients were in NYHA Class I at last follow-up. There were no late deaths or reoperation., Conclusions: Surgery can be safely undertaken beyond infancy in carefully selected patients of aortopulmonary window with acceptable early and mid-term outcomes., (© The Author 2017. Published by Oxford University Press on behalf of the European Association for Cardio-Thoracic Surgery. All rights reserved.)

Background: Tetralogy of Fallot and absent pulmonary valve (TOF/APV) is associated with significant pulmonary artery dilatation and airway compression., Methods: We performed a retrospective review of 73 consecutive patients who underwent repair for TOF/APV between January 2005-August 2015. Mean age was 6.4±5.6years (28days-22years). The right ventricular outflow tract (RVOT) was reconstructed using varied techniques. Freedom from RVOT gradients and re-operation was studied., Results: There were four (5.5%) early deaths, two each in infants and older children. Median ICU stay was 2days (range, 1-12days). Mean ICU stay for, infants, children and adults, was 6.5±6.04, 2.75±2.45, and 2.33±1.03days, respectively (p=0.0762). Median hospital stay was 6days (range, 4 to 15days). Mean hospital stay for, infants and children and adults was 7±2, 6.75±2.39, and 6.33±1.63days, respectively (p=0.325). Mean follow up was 65±36.6months (median 56 months, range 7-126 months). On follow up echocardiography, 14 (21.21%) had no pulmonary regurgitation. 21 (31.81%) had mild PR patients, 8 (12.12%) moderate PR and 19 (28.78%) had severe PR. There were five (7.5%) reoperations. Five and ten-year survival was 95%±2.12 and 92.3%±3.45 respectively. Freedom from RVOT reoperation was 93±2.62% and 89±3.87% at 5 and 10 years., Conclusions: In contrast to children and adults with TOF/APV, infants carry significant early mortality. But the mid-term outcome for patients who survive the initial repair of TOF/APV is acceptable. However, these patients require constant surveillance and irrespective of the methods of RVOT management, the reoperation rates are expected to be high as more of these patients survive into adulthood., (Copyright © 2017. Published by Elsevier B.V.)

An atrial septectomy is often required to create or enlarge a pre-existing restrictive atrial septal defect in patients with univentricular hearts undergoing the bidirectional superior cavopulmonary anastomosis. We describe an alternative surgical technique through the transected cardiac end of the superior vena cava without a right atriotomy successfully performed in 26 patients., (© 2017 Wiley Periodicals, Inc.)

Transesophageal echocardiography can be a useful adjunct in assessing the quality of repair in patients undergoing novel methods of reconstruction of the right ventricular outflow. We present one such patient here.

Objective: Aortopulmonary window (APW) is a rare congenital heart defect. We reviewed our experience with this condition over the last two decades., Methods: Between September 1993 and December 2013, 62 patients underwent surgery for APW. Depending on the associated lesions, they were divided into two groups: Simple (Group 1) or complex (Group 2). In the complex group, six patients had a ventricular septal defect, five patients had interrupted aortic arch, three patients had tetralogy of Fallot, two patients had double outlet right ventricle, and one patient had the right pulmonary artery arising from the ascending aorta., Results: Mean age at repair was 21.6 ± 32.02 months (median = 6, range 0.1-144 months). By preoperative echocardiographic assessment 27 out of 62 patients had severe pulmonary artery hypertension (52% of the cohort). Patch repair of APW was performed using the sandwich method (transwindow) (n = 27; 43.5%), transaortic (n = 18; 29%), and transpulmonary artery (n = 5; 8.1%) approaches; 10 patients (16.1%) underwent double ligation and two (3.2%) underwent division and suturing. Overall hospital mortality in group 1 was 6.97% (3/43) and in group 2 it was 21% (4/19), p = 0.085. Mean hospital stay in group 1 was 6.9 ± 2.4 days (median = 7 days) and in group 2 was 12 ± 6.1 days (median = 13 days), p = 0.0001. Follow-up in group 1 was 1.6-9.8 years (median = 6 years); in group 2, it was 1.8-8.9 years (median = 6.5 years). There were no late deaths. Two patients needed reintervention for distortion of the right pulmonary artery origin. All patients were in New York Heart Association Class I/II at last follow up., Conclusion: There are multiple acceptable surgical strategies for the treatment of aortopulmonary window. Despite a relatively advanced age and substantial number of patients with severe pulmonary hypertension the outcomes can still be good. Associated anomalies complicate the repair. Patients in the complex group had a protracted hospital course and a higher early mortality but similar late survival., (© 2017 Wiley Periodicals, Inc.)

Background and Objectives: Adequate nutritional supplementation in infants with cardiac malformations after surgical repair is a challenge. Critically ill infants in the early postoperative period are in a catabolic stress. The mismatch between estimated energy requirement (EER) and the intake in the postoperative period is multifactorial, predisposing them to complications such as immune deficiency, more infection, and growth failure. This study aimed to assess the feasibility and efficacy of enriched breast milk feed on postoperative recovery and growth of infants after open heart surgery., Methodology: Fifty infants <6 months of age were prospectively randomized in the trial for enteral nutrition (EN) postoperatively from day 1 to 10, after obtaining the Institute Ethics Committee's approval. They were equally divided into two groups on the basis of the feed they received: Control group was fed with expressed breast milk (EBM; 0.65 kcal/ml) and intervention group was fed with EBM + energy supplementation/fortification with human milk fortifier (7.5 kcal/2 g)/Simyl medium-chain triglyceride oil (7.8 kcal/ml). Energy need for each infant was calculated as per EER at 90 kcal/kg/day, as the target requirement. The intra- and post-operative variables such as cardiopulmonary bypass and aortic cross-clamp times, ventilation duration, Intensive Care Unit (ICU), and hospital length of stay and mortality were recorded. Anthropometric and hematological parameters and infection control data were recorded in a predesigned pro forma. Data were analyzed using Stata 14.1 software., Results: The duration of mechanical ventilation, length of ICU stay (LOIS), length of hospital stay (LOHS), infection rate, and mortality rate were lower in the intervention group compared to the control group although none of the differences were statistically significant. Infants in control group needed mechanical ventilation for about a day more (i.e., 153.6 ± 149.0 h vs. 123.2 ± 107.0 h; P = 0.20) than those in the intervention group. Similarly, infants in control group stayed for longer duration in the ICU (13.2 ± 8.9 days) and hospital (16.5 ± 9.8 days) as compared to the intervention group (11.0 ± 6.1 days; 14.1 ± 7.0 days) (P = 0.14 and 0.17, respectively). The LOIS and LOHS were decreased by 2.2 and 2.4 days, respectively, in the intervention group compared to control group. The infection rate (3/25; 5/25) and mortality rate (1/25; 2/25) were lower in the intervention group than those in the control group. The energy intake in the intervention group was 40 kcal more (i.e., 127.2 ± 56.1 kcal vs. 87.1 ± 38.3 kcal) than the control group on the 10th postoperative day., Conclusions: Early enteral/oral feeding after cardiac surgery is feasible and recommended. In addition, enriching the EBM is helpful in achieving the maximum possible calorie intake in the postoperative period. EN therapy might help in providing adequate nutrition, and it decreases ventilation duration, infection rate, LOIS, LOHS, and mortality.

We present a case with left isomerism, interruption of the inferior caval vein, anomalous systemic venous drainage with partially anomalous pulmonary venous drainage, and a sinus venosus type of atrial septal defect in the absence of a right superior caval vein. This report is of interest because of a rare combination of these anomalies, which was accurately diagnosed and successfully repaired., (© The Author(s) 2016.)

A 30-month-old female was admitted with recurrent spells and severe cyanosis. Preoperative echocardiography was diagnostic of tetralogy of Fallot with an atrial septal defect of the primum type, unroofed coronary sinus, and a left superior vena cava draining into the left atrium. At surgery the patient was found to have a complete atrioventricular septal defect in addition to these anomalies. Complete anatomical correction was achieved through the right atrial approach., (© 2015 Wiley Periodicals, Inc.)

Background: This prospective study proposed to determine the incidence, risk factors, and management protocols for early postoperative arrhythmias after pediatric cardiac surgery, with focus on outcomes, using a uniform protocol, and also to see if children operated on at a later age have different issues from those operated on earlier., Methods: Of 224 consecutive pediatric patients undergoing cardiac surgery from September 2013 to July 2014, 24 were excluded because their procedures were performed without cardiopulmonary bypass., Results: The median age was 24 months (mean 50.1 ± 62.4 months, range 0.5-216 months). Fifteen (7.5%) patients developed arrhythmia, the most common was junctional ectopic tachycardia (n = 7, 46.6%) followed by supraventricular tachycardia (n = 5, 33.3%). All junctional ectopic tachycardias occurred within 24 h of intensive care unit admission. Of the 7 patients with junctional ectopic tachycardia, 5 responded to conventional measures and 2 required amiodarone infusion. There was a significant longer cardiopulmonary bypass time in patients with arrhythmias compared to those without arrhythmias., Conclusion: We observed a very low incidence of arrhythmias, particularly junctional ectopic tachycardia, after open heart surgery in children. Other than a longer cardiopulmonary bypass time, no specific predictors were identified. It appears that the cause of arrhythmias following pediatric cardiac surgery is multifactorial and needs further study with a greater number of patients., (© The Author(s) 2015.)

Background: Multiple ventricular septal defects (VSDs) are difficult to close. In this report, we describe a simple and safe technique of closing multiple muscular and apical VSDs., Methods: Between January 2010 and December 2013, 52 patients with a muscular VSD either in isolation or in association with other congenital heart disease underwent surgery using this technique in which a black silk thread was passed through the suspected VSD opening into the left ventricle and brought out through the mitral valve and the interatrial septum. Sutures were placed around the silk thread to close the VSD., Results: Out of 52 patients, 34 were male; age ranged from 45 days to 5.5 years. In addition to the large subaortic/lage muscular VSD, three additional VSDs were present in 34 patients, two additional VSDs were present in 13 patients, and five patients had "Swiss Cheese septum." The VSDs were mid-muscular in 35 patients and were apical in 17 patients. Eleven patients had associated complex lesions. Intraoperative transesophageal echocardiograms did not reveal any significant residual shunt in any of these patients. There was no step-up on oximetry. There was one hospital death due to sepsis. Follow-up was available in 48 patients; three patients were lost to follow-up. At follow-up, no patient had a residual VSD., Conclusion: The biventricular approach through trans-right atrial, trans-interatrial septum using a thread through the hole method for closing multiple muscular VSD is effective with no persistent residual defects., (© 2015 Wiley Periodicals, Inc.)

Objective: The purpose of the present study was to study the midterm hemodynamic outcomes of unidirectional valved patch closure of ventricular septal defects (VSDs) in patients with VSD and pulmonary arterial hypertension (PAH)., Methods: From January 2006 to January 2012, 20 patients with VSD with PAH and a pulmonary vascular resistance index >8 Wood units underwent VSD closure with a unidirectional valved patch using the technique previously described by us. Of these, 13 patients agreed to follow-up cardiac catheterization and were studied at a mean follow-up of 34.7 ± 18.6 months (range, 2-56). The mean age of these 13 patients was 8.5 ± 4.4 years (range, 2-19; median, 9), and the mean preoperative systemic saturation was 94.1% ± 3.4% (range, 87-99; median, 95.0) The mean preoperative pulmonary artery systolic pressure was 96.2 ± 13.6 mm Hg (range, 75-115; median, 103.0), and the mean preoperative pulmonary vascular resistance index was 10.0 ± 2.1 Wood units (range, 8.0-15.1; median, 9.3)., Results: At follow-up cardiac catheterization, the mean systemic saturation had increased to 98.92%. The pulmonary vascular resistance index had decreased significantly to 5.8 ± 2.1 Wood units (P = .02). A significant decrease was seen in the pulmonary artery systolic, diastolic, and mean pressures (P = .000), and none of the patients had severe PAH. No patients died, and all patients were in New York Heart Association class I., Conclusions: Unidirectional valved patch closure of VSD is a promising technique for patients with a large VSD and severe PAH. It had a favorable effect on the immediate, early, and midterm clinical outcomes and hemodynamic parameters., (Copyright © 2014 The American Association for Thoracic Surgery. Published by Elsevier Inc. All rights reserved.)

Objective: Patients with tetralogy of Fallot (TOF) undergoing surgery in adulthood represent a challenge. We report our experience with such patients in or beyond the fourth decade of life., Design: Retrospective cohort., Setting: Multispeciality tertiary level referral center, Patients: Forty-one (age 30-52 years) with TOF undergoing surgery between January 2002 and March 2013. The hospital records of these patients were analyzed., Interventions: None., Outcome Measures: Early and late morbidity and mortality, duration of mechanical ventilatory support, inotropic score, intensive care unit and hospital stay, and correlation with various parameters., Results: Significant aortopulmonary collaterals were present in 28 patients; these were occluded in cardiac catheterization laboratory prior to repair. Median intraoperative right:left ventricular pressure ratio was 0.40 (range 0.2-0.8). Median inotropic score was 10 (range 5-30). Median duration of mechanical ventilation was 12 hours (range 6-48 hours). Preoperative oxygen saturation was negatively correlated with inotropic score (P = .001, r = -0.485), mechanical ventilatory support (P = .003, r = -0.460), intensive care unit stay (P = .004, r = -0.442), and hospital stay (P = .028, r = -353). Inotropic score was higher in patients with aortopulmonary collaterals (n = 28, P = .03), high preoperative hematocrit (n = 29, P = .029), and with right ventricular dysfunction (n = 6, P = .05). Patients with right ventricular outflow tract gradient >80 mm Hg (n = 19) had prolonged hospital stay (P = .002). Patients undergoing pure transatrial repair (n = 24) showed lower inotropic score (P = .045), less intensive care unit (P = .04), and hospital stay (P = .031). There were two early and two late deaths (one from trauma and one from unknown etiology). Median follow-up was 42 months. Thirty-one patients were in New York Heart Association class II and six were in class III., Conclusion: Repair of TOF in and beyond the fourth decade of life is feasible with acceptable results. Patients with high hematocrit, lower oxygen saturation, right ventricular dysfunction, aortopulmonary collaterals, and high preoperative right ventricular outflow tract gradients have a prolonged postoperative course., (© 2014 Wiley Periodicals, Inc.)

Objectives: Tetralogy of Fallot (TOF) with hemitruncus (HT) is a rare entity. In this report, we present our experience with this condition over the last 20 years., Methods: Between January 1994 and June 2013, 11 patients with HT and TOF underwent surgery at the All India Institute of Medical Sciences, New Delhi, India. All available clinical, radiographic, echocardiographic, cardiac catheterization, operative and follow-up data were reviewed., Results: The mean age was 73 ± 7.1 months (range 7 months to 18 years) and the mean weight was 15.7 ± 1.2 kg. The mean preoperative saturation was 79.3 ± 11.7% (range 62-92%). Six patients had anomalous left pulmonary artery (PA), whereas 5 had an anomalous right PA arising from the aorta. Surgical procedures consisted of complete intracardiac repair of TOF with direct implantation of the anomalous PA into the main PA (n = 7), intracardiac repair of TOF with an interposition saphenous vein graft between the right PA and main PA (n = 1), and reconstruction of the left PA with autologous pericardium with intracardiac repair of TOF (n = 1), direct implantation of the anomalous PA into the main PA with an innominate to right pulmonary artery shunt (n = 1) and a right PA banding with innominate to left PA shunt (n = 1). There were two early deaths. Follow-up ranged from 3 to 73 months. All survivors are in NYHA Class I and follow-up echocardiograms did not show any residual lesions., Conclusions: Surgical repair of HT with TOF results in acceptable early outcomes. The surgical strategy needs to be individualized to the anatomy of the patient., (© The Author 2014. Published by Oxford University Press on behalf of the European Association for Cardio-Thoracic Surgery. All rights reserved.)

A simplified technique to fix the commissural pillar of the pulmonary valve at the time of right ventricular outflow tract reconstruction during the arterial switch operation is presented., (Copyright © 2013 Australian and New Zealand Society of Cardiac and Thoracic Surgeons (ANZSCTS) and the Cardiac Society of Australia and New Zealand (CSANZ). Published by Elsevier B.V. All rights reserved.)

Since the anterior translocation of the pulmonary arteries in relation to the aorta (Lecompte maneuver) was first described in 1981, its indications have continued to expand. In this review, we discuss the physiological basis and the expanding indications for this maneuver.

Background: The study aimed to analyze the outcome of congenital heart surgery in a subset of Indian patients, using the Aristotle Basic Complexity score, the Risk Adjustment for Congenital Heart Surgery categories, and the Society of Thoracic Surgeons and European Association for Cardiothoracic Surgery mortality categories., Patients and Methods: 1312 patients <18 years of age undergoing congenital heart surgery were assigned the 3 scores and studied for outcome indices of difficulty (cardiopulmonary bypass time or duration of surgery >120 min), morbidity (intensive care unit stay >7 days), and mortality., Results: The overall mortality was 6.85%, with mean a Aristotle Basic Complexity score, Risk Adjustment for Congenital Heart Surgery category, and Society of Thoracic Surgeons and European Association for Cardiothoracic Surgery mortality category of 7.17 ± 2.04, 2.28 ± 0.78, and 2.24 ± 1.06, respectively. The mortality predictive capacity of the Risk Adjustment for Congenital Heart Surgery category (c = 0.76) was similar to that of the Society of Thoracic Surgeons and European Association for Cardiothoracic Surgery mortality category (c = 0.75); both were better compared to the Aristotle Basic Complexity score (c = 0.66). The Risk Adjustment for Congenital Heart Surgery category and Aristotle Basic Complexity score correlated with morbidity and difficulty outcomes., Conclusion: The study shows that the Aristotle Basic Complexity score, the Risk Adjustment for Congenital Heart Surgery category, and the Society of Thoracic Surgeons and European Association for Cardiothoracic Surgery mortality category are tools of case mix stratification to analyze congenital heart surgery outcomes in a subset of the Indian population.

We describe the technical aspects of performing an atrial switch operation in a 4-year-old boy with d-transposition of the great arteries and partial anomalous drainage of the left-sided pulmonary veins. The rarity of the condition is discussed.

We report a 3-year-old patient with tetralogy of Fallot (TOF), dextrocardia, situs inversus (SI), and total anomalous pulmonary venous return. This combination of anomalies is extremely unusual. The management is discussed and the literature is reviewed., (© 2013 Wiley Periodicals, Inc.)

We report an adult patient presenting with polycythemia and stroke, three decades following surgical closure of an atrial septal defect. The avoidance, diagnosis, and management of this complication are discussed., (© 2012 Wiley Periodicals, Inc.)

Background: There is a paucity of data about morphology, surgical procedure, and results in older patients with congenitally corrected transposition of great arteries (ccTGAs)., Patients and Methods: Between January 2002 and August 2012, 15 patients (7 males), median age 25 years, range 16 to 41 years underwent surgery for ccTGA. Associated lesions were tricuspid regurgitation (TR; n = 5) and ventricular septal defect (VSD) with pulmonary stenosis (PS; n = 10). Surgical procedures included tricuspid valve replacement (n = 4), tricuspid valve repair (n = 1), lateral tunnel Fontan (n = 2), extracardiac Fontan (n = 2), Kawashima procedure (n = 1), bidirectional (BD) Glenn (n = 2), Senning + Rastelli procedure (n = 1), and VSD closure + left ventricle to pulmonary artery conduit (n = 1). The details of these procedures and outcomes were analyzed., Results: There were no early or late deaths. Mean follow-up period was 49.9 ± 26 months. All patients who underwent tricuspid valve replacement are in New York Heart Association (NYHA) class I, with no progression of right ventricular (RV) dysfunction. One patient who underwent tricuspid valve repair is in NYHA class III and has progressed to severe RV dysfunction. None of the patients undergoing single ventricle palliation had any complications related to the surgery. Both patients who underwent anatomical and physiological biventricular (BV) repair had no complications., Conclusions: Older patients with ccTGA present a challenge. Fontan/BD Glenn remains a good option for patients who presented with VSD PS. Both anatomic and physiological BV repairs provide acceptable results. Tricuspid valve replacement is safe for patients presenting with TR who have improvement in functional class, though the right ventricular function may not improve.

We report the case of a 12-year-old patient with truncus arteriosus (common arterial trunk) who underwent successful surgical repair. The ventricular septal defect was closed using a unidirectional valved patch to act as a safeguard in the event of postoperative pulmonary hypertension and right ventricular decompensation.

Objective: To study the effect of cardiopulmonary bypass (CPB) on serum thyroid hormone profile in children undergoing open-heart surgery., Design: Prospective cross-sectional study., Setting: Multispecialty tertiary level referral center., Patients: One hundred consecutive patients (age 15.9 ± 14.6 months, weight 6.7 ± 2.5 kg) undergoing open-heart surgery under CPB., Interventions: None., Outcome Measures: Levels and trends of serum total thyroxine (TT4), free thyroxine (FT4), total tri-iodothyronine (TT3), free tri-iodothyronine (FT3) and thyroid stimulating hormone (TSH), survival, inotropic score, duration of mechanical ventilation, postoperative complications., Results: TT4 levels were 9.08 ± 3.6, 6.4 ± 2.5, 6.24 ± 2.1, 6.43 ± 2.4, 7.20 ± 3.0 µg/dL at baseline and at 1, 24, 48 and 72 hours; FT4 levels were 1.82 ± 0.5, 1.49 ± 0.3, 1.29 ± 0.3, 1.32 ± 0.4, and 1.43 ± 0.5 ng/dL; TT3 levels were 1.81 ± 0.4, 1.31 ± 0.3, 0.99 ± 0.2, 1.0 ± 0.37, and 1.17 ± 0.48 ng/ml; FT3 levels were 4.09 ± 1.0, 3.02 ± 0.8, 2.21 ± 0.6, 2.22 ± 0.7, and 2.66 ± 1.05 pg/ml; TSH levels were 5.40 ± 3.8, 2.0 ± 3.1, 1.24 ± 1.1, 2.90 ± 3.3, and 4.03 ± 3.4 mIU/L. There was significant fall (29.1% for FT4, 32.1% for TT4, 77% for TSH, 46% for FT3 and 45% for TT3, p < 0.0001). When area under curve (AUC) TT4 was compared between survivors (n = 87) and nonsurvivors (n = 12), significantly larger AUC was seen in survivors (492.81 ± 158.6) than nonsurvivors (360.75 ± 179.6 p = 0.0125). In survivors >72 hours, AUC TT4 was larger in patients with uneventful postoperative course versus those with postoperative complications (516.48 ± 18.6 vs. 394.78 ± 29.9, p = 0.001). AUC TT4 showed significant inverse correlation with inotropic score and borderline inverse correlation with duration of mechanical ventilation., Conclusion: Children undergoing surgery under CPB showed significant fall in thyroid hormones. Because TT4 level is modifiable, prophylactic administration of TT4 for improving outcomes needs to be studied further., (© 2012 Wiley Periodicals, Inc.)

Delayed presentation of ventricular septal defect (VSD) is common in developing countries. Such patients often have severe pulmonary arterial hypertension (PAH), which increases post-operative morbidity and mortality. To address these problems, we used our technique of unidirectional valved patch (UVP) for closure of VSD. Between January 2006 and December 2010, 17 patients (age 2-23 years, median 9 years) with a large VSD and severe PAH underwent VSD closure with UVP. Pre-operative mean indexed pulmonary vascular resistance (PVRI) was 10.9 ± 2.2 Wood units and mean pre-operative systemic saturation was 93.4 ± 2.6%. Shunt was bidirectional in 15 patients and predominantly right to left in two. After VSD closure, intra-operative transoesophageal echocardiography revealed a right to left shunt across the patch in three patients 2, 7 and 9 years of age who had pre-operative PVRI of 9.5, 9.8 and 11.1 Wood units, respectively. There were no in-hospital deaths and all patients had uneventful recovery. Mean follow-up was 30 ± 14.7 months and all patients are well without cyanosis. Echocardiography showed no shunt across the patch and all have systemic saturation >95%. We conclude that UVP is a promising technique in patients with large VSD and severe PAH.

Postoperative bleeding is a common complication after pediatric cardiac surgery. Use of recombinant activated factor VII for intractable hemorrhage after cardiac, pediatric, and neurosurgery has been shown to decrease postoperative bleeding, but data in children are limited. This retrospective study analyzed 20 children <15 years-old who underwent cardiac surgery and received recombinant activated factor VII for refractory postoperative hemorrhage. All patients underwent mediastinal reexploration before recombinant activated factor VII was administered as a bolus dose over 2-3 min as rescue therapy. If no significant decrease in chest tube drainage was observed, the dose was repeated after an interval of at least 2 h. The median dose of recombinant activated factor VII administered per bleeding episode was 83.33 μg·kg(-1) (range, 72.47-87.50 μg·kg(-1)), and the dose per patient was 154.16 μg·kg(-1) (range, 93.06-180.52 μg·kg(-1)). The median number of doses found to be effective in these children was 1.76. There were significant decreases in mediastinal chest tube drainage and the volume of packed red blood cells, platelet concentrates, and cryoprecipitate administered after recombinant activated factor VII. No complications were observed during the therapy.

Two patients with aortic right ventricular tunnel and anomalous origin of the right coronary artery from the tunnel are reported. The literature on this condition is reviewed. , (© 2011 Wiley Periodicals, Inc.)

We report our experience with a five-year-old child with d-transposition of great arteries (d-TGA), ventricular septal defect (VSD), and severe pulmonary arterial hypertension (PAH). A fenestrated unidirectional-valved patch was used to close the VSD and a standard arterial witch operation (ASO) was performed. Difficulties in assessment of operability and the choice of procedures in such patients are briefly discussed., (© 2010 Wiley Periodicals, Inc.)

We present a modified technique for replacement of the pulmonary valve with a pulmonary homograft. The principles of this technique are similar to the "scalloped subcoronary technique" for aortic valve replacement with a homograft. The advantages of using this technique in the pulmonary position are briefly discussed., (© 2010 Wiley Periodicals, Inc.)

Objective: Our objective was to assess the morbidity and mortality in children requiring extracorporeal membrane oxygenator support after cardiac surgery and to determine factors influencing outcome., Methods: Between January 2003 and June 2008, 58 patients required extracorporeal membrane oxygenator support after cardiac surgery. A retrospective study was performed and factors influencing outcome were determined by logistic regression modeling with the probability of outcome based on a combination of multivariate predictors., Results: Median age and weight were 12 days and 3.3 kg, respectively. Thirty-one patients had single ventricle repair and 27 had biventricular repair. Median duration of support with the oxygenator was 6 days. Thirty-nine (67%) patients were successfully weaned off the support, but only 24 (41%) survived to hospital discharge. Chief complications were renal failure (31%), neurologic complications (29%), and sepsis (16%). Multivariable logistic regression analysis identified 10 days or more of extracorporeal membrane oxygenation (odds ratio = 6.1), urine output less than 2 mL x kg(-1) x h(-1) in first 24 hours (odds ratio = 15), renal failure (odds ratio = 9.4), and pH less than 7.35 after 24 hours of extracorporeal membrane oxygenation (odds ratio = 82) as significant independent factors associated with failure to wean off extracorporeal membrane oxygenation. Factors associated with failure of hospital discharge despite successful decannulation were as follows: extracorporeal membrane oxygenator support time of 10 days or more, red blood cell transfusion of greater than 1000 mL/kg during the entire period of oxygenator support, and sepsis. Patients with single ventricle repair were at higher risk of hospital mortality., Conclusion: Longer duration of extracorporeal membrane oxygenator support, low pH and urine output in the first 24 hours, and renal failure are significant factors associated with mortality during extracorporeal membrane oxygenator support. Exposure to high amounts of blood transfusion during extracorporeal oxygenation, extended extracorporeal membrane oxygenator support, and sepsis increase risk of death after successful decannulation., (Copyright 2010. Published by Mosby, Inc.)

We report a 26-year-old patient with d-transposition of great arteries, intact ventricular septum, and an atrial septal defect. The relevant literature on late natural survivors with this condition is reviewed and the technical aspects of an alternative technique for accomplishing a successful atrial switch in this situation are discussed.

This report describes the uses of a large Eustachian valve as a cardiovascular patch. In one patient, the large Eustachian valve was used to close an atrial septal defect and in another patient it was used to create a tunnel during the lateral tunnel Fontan operation.

The objective of this study was to perform an audit of the use of homologous blood and blood products in patients undergoing open-heart surgery by a single surgical team that follows an in-house protocol for blood conservation. The hospital records of 310 consecutive patients (age >15 years) undergoing open-heart surgery over a period of 8 months were retrospectively reviewed to assess the comprehensive blood conservation protocol. Homologous blood and blood product usage during and after surgery, in the intensive care unit and up to hospital discharge was analyzed. Two hundred and fifty-six patients (82.6%) did not receive any blood or blood products. Only 54 patients (17.4%) received one or more units of allogenic transfusion either intraoperatively or postoperatively until discharge. Mean hemoglobin at discharge was 9.8 Grams% (8.9-12 Grams%). A standardized multidisciplinary approach to blood conservation in cardiac surgery decreases bleeding and transfusion requirements in a safe and cost effective manner.

Objective: Anatomic repair is being actively evaluated as the preferred option for congenitally corrected transposition of the great arteries. We present our 13-year experience with this approach., Methods: Between May 1994 and September 2007, 68 patients with congenitally corrected transposition of the great arteries underwent anatomic repair. Thirty-one patients (group 1, mean age of 94.8 +/- 42.3 months) underwent a combined Rastelli and atrial switch operation. Thirty-seven patients (group 2, mean age of 36.1 +/- 46.9 months) underwent an arterial switch operation and atrial rerouting. Eight patients in group 2 had an intact ventricular septum., Results: Group 1 had 5 early deaths (17%) but no late deaths. Three patients underwent conduit revision at a mean follow-up of 62 months. Group 2 had 5 early deaths (13.5%). There were 4 late reoperations (2 pulmonary baffle revisions, 1 mitral valve replacement, and 1 permanent pacemaker implantation) and 4 late deaths (1 secondary to progressive left ventricular dysfunction, 2 secondary to uncontrolled atrial tachyarrhythmia, and 1 secondary to pulmonary hypertension and right ventricular failure). In group 2, 4 patients have a left ventricular ejection fraction less than 40%, 5 patients have moderate aortic incompetence, 5 patients have symptomatic tricuspid incompetence, 1 patient has tricuspid stenosis, 1 patient has superior cava obstruction, and 3 patients are receiving antiarrhythmic therapy., Conclusion: The occurrence of left ventricular dysfunction indicate that anatomic repair in the arterial switch group is still fraught with imperfections. The Rastelli group required conduit revisions but has otherwise performed well.

We report the technical aspects of the arterial switch operation as we performed it in a 5-month-old infant who had situs inversus, dextrocardia, transposition of the great arteries, and inverted origin of the circumflex and right coronary arteries. The successful performance of this procedure required the use of a conduit-lengthening technique due to the much longer distance to the proposed site of coronary artery transfer.