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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: Hepatic derangements and liver injury following the Fontan operation (FO) may progress in some cases to hepatic fibrosis (HF) and cirrhosis. The respective roles of transient elastography (TE) or FibroScan, shear wave elastography (SWE), and aspartate transaminase to platelet ratio index (APRI) in assessing liver stiffness (LS) and possible liver injury in these patients are unclear., Methods: Thirty-nine patients (31 males), mean age 11.8 ± 5.7 years, median 11 years (interquartile range: 7-14.7 years), undergoing the FO between November 2017 and December 2018 were included. Liver stiffness and HF assessment was done with TE, SWE, and APRI in the preoperative period and postoperatively at 3-, 6-, and 12-month interval., Results: The LS values increased over time (postoperative values at 3, 6, and 12 months) as compared to preoperative values by both FibroScan and SWE. The change in liver stiffness measurement (LSM) was statistically significant: LSM0 to LSM3 ( P ≤ .0001), LSM0 to LSM6 ( P ≤ .0001), and LSM0 to LSM12 ( P = .001). Similarly, significant changes were observed on SWE: SWE0 to SWE3 ( P ≤ .0001), SWE0 to SWE6 ( P ≤ .0001), and SWE0 to SWE12 ( P = .001). There was no significant change in the APRI values over time., Conclusion: Noninvasive methods such as FibroScan and SWE may be of use to assess LS in follow-up of patients undergoing the FO for early recognition of hepatic changes.

We report the case of a six-year-old patient who underwent an extracardiac Fontan operation including bilateral bidirectional superior cavopulmonary anastomosis and direct inferior vena cava to main pulmonary artery connection that was performed without cardiopulmonary bypass.

Justification: 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 10th and 11th of August 2018 at the All India Institute of Medical Sciences, New Delhi. The meeting was supported by Children's HeartLink, a non-governmental organization based in Minnesota, USA., Objectives: To frame evidence based guidelines for (i) indications and optimal timing of intervention in common congenital heart diseases; (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 (atrial septal defect, ventricular septal defect, atrioventricular septal defect, patent ductus arteriosus and others), obstructive lesions (pulmonary stenosis, aortic stenosis and coarctation of aorta) and cyanotic congenital heart diseases (tetralogy of Fallot, transposition of great arteries, univentricular hearts, total anomalous pulmonary venous connection, Ebstein anomaly and others). In addition, protocols for follow-up of post surgical patients are also described, disease wise.

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.)

Objective: To determine the safety and efficacy of bivalirudin as an anticoagulant for pediatric open heart surgery (OHS) and to determine its appropriate dosage for this purpose., Design: Prospective, randomized controlled trial., Setting: Tertiary care hospital., Participants: Fifty acyanotic children aged 1-12 years undergoing OHS., Interventions: The children were randomized to receive either 4 mg/kg of heparin (n = 25, group H) or 1 mg/kg of bivalirudin bolus followed by 2.5 mg/kg/h infusion (n = 25, group B) as the anticoagulant. The doses were adjusted to maintain activated clotting time (ACT) above 480 seconds. At the conclusion of surgery, protamine (1.3 mg/100 U of heparin) was administered to children in group H., Measurements and Main Results: The children were comparable in both groups with regard to demographic characteristics. The mean age and weight were 51.5 months and 13.4 kg in group H, and 59.3 months and 13.4 kg in group B. The dose of anticoagulant required was 4.0 ± 0.2 mg/kg in group H and 1.7 ± 0.2 mg/kg followed by 3.0 ± 0.7 mg/kg/h infusion in group B (p < 0.001). One child in group H required an additional dose compared to 13 (54.2%) children in group B. Intraoperatively, the ACT achieved was higher in group H compared to group B (p < 0.05). The ACT returned to baseline value after protamine administration in group H, but it remained elevated for 2 hours after termination of cardiopulmonary bypass (CPB) in group B (p < 0.01). The ACT was higher in group B compared to group H for 6 hours after termination of CPB (p < 0.05). Heparin prolonged the onset of clotting, decreased the rate and strength of thrombus formation, and inhibited platelet function to a greater extent than bivalirudin on viscoelastic coagulation testing. The total duration of surgery was prolonged in group B. The postoperative chest tube drainage was similar in group B (4.9 mL/kg) as in group H (5.9 mL/kg) in spite of higher ACT. The transfusion requirements were similar. No adverse event occurred in any patient., Conclusion: Bivalirudin is a safe and effective anticoagulant for pediatric OHS. Though it is not suitable as a routine anticoagulant for this purpose, it may be used as a heparin alternative in instances when heparin cannot be used. The dose required to maintain ACT for more than 480 seconds was 1.7 ± 0.2 mg/kg followed by 3.0 ± 0.7 mg/kg/h infusion. The ACT remained elevated for 2 hours after stopping the infusion. Bivalirudin did not increase postoperative bleeding and transfusion requirement., (Copyright © 2018 Elsevier Inc. All rights reserved.)

Objectives: The Fontan procedure [total cavopulmonary connection (TCPC)] is the final palliation for patients with univentricular physiology. We studied differential perfusion ratio and percentage uptake of a radiotracer in different zones of each lung following TCPC., Methods: Between July 2015 and June 2017, 45 patients underwent 99mTc macroaggregated albumin lung perfusion scan at a mean follow-up period of 49.3 ± SD 26.1 days following TCPC. Differential perfusion ratio and percentage uptake of the radiotracer in the upper, middle and lower zones of each lung were calculated., Results: Post-foot injection [inferior vena cava (IVC) injection], preferential flow to the lungs was as follows: left lung (n = 13, 30.2%), right lung (n = 13, 30.2%) and uniformly to both lungs (n= 17, 39.6%). Post-arm injection [superior vena cava (SVC) injection], preferential flow to the lungs was as follows: left lung (n = 13, 30.2%), right lung (n = 22, 51.2%) and uniformly to both lungs (n= 8, 18.6%). The middle zone was perfused the most in both lungs. Total lower zone mean perfusion was higher than the upper zone following both SVC injection and IVC injection (34.1 ± SD 5.3% vs 17. ± SD 4.1% and 33 ± SD 5.0% vs 17.5 ± SD 4.1%, respectively). In patients with bilateral SVC, post-IVC injection, 6 (75%) patients had preferential flow to the right lung, whereas post-SVC injection, preferential flow to the left lung was visualized in 7 (87.5%) patients., Conclusions: Following TCPC, IVC blood was distributed uniformly in both lungs. SVC blood preferentially perfused the right lung. The middle zone was perfused the most in both lungs.

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.

Objectives: Studies on older patients undergoing primary Fontan operation (FO) are limited, with conflicting results. We review our experience with these patients beyond the first decade of life., Patients and Methods: Between January 2000 and December 2014, a total of 105 patients ≥10 years of age (mean 15.6 ± 4.9, range 10-31, median 15 years) underwent primary FO without a prior bidirectional superior cavopulmonary anastomosis (Bidirectional Glenn [BDG]). Mean preoperative New York Heart Association (NYHA) class was 2.2 ± 0.57., Results: Operative procedure was extra-cardiac FO in 62 patients (8 were fenestrated). Forty-three had a lateral tunnel FO (26 were fenestrated). There were 11 (10.5%) early deaths. Fourteen of the 94 early survivors experienced prolonged pleural effusions, 7 had arrhythmias, and 2 had thromboembolic events. Two patients underwent Fontan takedown. On univariate analysis, NYHA functional class III, mean pulmonary artery (PA) pressure ≥15 mm Hg, hematocrit ≥60%, preoperative ventricular dysfunction, and atrioventricular valve regurgitation (AVVR) were associated with early mortality. Median follow-up was 78 (mean 88.9 ± 6.3) months. In 94 survivors, 6 (6.4%) late deaths were encountered. At last follow-up, 81 (86.2%) survivors were in NYHA class I. Actuarial survival was 84.7% ± 3.7% at 5, 10, and 15 years., Conclusion: Carefully selected adolescents and young adults can safely undergo the primary FO. However, persistent pleural effusions, arrhythmias, thromboembolic events, and the need for reoperation mandate regular follow-up in such patients. Preoperative NYHA functional class III, mean PA pressure ≧ 15 mm Hg, hematocrit ≥ 60%, ventricular dysfunction, and AVVR were associated with early mortality, suggesting that primary FO should be avoided in such patients.

Objective: To analyze the intraoperative and early results of the bidirectional Glenn (BDG) procedure performed on cardiopulmonary bypass (CPB) using three different techniques., Methods: Between September 2013 and June 2015, 75 consecutive patients (mean age 42 ± 34.4 months) undergoing BDG were randomly assigned to either technique I: open anastomosis or technique II: superior vena cava (SVC) cannulation or technique III: intermittent SVC clamping. We monitored the cerebral near infrared spectrophotometry (NIRS), SVC pressure, CPB time, intensive care unit (ICU) stay, and neurocognitive function., Results: Patients in technique III had abnormal lower NIRS values during the procedure (57 ± 7.4) compared to techniques I and II (64 ± 7.5 and 61 ± 8.0, P = 0.01). Postoperative SVC pressure in technique III was higher than other two groups (17.6 ± 3.7 mmHg vs. 14.2 ± 3.5 mmHg and 15.3 ± 2.0 mmHg in techniques I and II, respectively = 0.0008). CPB time was highest in technique II (44 ± 18 min) compared to techniques I and III (29 ± 14 min and 38 ± 16 min, P = 0.006), respectively. ICU stay was longer in technique III (30 ± 15 h) compared to the other two techniques (22 ± 8.5 h and 27 ± 8.3 h in techniques I and II, respectively = 0.04). No patient experienced significant neurocognitive dysfunction., Conclusion: All techniques of BDG provided acceptable results. The open technique was faster and its use in smaller children merits consideration. The technique of intermittent clamping should be used as a last resort., (© 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.)

Objective: Persistent pleural effusions are a major source of morbidity after univentricular repair. These are often refractory to conventional conservative therapy. We adopted a strategy of diaphragmatic fenestration (DF) in such patients and report the results., Methods: Between January 2002 and 2014, we performed DF in 12 patients using an original technique that was first described by us. The medical records of all these patients were studied. Preoperative characteristics, amount and duration of effusions, and time to removal of chest tubes following DF were studied., Results: Mean age was 101 ± 57.9 months (range: 38-180 months), and mean body weight was 18.8 ± 5.8 kg (range: 11-28 kg). Five had a bidirectional Glenn, four had lateral tunnel Fontan, and three had an extracardiac Fontan as initial procedure. The average pleural drainage prior to DF was 352.5 ± 152 mL/24 h (18.75 mL/kg/24 h) for a median period of 33 days (bidirectional Glenn 216 ± 85 mL/24 h [16.5 mL/kg/24 h] for 30 days and total cavopulmonary connection 450 ± 104 mL/24 h [22.5 mL/kg/24 h] for 36 days). All patients underwent DF. Additionally, five patients underwent thoracic duct ligation on the left side. Postoperative chest drainage after DF was 25 mL/d for a median of 4 days, and the chest tubes could be removed in a median of 5.5 days (mean 7 days). There were no complications related to DF., Conclusions: In patients with persistent pleural effusions following univentricular palliation, DF is an attractive option when conventional therapies have failed. This original technique of DF is simple, reproducible, cost-effective, and free of any known complications., (© The Author(s) 2016.)

Objective: The adjustable pulmonary artery band (APAB) has been demonstrated by us earlier to be superior to the conventional pulmonary artery banding (CPAB), in terms of reduced early morbidity and mortality. In this study, we assessed the adequacy of the band and its complications over the mid-term., Methods: Between 2002 and 2012, 73 patients underwent adjustable PAB, and their operative and follow-up data were collected and analyzed., Results: There was one early death following the APAB. Follow-up data were available for 57 patients of which 44 patients (61.7%) underwent definitive repair, 10 were awaiting definitive repair, and 3 patients were kept on medical follow-up because of inadequate fall in pulmonary artery (PA) pressures. 14 patients (19%) were lost to follow-up. Major PA distortion or stenosis was absent in the majority. 1 patient had pseudoaneurysm of the main pulmonary artery (MPA) with sternal sinus infection and required surgical reconstruction. 1 patient had infective endocarditis of the pulmonary valve managed medically. Band migration was not encountered. There were two deaths after definitive repair and one after APAB., Conclusions: Patients undergoing APAB fulfilled the desired objectives of the pulmonary artery banding (PAB) with minimum PA complications in the mid-term. This added to the early postoperative benefits, makes the APAB an attractive alternative to the CPAB., (Copyright © 2015 Cardiological Society of India. Published by Elsevier B.V. All rights reserved.)

Objective: Patients who have undergone a previous bidirectional superior cavopulmonary anastomosis and have hypoxemia and anatomy considered unsuitable for the Fontan completion present a difficult subset. We performed common carotid artery-to-internal jugular vein (CCA-IJV) shunt in these patients., Methods: Between January 2010 and January 2015, eight patients underwent a CCA-IJV shunt. Their records were analyzed retrospectively., Results: There were no early deaths. The baseline arterial saturation prior to the shunt procedure was median 67% (range 60%-72%), which increased to median 83% (range 80%-90%) after the procedure. The preoperative arterial oxygen partial pressure (Pao 2) ranged from 30 to 49 mm Hg (median 40 mm Hg). The hematocrit dropped from a preoperative median of 65% (range 55%-72%) to a median of 45% (range 42%-48%) after the procedure. Median intensive care unit stay was 2.5 days, and the median hospital stay was 7 days. Median follow-up was 24 months. At the last follow-up, these patients were classified in New York Heart Association class II., Conclusions: In a selected group of patients who are considered to be unsuitable for completion of Fontan circulation following superior cavopulmonary anastomosis, creation of a CCA-IJV shunt may be helpful to improve saturations and improve ventricular function., (© The Author(s) 2015.)

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.)

Objective: The aim was to compare various pre-and post-operative parameters and to identify the predictors of mortality in neonates, infants, and older children undergoing Modified Blalock Taussig shunt (MBTS)., Materials and Methods: Medical records of 134 children who underwent MBTS over a period of 2 years through thoracotomy were reviewed. Children were divided into three groups-neonates, infants, and older children. For analysis, various pre-and post-operative variables were recorded, including complications and mortality., Results: The increase in PaO 2 and SaO 2 levels after surgery was similar and statistically significant in all the three groups. The requirement of adrenaline, duration of ventilation and mortality was significantly higher in neonates. The overall mortality and infant mortality was 4.5% and 8%, respectively., Conclusion: Neonates are at increased risk of complications and mortality compared with older children. Age (<30 days), weight (<3 kg), packed red blood cells transfusion >6 ml/kg, mechanical ventilation >24 h and post shunt increase in PaO 2 (P Diff) <25% of baseline PaO 2 are independent predictors of mortality in children undergoing MBTS.

A best evidence topic in cardiac surgery was written according to a structured protocol. The question addressed was 'whether conventional pulmonary artery banding (PAB) or adjustable PAB might be the superior intervention?' Using the PubMed search, 51 papers were found, of which seven papers represented the best evidence to answer the clinical question. We included only those papers that actually compared conventional PAB with adjustable PAB, and excluded those that studied only one of these interventions. Four studies qualified (one prospective and three retrospective) and analysed data in human patients, while three were experimental studies in animals. The end points in the prospective human study were death, debanding and follow-up to intracardiac repair. The three retrospective studies compared the incidence of early deaths, inotropic support, need for mechanical ventilatory support, reoperations and intensive care unit and hospital stay. Out of the four studies in humans, three studies noted a significant reduction in early deaths from 23 to 1.8%, 77 to 0% and 15 to 0% in conventional vs adjustable PAB. Need for early reoperations reduced from 18 to 3.5% and from 35 to 0% in 2 studies. Similarly, there was a reduction in the ventilatory times and the intensive care unit and hospital stay. The three experimental animal studies demonstrated that a much more reliable preparation of the ventricle was achieved with the use of an adjustable PAB. The results of all the seven studies led us to conclude that adjustable PAB provides superior early outcomes; reduces early mortality, need for inotropes and need for reintervention; and provides equivalent or superior band gradients when compared to conventional PAB. The use of the adjustable PAB was found to result in significant haemodynamic improvement by progressively reducing the pulmonary artery pressures and left-to-right shunt. The adjustable PAB was found to improve early survival and also made delayed repair feasible in a better clinical state, with reduced mortality and morbidity., (© The Author 2014. Published by Oxford University Press on behalf of the European Association for Cardio-Thoracic Surgery. All rights reserved.)

Objective: Altered cardiac autonomic control may play a role in the long-term outcome of patients undergoing univentricular heart repair. This study was undertaken to compare bidirectional superior cavopulmonary anastomosis with preserved antegrade pulmonary blood flow and total cavopulmonary connection, with regard to their effects on cardiac autonomic activity, as measured by heart rate variability indices, prior to and early after surgery., Methods: This prospective study included 46 patients (27 with bidirectional superior cavopulmonary anastomosis and 19 with total cavopulmonary connection. Heart rate variability was measured preoperatively and at 2 and 9 months postoperatively. The heart rate variability was measured by a 900-s electrocardiogram recording. Comparisons were drawn between and within groups, using standard statistical methods., Results: All heart rate variability parameters were comparable in the 2 groups preoperatively. At the first follow-up, all heart rate variability parameters had decreased in both groups, but the decreases were not statistically significant. Between-group comparisons showed significantly higher parasympathetic and lower sympathetic tone in the bidirectional superior cavopulmonary anastomosis group. At the second follow-up, the bidirectional superior cavopulmonary anastomosis group had a significant increase in overall cardiac autonomic tone, and the total cavopulmonary connection group had a significant increase in parasympathetic tone, compared to the first follow-up. Between-group comparisons showed higher cardiac autonomic tone in the bidirectional superior cavopulmonary anastomosis group., Conclusion: Total cavopulmonary connection leads to a significant reduction in overall cardiac autonomic tone, compared to bidirectional superior cavopulmonary anastomosis with antegrade pulmonary blood flow.

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.

A best evidence topic in cardiac surgery was written according to a structured protocol. The question addressed was: is sternotomy approach superior to a thoracotomy approach for a modified Blalock-Taussig shunt procedure? More than 58 papers were found using the search as described below, of which 11 papers represented the best evidence to answer the clinical question. The authors, journal, date and country of publication, patient group studied, study type, relevant outcomes and results of these papers are tabulated. Three of seven papers compared the sternotomy and thoracotomy approaches. The operative approach was a significant predictor of shunt failure. The criterion used to define early shunt failure was either the complete occlusion during hospitalization or the need to return to the operating room for a second shunt. The studies that compared the thoracotomy and sternotomy approaches observed increased shunt failure rates in the thoracotomy group. The sternotomy approach was associated with advantages like less pulmonary artery distortion, ease of technical performance, cosmetic advantage of a single sternotomy incision, ease of ligation of patent ductus, less phrenic nerve injury, less collateral formation in chest wall adhesions and less thoracotomy induced scoliosis. However, other papers studied either the sternotomy approach only or the thoracotomy approach and drew conclusions regarding risk factors for operative morbidity and mortality. We conclude that the sternotomy approach is beneficial to neonates and infants undergoing modified Blalock-Taussig shunt when compared with the conventional thoracotomy approach.

Objective: To compare cardiopulmonary exercise capacity of patients undergoing primary total cavopulmonary connection (TCPC) with those undergoing TCPC after a prior bidirectional Glenn (BDG)., Methods: Out of 42 patients in this study, 24 had undergone primary TCPC while 18 had undergone staged TCPC. The peak oxygen consumption (O(2) peak), O(2) pulse, chronotropic index, and other exercise parameters were studied., Results: There were no significant differences in O(2) peak (940 ± 538 vs. 1010 ± 417 mL/min, p=0.42) and O(2) pulse (5.76 ± 3.52 vs. 6.85 ± 2.95) of patients undergoing staged TCPC versus primary TCPC, respectively; however, chronotropic index (0.43 ± 0.23 vs. 0.30 ± 017, p=0.047) was significantly different. Exercise tolerance was the same in fenestrated versus nonfenestrated TCPC groups and age at TCPC less than or more than 7 years. However, O(2) peak and O(2) pulse of patients with extracardiac TCPC was better than patients with lateral tunnel TCPC (p values 0.05, 0.04 and respectively). Some parameters of exercise tolerance of patients with antegrade pulmonary blood flow (APBF) interrupted at the time of BDG were better than those with APBF open., Conclusion: There were no differences in the exercise parameters of patients undergoing a staged versus a primary TCPC, fenestrated versus nonfenestrated TCPC, and age at surgery less than or more than 7 years. Exercise parameters were better in the extracardiac conduit group versus lateral tunnel TCPC groups. Patients who had a TCPC after prior interruption of APBF had better exercise parameters.

Objective: We compared the early outcomes of patients undergoing extracardiac total cavopulmonary connection (TCPC) with or without cardiopulmonary bypass (CPB)., Methods: Between February 2012 and February 2013, 27 patients undergoing TCPC without CPB (off-pump group) were compared with matched 27 patients undergoing TCPC on CPB (on-pump group). Outcome parameters studied were inotropic score, time to extubation, intensive care unit (ICU) stay, first 12 hours mediastinal drainage in ICU, average pleural drainage, time to removal of chest tubes, total hospital stay, and saturation at discharge., Results: There was one early death in each group. No patient required conversion from off CPB to CPB. The inotropic score (6.1 ± 5.91 vs 10.1 ± 6.80, P = .03), time to extubation (8.7 ± 6.95 vs 10.31 ± 8.69 hours, P = .03), first 12 hours mediastinal drainage in ICU (611.9 ± 341.4 vs 922.2 ± 145.6 mL, P = .03), and ICU stay (1.6 ± 0.58 vs 2.9 ± 1.37 days, P = .001) were significantly less in the off-pump group when compared to the on-pump group, and saturation at discharge (99.7 ± 0.60 vs 98.6 ± 2.13, P = .026) was higher in the off-pump group. However, the average daily pleural drainage (125 ± 61.72 vs 150 ± 103.4 mL, P = .7), time to removal of chest tubes (12.69 ± 7.1 vs 15.44 ± 19.26 days, P = .45), and the total hospital stay (14.23 ± 7.4 vs 18.89 ± 19.9 days, P = .22) were no different. There were substantial savings in costs in patients undergoing off-pump TCPC (P = .016)., Conclusions: The TCPC without CPB is easy to perform, is cost-effective, and is associated with superior early postoperative outcomes as compared to TCPC on CPB. With appropriate modifications, this operation can be performed in almost all morphological subsets of patients who do not need an associated intracardiac procedure.

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.

Superior cavopulmonary anastomosis and total cavopulmonary anastomosis are the procedures of choice for the management of patients with a functionally univentricular heart. We review the various indications, sites, advantages, and complications of a systemic to pulmonary artery shunt after the creation of superior cavopulmonary anastomosis. Systemic pulmonary artery shunt may be useful as a palliative strategy in patients who have hypoxemia and completion of total cavopulmonary anastomosis is not feasible., (© 2013 Wiley Periodicals, Inc.)

Objective: To compare external and internal jugular venous pressures to monitor pulmonary artery pressure during the Fontan procedure., Background: During the Fontan procedure, the internal jugular vein is used to assess pulmonary artery pressure. The risk of venous thrombosis in the low-pressure Fontan circuit may compromise the long-term success of cavopulmonary anastomosis. This study compared external and internal jugular venous pressures, femoral venous pressure, and actual pulmonary artery pressure during the Fontan procedure., Method: 32 children undergoing a single-stage Fontan on cardiopulmonary bypass were monitored for mean external and internal jugular venous pressures and femoral venous pressure pre- and post-cardiopulmonary bypass. Pulmonary artery pressure was also recorded directly after cardiopulmonary bypass. The pressure data were analyzed using analysis of variance, and the coefficient of correlation was found., Results: The differences between external jugular, internal jugular, and femoral venous pressure pre-cardiopulmonary bypass, and between the 3 venous pressures and the pulmonary artery pressure after cardiopulmonary bypass were found to be non-significant. The coefficient of correlation of external jugular venous pressure and internal jugular venous pressure was 0.8163 (p < 0.0001) pre-cardiopulmonary bypass, and 0.6465 (p = 0.0001) post-cardiopulmonary bypass., Conclusion: The external jugular venous pressure correlates well with both internal jugular venous pressure and femoral venous pressure as a marker of pulmonary artery pressure in children undergoing the Fontan procedure. The use of external jugular venous pressure may also preclude the risk of thrombosis.

Background: This study aimed to compare preoperative baseline Sonoclot variables between acyanotic and cyanotic congenital heart disease patients., Methods: 100 patients aged from infancy to 9-years were studied. Fifty patients each from the cyanotic and acyanotic groups were studied for Sonoclot parameters preoperatively. After inhalational anesthetic induction, blood was collected from the arterial line and analyzed in a Sonoclot analyzer to measure Sonoclot activated coagulation time, clot rate, and platelet function., Results: Sonoclot activated coagulation time was normal in both groups, but lower in the cyanotic group (127.95 ± 51.4 s) than the acyanotic group (147.85 ± 45.48 s; p = 0.54). Both groups had abnormal clot rates and platelet function. The clot rate was significantly lower in the cyanotic group (19.31 ± 10.68 U·min(-1)) than acyanotic group (24.88 ± 9.23 U·min(-1); p = 0.009). Platelet function was deranged in 31% of patients (cyanotic, 59%; acyanotic, 8%; p <0.001). Platelet function was the most severely affected baseline parameter., Conclusions: Pediatric acyanotic and cyanotic congenital heart disease patients have deranged coagulation parameters as assessed by the Sonoclot analyzer. The abnormality is more marked in cyanotic patients. Platelet function is the most severely affected parameter. These baseline parameters in conjunction with post-bypass parameters for an individual patient will help in the formulation of specific blood component transfusion guidelines.

In the present report, we discuss a patient who developed persistent hypoxemia after an attempt at Fontan completion failed. As a bail-out procedure, a left common carotid artery to left internal jugular vein shunt was constructed, after which the hypoxemia was relieved., (Copyright © 2012 The Society of Thoracic Surgeons. Published by Elsevier Inc. All rights reserved.)

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.)

The anomalous origin of one pulmonary artery branch from the aorta (AOPA) is rare. We report our single-institution surgical experience with this condition. Between January 1994 and February 2011, 17 patients (age: 1 month-25 years) with AOPA underwent surgery at our institute. Thirteen patients had an anomalous origin of the right pulmonary artery (RPA) while four had an anomalous origin of the left pulmonary artery (LPA) from the aorta. In patients with anomalous RPA, 11 patients had the proximal type and two patients had the distal type of AOPA. Four patients had associated Tetralogy of Fallot (TOF). In 14 patients, direct implantation into the main pulmonary artery was performed, while three patients required interpositon of a graft. There was one operative death due to persistent hypoxia in a 7-month old child with TOF and an anomalous LPA from the aorta. At a median follow-up of 36.5 months (range: 2-192 months), all 16 survivors were asymptomatic. On echocardiography, two patients showed a gradient of 25 and 30 mmHg across the anastomosis and are being followed up. In our experience, early repair of AOPA results in acceptable haemodynamic and anatomic results. Long-term survival can be expected with a low incidence of re-operation or re-intervention.

Objective: The objective of this study was to assess the effectiveness of 0.5% ropivacaine used for parasternal intercostal blocks for postoperative analgesia in pediatric patients undergoing cardiac surgery., Design: A randomized, controlled, prospective, double-blind study., Setting: A tertiary care teaching hospital., Participants: Thirty children scheduled for cardiac surgery with a median sternotomy., Interventions: A 0.5% ropivacaine injection with 5 doses of 0.5 to 2.0 mL on each side in the 2nd to 6th parasternal intercostal space with a total dose of ropivacaine below 5 mg/kg or the same volume of saline before sternal wound closure., Measurements and Main Results: The time to extubation was significantly lower in patients administered the parasternal blocks with ropivacaine than in the control group; the mean values were 2.66 hours and 5.31 hours, respectively (p < 0.001). The pain scores were lower in the ropivacaine group compared with the saline group; mean values were 2.20 for the ropivacaine group and 4.83 for the saline group on a scale of 10. The cumulative fentanyl dose requirement over a 24-hour period was higher in the saline group than the ropivacaine group (p < 0.001)., Conclusions: Parasternal blocks with ropivacaine appear to be a simple, safe, and useful technique of supplementation of postoperative analgesia in pediatric patients undergoing cardiac surgery with a median sternotomy., (Copyright © 2012 Elsevier Inc. All rights reserved.)

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.)

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 studied the anatomic characteristics and results of surgery in 27 patients with total anomalous pulmonary venous drainage who were 15 years or older between January 1997 and July 2007. Mean age was 19.7+/-11.6 years (15-48 years). The anatomic subtypes were supracardiac (n=15), cardiac (n=7), and mixed (n=5). Fourteen patients were in NYHA class II and 13 were in NYHA class III. Eleven patients had severe and the rest had moderate pulmonary arterial hypertension; six patients had significant right ventricular dysfunction. All patients underwent complete repair. A small inter-atrial communication was left open in four patients and in two patients, a fenestrated unidirectional valved patch was used to close the atrial septal defect. There were no early or late deaths. Follow-up was 3-127 months (mean 61.2+/-36.1 months). Twenty patients were in NYHA class I and seven were in class II. Echocardiography showed normal right and left ventricular function in all patients with reduction of pulmonary arterial pressures in 26 patients. One patient underwent radiofrequency ablation for new onset atrial flutter. Surgery can be safely undertaken in a few naturally selected group of patients with total anomalous pulmonary venous drainage who survive beyond childhood.

A large number of patients in developing countries require surgical correction of congenitally malformed hearts. Unfortunately, only a limited number of centres offer these patients surgery at an affordable cost. In this review, we discuss the problems in managing these patients, with an emphasis on reduction of costs, so that the maximum number of patients can benefit. It is apparent that containing costs requires a multipronged approach, which begins with timely referral, and continues with early surgical correction and adequate postoperative care carried out in a scientific manner. Indigenization, innovation, training of manpower, and building a team, are essential to cut the costs, and to improve the quality of care.

Background: Conventional pulmonary artery banding (CPAB) is associated with high morbidity and mortality. We studied the changes in outcome with the use of an adjustable pulmonary artery band (APAB)., Methods: Between June 2001 and June 2006, 147 patients underwent PAB: 91 underwent CPAB and 56 underwent APAB., Results: The clinical profile of patients was similar in both groups. Inotropic drugs were used in 91 (100%) patients in the CPAB group and in 12 (21%) in the APAB group (p < 0.001). Early band related reoperation was required in 17 patients in the CPAB group compared with 2 patients in the APAB group (p = 0.014). There were 21 (23%) early deaths in CPAB group compared with 1 (1.8%) in the APAB group (p < 0.001). There was no difference in the intensive care unit stay, hospital stay, and final band gradients in the two groups. On a mean follow-up of 22.8 +/- 18.6 months (range, 4 to 72 months), there was PA distortion in 6 patients and band-migration in 4 patients in the CPAB group. These were not observed in the APAB group., Conclusions: Similar band gradients were achieved with the use of conventional or adjustable PAB. However, the use of this simple and inexpensive technique of APAB was associated with a significant reduction in the early band-related deaths, need for early multiple reoperations, and early adverse acute events, thus making it a safer alternative to CPAB, more so in unstable patients.

We present our experience with a patient in whom a homograft conduit was used to connect the superior vena cava (SVC) to the right pulmonary artery (RPA) in a functionally univentricular heart where corrective repair was impossible.

Background: We describe alternative surgical techniques for construction of systemic-to-pulmonary arterial shunts, and propose criterions for their application in selected patients., Patients and Methods: We constructed a variety of modified systemic-to-pulmonary arterial shunts, using polytetrafluoroethylene grafts, in 92 selected patients with cyanotic congenital heart disease and anomalies of the aortic arch and systemic veins. Their age ranged from 7 days to 3.6 years, with a mean of 7.08 months. We performed 88 operations through a thoracotomy. Of this cohort, 60 patients underwent a second-stage operation, with 15 receiving a superior cavopulmonary connection, 16 a total cavopulmonary connection, and 29 proceeding to biventricular repair after a mean interval of 15.6 months. We have 21 patients awaiting their second or final stage of palliation., Results: There were five early (5.4%) and six late deaths (6.8%), two of which were related to construction of the shunts. At a mean follow-up of 45.29 months, the increase in diameter of pulmonary trunk and its right and left branches was uniform and significant (p value less than 0.001). Pulmonary arterial distortion requiring correction at the time of second-stage operation was observed in 5 patients (6.1%). Adequate overall palliation was achieved in 98% of the cohort at 8 months, 91% at 12 months, and 58% at 18 months., Conclusions: Patients with a right- or left-sided aortic arch and right-sided descending thoracic aorta, those with anomalies of systemic venous drainage masking the origin of great arterial branches, and those with disproportionately small subclavian arteries, constitute the ideal candidates for our suggested modification of the construction of a modified Blalock-Taussig shunt. The palliation provided by these shunts was satisfactory, with predictable growth of pulmonary arteries, insignificant distortion in the great majority, and easy take-down.

Objective: Pulmonary artery banding is associated with a high morbidity and mortality. We describe a new technique of adjustable pulmonary artery banding to prevent these problems., Methods: Between December 2003 and May 2005, 32 patients aged 18 days to 2 years (mean age, 2.5 +/- 0.5 months) and weighing 2.1 to 6.3 kg (mean, 3.6 +/- 1.3 kg) underwent adjustable pulmonary artery banding., Results: All patients survived the operation. There were 2 deaths, one caused by meningitis and another caused by aspiration pneumonitis. Satisfactory band gradients were achieved between 3 and 10 days (7.2 +/- 2.6 days) in 3 to 6 sittings. Mean follow-up was 7.5 +/- 3.8 months (1-16 months). One patient required reoperation for unsatisfactory band gradient 2 weeks after discharge. There were no late deaths. Follow-up computed tomographic angiograms (n = 4) demonstrated proper band placement and ruled out distortion of the pulmonary arteries. Four patients underwent uneventful definitive operations after an interval of 7 to 13 months., Conclusion: This technique of percutaneously adjustable pulmonary artery banding is simple and inexpensive and allows easy band adjustments without the need for multiple reoperations.