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Anomalous origin of a pulmonary artery branch from the aorta is a rare congenital anomaly in which one of the pulmonary arteries arises from the aorta. These patients require early surgery to prevent development of severe irreversible pulmonary arterial hypertension. Multiple techniques have been described for repair of this condition. In this report, we describe a different technique compared with previously described procedures and discuss its advantages.

To link to full-text access for this article, visit this link: http://dx.doi.org/10.1016/j.jtcvs.2008.11.063 Byline: Arkalgud Sampath Kumar, Sachin Talwar, Anubhav Gupta Abbreviations: AF, atrial fibrillation; LA, left atrial; MRI, magnetic resonance imaging; MVR, mitral valve replacement; PA-MVR, mitral valve replacement with a pulmonary autograft Abstract: We performed mitral valve replacement with a pulmonary autograft using the technique described by us earlier and present the results. Author Affiliation: Cardiothoracic Centre, All India Institute of Medical Sciences, New Delhi, India Article History: Received 12 July 2008; Revised 21 September 2008; Accepted 25 November 2008

Background:In this prospective randomized controlled trial, we compared the standard del Nido cardioplegia solution (SDN) with the modified del Nido cardioplegia solution (MDN) in which the base solution was the plain Ringer solution. Methods:A total of 80 patients aged < 12 years undergoing intracardiac repair of Tetralogy of Fallot were randomized into SDN (n = 39) or MDN (n = 41) groups. The primary outcome was a change in cardiac index (CI). Secondary outcomes were ventricular arrhythmias after the release of aortic-cross clamp, postoperative inotropic score (IS), time to peripheral rewarming, duration of mechanical ventilation, intensive care unit (ICU) length of stay, and hospital length of stay, and electron microscopic differences between the 2 groups. Cardiac Troponin-I, inflammatory markers tumor necrosis factor-α (TNF-α), and interleukin-L (IL-6) were measured. Results:Applying the noninferiority confidence interval approach, the difference between the changes in CI between the 2 groups was −0.093 L/min/m2 (95% CI: −0.46-0.27 L/min/m2) which was within the noninferiority threshold of −0.5 indicating that CI was similar in both SDN and MDN. Ventricular arrhythmias postclamp release (P= .91), IS (P= .09), duration of mechanical ventilation (P= .27), ICU length of stay (P= .50), hospital length of stay (P= .57), IL-6 (P= .19), TNF-α (P= .17), Troponin-I (P= .15), electron microscopy changes (P> .05) were not different between groups. Conclusion:MDN was shown to be noninferior to the SDN cardioplegia in terms of preservation of cardiac index. In addition, other metrics indicative of myocardial protection were similar between groups. In developing nations where SDN is not available or is expensive, MDN cardioplegia is an acceptable alternative.

To link to full-text access for this article, visit this link: http://dx.doi.org/10.1016/j.jtcvs.2008.10.018 Byline: Pranava Sinha, Sachin Talwar, Achintya Moulick, Richard Jonas Author Affiliation: Department of Cardiovascular Surgery, Children's National Medical Center, Washington, DC Article History: Received 2 September 2008; Accepted 10 October 2008

Tetralogy of Fallot and absent pulmonary valve (TOF/APV) is associated with significant pulmonary artery dilatation and airway compression.

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.

We describe the anatomic findings in a 2-year-old patient with double outlet right ventricle with right-sided aorta in the setting of usual atrial arrangement and discordant atrioventricular connections, making comparison with a specimen from the pathological archive of the Birmingham Children’s Hospital in the United Kingdom having this rare combination of anatomic features. We discuss the challenges involved in diagnosis and management.

To link to full-text access for this article, visit this link: http://dx.doi.org/10.1016/j.jtcvs.2005.12.029 Byline: Arkalgud Sampath Kumar, Sachin Talwar, Devagourou Velayoudam Author Affiliation: Department of Cardiothoracic and Vascular Surgery, All India Institute of Medical Sciences, New Delhi, India Article History: Received 30 November 2005; Accepted 12 December 2005

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.

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.

Objective: We report our experience with surgery for total anomalous pulmonary venous connection (TAPVC) beyond first decade of life.Methods: Between January 1987 and July 2017, 98 patients ≥ten years underwent TAPVC repair. Their detailed case-records were analyzed.Results: Mean age was 19.05 (± 12.8; median 18) years. Anatomic subtypes were supracardiac (n = 62), cardiac (n = 20), and mixed (n = 16). An atrial septal defect (ASD) was present in all. Severe tricuspid valve regurgitation was present in four patients and severe rheumatic mitral regurgitation was present in one. On preoperative cardiac catheterization, mean pulmonary artery pressure was 67 ± 15.6 mm Hg (median 58; range 37-96). Mean pulmonary vascular resistance was 5.6 ± 3.9 Woods units·m2(median 4.7, range 2.9-11.8). Twenty-five patients had moderate and eight had severe pulmonary arterial hypertension. Right ventricular dysfunction was present in eight patients. At repair, a small ASD was left open in 87 patients while in 11 patients, the ASD was closed using a unidirectional valved patch. Follow-up was available for 90 (92%) patients. There were no late deaths over a mean follow-up of 163 ± 103.98 months (median 163, range 1-362). Eighty-two patients were in NYHA class I and eight were in class II. Right ventricular function normalized in 82 patients while 80 patients had reduction in pulmonary artery pressure. Event-free survival was 94.2% at 5 years, 92.3% at 10 years, and 90% at 20 and 30 years.Conclusions: Outcomes of surgical repair for TAPVC beyond first decade of life are satisfactory. Close follow-up, however, is necessary for possible persistence of pulmonary arterial hypertension.

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.

AbstractObjectivesArterial switch operation is the treatment of choice in infants with transposed arterial trunks. It is technically challenging to perform in patients having usual atrial arrangement and concordant atrioventricular connections but having a left-sided aorta. Correction in this setting requires surgical expertise and precision. Here we review our experience with such patients.MethodsBetween January, 2002 and October, 2013, the arterial switch operation was performed in 20 patients in the combination emphasised above. Patient records were analysed in detail for coronary arterial patterns, and for the techniques used for transfer of the coronary arteries and reconstruction of the great arteries. Outcomes were recorded in terms of in-hospital survival and left ventricular function at the most recent follow-up.ResultsAll patients survived the procedure. Ages ranged from 3 days to 18 months, with a median of 75 days; the weight of the patients ranged from 3 to 8.8 kg, with a median of 3.85 kg. The LeCompte manoeuvre was performed in only nine patients. The mean cardiopulmonary bypass time was 157.5±24.9, with a median of 161 minutes, and the mean aortic cross-clamp time was 101.2±23.8, with a median of 102 minutes. Subsequently, two patients died: the first due to a sudden onset of ventricular fibrillation and the second during a crisis of severe pulmonary hypertension. At the last follow-up, which ranged from 23 to 41 months, with a mean of 38.04±2.32 and a median of 38.4 months, all 18 survivors were in NYHA class I, with none requiring cardiac medications and all having normal bi-ventricular function without residual defects.ConclusionWith appropriate technical modifications, patients with concordant atrioventricular and discordant ventriculo-arterial connections with a left-sided aorta can undergo successful anatomical repair.

To link to full-text access for this article, visit this link: http://dx.doi.org/10.1016/j.jtcvs.2007.08.003 Byline: Shiv Kumar Choudhary, Sachin Talwar, Balram Airan Author Affiliation: Cardiothoracic Center, All India Institute of Medical Sciences, New Delhi, India. Article History: Received 17 July 2007; Accepted 7 August 2007

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.

AbstractA unidirectional superior cavopulmonary anastomosis was performed on the right side in a patient with a functionally univentricular heart, atresia of main and left pulmonary artery, bilateral superior caval veins, and a patent arterial duct in the right pulmonary artery. Anastomosis of the left superior caval vein to the right superior caval vein created a neo-innominate vein without using prosthetic material.

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.

Patients with right atrial isomerism and total anomalous pulmonary venous connection (TAPVC) in a functionally univentricular heart are a challenging subset with a high mortality rate. We present the case of a patient with univentricular heart who had right atrial isomerism and associated mixed TAPVC (supracardiac, cardiac, and infracardiac). The anatomy was delineated precisely, and the patient underwent first-stage univentricular palliation consisting of TAPVC repair and bilateral bidirectional superior cavopulmonary anastomosis.

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.

A 26-year-old patient with d-transposition of great arteries (d-TGA), bilateral superior vena cava, and juxtaposed atrial appendages underwent a successful atrial switch operation. It is extremely uncommon to encounter a previously unpalliated patient with d-TGA at this age. Unusual morphologic features in this patient necessitated technical modifications to successfully accomplish an atrial switch procedure.

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 (Pao2) 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 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.

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.

Surgical correction of patients with tetralogy of Fallot with pulmonary atresia is now one of the routine procedures performed by pediatric cardiac surgeons. In one variant, the pulmonary arterial supply is derived from a fistulous communication from the coronary arteries. This rare and interesting situation poses a diagnostic and therapeutic dilemma, as well as providing specific management challenges to the surgical team. Here, we discuss important aspects of this rare variant, specifically its morphology, presentation, evaluation and management.

AbstractBetween January, 2002 and December, 2011, 27 patients (19 boys) underwent pericardiectomy. The mean age was 9.3 ± 4.96 years (range 0.4 to 15 years) and the mean duration of symptoms was 16.9 ± 22.15 months. In all, 25 patients had dyspnoea; eight were in New York Heart Association (NYHA) class IV; six had bacterial pericarditis; and 18 were on anti-tuberculosis treatment, although only nine had records suggesting tuberculosis. There were nine patients who underwent pre-operative pigtail catheter drainage of pericardial fluid. Surgical procedures were complete pericardiectomy (n = 20), partial pericardiectomy (n = 6), and pleuropericardial window (n = 1).The mean pre-operative right atrial pressure was 20.4 ± 4.93 mmHg. There were six hospital deaths due to low cardiac output (n = 5) and arrhythmia (n = 1). The mean intensive care unit stay was 2.7 ± 1.2 days and mean post-operative stay was 9.9 days. The mean right atrial pressure dropped to 8.7 ± 1.15 mmHg. Adverse outcomes defined as death/prolonged intensive care unit stay, prolonged post-operative stay were not associated with sex, diagnosis of tuberculosis or pyopericardium, or the duration of symptoms or pre-operative right atrial pressure. Younger patients had prolonged intensive care unit stay (p = 0.03) but not increased mortality. Advanced NYHA class predicted death (p = 0.02). The mean follow-up was 23.1 ± 23.8 months. All except one survivor are in NYHA class I and off all cardiac medications. Despite adequate surgery, pericardiectomy in children is associated with a high mortality, which is related to delayed surgery and poor pre-operative general condition. No specific pre-operative variable other than worse pre-operative NYHA class is a predictor of survival. Therefore, early pericardiectomy should be undertaken in such patients.

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.

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.

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.

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.

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 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.

An 8-month-old boy with aortic origin of the right pulmonary artery, ventricular septal defect, and anomalous origin of the left main coronary artery from the right pulmonary artery, underwent uneventful single-stage surgical correction. The rarity and technical aspects of managing this condition are discussed.

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.

Background:Volatile anesthetic agents may precondition the myocardium and protect against ischemia and infarction. Preconditioning by volatile anesthetic agents is well documented in adults but is underinvestigated in children. The present study compares the effect of preconditioning in children by three volatile anesthetic agents along with several other variables associated with cardioprotection.Method:Eighty children scheduled for ventricular septal defect closure under cardioplegic arrest were assigned to preconditioning for five minutes after commencement of cardiopulmonary bypass (CPB) with one minimum alveolar concentration (MAC) of one of the following agents: isoflurane, sevoflurane, desflurane, or placebo (oxygen-air mixture). The plasma concentration of creatine kinase MB (CK-MB) was determined after initiation of CPB, and again 6 and 24 hours after admission to the intensive care unit (ICU) after surgery. Duration of inotropic support, mechanical ventilation, and length of ICU stay in all the groups were also recorded.Results:Preconditioning with isoflurane, sevoflurane, and desflurane was associated with significantly decreased postoperative release of CK-MB as compared to placebo group at 6 (group 1: 237.2 ± 189, group 2: 69.8 ± 15.8, group 3: 64.7 ± 37.8, and group 4: 70.4 ± 26.7) and 24 hours (group 1: 192.4 ± 158.2, group 2: 67.7 ± 25.0, group 3: 85.7 ± 66.8, and group 4: 50.4 ± 31.6) after admission to ICU. No significant differences were observed in the CK-MB levels among the three volatile anesthetic agents. Duration of inotropic support, mechanical ventilation, and length of ICU stay were greater in placebo group as compared to other groups without reaching statistical significance.Conclusion:Volatile anesthetic appear to provide definite cardioprotection to pediatric myocardium. No conclusion can be drawn regarding the best preconditioning agent among isoflurane, sevoflurane, and desflurane.

We report a two-year-old patient with isomerism of the right atrial appendages, a functionally univentricular heart, and associated totally anomalous pulmonary venous connection. The unusual finding was an intrapulmonary course of the vertical vein. We discuss the anatomical findings, management, and outcome.

In this report, we describe the surgical management of a patient with tetralogy of Fallot and pulmonary atresia, in whom a fistula from the anterior interventricular coronary artery was the predominant source of arterial supply to both the lungs.

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 Ucmin-1) than acyanotic group (24.88?±?9.23 Ucmin-1; p?=?0.009). Platelet function was deranged in 31% of patients (cyanotic, 59%; acyanotic, 8%; p?

Background:this study was performed to assess the results of aortic valve replacement in children with biological substitutes including homografts, pulmonary autografts (Ross procedure), and aortic valve reconstruction with autologous pericardium (Duran technique).Methods:between March 1992 and July 2009, 73 children with aortic valve disease (mean age, 11.8?±?2.7 years) underwent aortic valve replacement with biological substitutes including homografts, pulmonary autografts, and aortic valve reconstruction with autologous pericardium. Associated procedures were mitral valve repair in 32 and subaortic membrane resection in 3.Results:early mortality was 1.4% (1 patient). Median follow-up was 94 months. Sixty (83.3%) survivors had insignificant aortic regurgitation. Reoperation was required in 7 (9.6%) patients: for autograft dysfunction alone in 2, autograft failure and failed mitral valve repair in 2, autograft dysfunction with severe pulmonary homograft regurgitation in 1, severe homograft aortic valve regurgitation in 1, and right ventricular outflow tract obstruction in 1. There were 4 (5.4%) late deaths. Actuarial reoperation-free, event-free, and aortic valve dysfunction-free survival were 92.5%?±?4%, 93.4%?±?3.3 %, 94%?±?2.9%, 86.2%?±?4.3%, respectively, at 94 months.Conclusions:aortic valve replacement with biological substitutes is associated with acceptable hemodynamics and midterm results.

Objective: To study the effect of cardiopulmonary bypass (CPB) on serum thyroid hormone profile in children undergoing open‐heart surgery.

An alternative technique for channeling the pulmonary venous return towards the left atrium in patients with total anomalous pulmonary venous return to the coronary sinus is described. The advantages of this technique, which avoids the use of patch material and prevents arrhythmias, are discussed.

We describe a patient with concordant ventriculoarterial connections with parallel arterial trunks, divided left atrium (cor triatriatum sinister), ventricular septal defect, bilateral superior caval veins, and juxtaposed atrial appendages. The aorta was anterior and left sided. We discuss the morphological features of this rare condition, and the diagnostic dilemma it produced.

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.

Although rarely performed today, atrial switch operations continue to have an important role in the management of some forms of congenital heart disease. In developing countries, delayed diagnosis and presentation of patients with transposition of the great arteries is not uncommon. For some patients who are referred for surgery beyond the newborn period, the atrial switch operations are still considered to be the best option. Also, as part of surgical repair of congenitally corrected transposition, an atrial switch operation is combined with arterial switch or the Rastelli procedure as an alternative to physiologic repair. In isolated ventricular inversion (atrioventricular discordance with ventriculoarterial concordance), the atrial switch operation alone leads to complete anatomical correction. Finally, management of late complications of atrial switch operations requires a thorough understanding of the procedures.

Objective: For patients with dextro-transposition of great arteries (d-TGAs), ventricular septal defect (VSD), and severe pulmonary arterial hypertension (PAH), the common surgical options are palliative arterial switch operation (ASO) or palliative atrial switch operation leaving the VSD open. We evaluated the role of ASO with VSD closure using a fenestrated unidirectional valved patch (UVP). Methods: Between July 2009 and February 2011, six patients with TGAs, VSD, and severe PAH (mean age 39.8 ± 47.4 months, median 21, range 8-132 months), weighing 10.7 ± 9.2 kg (median 8.6, range 4.3-29 kg), underwent ASO with VSD closure using our simple technique of UVP. Mean pulmonary artery systolic pressure before the operation was 106 ± 12.7 mm Hg (median 107.5, range 95-126 mm Hg) and pulmonary vascular resistance was 9.5 ± 4.22 units (median 9.5, range 6.6-17.1 Wood units). Results: There were no deaths. All patients had a postoperative systemic arterial saturation of more than 95%, although there were frequent episodes of systemic desaturation due to right-to-left shunt across the valved VSD patch (as seen on transesophageal and transthoracic echocardiograms). Mean follow-up was 10 ± 7.6 months (median 7.5, range 1-22 months). At most recent follow-up, all patients had systemic arterial saturation of more than 95% and no right-to-left shunt through the VSD patch. In one patient, the follow-up cardiac catheterization showed a fall in pulmonary artery systolic pressure to 49 mm Hg. Conclusion: Arterial switch operation with UVP VSD closure is feasible with acceptable early results. It avoids complications of palliative atrial switch (arrhythmia and baffle obstruction) and partially or completely open VSD.

We report a 3-year-old patient with a rare combination of a sinus venosus interatrial communication, anomalous drainage of the right superior pulmonary vein to the right atrium, persistent left superior caval vein, and interruption of the inferior caval vein. We discuss features of anatomical and technical interest.