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A simplified technique of performing the extracardiac Fontan operation on cardiopulmonary bypass is described. The advantages of this technique are briefly discussed. [ABSTRACT FROM AUTHOR]

Total cavopulmonary connection (Fontan) without using cardiopulmonary bypass (CPB) may be superior to Fontan on CPB. In the experience of many, a Fontan operation without CPB may be associated with a reduced need for inotropic support, improved outcomes in the postoperative phase including faster time to termination of mechanical ventilatory support, reduced intensive care unit stay, lower volume of pleural and peritoneal effusions, and decreased hospital stay, thereby rendering it more cost-effective. However, the operation is technically more difficult to perform than Fontan on CPB and requires significant modifications of surgical technique and alteration in overall management strategy. In this article, an alternative technique of performing total cavopulmonary connection without CPB is described. Its advantages are briefly discussed. [ABSTRACT FROM AUTHOR]

Treating heart failure (HF) in postoperative congenital cardiac repair in children with an anomalous left coronary artery from the pulmonary artery (ALCAPA) is a daunting task. Regular anti-failure measures with angiotensin-converting enzyme (ACE) inhibitors may become inadequate or resistant in certain situations. Angiotensin receptor-neprilysin inhibitor (ARNI) in place of ACE inhibitors can be an alternative to help these postcardiac surgical children convalesce better. Sacubitril/valsartan is an ARNI-approved treatment for HF in adults, and in pediatric HF patients, it is showing promising benefits. However, in postcardiac surgical children with HF, its effects are not known. We used ARNI successfully in one of our postcardiac surgical infants with ALCAPA repair in HF, and we describe this case here. [ABSTRACT FROM AUTHOR]

In patients with univentricular heart, the Fontan procedure is the final palliation. This is usually staged. A systemic-to-pulmonary artery shunt is performed in the presence of episodes of cyanotic spells in the neonatal period or in some patients in infancy; a bidirectional superior cardiopulmonary anastomosis is preferred early in life. This is followed by the final Fontan palliation on an elective basis later. For an effective bidirectional superior cavopulmonary anastomosis and Fontan palliation, good-sized confluent pulmonary arteries (PAs) are mandatory in addition to favorable hemodynamic data. Patients with discontinuous PAs that are small in size present a surgical challenge at initial palliation as the one described in this report. [ABSTRACT FROM AUTHOR]

Total anomalous pulmonary venous connection (TAPVC) is a rare congenital heart disease with an incidence of 1%-3% of all congenital heart disease patients. The pulmonary veins returning oxygenated blood from the lungs are draining into the right side (right atrium or great veins), causing systemic hypoxia and cyanosis. TAPVC patients are mostly present at an early stage and need surgical correction to sustain life and prevent complications. However, some subsets of patients with TAPVC in whom the mixing of blood is happening may progress into adult life and have associated pulmonary arterial hypertension (PAH) and right ventricular (RV) dysfunction. We report a case of a young adult lady who presented to our hospital at 16 years of age with TAPVC along with severe PAH and RV dysfunction. During the perioperative period, the patient was successfully managed with pulmonary vasodilators and inotropic support. The objective of the case report was to describe the management strategy followed in the successful treatment of the patient. Due to congestive heart failure, low cardiac output status, and other factors, these individuals will also experience comorbidities such as PAH, RV dysfunction, and organ dysfunction. Patients require care in an environment that has experience handling a variety of problems and a PAH crisis. PAH can be controlled with the aid of pulmonary vasodilators such as sildenafil, bosentan, milrinone, NTG, and inhaled nitric oxide. Mechanical hyperventilation to lower pulmonary vascular resistance will also aid in treatment. When combined with intraoperative transesophageal echocardiography monitoring, the balanced anesthetic method can be used to successfully manage the majority of cases. [ABSTRACT FROM AUTHOR]

A 4.5-year-old patient with tricuspid atresia, pulmonary stenosis, bilateral superior vena cava veins with absent innominate vein, and hypoplasia of the left pulmonary artery required a bidirectional superior cavopulmonary anastomosis. An innominate vein was fashioned out of a 6 mm polytetrafluoroethylene graft. The technique is briefly discussed. [ABSTRACT FROM AUTHOR]

Thrombotic complications following balloon atrial septostomy (BAS) are unusual. We report a patient with thrombus formation at the site of BAS, extending into the inferior vena cava (IVC), following BAS for transposition of great arteries with intact ventricular septum (TGA-IVS). An urgent arterial switch operation (ASO) with removal of the thrombus was performed. [ABSTRACT FROM AUTHOR]

In this report, we describe a 25‐year‐old patient in whom a fistula between the right pulmonary artery and the left atrium was accurately diagnosed. The successful surgical management of this patient is described. Current diagnostic methods, evaluation, and treatment options for this uncommon cause of cyanosis in an adult patient are discussed. [ABSTRACT FROM AUTHOR]

Background : Neurodevelopmental abnormalities are common in congenital heart disease (CHD), more so in cyanotic CHDs. Perioperative factors have been known to affect neurodevelopmental outcomes. Aim : We aimed to determine the neurodevelopmental outcomes following open-heart surgery in cyanotic CHD. Methods : In this prospective observational study, eligible infants and children < 21 months with cyanotic CHD planned for open-heart surgery underwent preoperative neurodevelopmental assessment using Developmental Assessment Scale for Indian Infants (DASII) to look for any motor and/or mental delay. A second neurodevelopmental assessment was performed after 9 months ± 2 weeks of cardiac surgery. Follow-up DASII was conducted through interactive video conferencing in 23 of 60 patients due to COVID-19 pandemic. The univentricular and biventricular repair groups were compared in terms of their neurodevelopmental outcomes. Perioperative factors were compared between neurodevelopmental "delay" and "no delay" groups. Results: Of the 89 children enrolled, preoperative motor and mental delay were present in 29 and 24 children, respectively. Follow-up DASII could be performed in 60 children. At follow-up, motor delay was present in seven and mental delay in four children. Overall, there was a significant improvement in both motor and mental developmental quotient at follow-up. There was no significant difference in either motor or mental domains between univentricular and biventricular groups. Among the perioperative variables, only the postoperative length of stay in intensive care unit was significantly different between neurodevelopmental "delay" and "no delay" groups (P = 0.04). Conclusion : Neurodevelopmental delay occurred substantially among unoperated children with cyanotic CHD. The neurodevelopmental status improved significantly following open-heart surgery among the survivors. Delay was associated with length of stay in intensive care following cardiac surgery. [ABSTRACT FROM AUTHOR]

Background: Expected benefits of modified ultrafiltration (MUF) include increased hematocrit, reduction of total body water and inflammatory mediators, improved left ventricular systolic function, and improved systolic blood pressure and cardiac index (CI) following cardiopulmonary bypass (CPB). This prospective randomized trial tested this hypothesis.Methods: Seventy-nine patients undergoing intracardiac repair of tetralogy of fallot were randomized to conventional ultrafiltration (CUF) + MUF (n = 39) or only CUF group (n = 40). The primary outcome was a change in hematocrit. Secondary outcomes were changes in peak airway pressures, ventilatory support, blood transfusions, time to peripheral rewarming, mean arterial pressure, central venous pressure, inotrope score (IS), and CI. Serum inflammatory markers were measured.Results: Baseline hematocrit was 50.6 ± 10.02 in the only CUF group whereas it was 43.9 ± 5.55 in the CUF + MUF group (p = .36). Following MUF, the CUF + MUF group had higher hematocrit (44.7 ± 0.50 g/dl) compared to the only CUF group (37.2 ± 0.49 g/dl), p ≤ .001 after adjusting for baseline hematocrit. Central venous pressure (mmHg) immediately following sternal closure was 9.27 ± 3.12 mmHg in the CUF + MUF group and 10.52 ± 2.2 mmHg in the only CUF group (p = .04). In the intensive care unit (ICU), they were 11.52 ± 2.20 mmHg in the only CUF group and 10.84 ± 2.78 mmHg in the CUF + MUF group (p = .02). Time to peripheral rewarming was 6.30 ± 3.91 h in the CUF + MUF group and 13.67 ± 3.91 h in the only CUF group (p = .06). Peak airway pressures in ICU were 17 ± 2 mmHg versus 20.55 ± 2.97 mmHg in CUF + MUF group & only CUF group, respectively, p < .001). Duration of mechanical ventilation was 6.3 ± 2.7 h in CUF + MUF group compared to 14.7 ± 3.5 h in the only CUF group (p = .002). IS was 11.52 ± 2.20 in the only CUF group compared to 10.84 ± 2.78 in CUF + MUFs group. Eight of 39 (20.5%) patients in the CUF + MUF group had IS > 10 compared to 22 of 40 (55%) patients in the only CUF group (p = .02). Serum Troponin-T and interleukin-6 levels were lower in the CUF + MUF group; TNF-α and CPK-MB were similar. ICU and hospital stay were similar.Conclusion: Patients undergoing a combination of CUF and MUF had higher postoperative hematocrit, decreased duration of mechanical ventilation, lower need for inotropes and lower interleukin-6 and Troponin-T levels. This group had better postoperative outcomes. This study was registered with the Clinical trials registry of India (CTRI/2017/11/010512) before commencement. [ABSTRACT FROM AUTHOR]

Background: Total anomalous pulmonary venous connection with intact interatrial septum is extremely rare. Aims: In these patients, a right to left shunt through a ventricular septal defect or a patent ductus arteriosus is mandatory to maintain the systemic circulation. Mechanical or physiological restriction of shunting through these pathways results in rapid clinical deterioration. Discussion: We describe the anatomical findings with surgical repair in one such 10‐day‐old baby. [ABSTRACT FROM AUTHOR]

Objectives This study was aimed to assess the benefits of early tracheostomy (ET) compared with late tracheostomy (LT) on postoperative outcomes in pediatric cardiac surgical patients. Design Present one is a prospective, observational study. Setting The study was conducted at a cardiac surgical intensive care unit (ICU) of a tertiary care hospital. Participants All pediatric patients below 10 years of age, who underwent tracheostomy after cardiac surgery from January2019 to december2019, were subdivided into two groups according to the timing of tracheostomy: "early" if done before 7 days or "late" if done after 7 days postcardiac surgery. Interventions ET versus LT was measured in the study. Results Out of all 1,084 pediatric patients who underwent cardiac surgery over the study period, 41 (3.7%) received tracheostomy. Sixteen (39%) patients underwent ET and 25 (61%) underwent LT. ET had advantages by having reduced risk associations with the following variables: preoperative hospital stay (p = 0.0016), sepsis (p = 0.03), high risk surgery (p = 0.04), postoperative sepsis (p = 0.001), C-reactive protein (p = 0.04), ventilator-associated pneumonia (VAP; p = 0.006), antibiotic escalation (p = 0.006), and antifungal therapy (p = 0.01) requirement. Furthermore, ET was associated with lesser duration of mechanical ventilation (p = 0.0027), length of ICU stay (LOICUS; p = 0.01), length of hospital stay (LOHS; p = 0.001), lesser days of feed interruption (p = 0.0017), and tracheostomy tube change (p = 0.02). ET group of children, who had higher total ventilation-free days (p = 0.02), were decannulated earlier (p = 0.03) and discharged earlier (p = 0.0089). Conclusion ET had significant benefits in reduction of postoperative morbidities with overall shorter mechanical ventilation, LOICUS, and LOHS, better nutrition supplementation, lesser infection, etc. These benefits may promote faster patient convalescence and rehabilitation with reduced hospital costs. [ABSTRACT FROM AUTHOR]

Objectives To describe the outcomes of postoperative tracheostomy and determine the predictors of survival. Design Prospective, observational study. Setting Cardiac surgical intensive care unit of a tertiary care hospital. Participants All pediatric patients below 10 years of age who underwent tracheostomy after cardiac surgery from January 2019 to December 2019. Different variables were compared between survivors and nonsurvivors. Intervention Tracheostomy. Results Among 1084 pediatric patients who underwent cardiac surgery during the study period, 41 (3.7%) received tracheostomy. Survival rate was 71%. Earlier, sternal closure (SC) (p = 0.04), acute kidney injury (AKI) (p = 0.001), serum C-reactive protein (CRP) (p = 0.007), duration of total parenteral nutrition (TPN) (p = 0.005) and days of feed interruption (FI) (p = 0.02), activated partial thromboplastin time (aPTT) before tracheostomy (p = 0.006), and bleeding from tracheostomy site (p = 0.02) were significantly low in the survivor group. Among the peritracheostomy variables taken at different time points, the levels of pH on tracheostomy day 1 (p = 0.03), serum lactate on tracheostomy day 2 (p = 0.01) and day 3 (p = 0.01), and random blood sugar (RBS) on tracheostomy day 3 (p = 0.04) were significantly lower in the survivor group. The arterial oxygen saturation (SaO2) on tracheostomy day 1 (p = 0.04) and the platelet count before tracheostomy (p = 0.02) were significantly higher in the survivor group. Conclusions Our study demonstrated a survival of 71% among the study cohort. Lesser duration of open sternum, lower incidence of AKI, less number of days on TPN and FI, lower posttracheostomy aPTT, bleeding, RBS, lactate, and higher pretracheostomy platelet count and posttracheostomy SaO2 were found to be the predictors of survival. [ABSTRACT FROM AUTHOR]

A coronary artery crossing the right ventricular outflow tract is a subset of a larger pathomorphological cohort known as an anomalous coronary artery (ACA) in the tetralogy of Fallot (TOF). The best possible outcome in a patient with TOF and ACA is decided by judicious selection of optimum preoperative investigative information, the timing of surgery, astute assessment of preoperative surgical findings, and appropriate surgical technique from a wide array of choices. In most instances, the choice of surgical technique is determined by the size of the pulmonary annulus and the anatomical relation of ACA to the pulmonary annulus. In the present era, complete, accurate preoperative diagnosis and primary repair is a routine procedure with strategies to avoid a right ventricle-to-pulmonary artery conduit. [ABSTRACT FROM AUTHOR]

First introduced in 1971, the Fontan procedure is the final common destination for all patients with a functional single ventricle. The procedure itself has evolved tremendously over the last five decades. This review traces this journey and presents the importance, outcomes and future outlook of the procedure in the current era. [ABSTRACT FROM AUTHOR]

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

Background: Fenestration of the baffle/conduit is believed to reduce pleural effusion following the Fontan operation. However, equivocal results have been observed with or without fenestration. This study aims to evaluate the efficacy of fenestration on the amount and duration of pleural effusion following the Fontan operation. Methods: About 40 patients undergoing extracardiac Fontan (ECF) were randomized into two groups: one with fenestration (ECF‐F; n = 20) or without fenestration (ECF‐NF; n = 20). Primary outcome was the amount and duration of pleural effusions. Secondary outcomes were time to removal of the chest tubes, hospital stay, and readmission to the hospital because of recurrent pleural within 30 days of the operation. Results: Mean age was 11.5 ± 5.07 (range, 8.7‐13.5) years in the ECF‐F group and 13.6 ± 0.4 years (range, 10.5‐15.5) in the (ECF‐NF) group. The total drain output was 7.89 mL/kg/d in ECF‐NF compared with 6.9 mL/kg/d in the ECF‐group (P =.14). Time for removal of pleural tubes was 14.6 ± 0.95 days in the ECF‐NF group compared with 11.6 ± days in the ECF‐F group. Total duration of hospital stay was higher but not significant in the ECF‐NF group compared with the ECF‐F group. Two patients in ECF‐NF required readmission to the hospital within 30 days following discharge, while there were no readmissions in the ECF‐F group. Conclusion: Contrary to the literature, the creation of a fenestration in the ECF circuit was not clearly associated with a reduction in the amount and duration of pleural effusion compared with a non‐fenestrated Fontan. These findings may be debatable in high risks versus low risk candidates. However in the present study, in a low risk canditates undergoing the Fontan operation, the daily amount of pleural drainage was no different. Larger studies are needed to confirm these findings. [ABSTRACT FROM AUTHOR]

We hereby report rare occurrence of irreversible complete heart block in a child with tricuspid valve infective endocarditis. The tricuspid valve vegetation also caused complete closure of perimembranous ventricular septal defect, which was later discovered during surgery. [ABSTRACT FROM AUTHOR]

Aims Anatomic variations in hearts with common arterial trunk are well-known, although there is no large study of living patients. Detailed knowledge of the origins of the pulmonary and coronary arteries is vital for surgical management. We sought to clarify the variations using computed tomography. Methods and results We prospectively studied 70 consecutive patients using echocardiography and computed tomography. In 63 (90%) patients, there was aortic dominance, while 7 (10%) had dominance of the pulmonary component. In 27 (43%) patients with aortic dominance, part of the pulmonary segment arose from a truncal valvar sinus. A long confluent pulmonary channel was more common in patients with sinusal origin compared to those with non-sinusal origin of the pulmonary segment (19 vs. 0; P  = 0.0005). Close proximity between the orifices of the coronary arteries and the pulmonary component was also more frequent with sinusal origin (21 vs. 6; P  < 0.001) with 5 (19%) patients having pulmonary flow obstructed by a truncal valvar leaflet. Conclusion Sinusal origin of the pulmonary component is common with aortic dominance, frequently in association with a long confluent pulmonary segment, which may be in close proximity to the origin of a coronary artery. One-fifth of patients with sinusal origin of pulmonary component have a truncal valvar leaflet obstructing the pulmonary orifice. These morpho-anatomic findings have important implications for management. [ABSTRACT FROM AUTHOR]

Rupture of sinus of valsalva following repair of tetralogy of fallot (TOF) is very rare. It should be suspected as a cause of recurrent or prolonged pleural effusion and congestive cardiac failure in patients who have undergone repair of TOF. We report one such patient. [ABSTRACT FROM AUTHOR]

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

Objective: Since the intra-extracardiac Fontan (IECF) was popularized by Jonas in 2008, its claimed advantages over the traditional extracardiac or lateral tunnel Fontan are simplicity, suitability for nearly all subsets, flow characteristics, low risk of sinus node artery injury, and possibly, a lower incidence of arrhythmias. In this paper, we present our early experience with this modification of the Fontan operation. Methods: Between 2009 and 2018, 10 patients underwent IECF on cardiopulmonary bypass (CPB) and cardioplegic arrest at our institute. Analysis of preoperative, intraoperative, and early follow-up results was performed. A polytetrafluoroethylene (PTFE) graft was sutured proximally to the orifice of the inferior vena cava (IVC) and distally to the ipsilateral bidirectional superior cavopulmonary (BSCP) junction. Results: Nine patients had undergone a previous BSCP connection, and one patient had a primary IECF. Diagnoses were double outlet right ventricle (n = 2), unbalanced atrioventricular septal defect with associated atrioventricular valve regurgitation (n = 3), single ventricle with anomalies of cardiac situs (n = 2), and tricuspid atresia with borderline pulmonary arteries (n = 1) or tricuspid atresia with borderline pulmonary artery pressures (n = 2). Median aortic cross-clamp and CPB times were 42 min and 82 min respectively. There were no early or late deaths. Median intensive care stay was 3 days (1 to 23 days). There were no arrhythmias. Mean duration of pleural effusions was 9.5 (median 5.5) days. There were no arrhythmias at a median follow-up of 5 years (range 1 month to 9.3 years). Conclusion: The IECF is simple, particularly at reoperations, in borderline patients and those needing concomitant intracardiac procedures. Early results are promising. These patients need constant surveillance. [ABSTRACT FROM AUTHOR]

Ruptured sinus of Valsalva aneurysm from the left coronary sinus communicating through the mitral-aortic intervalvular fibrosa is rare. We report a 35-year-old patient who presented with only hemoptysis without any other cardiac complaint. The patient with this unusual diagnosis and presentation was managed successfully. [ABSTRACT FROM AUTHOR]

This review aims to present and compare different surgical techniques of root translocation of the great arteries except the Ross procedure. The historical aspects, technical considerations, and results are briefly elucidated. [ABSTRACT FROM AUTHOR]

Introduction: Even today, the search for the ideal cardiac valve continues. With advantages of having superior flow dynamics, avoidance of anticoagulation, and resistance to infection, homograft has been shown to have an edge over conventional prosthetic and bioprosthetic valves. But they suffer from disadvantages of limited availability and durability. Our center operates one of the oldest functioning valve banks in the country. We present our experience with homograft valve banking with antibiotic and cryopreserved homografts spread over a quarter century. Methods: For donor selection, procurement, sterilization, and preservation, the recommendations of the American Association of Tissue Banks are being followed in accordance with statutory provisions of the Transplantation of Human Organs Act, 1994. Results: During 25-year period (1993–2017), 777 hearts were procured. Age of the donors ranged from 2 to 60 years and hearts were procured within 24 h of death. A total of 1646 homografts (774 pulmonary, 774 aortic, 60 mitral valves, 20 descending thoracic aortae, and 18 monocusps) were harvested. A total of 546 (32%) homografts were rejected for various reasons. Nine hundred sixty-seven (56.7%) homografts were used in different procedures. Of these, 478 were pulmonary homografts, 425 were aortic homografts, 39 mitral homografts, 18 monocusps, and 7 descending thoracic aorta homografts. One hundred fifty-four (16%) homografts were antibiotic preserved and the rest 813 (84%) were cryopreserved. Conclusions: It is possible to run a homograft valve bank with minimum costs. Though, cryopreservation is more expensive, it provides an opportunity to store the valves for an indefinite period and maintain an uninterrupted supply of homografts. [ABSTRACT FROM AUTHOR]

Purpose: Vitamin K antagonists (VKAs), such as warfarin and acenocoumarol, exert their anti-coagulant effect by inhibiting the subunit 1 of vitamin K epoxide reductase complex (VKORC1). CYP2C9 is a hepatic drug-metabolizing enzyme in the CYP450 superfamily and is the primary metabolizing enzyme of warfarin. Three single nucleotide polymorphisms, two in the CYP2C9 gene, namely CYP2C9*2 and CYP2C9*3, and one in the VKORC1 gene (c.− 1639G > A, rs9923231), have been identified to reduce VKA metabolism and enhance their anti-coagulation effect. The purpose of this study is to evaluate the prevalence of CYP2C9 and VKORC1 polymorphism in Indians receiving VKA-based anti-coagulation after valve surgery and to evaluate the usefulness of genetic information in managing VKA-based anti-coagulation. Methods: In the current prospective observational study, 150 patients who underwent heart valve surgery and had stable INR were genotyped for VKORC1 (− 1639 G > A), CYP2C9*2, and CYP2C9*3. The VKA dosage was estimated from published algorithms and compared to the clinically stabilized dosage. Results: Out of 150 patients, 101 (67.33%) were on warfarin and 49 (32.66%) were on acenocoumarol. Majority of the patients, the 83 in warfarin group and the 40 in acenocoumarol group, had a wild CYP2C9 diplotype. The rest had a mutant (CYP2C9*2 or CYP2C9*3) diplotype. Similarly, 67 patients in the warfarin group and 35 patients in the acenocoumarol group had wild type (G/G) of VKORC1 genotype. The rest had a mutant (G/A or A/A) VKORC1 genotype. In the warfarin group, based on the genotype, 51.5% of the patients were extensive or normal metabolizers, and 47.4% of the patients were intermediate metabolizers of VKAs. In the acenocoumarol group, 61.2% of the patients were extensive or normal metabolizers, and 38.8% of the patients were intermediate metabolizers. Individually, alleles of VKORC1 (− 1639 G > A), CYP2C9*2, and CYP2C9*3 had mean dosage reduction effect on VKA dosage, which co-related to the clinically stabilized dosages (P < 0.0001). Among the VKORC1 (− 1639 G > A) cohort, the reduction in warfarin mean weekly dosage was 13.48 mg as compared to the wild-type category (P < 0.0001) and similarly, the reduction in the mean weekly acenocoumarol dose was 6.07 mg (P < 0.03) as compared to the wild type after adjusting for age, gender, and body mass index. Conclusion: Single nucleotide polymorphism in the CYP2C9 gene and in the VKORC1 gene is present in nearly 40% of Indian patients. VKORC1 (− 1639 G > A), CYP2C9*2, and CYP2C9*3 genotypes have significant dosage-lowering effects on VKA-based anti-coagulation therapy. The trend in estimated dosages of VKAs co-related to that of observed the clinically stabilized dosage in the cohort. The pharmacogenomic calculators used in this study tend to overestimate the VKA dosages as compared to clinical dosage due to the limitations in the algorithms and in our study. A new algorithm based on a larger dataset capturing the vast genetic variability across the Indian population and relevant clinical factors could provide better results. [ABSTRACT FROM AUTHOR]

The arrangement of aortic and pulmonary pathways is extremely variable in the hearts with a common arterial trunk. Almost always, interruption of the aortic arch is seen in the setting of hypoplasia of the ascending aorta and dominance of the pulmonary circulation. This subset poses substantial challenges in surgical repair and portends poor outcomes. In this report, we briefly describe the technique of ascending aorta reconstruction and other aspects of the surgical repair of this rare malformation. [ABSTRACT FROM AUTHOR]

Bulboventricular foramen (BVF) enlargement is often required to enlarge a restrictive interventricular communication in patients with univentricular hearts (UVH) to prevent the development of systemic ventricular outflow tract obstruction (SVOTO). We describe an alternative surgical technique through the transected pulmonary artery without an atriotomy, ventriculotomy or aortotomy that was successfully performed in a patient with double inlet left ventricle (DILV) with malposed great arteries (MPGA) and a restrictive BVF. [ABSTRACT FROM AUTHOR]

In this report, we describe a 3-year-old patient with a functionally univentricular heart (UVH), who had a combination of double outlet right ventricle (DORV) along with an unrouteable interventricular communication (VSD), severe infundibular and pulmonary valvar stenosis, and severe left pulmonary artery (LPA) ostial stenosis. This patient also had an interrupted inferior caval vein (IVC) with bilateral superior caval veins (SVC). We were able to undertake a successful Kawashima procedure with interruption of the antegrade pulmonary blood flow, reconstructing the LPA using a pedicled roll of the left atrial appendage (LAA). [ABSTRACT FROM AUTHOR]

In this review, we discuss various patch materials used for reconstruction of the right ventricular outflow tract. Their relative merits and demerits are discussed. Traditional patches and their results are detailed along with a brief description of newer developments in the field. [ABSTRACT FROM AUTHOR]

Objective: The bidirectional Glenn (BDG) procedure is a step in multistage palliation of univentricular heart (UVH). We aimed to report the factors determining the outcomes following BDG.Methods: Two hundred fifteen consecutive patients, 5.29 ± 5 years (range 1 month to 38 years, median 3 years) of age, weighing 13 ± 8.8 kg (range 2.6 to 51 kg, median 10 kg) with variable forms of UVH underwent BDG from 2003 to 2013. Their clinical records were reviewed retrospectively.Results: The most common anatomic diagnoses were tricuspid atresia (n = 87, 40.5%) and double outlet right ventricle (n = 78, 36%). Dextrocardia was present in 21 (9.86%) patients. Median left pulmonary (PA) and right PA diameters were 6 and 7 mm, respectively. One hundred sixty-two (77%) patients received unilateral BDG, and 45 had bilateral BDG. The antegrade pulmonary blood flow was closed in 199 and was left open in 16 patients. Concomitant procedures were reconstruction of pulmonary arteries for non-confluent PA (n = 28), atrial septectomy (n = 15), atrioventricular valve repair (n = 12) and repair of partial anomalous pulmonary venous connection (n = 1). A total of 37% of patients (n = 80) had a mean post-operative saturation of 90 ± 3.2%. There were four (1.86%) early deaths. Mean Glenn pressure was 14.7 ± 3.5 mm Hg, and mean inotropic score and Vasoactive inotropic score (VIS) were 1.64 ± 0.96 and 2.77 ± 2.63, respectively. Mean intensive care unit stay was 24.1 ± 26.4 (range 10-240) h, and mean duration of hospital stay was 7.15 ± 3.2 days. Mean saturation at the time of discharge was 92.4 ± 2.2% and on follow-up was 82 ± 2.16%. Follow-up cardiac catheterization data was available in 123 (60.3%). Sixty-nine (33.8%) patients underwent completion Fontan, and 135 patients were in follow-up or waiting for Fontan completion.Conclusion: BDG procedure can be performed safely with acceptable mortality. Age at presentation, pulmonary artery size and VIS were not related to mortality. Younger patients had similar outcomes but a longer hospital stay. Patients with preserved antegrade pulmonary blood flow had higher saturations. Those undergoing BDG without cardiopulmonary bypass had lower inotropic scores. [ABSTRACT FROM AUTHOR]

Background: Extracardiac Fontan (ECF) is currently the final operation of choice for patients with a univentricular heart. Performing this procedure without cardiopulmonary bypass (CPB) carries potential benefits. In this study, we report the early results of ECF without CPB.Patients and methods: Between 2012 and 2015, 72 consecutive patients underwent Fontan without CPB. Their medical records were examined in detail.Results: Mean age was 11.8 ± 5.2 (range 5 to 23, median 10) years. Intraoperative mean superior vena cava clamp time was 15.19 ± 3.8 min, and the inferior vena cava clamp time was 16.93 ± 3.31 min. There were three early deaths. No patient required conversion from off-CPB to CPB. Mean inotropic score was 4.73 ± 5.9 (range 0 to 25, median 2.5). Mean time to extubation was 9.5 ± 5.82 (range 3 to 29, median 8) hours. Pleural drainage in intensive care unit (ICU) was 551.57 ± 452.77 (median 470) ml, and mean ICU stay was 2.27 ± 3.09 (median 1.5) days. Mean daily pleural drainage after discharge from the ICU was 163.7 ± 88.01 (median 140) ml, and mean time to removal of pleural tubes was 15.76 ± 8.4 (median 14) days. Total hospital stay was 17.03 ± 8.62 (median 15) days. At an early follow-up of 2-40 (median 25) months, all survivors (n = 69) had a patent Fontan circuit with normal ventricular function on echocardiography. There were no late deaths or thromboembolic complications.Conclusions: Off-pump ECF is a low-risk procedure that avoids the harmful effects of CPB. Post-operative course of these patients is predictable with substantial savings in costs. [ABSTRACT FROM AUTHOR]

The commonly used technique to facilitate intracardiac exposure during transatrial repair of tetralogy of fallot involves considerable retraction of the tricuspid valve using retractors. We describe an alternative surgical technique in which it is possible to dispense away with the retractors. The advantages of such a technique are discussed. [ABSTRACT FROM AUTHOR]

Surgical strategies in patients with functionally or anatomically borderline right ventricles include a high-risk biventricular repair, a Fontan procedure, or a one and half ventricle repair (also referred to as the partial biventricular repair). One and half ventricle repair (1.5VR) circumvents the high early mortality of a biventricular repair and also the late morbidity of the Fontan. The two most common indications for a 1.5VR are a small pulmonary ventricle and a dilated poorly functioning pulmonary ventricle. Extension of 1.5VR to patients undergoing anatomical repair for congenitally corrected transposition of great arteries, straddling tricuspid valves, and severe Ebstein’s anomaly has facilitated biventricular repair with decreased mortality. We reviewed the relevant literature on this subject in detail and describe its rationale, indications and its early and late results. [ABSTRACT FROM AUTHOR]

Objectives: The Fontan operation is usually followed by significant pleural effusion. We aimed to study the factors associated with persistent pleural effusion with special reference to serum cortisol levels.Patients and methods: Thirty-eight patients undergoing the Fontan operation between September 2015 and November 2016 were prospectively studied. Parameters studied included age, weight, symptoms, atrio- ventricular valve regurgitation/stenosis/atresia, ventricular function, pulmonary artery pressures, oxygen saturation, aorto-pulmonary, and veno-venous collaterals, type of Fontan, duration of cardiopulmonary bypass, need for inotropes, duration of mechanical ventilation, conduit size, presence or absence of fenestration, and serum cortisol levels. The latter were measured before and after the Fontan operation and the co-relation between pleural effusion and change in serum cortisol levels was studied.Results: Mean age at operation was 13.1 ± 5.6 years (median 13 years). Mean duration and amount of pleural drainage was 15.76 ± 13.2 days (median 11.5 days) and 9.15 ± 4.6 mL/kg/day (median 9 mL/kg/day) respectively. Statistically significant risk factors for prolonged pleural effusion were higher pulmonary artery (PA) pressures (r = 0.328, p = 0.003, odds ratio 1.30), higher inotropic score (r = 0.4, p = 0.01), lower rise in serum cortisol (p = 0.03),elevated superior caval venous pressure (CVP) at 6 h (r = 0.44, p = 0.005) and 12 h (r = 0.4, p = 0.01) and higher duration of mechanical ventilation (r = 0.45, p = 0.005).Conclusions: PA pressures > 15 mmHg, higher inotropic score, higher CVP and lower rise in serum cortisol levels following the Fontan operation were associated with persistent pleural effusion. [ABSTRACT FROM AUTHOR]

We report a 15-year-old female patient with an unusual combination of discreet subaortic membrane, aortopulmonary window, severe aortic insufficiency and rheumatic mitral regurgitation. [ABSTRACT FROM AUTHOR]

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. [ABSTRACT FROM AUTHOR]

We report a 3-month-old male presenting with multiple aortic aneurysms arising de novo 2 months following the arterial switch operation. Successful repair of the aneurysms was performed under total circulatory arrest and at seven years follow-up, the patient has no recurrence. [ABSTRACT FROM AUTHOR]

Background : The optimal timing, need for primary/staged procedure in patients undergoing univentricular palliation, is debatable. Aims : We performed this study to assess the exercise performance of patients undergoing various forms of univentricular palliation. Setting and Design : This was a retrospective, prospective comparative study conducted at a multispecialty tertiary referral center. Patients and Methods : Between January 2012 and June 2015, 117 patients undergoing either bidirectional Glenn (BDG) (n = 43) or Fontan (total cavopulmonary connection [TCPC]) (n = 74) underwent exercise testing. Statistical Analysis : Comparisons between subgroups for continuous data were made with Student's t-test if normally distributed and Wilcoxon rank-sum test otherwise. Tests between subgroups for qualitative data were made with Pearson's Chi-square test. Results : Patients who underwent BDG with open antegrade pulmonary blood flow (APBF) had higher saturations (oxygen saturation [SpO2]) compared to those without it (87.5 ± 5.0% vs. 81.1 ± 4.8%; P = 0.0001). However, we found no differences in exercise parameters of patients undergoing BDG with or without APBF. Extracardiac TCPC (n = 42) patients demonstrated better exercise capacity (15.0 ± 7.7 vs. 11.2 ± 6.2 min; P = 0.02) and increased SpO2 on exercise (87.0 ± 8.0% vs. 83.4 ± 7.6%; P ≤ 0.05) compared to lateral tunnel TCPC (n = 32). Fenestrated TCPC (n = 30) patients had higher exercise capacity reflected by higher metabolic equivalents (METs) consumption (6.4 ± 2.3 vs. 5.2 ± 2.0 METs, P = 0.02), fewer pleural effusions (7.0 ± 3.2 vs. 9.2 ± 6.2 days, P ≤ 0.05), and lower hospital stay (9.5 ± 4.0 vs. 12.7 ± 7.7 days, P = 0.04) compared to nonfenestrated TCPC (n = 44) patients. Conclusions : We observed no differences in exercise parameters of patients undergoing BDG with or without APBF. Extracardiac TCPC patients had better exercise capacity but longer postoperative hospital stay and pleural effusions than patients with lateral tunnel Fontan. Fenestrated TCPC patients seemed to fare better than nonfenestrated ones. Patients undergoing TCPC had better exercise capacity than patients undergoing BDG alone. [ABSTRACT FROM AUTHOR]

We report a 50-day-old infant with infective endocarditis and a large fungal vegetation on the tricuspid valve (TV) obstructing the right ventricular inflow. Emergency excision of the vegetation and TV reconstruction failed. Because of failure to wean off cardiopulmonary bypass support, a rescue bidirectional superior cavopulmonary anastomosis (BDG) was performed. Various management options with appropriateness and efficacy of this procedure are discussed. [ABSTRACT FROM AUTHOR]

Background: We reviewed the long-term results of surgery for discrete subaortic membrane (SubAM) from a single institute.Methods: A retrospective review of medical records of all patients (n = 146) who underwent resection of a SubAM for discrete subaortic stenosis between 1990 and 2015 at the All India Institute of Medical Sciences, New Delhi, India was undertaken.Results: Median age at surgery was 9.0 years (9 months-47 years). There was one early death. Preoperative peak left ventricular outflow tract (LVOT) Doppler gradient was 83.4 ± 26.2 mmHg (range: 34-169 mmHg). On preoperative echocardiography, aortic regurgitation (AR) was absent in 69 (47.3%), mild in 35 (24%), moderate in 30 (20.5%), and severe in 12 (8.2%). After surgery, the LVOT gradient was reduced to 15.1 ± 6.2 mmHg (P < 0.001). Fourteen patients (9.6%) who had residual/recurrent significant gradients are currently being followed-up or awaiting surgery. There was improvement in AR for operated patients with freedom from AR of 92.6 ± 0.03% at 15 years. Kaplan-Meier survival at 25 years was 93.0 ± 3.9% (95% confidence interval: 79.6, 97.7). Freedom from re-operation at 25 years was 96.9 ± 1.8%.Conclusions: Long-term results of surgery for discrete SubAM are good. Resection of the membrane along with septal myectomy decreases the risk of recurrence. [ABSTRACT FROM AUTHOR]

Objectives: This study aims to compare the bidirectional superior cavopulmonary anastomosis (BDG) with or without cardiopulmonary bypass (CPB).Methods: 100 patients undergoing BDG were randomized into two groups: Off-CPB or on-CPB groups. All patients underwent near-infrared spectrophotometry (NIRS) and bispectral index (BIS) monitoring and pre- and postoperative serum 100 beta protein measurements (Sβ100) and neuro-cognitive evaluation. Postoperative intensive care unit (ICU) parameters were also studied.Results: The median age of patients in the on-CPB and off-CPB group were 42 and 48 months, respectively (p = 0.11). Median weights in the on-CPB group and off-CPB group were 13.5 (5-50) kg and 15 (7-36) kg, respectively (p = 0.927). There was a significant rise in superior vena cava (SVC) pressure on SVC clamping in the off-CPB group (23.12 ± 6.84 vs 2.98 ± 2.22 mmHg) on-CPB group (p < 0.001). There was a significant fall in NIRS and BIS values from baseline in the off-CPB group during the anastomosis but there was no statistically significant change in serum Sβ100from pre-clamp to post-clamp in either group. Inotropic support, duration of ventilation, ICU stay, and hospital stay were significantly less in the off-CPB group (p < 0.001). Assessment of Social Adaptive Functioning revealed no adverse sequelae. There were significant cost savings if surgery was performed off-CPB (p < 0.001).Conclusion: Off CPB-BDG is an economical and safe procedure. Duration of inotropic and mechanical ventilatory support, ICU, and hospital stay is significantly less. We did not observe any early adverse neurologic sequelae in patients undergoing off-CPB BDG. [ABSTRACT FROM AUTHOR]

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. [ABSTRACT FROM AUTHOR]

Background: The aim of this study is to evaluate the causal relation between hospital-acquired infection (HAI) and clinical outcomes following cardiac surgery in neonates and infants and to identify the risk factors for the development of HAI in this subset of patients. Materials and Methods: After Ethics committee approval, one hundred consecutive infants undergoing open heart surgery (OHS) between June 2015 and June 2016 were included in this prospective observational study. Data were prospectively collected. The incidence and distribution of HAI, the microorganisms, their antibiotic resistance and patients' outcome were determined. The Centers for Disease Control and Prevention criteria were used for defining HAIs. Univariate and multivariate risk factor analysis was done using Stata 14. Results: Sixteen infants developed microbiologically documented HAI after cardiac surgery. Neonatal age group was found to be most susceptible. Lower respiratory tract infections accounted for majority of the infections (47.4%) followed by bloodstream infection (31.6%), urinary tract infection (10.5%), and surgical site infection (10.5%). Klebsiella (36.8%) and Acinetobacter (26.3%) were the most frequently isolated pathogens. HAI was associated with prolonged ventilation duration (P = 0.005), Intensive Care Unit stay (P = 0.0004), and hospital stay (P = 0.002). Multivariate risk factor analysis revealed that preoperative hospital stay (odds ratio [OR] 1.22, 95% confidence interval (CI) 1.6-1.39, P = 0.004), and prolonged cardiopulmonary bypass (CPB) (OR 1.03, 95% CI 1.01-1.05, P = 0.001) were associated with the development of HAI. Conclusion: HAI still remains a dreaded complication in infants after OHS and contributing to morbidity and mortality. Strategies such as decreasing preoperative hospital stay, CPB time, and early extubation should be encouraged to prevent HAI. [ABSTRACT FROM AUTHOR]

Although the safety of paediatric heart surgery has been established, neurologic injuries are still a major source of comorbidity and mortality. The intra-uterine hypoxic milieu coupled with abnormal circulation poses threat to the developing brain. Neurologic injuries vary depending on the level and extent of injury as well as the aetiology, which may be multifactorial. Radiologic signs are late. Various biochemical markers and neuromonitoring modalities enable prediction of neuronal damage. Neuroprotection strategies in these children aim at skilful preoperative assessment, optimal cardiopulmonary bypass strategies, early detection and correction of metabolic parameters. In view of the complex mechanisms and multiple factors involved in neurologic insults, a thorough understanding of pathophysiology and meticulous attention to the details is required to prevent the same. [ABSTRACT FROM AUTHOR]