Rare associations of tetralogy of Fallot with anomalous left coronary artery from pulmonary artery and totally anomalous pulmonary venous connection.

We describe the cases of two patients with tetralogy of Fallot, aged 4 years and 8 months, who were incidentally detected to have concomitant anomalous left coronary artery from pulmonary artery and total anomalous pulmonary venous connection, respectively, on preoperative imaging. They underwent surgical correction with good mid-term outcomes. In this study, we discuss the embryological basis, physiological effects, and review the literature of these two unusual associations. Awareness of these rare associations will avoid missed diagnoses and consequent surgical surprises.

A single center, retrospective analysis of total anomalous pulmonary venous connection repair early outcome at a tertiary care center in India.

Context: In recent years, increasing awareness and early detection has made total anomalous pulmonary venous connection (TAPVC) a relatively common congenital heart condition presenting to children's heart centers in India. The condition was associated with significant morbidity and mortality in the past due to various reasons. Improvement in perioperative management has markedly changed the outcomes of TAPVC even in a developing country. Material and Methods: All patients with TAPVC operated between June 2013 and February 2018 at our center were included in the study. Post repair 30-days mortality and morbidity were analyzed. Results: A total of 166 patients were divided into supracardiac (91), infracardiac (45), cardiac (18), and mixed type (12). It also divided our cohort into obstructed and unobstructed types. The duration of inotrope usage in the obstructed group was significantly higher compared to the unobstructed group. Statistically, significant difference was noticed for the duration of ventilation 85.17 ± 80.94 h in obstructed type versus 49.23 ± 60.7 h in the unobstructed group, and in ICU stay (days) in obstructed (9.64 ± 5.96) and unobstructed group (6.29 ± 5.12). The morbidity parameters such as duration of an inotrope, ventilation, and length of ICU stay had a negative correlation between body surface area (BSA) of the patient but no correlation in respect to duration of CPB and ACC time. Mortality was found to be higher in lower BSA, infracardiac type (7/9), and obstructed variants of TAPVC (9/9) patients. Conclusions: Longer duration of inotrope usage, mechanical ventilation, and ICU stay were seen in obstructed TAPVC in comparison to unobstructed TAPVC patients. Duration of CPB or aortic cross-clamp had no effects on morbidity parameters. In our cohort of TAPVC patients, lower BSA was strongly associated with the longer requirement of inotropes, prolong ventilation time, and ICU stay. The risk factors for mortality in our study include lower BSA, infracardiac, and obstructed type of TAPVC.

Outcome of COVID-19-positive children with heart disease and grown-ups with congenital heart disease: A multicentric study from India.

BACKGROUND: Outcome data of children with heart disease who acquired COVID-19 infection are limited. AIMS: We sought to analyze outcome data and identify risk factors associated with mortality in children with heart disease and grown-ups with congenital heart disease (GUCH) who had a laboratory-confirmed COVID-19 infection. SETTINGS AND DESIGN: This is a retrospective, multicentric, observational study. MATERIALS AND METHODS: The study included children with heart disease and GUCH population, who presented with either symptomatic or asymptomatic COVID-19 infection to any of the participating centers. COVID-19-negative patients admitted to these centers constituted the control group. RESULTS: From 24 pediatric cardiac centers across India, we included 94 patients with a median age of 12.5 (interquartile range 3-96) months and 49 (52.1%) patients were males. Majority (83 patients, 88.3%) were children. One-third of the patients (n = 31, 33.0%) had acyanotic congenital heart disease, and 41.5% (n = 39) were cyanotic, with > 80% of the patients being unoperated. Only 30 (31.9%) patients were symptomatic for COVID-19 infection, while the rest were incidentally detected positive on screening. A total of 13 patients died (case fatality rate: 13.8%). The in-hospital mortality rate among hospitalized patients was significantly higher among COVID-19-positive cases (13 of 48; 27.1%) as compared to COVID-negative admissions (9.2%) during the study period (P < 0.001). On multivariate analysis, the independent predictors of mortality among COVID-19-positive cases were severity of illness at admission (odds ratio [OR]: 535.7, 95% confidence interval [CI]: 6.9-41,605, P = 0.005) and lower socioeconomic class (OR: 29.5, 95% CI: 1.1-814.7, P = 0.046). CONCLUSIONS: Children with heart disease are at a higher risk of death when they acquire COVID-19 infection. Systematic preventive measures and management strategies are needed for improving the outcomes.

Impact of COVID-19 pandemic on pediatric cardiac services in India.

BACKGROUND: COVID-19 pandemic has disrupted pediatric cardiac services across the globe. Limited data are available on the impact of COVID.19 on pediatric cardiac care in India. AIMS: The aims are to study the impact of COVID-19 pandemic on the care of children with heart disease in India in terms of number of outpatient visits, hospitalizations, catheter-based interventions, and cardiac surgeries. SETTINGS AND DESIGN: This is a retrospective, multicentric, observational study. METHODS: We collected monthly data on the number and characteristics of outpatient visits, hospitalizations, catheter-based interventions, and cardiac surgeries and major hospital statistics, over a period of 5 months (April to August 2020), which coincided with the first wave of COVID-19 pandemic in India and compared it with data from the corresponding months in 2019. RESULTS: The outpatient visits across the 24 participating pediatric cardiac centers decreased by 74.5% in 2020 (n = 13,878) as compared to the corresponding period in 2019 (n = 54,213). The reduction in the number of hospitalizations, cardiac surgeries, and catheterization procedures was 66.8%, 73.0%, and 74.3%, respectively. The reduction in hospitalization was relatively less pronounced among neonates as compared to infants/children (47.6% vs. 70.1% reduction) and for emergency surgeries as compared to elective indications (27.8% vs. 79.2%). The overall in-hospital mortality was higher in 2020 (8.1%) as compared to 2019 (4.8%), with a higher postoperative mortality (9.1% vs. 4.3%). CONCLUSIONS: The current COVID-19 pandemic significantly impacted the delivery of pediatric cardiac care across India with two-third reduction in hospitalizations and cardiac surgeries. In an already resource-constrained environment, the impact of such a massive reduction in the number of surgeries could be significant over the coming years. These findings may prove useful in formulating strategy to manage subsequent waves of ongoing COVID-19 pandemic.

Single-Stage Repair of Interrupted Aortic Arch With Aortopulmonary Window and Bovine-Type Aortic Arch Complicated by Left Subclavian Artery Origin Stenosis-A Case Report.

An aortopulmonary window is known to be associated in 5% of interrupted aortic arch cases. The combination of these lesions with a bovine-type aortic arch is much more uncommon. We report its embryological explanation and successful single-stage surgical repair in a three-month-old infant.

Indian Guidelines for Indications and Timing of Intervention for Common Congenital Heart Diseases: Revised and Updated Consensus Statement of the Working Group on Management of Congenital Heart Diseases. Abridged Secondary Publication.

JUSTIFICATION: A number of guidelines are available for management of congenital heart diseases from infancy to adult life. However, these guidelines are for patients living in high income countries. Separate guidelines, applicable to Indian children, are required when recommending an intervention for congenital heart diseases, as often these patients present late in the course of the disease and may have co-existing morbidities and malnutrition. PROCESS: Guidelines emerged following expert deliberations at the National Consensus Meeting on Management of Congenital Heart Diseases in India, held on 10th and 11th of August 2018 at the All India Institute of Medical Sciences, New Delhi. The meeting was supported by Children's HeartLink, a non-governmental organization based in Minnesota, USA. OBJECTIVES: To frame evidence based guidelines for (i) indications and optimal timing of intervention in common congenital heart diseases; (ii) follow-up protocols for patients who have undergone cardiac surgery/catheter interventions for congenital heart diseases. RECOMMENDATIONS: Evidence based recommendations are provided for indications and timing of intervention in common congenital heart diseases, including left-to-right shunts (atrial septal defect, ventricular septal defect, atrioventricular septal defect, patent ductus arteriosus and others), obstructive lesions (pulmonary stenosis, aortic stenosis and coarctation of aorta) and cyanotic congenital heart diseases (tetralogy of Fallot, transposition of great arteries, univentricular hearts, total anomalous pulmonary venous connection, Ebstein anomaly and others). In addition, protocols for follow-up of post surgical patients are also described, disease wise.

Indian guidelines for indications and timing of intervention for common congenital heart diseases: Revised and updated consensus statement of the Working group on management of congenital heart diseases.

A number of guidelines are available for the management of congenital heart diseases (CHD) 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 CHD, as often these patients present late in the course of the disease and may have coexisting morbidities and malnutrition. Guidelines emerged following expert deliberations at the National Consensus Meeting on Management of Congenital Heart Diseases in India, held on August 10 and 11, 2018, at the All India Institute of Medical Sciences. The meeting was supported by Children's HeartLink, a nongovernmental organization based in Minnesota, USA. The aim of the study was to frame evidence-based guidelines for (i) indications and optimal timing of intervention in common CHD; (ii) follow-up protocols for patients who have undergone cardiac surgery/catheter interventions for CHD; and (iii) indications for use of pacemakers in children. Evidence-based recommendations are provided for indications and timing of intervention in common CHD, including left-to-right shunts (atrial septal defect, ventricular septal defect, atrioventricular septal defect, patent ductus arteriosus, and others), obstructive lesions (pulmonary stenosis, aortic stenosis, and coarctation of aorta), and cyanotic CHD (tetralogy of Fallot, transposition of great arteries, univentricular hearts, total anomalous pulmonary venous connection, Ebstein's anomaly, and others). In addition, protocols for follow-up of postsurgical patients are also described, disease wise. Guidelines are also given on indications for implantation of permanent pacemakers in children.

Guidelines for the management of common congenital heart diseases in India: A consensus statement on indications and timing of intervention.

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

Malnutrition in children with congenital heart disease (CHD) determinants and short term impact of corrective intervention.

OBJECTIVE: To identify determinants of malnutrition in children with congenital heart disease (CHD) and examine the short-term effects of corrective intervention. METHODS: Patients with CHD admitted for corrective intervention were evaluated for nutritional status before and 3 months after surgery. Detailed anthropometry was performed and z-scores calculated. Malnutrition was defined as weight, height and weight/height z-score or= 2 children; height z-score

Native aortic coarctation in neonates and infants: Immediate and midterm outcomes with balloon angioplasty and surgery.

BACKGROUND: Native aortic coarctation in young infants can be treated surgically or with balloon angioplasty, although the latter approach remains controversial. We sought to compare midterm outcomes of balloon and surgical coarctoplasty at our center and to review the current practices in literature. METHODS: The study design was a retrospective record review. Patients with coarctation aged <1 year who underwent balloon or surgical coarctoplasty at our center (January 2010-January 2016) with >6-month post-procedure follow-up were studied. Patients with significant arch hypoplasia or interruption, long-segment coarctation, and associated intracardiac lesions were excluded from the study. Clinical, echocardiographic, and procedural details were recorded at initial presentation and follow-up. Need for reintervention was noted. RESULTS: Out of a total of 75 patients, 28 underwent balloon (Group A) and 47 underwent surgical coarctoplasty (Group B). There were two deaths in Group B (mortality 4.2%) and none in Group A. In neonates, 63.6% of Group A and 17.4% of Group B patients required reintervention (P = 0.007). Beyond the neonatal age, 29.4% of Group A and 41.6% of Group B patients required reintervention (not statistically significant). On univariate analysis, residual coarctation on predischarge echocardiogram was the only significant predictor of reintervention (P = 0.04). On Kaplan-Meier analysis, with neonatal presentation, freedom from reintervention in Group B was significantly higher than Group A (P = 0.028). CONCLUSION: In neonates with native aortic coarctation, surgery unequivocally yields better immediate and midterm results and is the preferred treatment modality. In older infants, balloon coarctoplasty has good early and midterm outcomes with acceptable reintervention rates and should be considered a viable alternative to surgery, especially in critical patients with high surgical risk.

Neonates with critical congenital heart defects: Impact of fetal diagnosis on immediate and short-term outcomes.

BACKGROUND: Fetal echocardiography is being increasingly used for prenatal diagnosis of congenital cardiac malformations, but its impact on the neonatal outcomes in low- and middle-income countries is still unknown. AIMS: The objective of this study is to determine the impact of fetal echocardiography on immediate postnatal and short-term outcome in a tertiary pediatric cardiac center. STUDY DESIGN: This is a prospective study. MATERIALS AND METHODS: One hundred consecutive patients with critical congenital heart defects (CHD) requiring active medical or surgical interventions in the 1st month of life were included in the study. The detailed history, postnatal examination findings, and fetal echocardiogram report were recorded. They were divided into two groups as antenatally diagnosed and postnatally diagnosed. Pre- and post-procedural variables were compared between the two groups. RESULTS: Twenty-nine neonates were diagnosed antenatally while 71 were diagnosed postnatally. Totally, 10 babies (34.5%) among the antenatally diagnosed group were delivered in a tertiary health-care setup. The mean age at presentation was 0 day in the antenatally diagnosed group while 10 days (0-30 days) in the postnatally diagnosed group (P = 0.01). A total of 17 (58.6%) patients in the antenatal group had duct dependent CHD, and 15 (88.2%) of these patients were transported on prostaglandin E1. In comparison, 19/34 (55.9%) patients in the postnatal group were transported on prostaglandin. The pH on admission in the antenatal group was 7.32 ± 0.05 as compared to 7.28 ± 0.05 in the postnatal group (P = 0.0004). There were 4 (5.6%) deaths in the postnatal group during transfer. There was no significant difference in the postoperative variables in both groups. CONCLUSIONS: Fetal echocardiography identifies patients with complex CHD resulting in better parental counseling, thus facilitating delivery at a tertiary care center and preoperative stabilization. This results in improved preoperative mortality and better stabilization.

Isolated Subclavian Artery: A Rare Entity Revisited.

Isolation of the subclavian artery is a rare aortic arch anomaly with fewer than 100 cases reported in the literature. Left subclavian artery isolation is seen in 0.8% of right aortic arches, and right subclavian artery isolation is seen four times less frequently. Other intracardiac or arch malformations are usually associated, among which tetralogy of Fallot is commonly described. We describe the presentation, preoperative imaging, surgical strategies, and outcomes of four patients with this rare condition and review the published literature. The embryological basis and interesting pathophysiology are also discussed. Surgical division and reimplantation of the isolated subclavian artery onto the aortic arch or common carotid artery is the treatment of choice, with good immediate and midterm outcomes.

Effect of dexmeditomidine on postoperative junctional ectopic tachycardia after complete surgical repair of tetralogy of Fallot: A prospective randomized controlled study.

INTRODUCTION: Incidence of junctional ectopic tachycardia (JET) after repair of tetralogy of Fallot (TOF) is 5.6-14%. Dexmeditomidine is a a-2 adrenoceptor agonist modulates the release of catecholamine, resulting in bradycardia and hypotension. These effects are being explored as a therapeutic option for the prevention of perioperative tachyarrhythmia. We undertook this study to examine possible preventive effects of dexmedetomidine on postoperative JET and its impact on the duration of ventilation time and length of Intensive Care Unit stay. METHODS: After obtaining approval from the hospitals ethics committee and written informed consent from parents, this quasi-randomized trial was initiated. Of 94 patients, 47 patients received dexmedetomidine (dexmedetomidine group) and 47 patients did not receive the drug (control group). RESULTS: Dexmedetomidine group had more number of complex variants like TOF with an absent pulmonary valve or pulmonary atresia (P = 0.041). Hematocrit on cardiopulmonary bypass (CPB), heart rate while coming off from CPB and inotrope score was significantly low in the dexmedetomidine group compared to control group. The incidence of JET was significantly low in dexmedetomidine group (P = 0.040) compared to control group. CONCLUSIONS: Dexmedetomidine may have a potential benefit of preventing perioperative JET.

Management of infants with large, unrepaired ventricular septal defects and respiratory infection requiring mechanical ventilation.

OBJECTIVES: We sought to describe the hospital management and early outcome of critically ill infants presenting with large ventricular septal defects and pneumonia requiring mechanical ventilation at a referral center in a developing country. Infants with large ventricular septal defects who have pneumonia might present with respiratory failure requiring mechanical ventilation. In the developing world this presentation is relatively common, but few data exist describing patient management strategies. METHODS: Hospital data of consecutive infants admitted with large ventricular septal defects and pneumonia requiring mechanical ventilation were reviewed and analyzed. RESULTS: We identified 18 infants (mean age, 3.6 +/- 3.0 months). On admission, all the infants were significantly malnourished, and echocardiography showed bidirectional shunting (predominantly right-to-left shunting) in 6 infants. Thirteen (72%) patients improved with intensive medical management that included mechanical ventilation for 1 to 16 days (median, 6.5 days); unequivocal left-to-right shunting was subsequently documented by means of echocardiography in all 13 patients. Twelve patients underwent surgical repair, and 11 (91.6%) were discharged after median mechanical ventilation of 100 hours (range, 42-240 hours) and intensive care unit stay of 8 days (range, 4-15 days). Five of 6 unoperated patients died, 4 of them within a few hours of admission. One child with multiple ventricular septal defects was discharged and subsequently underwent pulmonary artery banding. CONCLUSION: Corrective cardiac surgery for selected critically ill infants with large ventricular septal defects, severe malnutrition, and pneumonia requiring mechanical ventilation is feasible and should be considered a viable management strategy.

Closure of muscular ventricular septal defects guided by en face reconstruction and pictorial representation.

BACKGROUND: A surface reconstruction of the location and dimensions of muscular ventricular septal defects (VSDs) on right ventricular (RV) septal surface could serve as a better guide to surgical closure amid different classifications and confusing terminologies. METHODS: We reconstructed muscular VSD requiring surgery on an en-face view of the RV septal surface from echocardiographic orthogonal views in 34 consecutive patients. The location, dimensions of the defects, and relation to various RV septal landmarks are illustrated as a diagram. Recommendations are presented regarding surgical approach to the defects, along with predictions on the possibility of residual defects and heart block. RESULTS: Surgical findings were as predicted by the diagram in the 27 patients who underwent VSD closure. Seven infants (2.5 to 4.9 kg) underwent pulmonary artery (PA) banding based on predictions of heart block or major residual defects. Two patients with predicted risk of heart block underwent VSD closure with heart block ensuing in one of them. Based on the diagram limited ventriculotomy (n = 2) or detachment of tricuspid leaflets (n = 6) aided access to the VSD. Among patients undergoing VSD closure only 1 patient had a major residual defect that required PA banding. There were clinically insignificant residual defects in 8 patients. Four patients (12%) were anticipated preoperatively because of surgical inaccessibility and intentionally left alone. CONCLUSIONS: En-face reconstruction of single or multiple muscular VSDs is feasible from orthogonal echocardiographic views. It helps plan the surgical approach and predict the likelihood of heart block and residual defects after surgery.

Surgical experience of total anomalous pulmonary venous connection with mid-term follow-up in a developing country.

OBJECTIVE: To illustrate the incidence, the different age group, varied clinical presentation, incremental risk factors for surgery and follow-up results from this part of the world. EXPERIMENTAL DESIGN: Retrospective study with follow-up from 3 months to 5 years. SETTING: Institutional practice with hospitalised care. SELECTION PROCEDURE: Hospitalised patients with a diagnosis of total anomalous pulmonary venous connection. INTERVENTIONS: All patients who were diagnosed to have total anomalous pulmonary venous connection underwent corrective surgery. RESULTS: There were 20 hospital deaths postoperatively. The mean follow-up was 1.9 year. Logistic regression analysis identified age group, timing of surgery, associated anomalies preoperative symptoms, anatomical type of lesion, pulmonary artery hypertension, duration of ventilation, low cardiac output as risk factors for surgery. The mean survival time was 4-8 years. CONCLUSION: Mortality continues to be higher from this part of the world due to severe malnourishment, late presentation. Sociodemographic factors also play a major role in affecting surgical outcome apart from the identified risk factors for surgery.

Limited right posterior thoracotomy approach to atrial septal defect.

Experience of atrial septal defect closure via a limited posterior thoracotomy is described. From July 1999 to May 2001, 75 prepubertal girls with a median age of 7 years (range, 3 to 13 years) and a median weight of 18 kg (range, 10 to 46 kg) underwent atrial septal defect closure through a limited right posterior thoracotomy. All but 2 patients had an uneventful postoperative recovery. The median duration of ventilation was 13.3 hours (range, 4 to 24 hours). Median hospital stay was 6 days (range, 6 to 8 days). All patients were followed up for 7 to 32 months (mean, 15 months). The wounds healed well without any restriction of limb movement. The limited posterior thoracotomy gave excellent cosmetic results and can be used as a safe alternative approach for atrial septal defect closure in prepubertal females.