Early and Long-Term Clinical Outcomes of Ductal Stenting Versus Surgical Aortopulmonary Shunt Among Young Infants with Duct-Dependent Pulmonary Circulation.

Surgical aortopulmonary shunting (SAPS) and ductal stenting (DS) are the main palliations in infants with cyanotic congenital heart diseases (CHD). We aimed to study the safety and efficacy of DS and to compare it with SAPS as a palliative procedure in infants with CHD and duct-dependent pulmonary circulation. Retrospective institutional clinical data review of consecutive infants aged < 3 months who underwent DS or SAPS over 5 years. The primary outcome was procedural success which was defined as event-free survival (mortality, need for re-intervention, procedural failure) at 30 days post-procedure. The secondary outcome was defined by a composite of death, major adverse cardiovascular events, or need for re-intervention at 6 months and on long-term follow-up. We included 102 infants (DS, n = 53 and SAPS, n = 49). The median age at DS and SAPS was 4 days (IQR 2.0-8.5) and 8 days (IQR 4.0-39.0), respectively. The median weight at intervention was 3.0 kg (IQR 3.0-3.0) and 3.0 kg (IQR 2.5-3.0) in the two respective arms. Tetralogy of Fallot with pulmonary atresia was the most common indication for DS and SAPS. The 30-day mortality was significantly higher in SAPS group as compared with DS group (p < 0.05). However, 30-day major adverse cardiac events (MACE) rates were similar in both groups (p = 0.29). DS was associated with shorter duration of mechanical ventilation, duration of stay in the intensive care and hospital stay than with SAPS. At 6 months, there was no significant difference in terms of mortality or event-free survival. Long-term MACE-free survival was also comparable (p = 0.13). DS is an effective and safer alternative to SAPS in infants with duct-dependent pulmonary circulation, offering reduced procedure-related mortality and morbidity than SAPS. Careful study of ductal anatomy is crucial to procedural success. However, long-term outcomes are similar in both procedures.

A Lung ultrasound-based comparison of postoperative respiratory outcome after pediatric congenital heart surgery in COVID-19 recovered and COVID-19 unaffected children-a pilot study.

Background: COVID-19 is known to affect the alveolar-capillary membrane and interstitial tissue. Cardiopulmonary bypass (CPB) is proven to cause "pump-lung" syndrome. Little is known about the subgroup of COVID-19-recovered children undergoing cardiac surgeries under CPB. Aim: To compare the lung ultrasound score (LUSS) and morbidity of COVID-19-recovered children to those without COVID-19 after congenital cardiac surgery. Setting and Design: Prospective observational pilot study at a tertiary care institution. Materials and Methods: The study was carried out on 15 COVID-recovered children and 15 matched controls (Group B) posted for congenital cardiac surgery. COVID-recovered children were further divided into those who received in-hospital care for COVID (Group C) and those who did not (Group A). LUSS measured immediately after cardiac surgery was compared between the groups. PaO2/FiO2 ratio, incidence of pulmonary infection, re-intubation rate, duration of ventilation, in-hospital mortality, and 30-day mortality were also compared between the groups. Results: LUSS was comparable between Groups A and B (mean difference = 1.467, P = 0.721). Compared to the control group, Group C showed higher LUSS (mean difference = 11.67, P < 0.001), lower PaO2/FiO2 ratio (207.8 ± 95.49 Vs 357.6 ± 63.82, P = 0.003), and one in-hospital mortality. A significant negative correlation was seen between LUSS and PaO2/FiO2 ratio (rs = -0.75, P < 0.001). Conclusion: LUSS was not higher in COVID-recovered children. Children with a history of severe COVID-19 recorded higher LUSS, in-hospital mortality, duration of ICU stay, and duration of ventilation.

Pancycle aortic incompetence and double alternans in pediatric heart failure.

A 12-year-old boy presented with bicuspid aortic valve, severe aortic regurgitation, and dilated dysfunctional left ventricle in heart failure. He underwent aortic valve replacement with a 23 mm TTK Chitra heart valve prosthesis (tilting disk). He was gradually weaned off milrinone and noradrenaline in the intensive care. Echocardiography showed severe left ventricular dysfunction with an ejection fraction of 24%. The radial pulse was regular and of normal volume but exactly half that of the heart rate. Evaluation of the rhythm and echocardiography revealed an interesting hemodynamic phenomenon with double alternans.

Left cardiac sympathetic denervation in children with Jervell Lange-Nielsen syndrome and drug refractory torsades - A case series.

INTRODUCTION: Long QT syndrome is an inherited malignant channelopathy which leads to life-threatening arrhythmia, with multiple genotypes. Jervell and Lange-Nielsen syndrome (JLNS) is an autosomal recessive subtype of this disease, characterized by congenital sensorineural deafness and a high incidence of sudden cardiac death (SCD). METHODOLOGY: We prospectively followed up six children who underwent left cardiac sympathetic denervation (LCSD) for JLNS in view of high-risk features despite being on maximally tolerated doses of oral propranolol. RESULTS: Mean age at diagnosis was 2.75 ± 0.39 years, with a significant delay between onset of symptoms and diagnosis (mean 7.2 ± 3.5 months). All had sensorineural hearing loss, conforming to the JLNS phenotype. Mean QTc interval was 603 ± 93 ms, with T wave alternans (TWA) seen in all cases. All were started on propranolol and subsequently subjected to LCSD, and 3 underwent AAI permanent pacemaker implantation. Over a mean follow-up of 20 months, there was a significant reduction in QTc (603 ± 93 ms to 501 ± 33 ms, p = .04), which was persistent on follow-up (525 ± 41 ms) and only two out of six had persistent T wave alternans on ECG (p < .01). None of these children had presyncope, syncope, seizures, torsades de pointes, cardiac arrest or death on follow up following LCSD. CONCLUSION: Jervell Lange-Nielsen syndrome is a subtype of LQTS with high-risk features. LCSD, an effective therapeutic option for those having symptoms despite being on propranolol, results in significant reduction of QTc interval and amelioration of symptoms.

Paediatric quality of life in toddlers and children who underwent arterial switch operation beyond early neonatal period.

OBJECTIVES: The aim of this study was to evaluate the quality of life (QOL) of children who underwent the arterial switch operation (ASO) for Transposition of Great Arteries in our population and, specifically, to explore early modifiable factors and the influence of parental and socioeconomic factors on the QOL of these children. METHODS: Cross-sectional study using Paediatric Quality of Life Inventory™ 3.0 Cardiac Module was carried out on 3- to 12-year-old children who had undergone ASO between the years 2012-2018. Socioeconomic status was calculated using the modified Kuppuswamy scale (2019). Other clinical factors with possible bearing on the outcome were also analysed. RESULTS: Immediate survival after surgery was 196 out of 208 (94.2%) with an attrition of 19 patients (9.6%) over the follow-up period. Most surviving children (98.9%) had started formal schooling in age-appropriate classes. Two children had severe neuromotor impairment. The median cumulative health-related QOL score of the children was 97.9 (interquartile range 4.2) at 5.6 ± 1.27 years of life. The median scores each of the health-related QOL parameters, viz, heart problem symptoms, treatment compliance, perceived physical appearance, treatment-related anxiety, cognitive problems, and communication was 100 with negative skewing. CONCLUSIONS: Excellent QOL was observed in most children after ASO with the median total paediatric QOL scores in all domains of 97.9. Social factors did not show a statistically significant influence on the QOL parameters in the current cohort. The gradually declining trend across the age groups emphasizes the need for continued follow-up for early identification of possible correctable factors and initiating intervention to ensure good QOL into teenage and adulthood.

Heart failure in a newborn with tetralogy of Fallot: uncommon association of a common anomaly.

Heart failure in tetralogy of Fallot in the newborn period is rare and is usually due to either large aortopulmonary collaterals or absent pulmonary valve syndrome with severe pulmonary regurgitation. Pink tetralogy of Fallot and tetralogy of Fallot with disconnected pulmonary arteries from the aorta may present beyond the newborn period with heart failure when Pulmonary vascular resistance (PVR) falls. We describe the diagnostic and therapeutic pathway in a rare case of heart failure in newborn with tetralogy of Fallot.

Influence of age at surgery on left ventricular strain in patients with anomalous origin of left coronary artery from pulmonary artery.

OBJECTIVES: Myocardial strain abnormalities are described after surgical repair of anomalous left coronary artery from pulmonary artery (ALCAPA) even after recovery of ventricular function. The factors that predispose to the presence of these strain abnormalities in the presence of normal ventricular function are unknown. The aim of this study was to find out whether the age at repair influences the presence of global and regional strain abnormalities on follow-up. METHODS: Repaired ALCAPA patients from a single centre (n = 40) with good ventricular ejection fraction on follow-up were recruited. Baseline and follow-up data were collected from electronic records. Global and regional myocardial strain assessment was done by speckle tracking echocardiography prospectively. The association between age at repair and strain abnormalities on follow-up was analysed. RESULTS: The patients who presented earlier had significantly worse ventricular function pre-operatively compared to older patients (P < 0.0005). Global longitudinal strain was abnormal in 40% of patients with normal ventricular ejection fraction on follow-up. Presence of longitudinal strain abnormalities was more in patients who underwent repair at older age than in those who were repaired earlier (P < 0.0005). The probability of having normal longitudinal strain on follow-up was 81.6% if surgery was done before 7.8 months of age. If operated before 6 months, the odds of having normal myocardial strain was 11 times higher. Regional strain abnormalities of varying severity were present in all patients in the left and in some patients in the right coronary artery territories. CONCLUSIONS: Older age at ALCAPA repair is associated with increased incidence of myocardial strain abnormalities. Regional strain abnormalities were found in both left and right coronary artery territories.

Percutaneous Closure of Giant Aortic Pseudoaneurysm in a Child.

Although percutaneous closure of aortic pseudoaneurysms have been described in adults with good results, there are no reports in children. This case demonstrates that in selected high-risk cases where the anatomy is suitable, percutaneous closure may be feasible and safe in children.

Clinical outcomes of del nido cardioplegia and st thomas blood cardioplegia in neonatal congenital heart surgery.

OBJECTIVES: Cardioplegia is essential for adequate myocardial protection. There continues to remain ambiguity regarding the ideal cardioplegia for adequate myocardial protection in congenital heart surgery. This study compares clinical outcomes using St Thomas II solution and Del Nido cardioplegia in neonates undergoing cardiac surgery. METHODS: All neonates (<30 days) from 2011 to 2017 who underwent surgery requiring cardioplegic arrest were analyzed retrospectively. We divided the cohort into two groups depending on cardioplegia received, as group A (Blood cardioplegia with St Thomas II solution, n = 56) and group B (Del Nido cardioplegia, n = 48). Various demographic, intraoperative, early postoperative, and discharge variables were analyzed. RESULTS: Two groups were similar in age, gender, pre-operative diagnosis, and risk category. Cardiopulmonary bypass (CPB) time (P = 0.002), aortic cross-clamp (ACC) time (P = 0.018), and the number of doses of cardioplegia (P < 0.001) were significantly lower with Del Nido group. Though vasoactive inotropic score (VIS) (P = 0.036) was high during the first 24 h in the immediate postoperative period in group A, there was no difference in early mortality among both groups (P = 0.749). Both groups did not show significant differences related to various postoperative and discharge variables. CONCLUSION: When compared to St. Thomas solution, the use of Del Nido cardioplegia solution in neonates is associated with a significant decrease in CPB and ACC times and VIS in the first 24 h after surgery. The choice of cardioplegia (St Thomas/Del Nido) in neonates does not affect early mortality and early postoperative clinical outcomes.

Role of Transesophageal and Epicardial Echocardiography to Assess Surgical Repair in Double-Outlet Left Ventricle.

A double-outlet left ventricle (DOLV) is a congenital cardiac anomaly that rarely is encountered. This case report demonstrates the echocardiographic features of DOLV in the form of the pulmonary artery arising completely from the left ventricle with D-looped ventricles, especially when evaluated by intraoperative transesophageal echocardiography (TEE), along with the correlation of characteristic features with cardiac computerized tomography. The features pertinent to the differentiation of DOLV from double-outlet right ventricle and congenitally corrected transposition of the great arteries by echocardiography have been described. To the authors' knowledge, this was the first report of intraoperative TEE in the case of DOLV. In addition, the use of intraoperative echocardiography to rule out coronary compression as a cause for post-repair ventricular tachycardia, by use of TEE to rule out ventricular dysfunction and regional wall motion abnormalities, as well as epicardial echocardiography to demonstrate normal coronary blood flow, has been reported.

An unusual cause of heparin resistance - A case report.

Primary intestinal lymphangiectasia (PIL) is a rare disorder characterized by dilated intestinal lacteals that result in leakage of excessive serum proteins and lymphocytes into the gastrointestinal (GI) tract culminating in protein-losing enteropathy. The GI loss of protein and possible antithrombin III (AT-III) loss creates a prothrombotic environment. The surgical management of congenital heart disease (CHD) in presence of PIL can present with altered heparin response and can impose problems in instituting cardiopulmonary bypass (CPB). We report a case of surgical closure of ventricular septal defect with PIL with altered heparin response. Such an association of PIL with altered heparin response in CHD has not been reported in literature.

Intermediate-term neurodevelopmental outcomes and quality of life after arterial switch operation beyond early neonatal period.

OBJECTIVES: The study objective was to evaluate the cardiac, neurodevelopmental, psycho-social and health-related quality of life (HRQOL) outcomes of children who underwent an arterial switch operation (ASO). METHODS: Children who underwent ASO were evaluated on follow-up at 3-5 years with cardiovascular, neurodevelopmental and HRQOL assessment using validated tools. Children with developmental delay, attention-deficit hyperactivity disorder, autism spectrum disorder, neuromotor and speech and language impairment were considered to have neurodevelopmental disorder (NDD). The impact of socioeconomic status (Kuppuswamy classification), perioperative cardiac, nutritional and psycho-social factors on outcomes was analysed. RESULTS: There were 61 (89.7%) survivors at a mean follow-up of 50.9 ± 7.6 months. The median age at surgery was 41 days (22-74.5). One-third of patients had growth restriction. Two children had residual cardiovascular lesions requiring intervention. The mean HRQOL score was >90 in all scales of the Paediatric Quality of Life Inventory™ 3.0 Cardiac Module. Neurological abnormalities were seen in 19 patients (31.1%) of whom 17 (27.9%) patients had NDD and 12 had developmental delay. Speech and language impairment, attention-deficit hyperactivity disorder, and neuromotor impairment were found in 16.4%, 3.3% and 6.7% patients, respectively. On multivariate analysis, increasing time to lactate normalization and low socioeconomic status were associated with developmental delay after ASO. CONCLUSIONS: While intermediate-term cardiac outcomes and HRQOL after ASO were fairly satisfactory, NDD was identified in one-fourth of these children. Increasing time to lactate normalization after ASO and low socioeconomic status were associated with suboptimal intermediate neurodevelopment outcomes after ASO.

Mitral kissing lesion with anterior mitral leaflet aneurysm in a child.

Aortic valve endocarditis can lead to secondary involvement of aorto mitral curtain and the adjacent anterior mitral leaflet (AML). The secondary damage to AML is often caused by the infected diastolic jet of aortic regurgitation hitting the ventricular surface of the anterior mitral leaflet, or by the pronounced bacterial vegetation that prolapses from the aortic valve into the left ventricular outflow tract. This is called "kissing lesion". We describe a case of infective endocarditis of aortic valve in a 13-year-old child causing secondary mitral valve involvement with AML perforation and aneurysm formation.

Single collateral artery from descending thoracic aorta supplying pulmonary circulation-Computed tomography and echocardiographic images.

Pulmonary atresia (PA) with ventricular septal defect (VSD) is a rare congenital cardiac anomaly in which the pulmonary blood flow is supplied by major aorta pulmonary collateral arteries (MAPCAs). The complete repair includes unifocalization of MAPCAs, closure of VSD, and placement of a right ventricle-to-pulmonary artery conduit. We report a case of VSD with PA, where the computed tomography and echocardiography images described a large single collateral artery arising from descending thoracic aorta dividing into left and right branches and supplying the entire pulmonary circulation.

Intraoperative Three-dimensional Imaging of Ventricular Septal Defects in Children Using Epicardial Echocardiography: A Novel Approach.

OBJECTIVE: To assess the feasibility and accuracy of intraoperative three-dimensional (3D) epicardial en face images of ventricular septal defects (VSD) in children using the 3D transesophageal echocardiography (TEE) probe as an epicardial probe. DESIGN: This was a prospective nonrandomized blinded observational study. SETTING: The study was conducted at a single tertiary cardiac care center. PARTICIPANTS: Fifty pediatric patients, weighing between 5.0 kg and 20 kg, who were scheduled for elective surgical closure of their VSDs, prospectively were recruited for this study. INTERVENTIONS: Epicardial 3D images were acquired using the full-volume mode. VSD location and size were determined from the 3D data sets using multiplanar reconstruction mode (QLAB 9) by a blinded investigator. The accuracy in terms of location and size was compared with surgical findings. MEASUREMENTS AND MAIN RESULTS: In 50 study subjects, 54 VSDs were located by epicardial 3D echocardiography (3DE): 12 perimembranous, 17 inlet, eight muscular, 15 malaligned, and two outlet. Average image acquisition time was 4.96 (±1.47) minutes. Average image reconstruction time was 6.18 (±1.93) minutes. Good en face views of the VSD could be rendered in all patients (100% feasibility). The highest image quality was for perimembranous and malaligned VSDs. The location of VSD by 3DE corresponded precisely with surgical findings (100% accuracy). With linear regression analysis, excellent correlation was observed between the diameters measured by surgery and that measured by 3DE from the right ventricle side (r2 = 0.97, p < 0.001). CONCLUSIONS: The authors' study demonstrated that diagnostic quality 3D en face images of VSD can be obtained intraoperatively using 3D epicardial echocardiography. The short acquisition and reconstruction times make this technique clinically applicable.

Transthoracic intracardiac catheters in perioperative management of pediatric cardiac surgery patients: a single-center experience.

BACKGROUND: Transthoracic intracardiac catheters inserted under direct vision in the pulmonary artery and left atrium during cardiac surgery play major roles in the management of patients with complex congenital heart disease. We aimed to analyze the utility of transthoracic intracardiac catheters in the perioperative management of pediatric cardiac surgery patients and review catheter-related morbidity. METHODS: The computerized register of all pediatric cardiac surgery patients in whom transthoracic intracardiac catheters were inserted from 2012 to 2019 in a tertiary referral center were reviewed. RESULTS: Transthoracic pulmonary artery and left atrial catheters were inserted in 89 and 71 patients, respectively. The most common indications for pulmonary artery and left atrial catheters were total anomalous pulmonary venous connection (52%) and total cavopulmonary connection (58%) respectively. The most common reason for elevated pulmonary artery and left atrial pressure after cardiopulmonary bypass was left ventricular dysfunction. Transthoracic pulmonary artery catheters helped in diagnosing pulmonary hypertensive crisis (29%), surgical decision-making (14%), and ventilator therapy (16%). Left atrial catheters helped in the diagnosis of left ventricular dysfunction (54%). The incidence of morbidity was 8.9% for transthoracic pulmonary artery catheters and 9.8% for left atrial catheters. CONCLUSION: Transthoracic pulmonary artery catheters help in the diagnosis and management of pulmonary hypertensive crisis, for making perioperative surgical decisions, and during ventilator therapy. Transthoracic left atrial catheters help in the diagnosis of left ventricular dysfunction in the perioperative period. The diagnostic and treatment benefits provided by transthoracic intracardiac catheters outweigh the minor adverse events, supporting their continued use in the perioperative period.