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.

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.

Major Aortopulmonary Collateral Arteries.

Major aortopulmonary collateral arteries (MAPCAs) are congenital vessels that arise from the aorta or its first-order branches and are distally connected to the pulmonary arterial vasculature, thereby providing pulmonary blood flow. MAPCAs are commonly associated with several congenital heart diseases that have compromised pulmonary circulation due to severe stenosis involving pulmonary valves or arteries or due to pulmonary atresia. Embryologically, MAPCAs are presumed to be persistent segmental arteries. MAPCAs can be imaged with CT and MRI, and such imaging findings are important for surgeons and interventionists. The management options for MAPCAs include unifocalization, surgical ligation, and endovascular interventions, such as coil embolization. This review highlights the role of reporting certain critical features of MAPCAs at CT and MRI, which will help to facilitate management decisions for systemic-to-pulmonary collateral vessels observed in patients with congenital heart disease. Keywords: Pediatrics, CT Angiography, Image Postprocessing, Interventional-Vascular, MR Angiography, Embolization, Stents, Cardiac, Vascular, Aorta © RSNA, 2022.

Outcomes of repair of anomalous origin of pulmonary artery branch from aorta ascendens with autologous tissue: a rare condition revisited.

BACKGROUND: Anomalous origin of pulmonary artery branch from ascending aorta (APA) in the presence of two separate semilunar valves is an uncommon entity necessitating early diagnosis and surgery to prevent development of irreversible pulmonary vascular disease. We evaluated our experience with the technique and outcome of 11 patients with this condition. METHODS: Between January 2000 and December 2019, 11 patients were diagnosed with APA. Echocardiographic data were collected from the records, including the site of origin of the anomalous pulmonary artery, additional defects, pulmonary artery pressures, and biventricular function. Intraoperative charts were reviewed for the details of the surgical procedure and cardiopulmonary bypass. Post-operative data included survival, ventilatory support, and duration of hospital stay. After discharge, children were reviewed at 1 month, 3 months, 6 months, and then at yearly intervals. RESULTS: Of the 11 patients, females were more than males (7:4) with a median age of 6 months (15 days-28 years) and median weight of 5.7 kg (1.8-40 kg). Nine patients underwent direct re-implantation of anomalous pulmonary artery branch to main pulmonary artery. The survival rate was 88.8% in our series. On follow-up, no re-operations or re-interventions were required and all surgically corrected patients were in stable clinical condition. CONCLUSION: Early- and midterm outcomes of children who underwent surgery for APA is convincing. Early direct re-implantation of the anomalous branch pulmonary artery to main pulmonary artery without any graft material is the optimal surgical strategy for these patients.

Repercussions of a giant major aortopulmonary collateral aneurysm adjacent to vertebral artery origin.

An aneurysm of major aortopulmonary collateral in an adult with congenital cyanotic heart disease was detected incidentally following a hemoptysis episode. The location and size of the aneurysm needed special concern during treatment to avoid aneurysm-related complications and thromboembolism secondary to intervention.

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.

Recurrent pericardial effusion in a boy: A clue to underlying pericardial and pulmonary lymphangiectasia.

Pulmonary lymphangiectasia is a rare congenital malformation of lymphatic vessels. We report the case of a 5-year-old boy with recurrent pericardial effusion which was diagnosed to be due to pericardial and pulmonary lymphangiectasia.

Hemianomalous pulmonary venous drainage and type IIb coronary sinus septal defect - Utility of subxiphoid and right parasternal views.

A 3-year-old boy presented with history of recurrent respiratory tract infections in infancy. Clinically he had hemodynamically significant pre-tricuspid left-to-right shunt and no pulmonary hypertension. Transthoracic echocardiography delineated anomalous drainage of the left sided pulmonary veins to a dilated coronary sinus opening into the right atrium. Closer evaluation from the subxiphoid and right parasternal views led to the diagnosis of an associated type IIb coronary sinus septal defect.

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.

Midterm results of homografts in pulmonary position: a retrospective single-center study.

OBJECTIVE: Valved conduits play a pivotal role in the right ventricular outflow tract (RVOT) reconstruction in patients with congenital heart disease (CHD), and valved homografts have become the most commonly used conduits in pediatric cardiac surgery. This study aimed to assess the midterm performance of aortic and pulmonary homografts used in pulmonary position in patients with CHD. METHODS: Ninety-eight patients underwent surgical RVOT reconstruction with a homograft from January 2012 to December 2017. We collected all patient details from the institute patient record system. Echocardiographic data were obtained from the records. Postoperative data included survival and follow-up echocardiography. The primary endpoints of the study were homograft failure, homograft dysfunction, and freedom from re-intervention. RESULTS: We observed allograft failure in two patients (2%), one each due to pseudo-aneurysm formation and infective endocarditis. There were 8 early deaths (8/98, 8.2%) and 9 late deaths (9/98, 9.2%). The mean survival time was 6.1 years, and the postoperative survival rate at 1 and 5 years was 89.73% and 82.65%, respectively. Homograft regurgitation and homograft dysfunction were higher in the pulmonary homograft group. However, the re-intervention rates were more in the aortic homograft (24.24%) group due to the higher incidence of calcification associated with them. CONCLUSION: Homografts used for RVOT reconstruction provide excellent intermediate-term outcomes, irrespective of the type of homograft used. Pulmonary homografts are more durable than aortic homografts and provide satisfactory results even in infants.

Correction to: Midterm results of homografts in pulmonary position: a retrospective single-center study.

[This corrects the article DOI: 10.1007/s12055-020-01065-1.].

Congenital long QT syndrome and patent ductus arteriosus: A rare surgical scenario.

Congenital long QT syndrome (LQTS) is a rare cardiac condition characterized by abnormality of either sodium or potassium ion channels resulting in prolongation of QT interval and thereby predisposing to life-threatening arrhythmia. Once the syndrome is diagnosed, measures should be taken to avoid sudden cardiac death. We present a rare case of LQTS associated with patent ductus arteriosus in a child, and a unique approach was used in managing both conditions.

Overdrive suppression of postoperative sustained ventricular tachycardia by atrial pacing and its hemodynamic effect.

Sustained ventricular tachycardia (VT) in the early postoperative period following intracardiac repair for tetralogy of Fallot is rare. In stable VT, amiodarone forms the mainstay of management. However, where amiodarone and other antiarrhythmic drugs are contraindicated, suppressive overdrive atrial pacing can be used as a safe and efficient alternative to maintain cardiac output. We present a case of 1-year 5-month-old child who developed VT with low cardiac output syndrome with deranged hepatic function, who was managed efficiently using suppressive atrial pacing to ameliorate the effects of sustained VT.

Open heart surgery in an infant with hereditary spherocytosis and a review of literature.

Hemolytic anemia often challenges congenital heart surgery. Hereditary spherocytosis is a rare familial hemolytic anemia. When associated with congenital heart disease, the safe performance of cardiopulmonary bypass becomes a priority. The increased risk of hemolysis during cardiopulmonary bypass could potentially lead to significant secondary organ damage. Till now, only very few reports of successful repair of a congenital heart defect in patients with hereditary spherocytosis have been reported. We report the only case of successful repair of a congenital heart defect in an infant with hereditary spherocytosis.