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An anomalous right coronary artery from the pulmonary artery (ARCAPA) is a rare congenital anomaly that does not have the typical presentation of the more common anomalous left coronary artery. We present an infant with tetralogy of Fallot with atypical findings on the preoperative echocardiogram. A cardiac computerized tomographic (CT) scan showed ARCAPA. This was confirmed intraoperatively and repaired successfully. Close attention to coronaries on echocardiography and a low threshold for additional imaging can successfully diagnose ARCAPA in the presence of additional congenital heart defects. [ABSTRACT FROM AUTHOR]

In a retrospective analysis of echocardiograms, the incidence of retroaortic innominate vein was found to be 0.55% amongst children with congenital heart disease. It was most commonly associated with tetralogy of Fallot and right aortic arch.

Background : Various operative strategies are described for the Fontan procedure. In this study, we describe our short-term results and technique of Fontan procedure on cardiopulmonary bypass (CPB) and deep hypothermic circulatory arrest (DHCA). Methods : This was a retrospective study of 32 patients, median age of 6 years (4–19 years) and median weight of 20 kg (13–51 kg), who underwent Fontan procedure on CPB and DHCA from July 2016 to July 2021. Results : The median CPB time was 125 min (77–186 min), the median DHCA time was 42 min (27–50 min), and the median Fontan pressure was 14 mmHg (10–18 mmHg). The median time to extubation was 4 h (1–20 h), the duration of chest tube drainage was 8 days (5–24 days), and the median intensive care unit stay was 4 days (3–8 days). The presence of heterotaxy was associated with longer duration of pleural drainage (P = 0.01). There was no operative mortality and no major adverse events such as seizures, gross neurological deficits, or arrhythmias in the postoperative period. Conclusions : Fontan procedure can be safely performed on CPB and DHCA with good operative results. This operative strategy may be used in special circumstances like in patients with situs and systemic venous anomalies and those requiring repair of a complex intracardiac defect. Long-term follow-up will be required to evaluate if this strategy has any impact on the neurodevelopmental outcome and the long-term sequelae of Fontan. [ABSTRACT FROM AUTHOR]

Unifocalization of the major aortopulmonary collaterals (MAPCAs) followed by intracardiac repair with ventricular septal defect (VSD) closure and restoration of right ventricle-to-pulmonary artery continuity is the ultimate treatment goal in a case of VSD with pulmonary atresia and MAPCAs. It may be achieved in a single stage or may require multiple surgeries. We present a case of a 2-year-old boy with VSD with pulmonary atresia who underwent single-stage unifocalization of MAPCAs through the midline followed by intracardiac repair using 2 polytetrafluoroethylene tube grafts: one for unifocalization and other as a bicuspid valved right ventricle-to-pulmonary artery conduit. [ABSTRACT FROM AUTHOR]

We report the use of Bosentan in the post-operative period of a neonate with obstructed infradiaphragmatic total anomalous pulmonary venous connection and severe pulmonary arterial hypertension. To our knowledge, this is the first report of use of Bosentan in this situation.

Background: Pediatric heart transplantation is a now a well-established and standard treatment option for end stage heart failure for various conditions in children. Due to logistic issues, it is not an option for in most pediatric cardiac centres in the third world. Aim: We sought to describe our early experience in the current era in India. Methods: This is a short term retrospective chart review of pediatric patients who underwent heart transplantation at our centre. Mean/Median with standard deviation /range was used to present data. Results: Twenty patients underwent orthotopic heart transplant between January 2016 and June 2019. The median age at transplant was 12.4 years (range 3.3 to 17.3 years). The median weight was 23.2kg (range 10 80kg). The mean donor/recipient weight ratio was 1.62± 0.84. The mean ICU stay was 12.1days. The mean follow up post transplant was 2.03± 0.97years (range 10 days 3.57 years). The 1 month and the 1 year survival was 100%. Biopsies were positive for significant rejection in 7 patients (35%). At the time of last follow up, 3 patients (15%) had expired. The major post transplant morbidities were mechanical circulatory support (n=3), hypertension with seizure complex (n=3), post transplant lympho proliferative disorder (n=1), pseudocyst of pancreas (n=1), coronary allograft vasculopathy (n=3) and systemic hypertension (n=7). All surviving patients (n=17) were asymptomatic at last follow up. Conclusion: The results suggest acceptable short term outcomes in Indian pediatric patients can be achieved after heart transplantation in the current era. Significant rejection episodes and coronary allograft vasculopathy need careful follow up. [ABSTRACT FROM AUTHOR]

Fetal and neonatal manifestations of long QT syndrome are not well documented except few case reports. We report a neonate presenting with potential brief resolved unexplained events like episode, long QT interval was noted on surface electrocardiogram, and molecular diagnosis revealed a novel heterozygous nonsynonymous missense substitution in exon 17 of the A-kinase anchor protein 9 gene. The proband responded to propranolol at a dose of 1 mg/kg/day. [ABSTRACT FROM AUTHOR]

A 3-year-old child with a complex variant of double outlet right ventricle achieved a successful biventricular repair after detailed delineation of the intracardiac anatomy on multimodality imaging. A virtual three-dimensional (3D) model based on computed tomography was used successfully in the absence of an actual 3D-printed model. This case report seeks to highlight and hence increase the utilization of the virtual 3D model in resource-limited settings. [ABSTRACT FROM AUTHOR]

Horseshoe lung, usually associated with pulmonary venolobar syndrome, is a rare congenital anomaly involving the fusion of the postero-basal segments of the right and left lungs across the midline. The fused segment or the isthmus lies posterior to the pericardium and anterior to the aorta.The associated pulmonary venolobar syndrome involves anomalous systemic arterial supply and anomlaous systemic venous drainage of the right lung. With the advent of MDCT imaging, we can diagnose this rare condition as well all its associated anomalies non-invasively. Volume-rendered techniques greatly simplify the complex anatomy and provide easy understanding of the same. [ABSTRACT FROM AUTHOR]

Objectives: This study was designed to evaluate the outcome of stent placement (SP) for conduit discrete stenosis using predefined criteria. Background: Right ventricle (RV) to pulmonary artery (PA) conduits are often associated with complications, such as stenosis, requiring multiple surgical replacements. Methods: Patients who underwent primary or repeat SP were included. Indications for SP were clinical symptoms and/or RV to systolic blood pressure (SBP) ratio (RV:SBP) >0.65 by echocardiography. Our definition of success was a decrease in RV:SBP by >20%, a final RV:SBP ratio of <0.65, or resolution of symptoms. Results: Stents were placed successfully in 28 of 31 patients (90%), including 3 patients who underwent the procedure solely for symptoms. The RV:SBP ratio decreased (0.75 ± 0.17 vs. 0.52 ± 0.12, p < 0.001), and the conduit diameter increased (postero-anterior 9.1 ± 2.9 vs. 12.0 ± 2.8 mm, lateral 8.3 ± 2.2 vs. 11.6 ± 2.4 mm, p < 0.001). In the 28 patients with successful SP, 8 (29%) remained free from second intervention. In the remaining patients, the median time to re-intervention was 16 months (range 6 to 44 months). Second transcatheter interventions (4 SP, 4 balloon dilation) were successful in 8 of 13 patients. Complications included balloon rupture (n = 4), stent fracture (n = 2), and pseudoaneurysm formation (n = 1). Conclusions: Initial SP has excellent intermediate outcomes, successfully postponing surgical intervention for the majority of patients. Conduit restenosis may be successfully treated with a second transcatheter intervention. On the basis of these data, SP is likely the procedure of choice for patients with a discrete stenosis of the RV to PA conduit. [Copyright &y& Elsevier]

Pretreatment with heat confers cardiopulmonary protection in endotoxemic animals. This mechanism may be through suppression of pro-inflammatory mediator production. The objectives of this study were to determine the effect of heat stress on tumor necrosis factor-α (TNF-α) and macrophage inflammatory protein-2 (MIP-2) in a lipopolysaccharide-exposed macrophage cell line and to study the relationship between TNF-α and MIP-2 production. Heat pretreatment resulted in decreased TNF-α transcription and translation by lipopolysaccharide-exposed macrophages; and increased MIP-2 concentration without additional effect in transcription. Administration of TNF-α antibody prior to exposure to lipopolysaccharide resulted in increased MIP-2 concentration suggesting that TNF-α acts to down-regulate MIP-2 production. The mechanism by which heat stress causes an increase in MIP-2 concentration may be secondary to its suppressing effect on TNF-α production. [ABSTRACT FROM AUTHOR]

Pulmonary artery thrombosis in neonates is a rare entity. We describe two neonates with this diagnosis; their presentation, evaluation, and management. These cases highlight the importance of this differential diagnosis when evaluating the cyanotic neonate. [ABSTRACT FROM AUTHOR]

The authors discuss a case of a 39-year-old-woman, previously diagnosed with systemic lupus erythematosus (SLE) and presenting with diamniotic dichorionic twins for fetal echocardiography at 28 weeks of gestation. She had an emergency cesarean section at 29 weeks because of intrauterine fetal death of twin A; twin B was found to have congenital complete heart block (CHB). He was given staged pacing using temporary epicardial pacing after which he remained hemodynamically stable.

Background: Excellent outcomes of right ventricle to pulmonary artery conduits with polytetrafluoroethylene (PTFE) valves have been reported. The purpose of this study was to analyze the short-term results of our handmade PTFE bicuspid valved conduit (VC) for right ventricular outflow tract reconstruction. Methods: Between September 2019 and May 2023, bicuspid PTFE-VC was implanted in 17 patients at a median age of 2.5 years (range, 3 months to 13.6 years). The PTFE-VC was fashioned from a commercially available PTFE tube graft (14 mm in three patients, 16 mm in three patients, 18 mm in one patient, 20 mm in three patients, and 22 mm in seven patients) and 0.1 mm thick PTFE membrane for the leaflet material. Valve function was assessed by echocardiogram after the implantation. The conduit reoperation and the conduit dysfunction were analyzed. There were no early deaths, but there was one late death. Results: There were no postoperative in-hospital deaths. Follow-up echocardiograms were available for 14 of 17 patients. The median follow-up was 21 months (range, 7-49 months). Conduit stenosis was none or trivial in 11 patients while it was mild in two and moderate in one patient and severe in 0 patients. Conduit insufficiency was mild or trivial in all 14 patients. By the end of the study period, freedom from reoperation/reintervention was 100%. There were no episodes of aneurysmal dilatation of the conduit or endocarditis. Conclusions: Handmade bicuspid PTFE VC shows good short-term outcome, with no significant valve dysfunction and no reintervention. A longer follow-up is necessary to evaluate the long-term advantages of using the handmade bicuspid PTFE VC., Competing Interests: Declaration of Conflicting InterestsThe author(s) declared no potential conflicts of interest with respect to the research, authorship, and/or publication of this article.

Different surgical techniques have been described for the primary repair of anomalous left coronary artery arising from the pulmonary artery (ALCAPA); however, very few techniques are described for management of coronary artery occlusion following ALCAPA repair. We present a case of a 7-year-old girl with left main coronary atresia status-post left coronary button transfer for ALCAPA in infancy. She underwent redo-sternotomy and left subclavian artery-to-left main coronary artery bypass plus mitral valve repair and had an uneventful postoperative course.

Right atrial aneurysm (RAA) is a rare congenital anomaly with a diverse clinical spectrum. We present a case of antenatal detection of a giant RAA. The infant had 3 episodes of staring spells presumed to be thrombo-embolic phenomena originating from the RAA. The infant underwent successful RAA resection with preservation of the right coronary artery that was displaced from its usual position due to invagination of the RAA in the subepicardial space of the right atrio-ventricular groove.

Aortic regurgitation after transcatheter device closure of a perimembranous ventricular septal defect is a known complication. We present the case of an 11-year-old boy with severe aortic valve regurgitation due to cusp perforation complicating previous device closure of a ventricular septal defect. The patient underwent successful aortic valve repair (neo-cusp reconstruction technique) by replacement of a cusp and shaving off of a rim of the device 5 years after device closure.

Purpose: Numerous attempts have been made to extend the boundaries of arterial switch operation (ASO) in children presenting late with transposition of great arteries with intact ventricular septum (TGA/IVS) and regressed left ventricle (rLV). Many children tolerate the delayed ASO uneventfully, whereas others need mechanical circulatory support (MCS) to sustain the systemic circulation while the left ventricle undergoes retraining., Description: In this article, we describe six consecutive children with TGA/IVS and rLV who underwent primary ASO., Results: Three were managed medically, while three required MCS in the form of Centrimag left ventricular assist device (LVAD). All patients survived the operation and were discharged home in a stable condition., Conclusions: Primary ASO can be safely performed in children with TGA/IVS and rLV, provided the center has MCS options. Supporting the rLV with LVAD is feasible and can be achieved safely.

The preferred approach for patients with D-transposition of the great arteries with an intact ventricular septum (DTGA/IVS) is the arterial switch operation (ASO). In those patients presenting late, with regressed left ventricle (LV), successful LV preparation is of paramount importance to achieve this goal. We present a toddler with DTGA/IVS who underwent ASO followed by successful left ventricular retraining with postoperative left ventricular assist device support with CentriMag centrifugal pump.

A three-dimensional (3D) printed heart model based on contrast-enhanced MR angiography data was obtained in an 8-month-old male child with double-outlet right ventricle. The model could successfully show the spatial relationship between the aortic annulus, the pulmonary valve and the ventricular septal defect. The patient underwent complete intracardiac repair based on the 3D model. MR angiography images could be successfully used to create a true-size 3D heart model, which significantly helped in assessing the routability of the ventricular septal defect to the aorta, leading to successful intracardiac repair in our patient.

Background: Double outlet right ventricle (DORV) with two well-developed ventricles and with a remote ventricular septal defect (VSD) may present a therapeutic challenge. Echocardiographic imaging of such complex cases does not always provide all of the information required to decide on an operative approach (biventricular or univentricular) and to design an intracardiac baffle to direct left ventricular outflow through the VSD and to the aorta for biventricular repair. A three dimensional (3D) printed model of the heart based upon data derived from computed tomography (CT) or magnetic resonance imaging (MRI) may contribute to a more complete appreciation of the intracardiac anatomy., Methods: From April to September 2015, six consecutive patients with DORV and remote VSD underwent CT/MRI scans. Data sets from these studies were used to generate life-size 3D models using a 3D printer. We compared the assessment of 3D printed heart model findings with information obtained from echocardiography, CT, or cardiac MRI and with details of the surgeon's intraoperative direct observations when available. Quantification of the information provided by the 3D model was achieved using a unique scale that was created for the purpose of this study. The accuracy and utility of information derived preoperatively from the models were assessed., Results: Six data sets from six patients were analyzed. Five data sets could be successfully used to create sandstone models using 3D printing. The five patients ranged from 7 months to 11 years of age and weighed 6.7 to 26 kg. The spatial orientation of the heart in the thorax, the relationships of the great arteries and the semilunar valves, the size and location of the VSD were well appreciated in all models, as were the anticipated dimensions and orientation of a surgically planned interventricular baffle. Three of the five patients underwent successful biventricular repair., Conclusion: The 3D printed models scored higher than conventional imaging, with respect to most aspects of the surface spatial orientation and intracardiac anatomy. The models are a useful adjunct in preoperative assessment of complex DORV. The unique scale helps quantify the advantages and limitations of the 3D heart models., (© The Author(s) 2016.)