ObjectiveTo compare the effectiveness and safety of Celsior® crystalloid solution to St Thomas® solution as cardioplegia in pediatric arterial switch surgery.MethodsA retrospective study was conducted on 180 patients who underwent arterial switch operation (ASO) between 2005 and 2019. The patients were divided into two groups: the St Thomas group receiving St Thomas solution and the Celsior® group receiving Celsior® solution. The study aimed to assess myocardial protection while evaluating clinical outcomes of patients between groups.ResultsBaseline characteristics not different between groups. The postoperative troponin release trends and blood lactate levels were not different between groups. However, the Celsior® group had a significant lower incidence of delayed sternal closure (9.7% vs. 19.5%; p = 0.09) and mechanical circulatory support (ECMO) (4.9% vs. 24.7%; p < 0.001) compared to the St Thomas group. The length of stay in the intensive care unit (ICU) was significantly shorter in the Celsior® group (4.6 ± 3.36 days vs. 8.72 ± 5.08 days, respectively; p < 0.001). There was no significant difference in 30-day mortality between the two groups (2.9% vs. 2.6%; p = 0.147).ConclusionThe study suggests that Celsior® solution is effective and safe for myocardial protection in pediatric arterial switch surgery. It may offer potential benefits such as reduced need for delayed sternal closure and ECMO support, as well as shorter ICU stay. However, the study has limitations including its retrospective design and the use of different cardioplegic solutions during different time periods. Further prospective randomized trials are needed for confirmation. Clinical Registration NumberClinicalTrials.gov, ID: NCT04616222.

Background: Anomalous aortic origin of the right coronary artery is known to be a cause of sudden cardiac death in athletes. There are no specific guidelines concerning treatment strategy in the literature. The aim of this study is to describe and report our experience of direct reimplantation technique in the treatment of this anomaly. Methods: This was a retrospective single center study of 30 patients who underwent surgery in the congenital heart disease unit of Louis Pradel Heart and Lung Hospital between January 2003 and December 2016. The mean follow-up was seven years (3 months-17 years). Results: Thirty patients underwent surgery. The median age was 17 years (0.2-52 years). There were 24 males. The median weight was 58 kg (3.6-118 kg). Fourteen patients were actively engaged in sports. Twenty-six patients had exertional chest pain or syncope. The median time lapse between diagnosis and intervention was 4.5 months (0.5-179 months). Twenty-seven (90%) patients underwent reimplantation of the anomalous coronary artery without transverse aortotomy, while in 3 (10%) patients transverse aortotomy was used to facilitate reimplantation to avoid tension at the anastomosis. There was no early death; one late death occurred in the third postoperative month. At the last follow-up, all patients had returned to normal physical activity without evidence of ischemia. Conclusions: Direct reimplantation allows for a complete restoration of the coronary anatomy and enables patients to return to normal physical activity. Our study shows encouraging results using a direct reimplantation technique without aortotomy.

Anomalous aortic origin of a coronary artery, particularly in the presence of inter-arterial course between the great arteries, has been found to be associated with sudden death in young people.This study reports a single-center experience in the management of anomalous aortic origin of a coronary artery, with or without inter-arterial course, by focusing specifically on presentation, diagnosis, and patient outcome.From March 1993 to February 2018, 100 patients (70 males) were diagnosed with anomalous aortic origin of a coronary artery, including 27 left coronary artery from the right sinus, 60 right coronary artery from the left sinus, and 13 other anomalies. Patients with inter-arterial course between the great arteries presented more frequently with syncope and/or sudden death compared to patients without (23.4% vs. 0%, p = 0.026). Diagnosis was reached using first-line transthoracic echocardiography in 65% of cases. Surgical repair was performed in 61 patients (61%). All operated patients (60 direct implantations, 1 unroofing) had been diagnosed with inter-arterial course between the great arteries, and were asymptomatic at a mean (±SD) postoperative follow-up of 4.9 ± 5.3 years.An inter-arterial course of the anomalous right or left coronary arteries arising from the opposite sinus is associated with life-threatening events. Direct reimplantation of coronary artery is reliable, and should be discussed even in asymptomatic patients.

Between 1983 and 2016, we operated on 14 children with tetralogy of Fallot with an anomalous coronary artery crossing the pulmonary infundibulum, which is an anomaly that makes the repair complex. The technique used was the enlargement of the right ventricular outflow tract underneath the mobilized coronary artery. All patients had right ventricular outflow tract relief without coronary artery injury. Only one patient required the use of an extracardiac conduit. There was neither in-hospital mortality nor coronary anomaly requiring reintervention. Mobilizing the anomalous coronary artery in tetralogy of Fallot repair often allows relief of obstruction without using an extracardiac conduit.

Background: Progress in the management of complex congenital heart disease (CHD) led to an improvement in survival rates of adults with a Fontan-like circulation. The objective of this study was to assess the subjective health status and quality of life of this population. Methods and results: Patients aged more than 18 years at the time of the study, who underwent a Fontan-like procedure. Subjective health status was assessed by the SF-36 questionnaire and a linear analog scale was used to score patients’ self-perception of their quality of life; cardiac and demographic parameters were collected. Results: Among 65 eligible patients, 60 (23 females; mean ± SD age: 25.7 ± 7.2 years) answered the SF-36 questionnaire and 46 of these were interviewed to evaluate their perceived quality of life. Among them, 20 (33.3%) were working full-time and 21 (35%) experienced arrhythmias. The physical SF-36 scores were lower in patients than in the general population (p ≤ 0.05). The New York Hear Association (NYHA) class and occupation were correlated with SF-36 scores of physical activity (respectively, p = 0.0001 and p = 0.025). SF-36 scores of psychological status were associated with the number of drugs and occupation (respectively, p = 0.0001 and p = 0.02). The mean ± SD quality of life score measured using a linear analog scale was 7.02 ± 1.6 and was linked to education and occupation (p ≤ 0.05) but not with cardiac parameters. Conclusion: Adult Fontan patients perceive an impaired physical health but report a good overall quality of life. Education and occupation impacts significantly on Fontan patients’ quality of life.

Apparently, balanced chromosomal rearrangements usually have no phenotypic consequences for the carrier. However, in some cases, they may be associated with an abnormal phenotype. We report herein the case of a 4-year-old boy presenting with clinically isolated supravalvular aortic stenosis (SVAS). No chromosomal imbalance was detected by array CGH. The karyotype showed a balanced paracentric chromosome 7 inversion. Breakpoint characterization using paired-end whole-genome sequencing (WGS) revealed an ELN gene disruption in intron 1, accounting for the phenotype. Family study showed that the inversion was inherited, with incomplete penetrance. To our knowledge, this is the first case of a disruption of the ELN gene characterized by WGS. It contributes to refine the genotype-phenotype correlation in ELN disruption. Although this disruption is a rare etiology of SVAS, it cannot be detected by the diagnostic tests usually performed, such as array CGH or sequencing methods (Sanger, panel, or exome sequencing). With the future perspective of WGS as a diagnostic tool, it will be important to include a structural variation analysis in order to detect balanced rearrangements and gene disruption.

Correct visualization of the vascular lumen is impaired in standard computed tomography (CT) because of blooming artifacts, increase of apparent size, induced by metallic stents and vascular calcifications. Recently, due to the introduction of photon-counting detectors in the X-ray imaging field, a new prototype spectral photon-counting CT (SPCCT) based on a modified clinical CT system has been tested in a feasibility study for improving vascular lumen delineation and visualization of coronary stent architecture. Coronary stents of different metal composition were deployed inside plastic tubes containing hydroxyapatite spheres to simulate vascular calcifications and in the abdominal aorta of one New Zealand White (NZW) rabbit. Imaging was performed with an SPCCT prototype, a dual-energy CT system, and a conventional 64-channel CT system (B64). We found the apparent widths of the stents significantly smaller on SPCCT than on the other two systems in vitro (p < 0.01), thus closer to the true size. Consequently, the intra-stent lumen was significantly larger on SPCCT (p < 0.01). In conclusion, owing to the increased spatial resolution of SPCCT, improved lumen visualization and delineation of stent metallic mesh is possible compared to dual-energy and conventional CT.

Ventricular septal defect (VSD) including outlet VSD of double outlet right ventricle (DORV) and perimembranous VSD are among the most common congenital heart diseases found at birth. HOXB1 encodes a homeodomain transcription factor essential for normal cardiac outflow tract development. The aim of the present study was to investigate the possible genetic effect of sequence variations in HOXB1 on VSD. The coding regions and splice junctions of the HOXB1 gene were sequenced in 57 unrelated VSD patients. As a result, a homozygous c.74_82dup (p.Pro28delinsHisSerAlaPro) variant was identified in one individual with DORV. We also identified five previously reported polymorphisms (rs35114525, rs12946855, rs14534040, rs12939811, and rs7207109) in 18 patients (12 DORV and 6 perimembranous VSD). Our study did not show any pathogenic alterations in the coding region of HOXB1 among patients with VSD. To our knowledge this is the first study investigating the role of HOXB1 in nonsyndromic VSD, which provide more insight on the etiology of this disease.

Aortic pseudocoarctation is a rare congenital anomaly characterized by elongation and deformity of the aortic arch and is known to be associated with aneurysmal formation. Several studies unite to say it leads to a surgical sanction as soon as symptomatic or associated with aneurysms of the aortic arch. Our patient is a 12 years old boy, followed since birth for a little tight pseudocoarctation with a cervical aortic arch and transverse aortic arch hypoplasia. Close clinical and paraclinical monitoring including angioscans, showed the gradual enlargement of the superior mediastinum, in relation with the appearance of three aneurysms of the aortic arch. The intervention, performed by sternotomy, has consisted of the resection of the aneurysmal area and the interposition of a Dacron tube to repair the aortic arch and the reimplantation of the left subclavian artery into the left carotid artery. The postoperative course was uneventful. Management of pseudocoarctation associated with cervical aortic arch and aneurysms remains surgical. Close monitoring of patients with pseudocorctation, seems to be essential to avoid fatal complications such as aneurysmal rupture.

Atrial septal defect (ASD) is a common congenital heart defect. Transcatheter closure become now the standard approach for ostium secondum type ASD closure against surgery. However, this procedure is not free of complications Device migration is the most common complication. We report a case of an obstruction of tricuspid valve by migration of a transcatheter ASD device occlude in a 9 years-old girl reveled by severe ventricular arrhythmias. She underwent successful emergent surgical removal under cardiopulmonary bypass and ASD closure with a synthetic patch. The postoperative course was favorable.

Background Total anomalous pulmonary venous connection is a rare cardiac malformation associated with significant morbidity and mortality rates. We report a large surgical series study to evaluate mid-term and long-term results of conventional surgical techniques.Methods and Results We performed a retrospective analytic study of all patients operated on for simple total anomalous pulmonary venous connection in the University Hospital of Lyon, France, between January 1973 and June 2014. A total of 180 patients were included (43% supracardiac, 27% intracardiac, 19% infracardiac, and 11% mixed types). Mean cardiopulmonary bypass and aortic cross clamp times were respectively 66 and 39 minutes. Overall mortality was 27.1%, including 38 early deaths (21.1%) and 12 late deaths (6.1%). The percentage of early death greatly decreased over the eras, from 42.1% in the seventies to 7.4% after 2010. Besides the earlier era of intervention (p < 0.0001), significant risk factors for death in multivariate analysis were preoperative pulmonary hypertension, acidosis, and cardiopulmonary bypass time. There were 24 reoperations, including 7 for pulmonary venous obstruction; 6 died. Factors directly and independently associated with late complications were the anatomic type (mixed forms, p = 0.0023), and length of aortic cross clamp time (p = 0.01). Long-term results for survivals are excellent. We report 84.7% of asymptomatic patients with a mean follow-up of 10.8 years.Conclusions The overall prognosis of total anomalous pulmonary venous connection repair with conventional procedures has greatly improved over the years with excellent long-term results. A thorough evaluation of all preoperative characteristics is imperative to achieve the best outcome.

Long term oral anti-coagulation with vitamin K antagonists is a risk factor of hemorrhagic or thromebomlic complications. Periodic laboratory testing of international normalized ratio (INR) and a subsequent dose adjustment are therefore mandatory. The use of home testing devices to measure INR has been suggested as a potential way to improve the comfort and compliance of the patients and their families, the frequency of monitoring and, finally, the management and safety of long-term oral anticoagulation. In pediatric patients, increased doses to obtain and maintain the therapeutic target INR, more frequent adjustments and INR testing, multiple medication, inconstant nutritional intake, difficult venepunctures, and the need to go to the laboratory for testing (interruption of school and parents' work attendance) highlight those difficulties. After reviewing the most relevant published studies of self-testing and self-management of INR for adult patients and children on oral anticoagulation, it seems that these are valuable and effective strategies of INR control. Despite an unclear relationship between INR control and clinical effects, these self-strategies provide a better control of the anticoagulant effect, improve patients and their family quality of life, and are an appealing solution in term of cost-effectiveness. Structured education and knowledge evaluation by trained health care professionals is required for children, to be able to adjust their dose treatment safely and accurately. However, further data are necessary in order to best define those patients who might better benefit from this multidisciplinary approach.

The aim of the study was to assess the long term outcome of patients with ALCAPA

Standardized techniques of mitral valve repair (MVR) have recently witnessed the introduction of a 'respect rather than resect' concept, the strategy of which involves the use of artificial chordae. MVR displays several advantages over mitral valve replacement in degenerative mitral regurgitation' (MR), but the risk of reoperation for MVR failure must be taken into account. Different mechanisms could be advocated as the leading cause of MVR failure; procedure-related mechanisms are usually involved in early MVR failure, while valve-related mechanisms are common in late failure. Here, the case is reported of an early failure of MVR using artificial chordae that could be explained by an unusual procedure-related mechanism, namely anterior papillary muscle necrosis. MVR failure is a well-known complication after surgical repair of degenerative MR, but anterior papillary muscle partial necrosis might also be considered a possible mechanism of procedure-related MVR failure, especially when considering the increasing use of artificial chordae. Owing to the encouraging results obtained, mitral valve re-repair might be considered a viable solution, but must be selected after only a meticulous evaluation of the underlying mechanism of MVR failure.

The aim of the study was to assess the long-term outcome of patients with ALCAPA.

Anomalous left coronary artery to the pulmonary artery is a rare pathology with a mortality rate of 90% in the first year of life, directly related to left ventricular function and coronary perfusion, although several adult cases have been reported. Surgical correction consists of ligation of the anomalous left coronary artery associated with coronary artery bypass grafting. We describe the exceptional case of a 65-year-old woman who underwent reimplantation of the left coronary artery in the anatomical position without bridging.