Catheter ablation of atrial tachyarrhythmias in patients with atrioventricular septal defect.

AIMS: The incidence of atrial tachyarrhythmias is high in patients with atrioventricular septal defect (AVSD). No specific data on catheter ablation have been reported so far in this population. We aimed to describe the main mechanisms of atrial tachyarrhythmias in patients with AVSD and to analyse outcomes after catheter ablation. METHODS AND RESULTS: This observational multi-centric cohort study enrolled all patients with AVSD referred for catheter ablation of an atrial tachyarrhythmia at six tertiary centres from 2004 to 2022. The mechanisms of the different tachyarrhythmias targeted were described and outcomes were analysed. Overall, 56 patients (38.1 ± 17.4 years, 55.4% females) were included. A total of 87 atrial tachyarrhythmias were targeted (mean number of 1.6 per patient). Regarding main circuits involved, a cavo-annular isthmus-dependent intra-atrial re-entrant tachycardia (IART) was observed in 41 (73.2%) patients and an IART involving the right lateral atriotomy in 10 (17.9%) patients. Other tachyarrhythmias with heterogeneous circuits were observed in 13 (23.2%) patients including 11 left-sided and 4 right-sided tachyarrhythmias. Overall, an acute success was achieved in 54 (96.4%) patients, and no complication was reported. During a mean follow-up of 2.8 ± 3.8 years, 22 (39.3%) patients had at least one recurrence. Freedom from atrial tachyarrhythmia recurrences was 77.5% at 1 year. Among 15 (26.8%) patients who underwent repeated ablation procedures, heterogeneous circuits including bi-atrial and left-sided tachyarrhythmias were more frequent. CONCLUSION: In patients with AVSD, most circuits involve the cavo-annular isthmus, but complex mechanisms are frequently encountered in patients with repeated procedures. The acute success rate is excellent, although recurrences remain common during follow-up.

Perioperative Hemostatic Management of a Newborn With Hereditary Hemophilia A Undergoing Emergent Surgery for Dextro-Transposition of the Great Arteries.

Hemophilia A is an inherited bleeding disorder characterized by a lack of plasma clotting factor VIII (FVIII). In prophylaxis or during surgery, FVIII infusions are necessary to prevent bleeding. The authors describe the perioperative challenges and application of a multidisciplinary hemostatic management approach to a Caucasian male newborn, with antenatal diagnoses of moderate hemophilia A (2 IU/dL) and dextro-transposition of the great arteries requiring arterial switch surgery within the first month of life. Because both conditions are rare, only few reports in the literature are available describing perioperative management of hemophilia in neonates and children undergoing cardiac surgery. After baseline FVIII determination and normal standard coagulation studies, iterative intravenous pharmacist-prepared plasma-derived FVIII boluses were calculated (35 IU/kg) and administered intravenously every 6 hours for 24 hours, then switched to a continuous infusion and guided by daily chromogenic clotting FVIII activity assay for targeted values between 80 and 100 IU/dL. Successful cardiac surgery, using cardiopulmonary bypass, was performed with continuous infusion of FVIII at 5 IU/kg/h. Thirteen days after surgery, the FVIII antibody screening remained negative and continuous infusion was switched in favor of a daily intravenous bolus treatment to facilitate reconciliation to the center of origin. The authors' multidisciplinary strategy, established antenatally, allowed for successful care in this highly complex and rare situation.

Berlin Heart EXCOR Paediatric Ventricular Assist Device: Does Weight Matter?

BACKGROUND: Berlin Heart EXCOR (BH) ventricular assist devices provide mechanical long-term circulatory support in children with end-stage heart failure, as a bridge to transplantation or to recovery. Most studies are from large-volume paediatric cardiac centres. AIM: The aim of this study was to analyse the experiences of three French centres and to compare these with available published data. METHOD: We performed a retrospective observational study of three paediatric cardiac intensive care units. All children supported with BH devices were included. Morbidity and mortality data were collected and risk factors analysed. RESULTS: Fifty-four (54) patients (54% male) were included. Survival rate was 73% while on a BH device. Median age at BH device implantation was 17 months (range 2-180 months). The predominant indication was dilated cardiomyopathy (61%). Bi-ventricular assist device was used in 25 (46%) cases. The total length of long-term circulatory support was 3,373 days, with a mean length per patient of 62.5 days (range 5-267 days). Thirty-two (32) patients were transplanted (59%) and seven (13%) were successfully weaned. Type and length of support did not influence morbidity. Main complications were renal dysfunction (57%), bleeding (41%), and infection (39%). In multivariate analysis, a weight <5 kg was significantly associated with higher mortality. CONCLUSIONS: The weight seems to be the most important risk factor of mortality in this precarious condition.

Total Anomalous Pulmonary Venous Connection: A 40 years' Experience Analysis.

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.

[About a case of a recurrent glandular cardiac myxoma in a child]. / À propos d'un cas de myxome cardiaque avec éléments glandulaires récidivant chez un enfant.

Primary cardiac tumors are extremely rare and mainly benign. The majority of these are myxomas (40%). Myxoma are generally sporadic tumors which occur most commonly in adult females between 30 and 40 years, and are seldom found in the paediatric population (5%). Seven percent are associated with igenetic diseases. We report the case of an eight-year-old boy presenting a recurrent glandular cardiac myxoma. In 2011, he presented a deterioration of the general state. An echocardiography highlighted a left atrial mass on the interatrial septum, with a pedicular insertion. On the microscope, it consisted of a proliferation of stellate cells isolated or clustered in rudimentary vessels in a myxoid stroma presenting haemorrhage changes. These cells expressed CD34 and calretinine. Glandular elements without atypia were clustered within the myxomatous proliferation. They expressed cytokeratin (CK) 7. Surgical resection was macroscopically complete. In 2014, the boy had a sudden neurological deficit during a football match. An echocardiography revealed a recurrence at the same location. The lesion was excised and addressed in several fragments. Classical myxoma was associated with glands without atypia. This last component expressed CKAE1/AE3 and CK7. Ki67 index of proliferation was low. The surgical reintervention was macroscopically complete. The final diagnosis was glandular cardiac myxoma. A genetic survey was conducted, showing the presence of Carney complex. This is the first description in the litterature of a recurrent glandular cardiac myxoma occuring in a child.

Could anterior papillary muscle partial necrosis explain early mitral valve repair failure?

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.

Tissue hemoglobin monitoring is unable to follow variations of arterial hemoglobin during transitions from pulsatile to constant flow in cardiac surgery.

OBJECTIVE: To test whether the variations of tissue hemoglobin concentration (∆THb) measured by the FORE-SIGHT(TM) cerebral oximeter can accurately detect changes in arterial hemoglobin concentration (∆AHb) before, during, and after cardiopulmonary bypass. DESIGN: A prospective clinical study. SETTING: Cardiac surgery operating room. PARTICIPANTS: Thirty patients scheduled for cardiac surgery. INTERVENTIONS: Tissue hemoglobin concentration (THb) was recorded continuously via 2 sensors applied on the forehead and connected to the cerebral oximeter. Arterial hemoglobin concentration (AHb) was measured in a hematology analyzer laboratory. Hemodynamic and respiratory parameters as well as epidemiologic data also were noted. Data were collected at 3 perioperative times: After induction of anesthesia, 10 minutes after cardioplegia, and at the end of the surgery. MEASUREMENTS AND MAIN RESULTS: Ninety pairs of data were collected. The coefficient of linear regression between ∆THb and ∆AHb was 0.4 (p<0.001). After exclusion of Hb variations<5%, the trending ability of THb to predict ∆AHb was 87%. However, the Bland and Altman plot graph for THb and AHb showed major limits of agreement (2.4 times the standard deviation). Central venous pressure and carbon dioxide tension were linked independently and positively with THb (p = 0.03). CONCLUSIONS: Continuous monitoring of THb cannot accurately track variations of AHb during the transition from pulsatile to continuous flow and vice versa in cardiac surgery. Local hemodynamic factors such as PaCO2 and vasodilation significantly impact THb. In this setting, THb monitoring should not be used to guide eventual blood transfusion management.

Celsior® vs. St Thomas® cardioplegia: analysis of myocardial protection and clinical safety in neonates.

Objective: To compare the effectiveness and safety of Celsior® crystalloid solution to St Thomas® solution as cardioplegia in pediatric arterial switch surgery. Methods: A 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. Results: Baseline 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). Conclusion: The 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 Number: ClinicalTrials.gov, ID: NCT04616222.

Focus on echocardiographic and Doppler analysis of coronary artery abnormal origin from the pulmonary trunk with mild myocardial dysfunction.

BACKGROUND: Late presentation of abnormal origin of coronary artery from the pulmonary artery (ACAPA) is uncommon compared with early presentation, which usually induces extended myocardial necrosis and severe heart failure. The late presentation is characterized by abundant development of intercoronary collaterals resulting in mild and rare symptoms, but nevertheless can cause sudden cardiac death. Our objective was to describe presentation, cardiovascular imaging methods for diagnosis and outcomes of patients with late presentation of ACAP. METHODS: The study is a retrospective review of a single-center database to identify all patients diagnosed with ACAPA beyond the first year of life. RESULTS: From 1976 to 2011, 10 patients were identified with ACAPA at the age of 1.1-64 years: 6 with left coronary artery from the pulmonary artery (ALCAPA) and 4 with right coronary artery from the pulmonary artery (ARCAPA). Echocardiography and Doppler imaging evidenced: (1) direct signs: the abnormal coronary ostium arising from the pulmonary trunk with retrograde coronary artery flow and (2) indirect signs: abundant intercoronary septal collaterals with anterograde flow (ARCAPA) or retrograde flow (ALCAPA) and dilatation of the controlateral normally originated coronary artery. Nine patients underwent surgical implantation of the ACAPA into the ascending aorta. After 7.9 years mean follow-up, all were asymptomatic except one who required a second surgery. CONCLUSIONS: Noninvasive cardiovascular imaging, namely transthoracic echocardiography and Doppler specific parameters, can reach diagnosis of late presentation of ACAPA. Direct aortic implantation is a reliable and effective to establish dual coronary artery circulation and prevent risks due to myocardial ischemia.

Ability of the third-generation FloTrac/Vigileo software to track changes in cardiac output in cardiac surgery patients: a polar plot approach.

OBJECTIVE: To evaluate the ability of the third-generation (3.01) of FloTrac/Vigileo monitor (Edwards Lifesciences, Irvine, CA) to follow variations in cardiac output (∆CO) using the new polar plot approach. DESIGN: Prospective interventional study. SETTING: Single hospital university study. PARTICIPANTS: Twenty-five patients referred for cardiac surgery. INTERVENTIONS: CO was measured simultaneously by 3 to 5 bolus thermodilution (COtd measurements), using a pulmonary artery catheter and by arterial pulse contour analysis, using the FloTrac/Vigileo (COvi). Data were collected at eight time points: before incision, after sternotomy, before and after protamine sulfate infusion, at the start of sternal closure, at the end of surgery, on arrival to intensive care unit, and after a standardized volume expansion with 500 mL of hetastarch 6%. MEASUREMENTS AND MAIN RESULTS: One-hundred thirty-five pairs of CO data were collected; the mean bias of all CO measurements corrected for repeated measures was 0.2 L/min with limits of agreements of -3.3 L/min and +2.9 L/min. The percentage error was 66.5%. The polar plot analysis included 71 significant ∆CO and showed a mean polar angle of -3.4 degrees with 95% polar percentage error equivalent limits of -61 to 55; 69% of analysed data points fell within the 30-degree limits and provided a correct polar concordance rate. CONCLUSIONS: Third-generation FloTrac/Vigileo software still lacks the accuracy to reliably detect changes in cardiac output (∆CO) in cardiac surgery. Improvements to FloTrac/Vigileo CO algorithm and software still are needed in this particular setting.

Systematic Electrophysiological Study Prior to Pulmonary Valve Replacement in Tetralogy of Fallot: A Prospective Multicenter Study.

BACKGROUND: Ventricular arrhythmias and sudden death are recognized complications in tetralogy of Fallot. Electrophysiological studies (EPS) before pulmonary valve replacement (PVR), the most common reintervention in tetralogy of Fallot, could potentially inform therapy to improve arrhythmic outcomes. METHODS: A prospective multicenter study was conducted to systematically assess EPS with programmed ventricular stimulation in patients with tetralogy of Fallot referred for PVR from January 2020 to December 2021. A standardized stimulation protocol was used across all centers. RESULTS: A total of 120 patients were enrolled, mean age 39.2±14.5 years, 53.3% males. Sustained ventricular tachycardia was induced in 27 (22.5%) patients. When identifiable, the critical isthmus most commonly implicated (ie, in 90.0%) was between the ventricular septal defect patch and pulmonary annulus. Factors independently associated with inducible ventricular tachycardia were history of atrial arrhythmia (odds ratio, 8.56 [95% CI, 2.43-34.73]) and pulmonary annulus diameter >26 mm (odds ratio, 5.05 [95% CI, 1.47-21.69]). The EPS led to a substantial change in management in 23 (19.2%) cases: 18 (15.0%) had catheter ablation, 3 (2.5%) surgical cryoablation during PVR, and 9 (7.5%) defibrillator implantation. Repeat EPS 5.1 (4.8-6.2) months after PVR was negative in 8 of 9 (88.9%) patients. No patient experienced a sustained ventricular arrhythmia during 13 (6.1-20.1) months of follow-up. CONCLUSIONS: Systematically performing programmed ventricular stimulation in patients with tetralogy of Fallot referred for PVR yields a high rate of inducible ventricular tachycardia and carries the potential to alter management. It remains to be determined whether a standardized treatment approach based on the results of EPS will translate into improved outcomes. REGISTRATION: URL: https://clinicaltrials.gov/ct2/show/NCT04205461; Unique identifier: NCT04205461.

Long term outcome of laryngeal mobility disorder and quality of life after pediatric cardiac surgery.

INTRODUCTION: Laryngeal mobility disorder after a pediatric heart surgery is common (between 5 and 10% of cases), and has important consequences on swallowing, breathing and speaking. After reviewing the literature, the recovery rate is variable and the postoperative follow-up is often done on a short time frame. The primary objective of the study is to describe the recovery from laryngeal mobility disorder with a follow-up time of at least 5 years. The secondary objective is to describe of the quality of life of the child in terms of phonation and swallowing, and to identify potential risk factors for a lasting laryngeal mobility disorder. METHODS: We collected data (morphological characteristics and details of the procedures and medical care) on children who had undergone a heart surgery with risks of complications, between 2010 and 2015, and with a laryngeal mobility disorder detected after the surgery through nasal flexible laryngoscopy. During a follow-up consultation, carried at least 5 years after the surgery, we performed a nasal flexible laryngoscopy to assess whether or not the patient had recovered a full mobility of the larynx. Two questionnaires were also given to the patients, the pVHI and the PEDI EAT-10, to assess respectively the quality of their speech and of their swallowing function. RESULTS: The recovery rate for a laryngeal mobility disorder more than 5 years after surgery was found to be 65% (9 children out of the 14 included in the study). We identified a risk factor for the persistence of a laryngeal mobility disorder after surgery: the presence of an associated genetic syndrome, p = 0.025. Children with persistent laryngeal mobility disorder have an impaired quality of life score, using the pVHI scale, which correlates well with the flexible laryngoscopy findings, p = 0.033. CONCLUSION: Children with a lasting laryngeal mobility disorder have disabling respiratory and vocal symptoms in their daily lives. Nasal flexible laryngoscopy should therefore be systematically performed postoperatively after a surgery carrying risks. For improved patient management, early detection of these disorders by pharyngolaryngeal nasal flexible laryngoscopy in the aftermath of high-risk cardiac surgery is strongly advised, with prolonged follow-up.

Incidence, Risk Factors, and Outcomes of Atrial Arrhythmias in Adult Patients With Atrioventricular Septal Defect.

OBJECTIVES: This study aimed to assess the incidence, associated factors, and outcomes of atrial arrhythmias in adults with atrioventricular septal defect (AVSD). BACKGROUND: Data regarding atrial arrhythmias in adults with AVSD are particularly scarce. METHODS: Data were analyzed from a multicentric cohort of adult patients with AVSD. Lifetime cumulative incidences of atrial arrhythmias were studied. Multiple logistic regression models were used to identify risk factors. RESULTS: A total of 391 patients (61.6% women) were enrolled with a mean age of 36.3 ± 16.3 years and a mean follow-up of 17.3 ± 14.2 years after initial surgical repair. Overall, 98 patients (25.1%) developed at least 1 episode of atrial arrhythmia at a mean age of 39.2 ± 17.2 years. The mean ages of patients at first episode of intra-atrial re-entrant tachycardia (IART)/ focal atrial tachycardia (FAT) and atrial fibrillation were 33.7 ± 15.3 and 44.3 ± 16.5 years, respectively. The lifetime risks for developing atrial arrhythmia to ages 20, 40, and 60 years were 3.7%, 17.8%, and 55.3%, respectively. IART/FAT was the leading arrhythmia until the age of 45, then atrial fibrillation surpassed IART/FAT. Age (odds ratio [OR]: 1.4; 95% confidence interval [CI]: 1.2-1.6), number of cardiac surgeries (OR: 4.1; 95% CI: 2.5-6.9), left atrial dilatation (OR: 3.1; 95% CI: 1.4-6.8), right atrial dilatation (OR: 4.1; 95% CI: 1.7-10.3), and moderate or severe left atrioventricular valve regurgitation (OR: 3.7; 95% CI: 1.2-11.7) were independently associated with a higher risk of atrial arrhythmias, whereas the type of AVSD and the age at repair were not. The occurrence of atrial arrhythmias was associated with pacemaker implantation (41.8% vs. 8.5%; P < 0.001), heart failure (24.5% vs. 1.0%; P < 0.001), and cerebrovascular accidents (11.2% vs. 3.4%; P = 0.007). CONCLUSIONS: The lifetime risk of atrial arrhythmias in patients with AVSD is considerable with more than half of patients developing ≥1 atrial arrhythmia by the age of 60 and is associated with a significant morbidity. The risk in partial/intermediate AVSD is as high as in complete AVSD and is not impacted by age at repair.

Transpulmonary thermodilution in a pediatric patient with an intracardiac left-to-right shunt.

Monitoring of cardiac output (CO) in the perioperative period and in seriously ill pediatric patients is of major importance for medical management. Hemodynamic monitoring, using transpulmonary thermodilution (TPTD) via a single thermal indicator injection, allows for measurements of CO, volumetric variables and extravascular lung water (EVLW). We describe and explain the influence of a left-to-right shunt on TPTD curve characteristics and EVLW measurements in a young child undergoing a surgical atrial septal defect repair. We suggest that these specific changes in the TPTD curve and the overestimation of EVLW detected by current device, in absence of gas exchange abnormalities, could be indicators of existing circulatory shunts in pediatric patients.

Excision of Wilms' Tumor With Atrial Extension Under Moderate Hypothermia and Cerebral Perfusion.

BACKGROUND: Wilms' tumor is the most common pediatric renal tumor. Almost half of all cases have involvement of the inferior vena cava, which must be addressed at the time of surgical excision. Further extension into the right atrium may pose an immediate vital risk and necessitates special operative techniques that employ cardiopulmonary bypass. CASE REPORT: We report the case of a child with a left Wilms' tumor with inferior caval and right atrial involvement, which led to significant hemodynamic compromise and urgent surgery. A left nephrectomy and cavoatrial thrombectomy were performed via a sterno-laparotomy. Our strategy employed moderate hypothermic circulatory arrest at 26°C and antegrade cerebral perfusion in order to improve visualization and ensure complete thrombectomy and protection of the abdominal organs. CONCLUSION: This case emphasizes the advantages of moderate hypothermic circulatory arrest compared to deep hypothermic circulatory arrest and normothemic cardiopulmonary bypass.

Health Profile of Precarious Migrants Attending the Médecins Du Monde's Health and Social Care Centres in France: a Cross-Sectional Study.

Objective: The present study aimed to compare the precarious migrants' health problems managed in Médecins du Monde's health and social care centres (CASO) with those of patients attending general practice in France. Methods: We compared the most frequent health problems managed in the 19 CASO in metropolitan France with those of a national sample of usual general practice consultations, after standardisation for age and sex. Results: Precarious migrants had fewer health problems managed per consultation than other patients (mean: 1.31 vs. 2.16), and these corresponded less frequently to chronic conditions (21.3% vs. 46.8%). The overrepresented health problems among CASO consultations were mainly headache (1.11% vs. 0.45%), viral hepatitis (1.05% vs. 0.20%), type 1 diabetes (1.01% vs. 0.50%) and teeth/gum disease (1.01% vs. 0.23%). Their underrepresented health problems were mainly lipid disorder (0.39% vs. 8.20%), depressive disorder (1.36% vs. 5.28%) and hypothyroidism (0.50% vs. 3.08%). Prevention issues were nominal in precarious migrants (0.16%). Conclusion: Both chronic somatic and mental conditions of precarious migrants are presumably underdiagnosed. Their screening should be improved in primary care.

Direct Reimplantation Procedure in Anomalous Aortic Origin of the Right Coronary Artery: Long-Term Single Center Outcomes.

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.

Impact of Pulmonary Valve Replacement on Ventricular Arrhythmias in Patients With Tetralogy of Fallot and Implantable Cardioverter-Defibrillator.

OBJECTIVES: This study aimed to assess the impact of pulmonary valve replacement (PVR) on ventricular arrhythmias burden in a population of tetralogy of Fallot (TOF) patients with continuous cardiac monitoring by implantable cardioverter-defibrillators (ICDs). BACKGROUND: Sudden cardiac death is a major cause of death in TOF, and right ventricular overload is commonly considered to be a potential trigger for ventricular arrhythmias. METHODS: Data were analyzed from a nationwide French ongoing study (DAI-T4F) including all TOF patients with an ICD since 2000. Survival data with recurrent events were used to compare the burden of appropriate ICD therapies before and after PVR in patients who underwent PVR over the study period. RESULTS: A total of 165 patients (mean age 42.2 ± 13.3 years, 70.1% male) were included from 40 centers. Over a median follow-up period of 6.8 (interquartile range: 2.5 to 11.4) years, 26 patients (15.8%) underwent PVR. Among those patients, 18 (69.2%) experienced at least 1 appropriate ICD therapy. When considering all ICD therapies delivered before (n = 62) and after (n = 16) PVR, the burden of appropriate ICD therapies was significantly lower after PVR (HR: 0.21; 95% confidence interval [CI]: 0.08 to 0.56; p = 0.002). Respective appropriate ICD therapies rates per 100 person-years were 44.0 (95% CI: 35.7 to 52.5) before and 13.2 (95% CI: 7.7 to 20.5) after PVR (p < 0.001). In the overall cohort, PVR before ICD implantation was also independently associated with a lower risk of appropriate ICD therapy in primary prevention patients (HR: 0.29 [95% CI: 0.10 to 0.89]; p = 0.031). CONCLUSIONS: In this cohort of high-risk TOF patients implanted with an ICD, the burden of appropriate ICD therapies was significantly reduced after PVR. While optimal indications and timing for PVR are debated, these findings suggest the importance of considering ventricular arrhythmias in the overall decision-making process. (French National Registry of Patients With Tetralogy of Fallot and Implantable Cardioverter Defibrillator [DAI-T4F]; NCT03837574).

Congenital native interruption of aortic arch in an adult: extra-anatomic approach by right-side thoracotomy.

We outline the case of an 18-year-old male patient with a congenital nonoperated interruption of the aortic arch. A right thoracotomy without cardiopulmonary bypass facilitated repair through an extra-anatomic bypass between the ascending and the supradiaphragmatic descending aorta. Results for the immediate and 2-year radiologic and clinical check-up were satisfactory. The most common complications in anatomic correction are stroke under selective cerebral perfusion, risk of paraplegia, and hemorrhage. We present a new technique for repair of interruption of the aortic arch in adults that avoids the need for extended dissection of the aorta and a partial occlusion clamp during anastomosis and allows for cerebral and medullar perfusion.

Subtle bacterial endocarditis due to Kingella kingae in an infant: a case report.

A 9-month-old infant presented with fever, dyspnoea, and a murmur. Echocardiography showed a mitral vegetation with significant regurgitation. Mitral valve plasty was performed on day 6, and was polymerase chain reaction positive for Kingella kingae. The cardiac outcome was favourable. This case illustrates a subtle presentation of K. kingae mitral valve infective endocarditis in a normal-cardaic infant, treated with early surgery, and the agent belonged to the HACEK (Haemophilus spp Actinobacillus actinomycetemcomitans, Capnocytophaga spp, Cardiobacterium hominis, Eikenella corrodens, and Kingella kingae) group.