Single-Shot Cold Histidine-Tryptophan-Ketoglutarate Cardioplegia for Long Aortic Cross-Clamping Durations in Neonates.

OBJECTIVE: More than 30% of European pediatric cardiac surgery centers use single-dose cold histidine-tryptophan-ketoglutarate cardioplegia (Custodiol; Dr Franz Köhler Chemie GmbH, Bensheim, Germany). In neonates with transposition of the great arteries, arterial switch surgery (ASO) implies aortic division, and it is unknown whether repeated ostial cannulation causes intimal insult and affects long-term results, and therefore, single-dose Custodiol is appealing. The present study investigated the association among myocardial no-flow duration, postoperative troponins, and postoperative outcomes in neonates undergoing ASO with Custodiol cardioplegia. DESIGN: Retrospective analysis of the association among myocardial no-flow duration, postoperative troponin release (concentration magnitude × measurement duration within 48 h), and outcomes using stratification according to coronary anatomy and attending surgeon. SETTING: Single-institutional, tertiary pediatric cardiac surgery unit of a university hospital. PARTICIPANTS: The study comprised 101 neonates undergoing ASO. INTERVENTIONS: None. MEASUREMENTS AND MAIN RESULTS: The mean age of patients was 6.1 ± 5.4 days, the cardiopulmonary bypass duration was 108.7 ± 54.1 minutes, the temperature during cross-clamping was 31.1°C ± 1.7°C, the duration of mechanical ventilation was 4 (3-6) days, the length of intensive care unit stay was 7 (5-8) days, delayed sternal closure occurred in 32 (31.7%) patients, and no patients died. The myocardial no-flow duration averaged 62.3 ± 14.6 minutes and was linked with both troponin release (p = 0.04) and low cardiac output syndrome, as assessed by the requirement for delayed sternal closure (p = 0.03), regardless of cardiopulmonary bypass duration and temperature. Eighty-two percent of the patients with myocardial no-flow duration >74 minutes necessitated delayed sternal closure. CONCLUSIONS: Single-dose Custodiol may be inadequate for prolonged cross-clamping durations without myocardial perfusion in neonates.

Crossed pulmonary arteries as additional cause of dysphagia in association with right aortic arch and Kommerell diverticulum.

We describe an uncommon association of crossed pulmonary arteries and a right aortic arch with a Kommerell diverticulum and a left ligamentum arteriosum, resulting in disabling dysphagia in a 33-year-old woman. First, endovascular exclusion of the Kommerell diverticulum was performed using a thoracic stent graft, associated with left subclavian-carotid transposition. Second, open aneurysmorrhaphy and division of the left ligamentum arteriosum allowed a proper release of the oesophageal compression. Dysphagia completely disappeared in the postoperative course. Control computed tomography angiography at 6-month follow-up showed a satisfactory hybrid repair. A complete understanding of the combined effects of these two anatomical variations on oesophageal compression led to a suitable surgical management.

Anomalous Aortic Origin of a Coronary Artery is Always a Surgical Disease.

Anomalous aortic origin of a coronary artery is a congenital anomaly in which a major coronary artery arises from the wrong sinus of Valsalva (left coronary from right sinus or right coronary from left sinus) and courses between the great arteries before reaching its normal epicardial course. Although the risk of sudden death is clearly established, the indications for surgery remain controversial. The risk of sudden death is increased in symptomatic patients, in anomalous left coronary artery, as well as in the presence of some risk factors (intense physical activity, young age [<35 years], aggravating anatomical features [intramural interarterial course, slit-like ostium, acute angle of take-off]). As far as is currently known, surgical management using an anatomical repair can prevent sudden death, provided that normal coronary anatomy and function are achieved and that extensive return-to-play testing is performed. A precise evaluation of the benefit/risk ratio is mandatory on an individual basis, but surgery may be indicated in the vast majority of patients.

[Anomalous aortic origin of coronary arteries: a frequent and curable cause of sudden death]. / Les anomalies de naissance des artères coronaires à partir de l'aorte: une cause fréquente et évitable de mort subite.

Anomalous aortic origin of coronary arteries is a congenital heart defect in which one coronary artery arises from the opposite sinus (the left coronary artery from the right coronary sinus, or the right coronary artery from the left sinus). The initial segment of the abnormal artery courses between the great arteries and is usually located within the aortic wall. These anomalies are far from rare, affecting 0.1% to 0.3% of the population, and carry a high risk of sudden cardiac death. It is thought that the main mechanism of cardiac death is external compression of the abnormal coronary artery between the great arteries as they expand during strenuous exercise. The risk of sudden death is particularly high in patients with an anomalous left coronary artery or associated anatomic lesions (stenotic intramural segment, slit-like deformation of the ostium), and also in young athletes. A common presentation is sudden unexplained death. The diagnosis can be made by echocardiographic examination. Silent myocardial ischemia must be sought, with stress echocardiography and stress nuclear imaging. Various surgical techniques have been described. Our own technique involves the creation of a neo-ostium in the appropriate sinus. Surgical treatment is relatively safe and provides satisfactory results, most patients having no residual myocardial ischemia under stress. Surgery is recommended for all patients with an anomalous left coronary artery, regardless of symptom status, as well as for symptomatic patients with an anomalous right coronary artery (aborted sudden death, symptoms under stress, myocardial ischemia under stress). Because of the incidence and severity of these anomalies, careful echocardiographic evaluation should be performed routinely, at least in young athletes.

[Mechanical circulatory support and replacement in heart failure]. / Assistance et suppléance mécaniques de la fonction cardiaque défaillante.

MECHANICAL CIRCULATORY SUPPORT AND REPLACEMENT IN HEART FAILURE. Technological developments have led to a panel of mechanical devices making possible circulatory support and cardiac replacement in patients with heart failure. Some devices (namely extracorporeal membrane oxygenation or ECMO) allow short-term support in patients with acute heart failure and improve the prognosis. Other devices (left ventricular assist device and total artificial heart) are designed for long-term use in patients with advanced heart failure; although heart transplantation remains the gold standard for these patients, mechanical support devices may keep patients alive until transplantation or become destination therapy if transplantation is contraindicated. The optimal use of mechanical circulatory support needs a strong organizational network of specialized multidisciplinary centers.

ASSISTANCE ET SUPPLÉANCE MÉCANIQUES DE LA FONCTION CARDIAQUE DÉFAILLANTE. Les progrès de la technologie ont permis la mise au point de dispositifs efficaces d'assistance et de suppléance de la fonction cardiaque défaillante. Les uns, destinés à une utilisation de courte durée (essentiellement extracorporeal membrane oxygenation [ECMO]), sont mis en place en urgence et donnent des chances de survie à des patients présentant une défaillance cardiaque aiguë menaçant le pronostic vital à très court terme. Les autres (dérivations ventriculaires implantées et coeurs artificiels) sont destinés à une utilisation prolongée ; ils sont utilisés chez les patients en insuffisance cardiaque avancée, en complémentarité avec la transplantation cardiaque, qui reste le traitement optimal ; ils permettent soit l'attente d'un greffon, soit une survie dans des conditions acceptables si la greffe n'est pas réalisable. L'utilisation optimale des techniques d'assistance circulatoire nécessite l'organisation d'un réseau performant de centres multidisciplinaires labélisés.

Outcomes of aortic valve repair in children stratified by complexity: Which outcome for which lesion?

OBJECTIVE: Aortic valvuloplasty frequency has significantly increased over the past 15 years. Surgical repair varies in complexity depending on valvular lesions. Our aim is to report results on the whole spectrum of aortic valvuloplasty techniques. METHODS: All children who consecutively underwent aortic valvuloplasty for aortic stenosis and/or aortic insufficiency between January 2006 and December 2020 at Necker Sick Children's Hospital (Paris, France) were included in a retrospective cohort study. Aortic valvuloplasty techniques were classified into 3 difficulty levels: (1) simple repair, corresponding to commissurotomy and/or shaving in aortic stenosis (AS) in neonates (group 1) and children >1 month (group 2); (2) intermediate-complexity repair, corresponding to commissuroplasty, leaflet resuspension, and fenestration closure in aortic insufficiency (leaflet prolapse in connective tissue disease, isolated leaflet prolapse and Laubry-Pezzi groups); and (3) complex repair requiring a pericardial patch to restore a functional aortic valve in mixed aortic valve disease (bicuspidization with neocommissure and cusp extension groups). RESULTS: During the study period, 324 children underwent aortic valvuloplasty. Survival and freedom from aortic valve reintervention at 10 years were, respectively, 86.1% and 50.9% in neonates with AS, 95.2% and 71.7% in children >1 month with AS, 93.8% and 79.5% in leaflet prolapse in connective tissue disease, 97.7% and 91.9% in isolated leaflet prolapse, 100% and 88% in those with Laubry-Pezzi syndrome, 97.4% and 84.8% in bicuspidization with neocommissure, and 100% and 54.2% in the cusp extension. CONCLUSIONS: Durability of aortic valvuloplasty techniques is satisfactory and offers the possibility to delay the Ross procedure, regardless of the lesion's complexity.

Pitfalls in repair of conotruncal anomalies.

Despite a wide anatomic diversity, the complete repair of all conotruncal anomalies includes two surgical steps. 1) An intracardiac tunnel is created to connect the left ventricle to one of the arterial orifices (usually the aortic, sometimes the pulmonary), through the conoventricular ventricular septal defect. Any conal septum should be resected to create a short, large, and straight tunnel. Abnormal insertions of the atrioventricular valves (tricuspid and mitral) on the conal septum should be preserved. "Intramural" residual ventricular septal defects must be avoided by anchoring the intracardiac patch directly to the arterial annulus. 2) To connect the right ventricle to the pulmonary artery, either an intracardiac or an extracardiac reconstruction is carried out, according to the distance between the tricuspid valve and the pulmonary orifice. When extracardiac reconstruction is indicated, it is usually performed without prosthetic conduit (with or without French maneuver, eventually using the left atrial appendage). In most patients, complete repair can be performed as a primary operation during infancy.

[Quality of life after pediatric heart transplantation]. / Qualité de vie après transplantation cardiaque chez L'Enfant.

The first pediatric heart transplant was performed more than 25 years ago. The results, in terms of mortality and morbidity, have gradually improved over the years, and quality of life and development are thus becoming increasingly important issues. Functional capacity is considered excellent by most recipients: 90% have few if any symptoms. Objective exercise capacity is usually subnormal, however, mainly owing to the inability of the heart rate to increase with exercise. Early cardiac rehabilitation should be encouraged in order to optimize the capacity for physical activity. Recipients can be expected to have neurodevelopmental outcomes in the low-to-normal range, with a 10- to 15-point deficit compared with normal children. Global cognitive abilities and school performance can be affected, particularly in mathematics. Moreover, 20% to 30% of these patients have behavioral and psychological disorders, which are relatively stable over time. Neurodevelopmental outcome is poorer in patients grafted because of a congenital heart defect and is consistent with that of other children with complex congenital heart diseases requiring surgical intervention. This suggests that these problems may be related more to the underlying heart disease than to transplantation itself. Neurodevelopmental outcome is better in families with good intellectual and socioeconomic status. A global, multidisciplinary approach is needed to manage these problems, both early after transplantation and later during follow-up, particularly during the transition from childhood and adulthood.

Long-term results after the réparation à l'étage ventriculaire procedure for transposition of the great arteries and double-outlet right ventricle with pulmonary stenosis.

OBJECTIVES: The purpose of this study is to describe the long-term results of the 'réparation à l'étage ventriculaire' (REV) technique for double-outlet right ventricle and transposition of the great arteries (TGA) with pulmonary stenosis (PS). METHODS: Between 1980 and 2021, 157 patients underwent a REV procedure (median age and weight: 20.8 months and 7.7 kg). The most frequent anatomical presentation was the association between TGA, ventricular septal defect and PS (n = 116, 73.9%). RESULTS: Sixty-seven patients (42.7%) underwent a Rashkind procedure, and 67 patients (42.7%) a prior surgical palliation (including 62 systemic-to-pulmonary artery shunts). Resection of the conal septum and/or ventricular septal defect enlargement was performed in 109 patients (69.4%). Thirteen patients (8.3%) died, including 4 during the first postoperative month and 2 after heart transplant. Overall survival at 40 years was 89.3%. Thirty-seven patients (23.6%) required 68 reinterventions on the right ventricular outflow tract (RVOT), including 49 reoperations, with a median delay of 9 years after the REV (8 months to 27 years). Twenty patients (12.7%) underwent RVOT valvulation (16 surgical and 4 interventional). Freedom from RVOT reintervention and reoperation at 40 years were 60.3% and 62.6%, respectively. Four patients (2.5%) required reoperation for left ventricular outflow tract obstruction, with a median delay of 4.8 years. CONCLUSIONS: The REV procedure is a good alternative for TGA and double-outlet right ventricle with PS patients. Only a quarter of the patients required redo surgery on the RVOT. Reoperations for left ventricular outflow tract obstruction are scarce.

Early initiation of peritoneal dialysis in neonates and infants with acute kidney injury following cardiac surgery is associated with a significant decrease in mortality.

Association between early renal replacement therapy and better survival has been reported in adults with postoperative kidney injury, but not in children undergoing cardiac surgery. We conducted a retrospective cohort study of 146 neonates and infants requiring peritoneal dialysis following cardiac surgery in a tertiary referral hospital. A propensity score was used to limit selection bias due to timing of dialysis, and included baseline and intraoperative characteristics, requirement for postoperative extracorporeal membrane oxygenation, and creatinine clearance variation. Inverse probability of treatment weighting resulted in good balance between groups for all baseline and intraoperative variables. After weighting, 30-day and 90-day mortality were compared between the 109 patients placed on dialysis early, within the first day of surgery, and those with delayed dialysis, commencing on the second day of surgery or later, using logistic regression and survival analysis. Mortality was 28.1% at 30 days, and was 36.3% during follow-up. Early dialysis was associated with a 46.7% decrease in the 30-day and a 43.5% decrease in the 90-day mortality rate when compared with delayed dialysis. All other short-term outcome variables were similar. Thus, initiation of peritoneal dialysis on the day of or the first day following surgery was associated with a significant decrease in mortality in neonates and infants with acute kidney injury.

[Transposition of the great arteries: a curable congenital heart defect?]. / Transposition des gros vaisseaux. Une malformation congenitale curable?

Transposition of the great arteries (TGA) is a common congenital heart malformation, involving the inversion of both great vessels (aorta and pulmonary artery). It is not compatible with life in the absence of surgical treatment. The prognosis of this malformation has been transformed by the development of neonatal cardiac surgery. "Anatomic" repair has been introduced in the 80s; its goal is to provide near-normal cardiac anatomy. The mid-term results of anatomic repair of TGA can be summarized as follows: overall mortality is 5% (most deaths occur during the first postoperative year and are related to coronary complications), 5% of patients need reoperation for various reasons (mainly, coronary obstruction and pulmonary stenosis), major sequelae are rare (1-2%), minor sequelae are frequent (15-20%) and require strict surveillance, most patients (70-75%) are leading a normal life and can be considered as "cured". Very long-term results remain to be determined. The management of TGA includes several steps: prenatal diagnosis, planned delivery close to a pediatric cardiology center, immediate treatment with Rashkind maneuver and/or PGE1 infusion, anatomic repair during the first week of life, life-long surveillance by a congenital cardiologist. This complex multidisciplinary approach emphasizes the need for specialized centers of congenital cardiology from fetal life to adulthood.

[Transplantation cardiaque pédiatrique : quel futur ?] / The future of pediatric heart transplantation.

The future of pediatric heart transplantation. Pediatric heart transplantation developed in the 1980s, following the introduction of cyclosporine. The International Registry includes more than 14 000 patients (10 % of the whole). The results improved progressively. However, two drawbacks persist : a shortage of donors, particularly in infants and a high morbi-mortality on the long term. The rapid achievement of a pediatric artificial heart is unlikely. The future offers two directions. Firsly xenotransplantation : the production of genetically-modified pigs and new immuno-suppressive modalities allow long-term survival in heterologous pig/primate transplantation. Human clinical trials may begin soon, particularly in neonates. Secondly tissue engineering : constant advances (scaffolds, cells lines, growth factors) may make possible the production of a functional heart from the receivor's own stem-cells.

Transplantation cardiaque pédiatrique : quel futur ? La transplantation cardiaque pédiatrique s'est développée dans les années 1980, avec l'avènement de la ciclosporine. Le Registre International compte plus de 14 000patients (10 % de l'ensemble). Les résultats se sont progressivement améliorés, mais deux écueils persistent : la pénurie de donneurs, en particulier chez le nourrisson, et la morbi-mortalité élevée à très long terme. Le développement rapide d'un coeur artificiel adapté à l'enfant est peu probable et l'avenir s'oriente dans deux directions. D'une part la xénotransplan¬tation, la production de porcs génétiquement modifiés et des trai¬tements immunosuppresseurs innovants ayant permis la survie prolongée de greffons cardiaques entre porc et primate. Les essais cliniques semblent proches, en particulier chez le nouveau-né. D'autre part l'ingénierie tissulaire ; en effet des progrès constants (matrices, lignées cellulaires, facteurs de croissance) laissent en¬trevoir la possibilité de créer un coeur fonctionnel à partir des propres cellules du receveur.

Ross procedure or complex aortic valve repair using pericardium in children: A real dilemma.

OBJECTIVE: Difficult to repair aortic valve lesions, requiring the use of a valve substitute, remain controversial in the face of the Ross procedure, despite undeniable technical advances. This study was undertaken to compare midterm outcomes of children treated using the Ross procedure or aortic valvuloplasty for complex aortic valve lesions. METHODS: Between January 2006 and December 2017, 126 patients aged younger than 18 years were treated for complex aortic stenosis and/or aortic insufficiency and were included in this retrospective study. Only aortic valve lesions requiring repair with an autologous or heterologous pericardial patch were considered complex lesions. Propensity score framework analyses were used to compare outcomes of the Ross and aortic valvuloplasty groups while controlling for confounders. RESULTS: Among the 126 patients with complex aortic valve lesions, propensity score matching selected 34 unique pairs of patients with similar characteristics. Survival (aortic valvuloplasty, 94.1%; Ross, 91%; P = .89), freedom from overall reintervention (aortic valvuloplasty, 50.1%; Ross, 69%; P = .32), and freedom from infective endocarditis at 8 years (aortic valvuloplasty, 100%; Ross, 85.9%; P = .21) were similar. However, freedom from reintervention in the left ventricular outflow tract at 8 years was lower after aortic valvuloplasty than after the Ross procedure (50.1% vs 100%, respectively; P = .001). CONCLUSIONS: Aortic valvuloplasty and the Ross procedure yielded similar 8-year outcomes regarding death, reoperation, and infective endocarditis although aortic valvuloplasty tended to be associated with fewer cases of infective endocarditis. Aortic valvuloplasty using a pericardial patch can be chosen as a first-line strategy for treating complex aortic valve lesions and might offer the possibility of a later Ross procedure.

[Anatomic correction of transposition of the great arteries: a model of surgical "cure" in pediatric cardiology?]. / La correction anatomique de la transposition des gros vaisseaux: un modèle de "guérison" en chirurgie cardiaque pédiatrique?

Transposition of the great arteries (TGA) is a common congenital heart malformation, involving inversion of the two great vessels (aorta and pulmonary artery). Without surgical treatment, TGA is not compatible with life. The prognosis of this malformation has been dramatically changed by the development of neonatal cardiac surgery. "Anatomic" repair was introduced in the 1980s, with the goal of providing near-normal cardiac anatomy. It is carried out in neonates with isolated TGA (so-called simple TGA), as well as in those with associated anomalies (ventricular septal defect, aortic coarctation). Since January 1987, 1020 neonates have undergone anatomic repair of TGA in the pediatric cardiac surgery department of Groupe Hospitalier Laennec-Necker-Enfants Malades in Paris. The overall mortality rate was 4.8% (49 patients), and all deaths occurred within the first postoperative year. Among the 966 survivors who could be monitored (mean follow-up 6.2 years), 52 children (5.4%) underwent reoperation, with no additional mortality. Nearly all the survivors have normal cardiac function and quality of life. Despite significant mortality (albeit currently low) anda low risk of reoperation, the mid-term results of anatomic repair of TGA are excellent and represent a surgical "cure" in most cases. Very-long-term outcome remains to be determined.

[Pediatric heart surgery in developing countries. Twenty years experience of La Chaine de L'espoir (Chain of Hope)]. / La chirurgie cardiaque pédiatrique dans les pays en voie de développement. Vingt ans d'expérience de la chaîne de l'Espoir.

Because of their high incidence, cardiac disorders in children are now a public health issue. These children require multidisciplinary management, but surgery is sometimes unavoidable. The two most frequent types of cardiac disorder in children are congenital defects and rheumatic valve disease. La Chaine de l'Espoir (Chain of Hope) is present in more than 30 countries in Africa, Asia and the Middle East. Due to the socio-economic context and lack of technical resources, pediatric heart surgery has several particular characteristics in developing countries. Infants rarely undergo cardiac surgery, given the complexity of their management and particularly the need for intensive care. Another specificity is the need to limit treatment to reparatory or conservative methods. Surgery is rarely attempted in terminally ill patients. Costs must be kept to a minimum, and this is why we have optedfor local manufacture of mitral rings, led by Daniel Roux. After 20 years of practice we have found that regional hospitals are the smallest structures that can offer acceptable results in terms of patient care and professional training

Three-dimensional geometry of coronary arteries after arterial switch operation for transposition of the great arteries and late coronary events.

OBJECTIVE: Using 3-dimensional (3D) modeling to predict late coronary events after the arterial switch operation (ASO) for transposition of the great arteries (TGA). METHOD: We reviewed 100 coronary computed tomography scans performed after ASO randomly selected from free-from-coronary-event patients and 21 coronary computed tomography scans from patients who had a coronary event later than 3 years after ASO. Using 3D modeling software, we defined and measured 6 geometric criteria for each coronary artery: Clockwise position of coronary ostium, First centimeter angle defined as the angle between of the coronary artery ostium and the first centimeter of the vessel, Minimal 3D angle between the coronary first centimeter and the aortic wall, ostium height defined as the distance between the ostium and the aortic valve, distance between the coronary ostium and the pulmonary artery, and distance between the coronary first centimeter and the pulmonary artery. RESULTS: None of the right ostium geometric parameters were associated with coronary events. Four out of 6 criteria of left coronary artery geometry were associated to coronary events: Clockwise position of the left ostium >67° (P < .001), First centimeter angle >62° (P < .01), minimal 3D angle <39° (P = .003), distance between the coronary ostium and the pulmonary artery <1 mm/mm (P = .03). The association of first centimeter angle >62° and minimal angle in 3D <39° had a 88% sensitivity and a 81% specificity to predict coronary events (receiver operator characteristics curve, 0.847; 95% confidence interval, 0.745-0.949; P < .001). CONCLUSIONS: The acquired geometric characteristics of the transferred left coronary artery are associated with coronary events. Imaging coronary arteries after ASO might be useful to select patients at higher risk of coronary events and to tailor surveillance.

Potts anastomosis in children with severe pulmonary arterial hypertension and atrial septal defect.

AIMS: Potts shunt has been proposed as a bridge or alternative to lung transplantation for children with severe and drug-refractory suprasystemic pulmonary arterial hypertension (PAH). We describe the management of the atrial shunt when a Potts shunt is planned in refractory PAH. METHODS AND RESULTS: We report a case series of children in whom a Potts shunt was done for severe PAH associated with an atrial septal defect to illustrate the different clinical and haemodynamic scenarios. Five children (2 to 13 years) underwent a Potts shunt: three surgical, one percutaneous Potts shunt, and one percutaneous stenting of a restrictive arterial duct. All had associated atrial septal defect. Those who had generalized cyanosis before the procedure had a complicated postoperative course and required longer ventilatory and inotropic support, except the one who had atrial septal defect closure before the Potts shunt. One of the three cyanotic patients died. Two patients with left-to-right shunt before the Potts shunt had an uncomplicated postoperative course. CONCLUSIONS: Shunt physiology is only partially predictable after the Potts shunt in children with PAH and atrial septal defect. Abrupt drop in left ventricle preload while the right ventricle is decompressed can potentially be prevented by atrial septal defect closure prior to the Potts shunt.

Catheter ablation in adults with congenital heart disease: A 15-year perspective from a tertiary centre.

BACKGROUND: With the growing adult congenital heart disease (ACHD) population, the number of catheter ablation procedures is expected to dramatically increase. Data reporting experience and evolution of catheter ablation in patients with ACHD, over a significant period of time, remain scarce. AIM: We aimed to describe temporal trends in volume and outcomes of catheter ablation in patients with ACHD. METHODS: This was a retrospective observational study including all consecutive patients with ACHD undergoing attempted catheter ablation in a large tertiary referral centre over a 15-year period. Acute procedural success rate and freedom from recurrence at 12 and 24 months were analysed. RESULTS: From November 2004 to November 2019, 302 catheter ablations were performed in 221 patients with ACHD (mean age 43.6±15.0 years; 58.9% male sex). The annual number of catheter ablations increased progressively from four to 60 cases per year (P<0.001). Intra-atrial reentrant tachycardia/focal atrial tachycardia was the most common arrhythmia (n=217, 71.9%). Over the study period, acute procedural success rate increased from 45.0% to 93.4% (P<0.001). Use of irrigated catheters (odds ratio [OR] 4.03, 95% confidence interval [CI] 1.86-8.55), a three-dimensional mapping system (OR 3.70, 95% CI 1.72-7.74), contact force catheters (OR 3.60, 95% CI 1.81-7.38) and high-density mapping (OR 3.69, 95% CI 1.82-8.14) were associated with acute procedural success. The rate of freedom from any recurrence at 12 months increased from 29.4% to 66.2% (P=0.001). Seven (2.3%) non-fatal complications occurred. CONCLUSIONS: The number of catheter ablation procedures in patients with ACHD has increased considerably over the past 15 years. Growing experience and advances in ablative technologies appear to be associated with a significant improvement in acute and mid-term outcomes.

Circulating endothelial cells: a new candidate biomarker of irreversible pulmonary hypertension secondary to congenital heart disease.

BACKGROUND: Congenital heart disease can be complicated by pulmonary arterial hypertension (PAH), the reversibility of which is often difficult to predict. We recently reported a lung biopsy study showing impaired apoptotic regulation of endothelial cells in irreversible PAH. The objective of the present study was to identify noninvasive biomarkers of endothelial turnover that could be used to identify congenital heart disease patients at risk of irreversible PAH. METHODS AND RESULTS: Circulating endothelial cells (CECs) isolated with CD146-coated beads and circulating CD34(+)CD133(+) progenitor cells (CPCs) were quantified in peripheral vein, pulmonary artery, and pulmonary vein blood samples from 26 patients with congenital heart disease (16 with reversible PAH [median age 2 years] and 10 with irreversible PAH [median age 9 years]) and 5 control patients. Surgical lung biopsy was performed in 19 cases. As expected, endothelial remodeling was observed in irreversible PAH but not in reversible PAH. CEC and CPC numbers were each similar in the 3 types of blood samples. CEC numbers were significantly higher in patients with irreversible PAH (median 57 CEC/mL) than in patients with reversible PAH and control subjects (median 3 CEC/mL in the 2 groups). In contrast, CPC numbers did not differ among patients with irreversible or reversible PAH and control subjects (median 84, 64, and 44 CPC/10(5) lymphocytes, respectively, in the 3 groups). CONCLUSIONS: Irreversible PAH in congenital heart disease is associated with endothelial damage and with increased circulating endothelial cell counts. The present study suggests that CECs could be a valuable tool to define therapeutic strategies in congenital heart disease patients with PAH.