Outcomes after the Ross procedure with pulmonary autograft reinforcement by reimplantation.

Background: Pulmonary autograft reinforcement to prevent dilatation and subsequent neo-aortic valve regurgitation has been reported; however, data on long-term function of the neo-aortic valve after this modified Ross procedure are lacking. Our objective here was to assess long-term outcomes of the modified Ross procedure with autograft reinforcement using the reimplantation technique. Patients: The outcomes of 61 consecutive patients managed using the Dacron-conduit reinforced Ross procedure between 2009 and 2021 were reviewed. Most patients had a unicuspid or bicuspid aortic valve (n = 52; 85%), predominant aortic valve regurgitation (n = 42; 77%), and >30 mm dilatation of the ascending aorta (n = 33; 54%). A prior aortic valve procedure was noted in 47 patients (77%) patients, including 38 (62%) with surgical repair and 9 (15%) with balloon dilatation. The pulmonary autograft was reimplanted within a Dacron conduit with a median diameter of 25.6 mm (range, 20-30 mm) using the David valve-sparing aortic root replacement technique. Results: All patients survived. The median age at surgery was 16.8 years (range, 6-38 years). Neo-aortic valve replacement was required in 3 patients (4.9%; 95% CI, 0.34%- 12.7%) because of infective endocarditis, left ventricular false aneurysm, and leaflet perforation, respectively; the repeat procedure was done early in 2 of these patients (2 of 61; 3%). Six patients required right ventricular outflow conduit replacement, 5 by surgery and 1 percutaneously. The median duration of follow-up was 90 months (range, 10-124 months). The 5- and 10-year rates of reintervention-free survival were 84.3% (95% CI, 74%-95%) and 81.6% (95% CI, 72%-93%), respectively, and 5-year survival without aortic reintervention was 94.5% (95% CI, 88%-100%), with little change at 10 years. No patients experienced deterioration of initial neo-aortic valve function (ie, regurgitation or stenosis). Conclusions: Autograft reinforcement using the reimplantation technique allowed expansion of Ross procedure indications to all patients requiring aortic valve replacement and prevented neo-aortic root dilatation. Failures were uncommon. Long-term follow-up data showed stable neo-aortic valve function.

Fusión de imágenes de tomografía computarizada cardiaca y ecocardiografía: un nuevo enfoque en las cardiopatías congénitas / Cardiac tomography-echocardiography imaging fusion: a new approach to congenital heart disease

Introducción y objetivos: El diagnóstico y manejo terapéutico de pacientes con cardiopatías congénitas se basa en gran medida en los resultados de la ecocardiografía. Las diferentes técnicas de fusión de imagen han supuesto un reciente avance en la obtención de imágenes cardíacas. Nuestro objetivo ha sido el de evaluar la viabilidad de la fusión de la tomografía computarizada (TC) y la ecocardiografía transtorácica (ETT) 3D en niños y adultos con cardiopatías congénitas. Métodos: Se incluyeron de forma prospectiva 13 pacientes con cardiopatías congénitas y 1 paciente con corazón normal los cuales se sometieron a TC y ETT 3D como parte de su seguimiento habitual. Se describieron los pasos necesarios para completar el proceso de fusión (alineación, puntos de referencia y superposición), la navegación y la evaluación de las imágenes. Resultados: La edad media fue de 9,5 años [2,7-15,7], el 57% eran varones y la superficie corporal media fue de 0,9 m2 [0,6-1,7]. Se clasificaron las cardiopatías como simples (n=4, 29%), moderadas (n=4, 29%) o complejas (n=6, 42%). La fusión 3D ETT-TC fue exitosa en todos los pacientes. La mediana del tiempo total para completar el proceso de fusión fue de 735 segundos [628-1163], sin diferencias significativas según el grado de complejidad de la cardiopatía. Los puntos de referencia se modificaron significativamente en las cardiopatías congénitas complejas. Conclusiones: La técnica de fusión 3D ETT-TC en una población de niños y adultos con diversas cardiopatías congénitas es viable y precisa según demostramos. La visualización simultánea de diferentes estructuras cardíacas podría ayudar a comprender las características anatómicas de las cardiopatías congénitas sin limitaciones en cuanto a la edad, el peso o la complejidad.(AU)

Introduction and objectives: Diagnosis, management, and surgical decision-making in children and adults with congenital heart disease are largely based on echocardiography findings. A recent development in cardiac imaging is fusion of different imaging modalities. Our objective was to evaluate the feasibility of computed tomography (CT) and 3-dimensional (3D) transthoracic echocardiography (TTE) fusion in children and adults with congenital heart disease. Methods: We prospectively included 14 patients, 13 of whom had congenital heart disease, and who underwent both CT and 3D TTE as part of their usual follow-up. We described the steps required to complete the fusion process (alignment, landmarks, and superimposition), navigation, and image evaluation. Results: Median age was 9.5 [2.7-15.7] years, 57% were male, and median body surface area was 0.9 m2 [0.6-1.7]. Congenital heart disease was classified as simple (n=4, 29%), moderate (n=4, 29%), or complex (n=6, 42%). 3D TTE-CT fusion was successful in all patients. Median total time to complete the fusion process was 735 [628-1163] seconds, with no significant difference according to the degree of complexity of the defects. Landmarks were significantly modified in complex congenital heart disease. Conclusions: We established the feasibility and accuracy of 3D TTE-CT fusion in a population of children and adults with a variety of congenital heart diseases. The simultaneous visualization of many intracardiac structures may help to understand the anatomical features of congenital heart disease without limitations regarding age, weight, or complexity of the congenital defects.(AU)

Cardiac tomography-echocardiography imaging fusion: a new approach to congenital heart disease.

INTRODUCTION AND OBJECTIVES: Diagnosis, management, and surgical decision-making in children and adults with congenital heart disease are largely based on echocardiography findings. A recent development in cardiac imaging is fusion of different imaging modalities. Our objective was to evaluate the feasibility of computed tomography (CT) and 3-dimensional (3D) transthoracic echocardiography (TTE) fusion in children and adults with congenital heart disease. METHODS: We prospectively included 14 patients, 13 of whom had congenital heart disease, and who underwent both CT and 3D TTE as part of their usual follow-up. We described the steps required to complete the fusion process (alignment, landmarks, and superimposition), navigation, and image evaluation. RESULTS: Median age was 9.5 [2.7-15.7] years, 57% were male, and median body surface area was 0.9 m2 [0.6-1.7]. Congenital heart disease was classified as simple (n=4, 29%), moderate (n=4, 29%), or complex (n=6, 42%). 3D TTE-CT fusion was successful in all patients. Median total time to complete the fusion process was 735 [628-1163] seconds, with no significant difference according to the degree of complexity of the defects. Landmarks were significantly modified in complex congenital heart disease. CONCLUSIONS: We established the feasibility and accuracy of 3D TTE-CT fusion in a population of children and adults with a variety of congenital heart diseases. The simultaneous visualization of many intracardiac structures may help to understand the anatomical features of congenital heart disease without limitations regarding age, weight, or complexity of the congenital defects.

Electric toy car to reduce anxiety before a cardiac catherisation: randomised controlled trial.

BACKGROUND: Anxiety before an invasive intervention is associated in children with persistent psychological disorders. We studied the effect of the transfer to the catheterisation room by an electric toy car on the anxiety of children and their parents before a cardiac catheterisation. METHODS: Forty-eight children with a median age of 5.6 years [4.2-7.0] were randomised to either riding on an electric car to go to the catheterisation laboratory or being transported lying supine on a gurney. Anxiety assessments were performed by a physician blinded to group allocation on the day before the procedure (T0) and at anaesthesia induction (T1). The modified Yale Preoperative Anxiety Scale Short Form (mYPAS-SF) and visual analogue scale for anxiety (VAS-A) were used in the children and the VAS-A in the parents. RESULTS: The mYPAS-SF, VAS-A-child, and the VAS-A-parent scores were significantly higher at T1 than at T0 (p < 0.001, p < 0.001, and p = 0.005, respectively). The primary outcome (the median mYPAS-SF score at T1) was not significantly different in the two groups when males and females were combined. At T1, the VAS-A-child score, however, was significantly lower in the intervention than the control group (22 versus 55, p < 0.001). In the boys, the median mYPAS-SF score at T1 was significantly lower in the intervention group (25.0 versus 51.0, p = 0.024). No difference was observed in girls. The VAS-A parent score was lower at T1 in the intervention group (60 versus 87, p = 0.05). CONCLUSION: Riding to the catheterisation laboratory on an electric toy car decreased anxiety in boys and decreased parental anxiety.

Anomalous aortic origin of coronary arteries: is the unroofing procedure always appropriate?

OBJECTIVES: Anomalous aortic origin of the coronary artery (AAOCA) with an interarterial ± an intramural course is a rare anomaly that carries a high risk of ischaemic events and even sudden death. The unroofing of the intramural course has been adopted as the gold standard surgical treatment. However, some anatomical forms need alternative techniques. METHODS: We reviewed the surgical cohort with AAOCA managed at our institution between 2005 and 2019 and analysed the anatomical and clinical outcomes. RESULTS: Thirty-nine patients underwent surgical interventions. The median age was 14 years (10-26 years). Twenty-eight patients (72%) had right AAOCA, and 11 (28%) had left AAOCA. Thirty-one (80%) patients presented with symptoms. The symptoms were chest pain in 22 patients (56%), syncope in 5 patients (13%), cardiac arrest during exercise in 2 patients (5%), dyspnoea in 6 patients (15%) and dizziness in 13 patients (33%). An ischaemic test was performed in 32 patients: Only 4 patients (10%) had positive results from the ischaemic test. All patients had computed tomography angiography scans to confirm the precise anatomical features of the anomaly. Repair techniques included 30 unroofing procedures (77%) with an associated translocation of the pulmonary artery for 11 patients in our early experience. In 6 patients the unroofing procedure was not feasible because of the absence of an intramural distinct segment or was judged intraoperatively not appropriate. A reimplantation of the anomalous coronary artery was performed in 2 patients (5%); 3 patients had coronary artery bypass grafting procedures (7%); and 3 (8%) had an isolated translocation of the pulmonary artery. There were no early or late deaths. All patients were free of symptoms. Computed tomography angiography scans performed in 31 cases showed a patent, non-restrictive coronary artery ostium. Seventeen patients underwent postoperative ischaemia testing and showed no evidence of ischaemia. CONCLUSIONS: Surgical correction in AAOCA is mandatory both for symptomatic and for asymptomatic patients with evidence of myocardial ischaemia under stress or with a restricted coronary artery segment. Surgical unroofing remains the gold standard but is not appropriate for all forms: alternative techniques should be considered. Surgical results are promising.

4D flow cardiac magnetic resonance in children and adults with congenital heart disease: Clinical experience in a high volume center.

BACKGROUND: Cardiac magnetic resonance (CMR) imaging with velocity encoding along all three directions of flow, known as 4DFlow CMR, provides both anatomical and functional information. Few data are available on the usefulness of 4DFlow CMR in everyday practice. Here, our objective was to investigate the usefulness of 4DFlow CMR for assessing congenital heart disease (CHD) in everyday practice. METHODS: From 2017 to 2019, consecutive patients who underwent 4DFlow CMR were included prospectively at a single high-volume centre. The parameters recommended by an expert's consensus statement for each diagnosis (congenital valvulopathy, septal defect, complex CHD, tetralogy of Fallot, aortic abnormalities) were assessed by two blinded experienced readers. 4DFlow CMRs that provided all recommended parameters were considered successful. Inter-observer and intra-observer agreement were investigated. RESULTS: We included 187 adults and 60 children covering broad ranges of weight (4.5-142 kg) and age (0.1-67 years). 4DFlow CMR was always the second-line imaging modality, after inconclusive echocardiography, and was successful in 231/247 (91%) patients, with no significant difference between children and adults (54/60, 90%; and 177/187, 95%; respectively; p = .13). Longer time using 4DFlow CMR at our centre was associated with success; in children, older age was also associated with exam success. There was an about 12-month learning curve in children. The success rate was lowest in neonates. Inter-observer and intra-observer agreement were substantial. CONCLUSION: Our results suggest that 4DFlow CMR usually provides a comprehensive assessment of CHD in adults and children. A learning curve exists for children and the investigation remains challenging in neonates.

Critical Congenital Heart Diseases in Preterm Neonates: Is Early Cardiac Surgery Quite Reasonable?

Prematurity is a recognized risk factor for morbidity and mortality following cardiac surgery. Postoperative and long-term outcomes after cardiac surgery performed in the preterm period are poorly described. The aim of this study was to analyze a population of preterm neonates operated on for critical congenital heart disease (CHD) before 37 weeks of gestational age (wGA) with special attention given to early and late mortality and morbidity. Between 2000 and 2013, 28 preterm neonates (median gestational age (GA) 34.3 weeks) underwent cardiopulmonary bypass (CPB) surgery for critical CHD before 37 wGA; records were retrospectively reviewed. All patients except three with single ventricle physiology had a single-stage anatomic repair. Overall mortality was 43 % (95 % CI 25-62). Risk factors for death were birth weight (p = 0.032) and weight at surgery (p = 0.037), independently of GA, preoperative status, CPB and aortic clamp time. Seven patients, including those with univentricular hearts, died during the postoperative period, and five in the first year after surgery. Median follow-up was 5.9 years (range 1 month-12.8 years). Kaplan-Meier survival rate was 75 % (95 % CI 59-91) at 1 month, and 57 % (95 % CI 39-75) at 1 and 5 years. Eight patients required reoperations after a delay of 2.8 ± 1.3 months; eight had bronchopulmonary dysplasia. At the end of follow-up, nine patients were asymptomatic. One-stage biventricular repair for critical CHD on preterm neonates was feasible. Mortality remained high but acceptable, mainly confined to the first postoperative year and related to small weight. Despite reoperations, long-term clinical status was good in most survivors. Further long-term prospective investigations are necessary to evaluate neurodevelopmental outcomes.

Feasibility of creating a novel animal heart model to test transcatheter techniques for a cavocaval connection that mimics a Fontan completion.

OBJECTIVE: The objective of this study was to create a novel animal model to foster the future development of interventional techniques for a cavocaval connection that mimics a Fontan completion. METHODS: Ten sheep were studied. All had the superior vena cava-right atrium junction closed using a polytetrafluoroethylene membrane. A valveless Gore-Tex conduit connecting the terminal portion of the superior vena cava to the right atrium was used to bypass the polytetrafluoroethylene occlusion and to allow normal venous drainage through the right atrium. Radio-opaque nitinol rings were placed around the inferior vena cava near its entry in the right atrium to allow better fluoroscopic visualization and to enhance stent stability during transcatheter cavocaval connection. RESULTS: The first 3 animals died during the learning curve as a result of technical issues. The subsequent 7 surviving sheep showed good flow dynamics on cardiac catheterization. Transcatheter cavocaval connection was performed successfully performed in 6 sheep at 0 to 9 months after the initial surgery. Sacrifice was done electively in all animals at 1 to 9 months per protocol. One animal was euthanized early because of an untreatable infection. One sheep was observed as a control without a transcatheter cavocaval connection and was sacrificed at 12 months. CONCLUSIONS: A novel, chronic ovine model to foster development of transcatheter techniques for cavocaval connection to mimic a Fontan completion was created. The successful model is easily reproducible after a short learning curve and shows good survival.

Outcomes and safety of transcatheter pulmonary valve replacement in patients with large patched right ventricular outflow tracts.

BACKGROUND: Although globally accepted, the indication for implantation of the Melody(®) (Medtronic Inc., Minneapolis, MN, USA) transcatheter pulmonary valve is limited to the treatment of haemodynamically dysfunctional right ventricular outflow tract (RVOT) with right ventricle to pulmonary artery (PA) obstruction. The use of the Melody valve for haemodynamically significant isolated pulmonary regurgitation has not been evaluated. AIM: We evaluated the outcomes of Melody valve insertion in patients with a large patched RVOT. METHODS: We analysed procedural and short-term outcomes data from 13 patients who underwent Melody valve implantation for a large RVOT with significant pulmonary regurgitation as the primary lesion. RVOT preparation was done in all patients using the Russian dolls technique and/or the PA jailing technique. Melody valve insertion was performed concomitantly in 10 patients and after 1 to 3 months in three patients. RESULTS: All procedures were successful. The mean follow-up period was 30 ± 4 months after the procedure. There was no incidence of stent fracture, migration or embolization. Only one patient who underwent the jailing technique developed a significant paraprosthetic leak and is scheduled for redilatation of the Melody valve. CONCLUSIONS: Careful patient selection, balloon sizing and RVOT preparation with prestenting using the Russian dolls technique and/or the PA jailing technique are required to modify the RVOT for transcatheter valve implantation. Short-term follow-up showed competent valves with no stent fracture or migration and appears promising. Wider experience with long-term outcomes may be required to standardize the procedure in such a subset of patients.

Fetal cardiac interventions: myths and facts.

An early, primary, in utero cardiac abnormality may prevent normal heart development and cause irreversible secondary structural changes. The idea of foetal cardiac intervention stems from this understanding and focuses on antenatal intervention targeting the primary abnormality to allow normal flow and haemodynamics and thus normal heart development. Crucial aspects of foetal vascular access, varying foetal lie and structural complexity make it very hard to set procedural standards. The procedures are complex and are associated with significant maternal and foetal morbidity and mortality. The high risk-benefit ratio clearly explains the investigational nature of such therapies. With the development of minimally invasive techniques and continued animal experiments, foetal interventional therapy may see a low rate of morbidity and mortality, improving the prognosis of newborns with congenital heart disease previously considered incurable.

[Follow-up of tetralogy of Fallot after repair]. / Surveillance d'une tétralogie de Fallot opérée.

Repair of tetralogy of Fallot (TOF) exists for more than 40 years. This repair results in a pulmonary regurgitation, which is usually well tolerated for two decades or so, but eventually this is injurious for the right ventricle (RV). The RV enlargement and severe RV dysfunction increase risk for ventricular tachycardia (VT) and sudden death in the long-term. The pulmonary valve replacement (PVR) is shifting earlier to preserve RV function before patients develop symptoms. Several parameters have to be considered to facilate correct timing for PVR (surgically of by catheterization) : echocardiography, magnetic resonance imaging, electrocardiogram and cardiopulmonary exercise. All patients should have regular follow-up in a specialized grown-up congenital heart disease (GUCH) center to detect as soon as possible pathological signs of RV enlargement. Implantable cardioverter-defibrillator (ICD) implantation for primary prevention and programmed ventricular stimulation in repaired TOF remain controversal.

Prevalence of preoperative arrhythmias in children with delayed treatment of severe congenital heart disease.

AIM: Our aim was to determine the real importance of rhythm and conduction disorders in children with unoperated severe congenital heart disease. METHODS: Consecutive children with delayed treatment of severe congenital heart disease were prospectively studied for the occurrence of arrhythmias before any invasive investigation or surgical procedure was performed. RESULTS: All 168 children were in sinus rhythm. One hundred and fifty-eight patients (94%) had no significant preoperative findings. One child with double discordance had an intermittent complete atrioventricular block, and another one had a long QT syndrome. Children with severe ventricular dysfunction had paroxysmal atrioventricular re-entry tachycardia in 3 cases and abnormally frequent premature ventricular complexes in 3 other cases. Children with severe left atrial dilatation had periods of atrial ectopic tachycardia in one case and atrial fibrillation in another case. CONCLUSIONS: The prevalence of rhythm and conduction disorders is relatively low in children with delayed treatment of severe congenital heart disease. Only those with congenital heart disease classically combined with such disorders and those with prolonged severe ventricular dysfunction and/or atrial dilatation are at risk of developing significant arrhythmias and should undergo a preoperative assessment of arrhythmias.