Long-term outcome after neonatal intervention for congenital critical aortic stenosis.

BACKGROUND: This study explored long-term outcome and functional status of patients born with critical aortic stenosis (CAS) following neonatal surgical or catheter interventions. METHODS: A 40-year retrospective review of all consecutive patients within a large, single-center referral unit who required neonatal (<30 days) intervention for CAS. Additional detailed evaluation of surviving patients >7 years age was performed, with clinical assessment, objective cardiopulmonary exercise testing and state-of-the-art characterization of myocardial function (advanced echocardiography and cardiac MRI). RESULTS: Between 1970 and 2010, ninety-six neonates underwent CAS intervention (mean age 9 ± 7.5 days). Early death occurred in 19 (19.8%) and late death in 10 patients. Overall survival at 10 and 30 years was 70.1% and 68.5%, freedom from reintervention was 41.8% and 32.9% respectively. Among the 25 long-term survivors available for detailed assessment (median age 15.7 ± 6.4 years), 55% exhibited impaired peak oxygen uptake. Mean left ventricle (LV) ejection fraction was 65 ± 11.2%, with a mean LV end-diastolic volume z-score of 0.02 ± 1.4. Mean LV outflow tract Vmax was 2.3 ± 1.02 m/s. CAS patients had reduced LV longitudinal and increased radial strain (p = 0.003, p < 0.001 respectively). Five patients had severe LV diastolic dysfunction associated with endocardial fibroelastosis (EFE) (p = 0.0014). CONCLUSION: Despite high early mortality rate, long-term survival of patients with CAS is reasonable at the expense of high reintervention rate. With successful intervention, there remained long-term clinical and subclinical LV myocardial impairment, of which EFE was one marker. Long-term follow-up of all CAS patients is crucial, involving detailed myocardial functional assessment to help elucidate physiology and optimise management.

Impact of Tracheal Arborization and Lung Hypoplasia in Repair of Pulmonary Artery Sling in Combination With Long-Segment Tracheal Stenosis.

Background: Reimplantation of the left pulmonary artery (LPA) and slide tracheoplasty has been our standard approach of care for patients with pulmonary artery sling (PAS) and tracheal stenosis. We present our experience, with emphasis on tracheal arborization and hypoplastic lungs; and their impact on long-term outcome of children with PAS and tracheal stenosis. Methods: It is a retrospective comparative study. Data were classified and analyzed based on the type of tracheobronchial arborization and normal versus hypoplastic lungs. Results: Seventy-five children operated between January 1994 and December 2019 (67 with normal lungs and 8 with lung hypoplasia/agenesis) were included. Patients with hypoplastic lungs had higher rates of preoperative ventilation (87.5%), postoperative ventilation (10 vs 8 days, P = .621), and mortality (50% vs 9%, P = .009) compared with those with normal lungs. Nineteen patients had tracheal bronchus (TB) variety and 30 patients had congenital long-segment tracheal stenosis (CLSTS) variety of tracheobronchial arborization. Endoscopic intervention was needed in 47.4% of patients with TB type and 60% with CLSTS type. CLSTS patients had higher rates of preoperative ventilation (60% vs 47.4%, P = .386), longer periods of postoperative ventilation (13 vs 6.5 days, P = .006), and ICU stay (15 vs 11 days, P = .714) compared with TB type. Conclusion: Surgical repair of PAS with tracheal stenosis has good long-term outcomes. All variations of tracheal anatomy can be managed with slide tracheoplasty. Persistence of airway problems requires intervention during follow-up as tracheal stenosis continues to be the Achilles heel.

Percutaneous heart valves demonstrating long-term durability: A case series of Melody valves in the pulmonary position lasting up to 19 years.

It is uncertain how long catheter delivered percutaneous heart valves may last. In congenital cardiology, stenosis and regurgitation of right ventricular to pulmonary artery conduits and valves is common, leading to repeated operations for young patients with concomitant mortality and morbidity. It has also been unclear whether percutaneous pulmonary valves last as long as surgical pulmonary valves. When the current generation of the percutaneous pulmonary valve was first implanted in the United Kingdom from 2003, randomized trials were initially not performed, decided on a case-by-case basis in congenital cardiology, nor long-term registries kept. We describe three cases where such percutaneous heart valves have lasted up to 19 years. All valves were working without significant stenosis and minor degrees of regurgitation on long-term echocardiographic follow-up, patients being asymptomatic. This demonstrates that percutaneous pulmonary valves can achieve long-term durability and may prevent the need for otherwise high-risk surgery in congenital cardiac patients.

Successful retrieval of embolised Occlutech atrial septal defect occluder from descending aorta in a 4-year-old child using a "mother and child" technique: a case report and literature review.

A 9 mm Occlutech septal occluder Flex II device was retrieved in a 4-year-old 22 kg child; A 6 Fr Cook® Flexor sheath (child) was inserted into a 9 Fr Occlutech® ASD Delivery Set (mother). Once the tip of the smaller sheath was close to the device, a 4 Fr right Judkins catheter was introduced with a snare. The right atrial hub was captured and withdrawn to the level of the 6 Fr sheath which was then withdrawn into the 9 Fr sheath before being removed completely. The "mother and child" technique offers a greater likelihood of slenderising and retrieving embolised devices.

Cardiac outcomes in severe acute respiratory syndrome coronavirus-2-associated multisystem inflammatory syndrome at a tertiary paediatric hospital.

INTRODUCTION: We describe a cohort of children referred with multisystem inflammatory syndrome in children associated with severe acute respiratory syndrome coronavirus 2 and compare this cohort with a 2019 cohort of children with Kawasaki disease. METHODS: We conducted a retrospective cohort study of 2019 and 2020 referrals to the inflammatory cardiology service at Great Ormond Street Hospital for Children. We compared cardiac and inflammatory parameters of a sub-section of the 2020 cohort who presented with reduced left ventricular ejection fraction with the remainder of the cohort. RESULTS: Referrals significantly increased between February and June 2020 compared to 2019 (19.8/30 days versus 3.9/30 days). Frequency of coronary artery aneurysms (11/79 (13.9%) versus 7/47 (14.9%)) or severe coronary artery aneurysms (6/79 (7.6%) versus 3/47 (6.4%)) was similar between 2020 and 2019, respectively. The 2020 cohort was older (median age 9.07 years versus 2.38 years), more likely to be of Black, Asian, or other minority ethnic group (60/76 (78.9%) versus 25/42 (59.5%)), and more likely to require inotropic support (22 (27.5%) versus 0 (0%)). Even children with significantly reduced left ventricular ejection fraction demonstrated complete recovery of cardiac function within 10 days (mean 5.25 days ± 2.7). DISCUSSION: We observed complete recovery of myocardial dysfunction and an overall low rate of permanent coronary sequelae, indicating that the majority of children with multisystem inflammatory syndrome in children are unlikely to encounter long-term cardiac morbidity. Although the frequency of myocardial dysfunction and inotropic support requirement is not consistent with a diagnosis of Kawasaki disease, the frequency of coronary artery abnormalities and severe coronary artery abnormalities suggests a degree of phenotypic overlap.

Repair of pulmonary artery sling with tracheal and intracardiac defects.

BACKGROUND: Pulmonary artery sling is commonly associated with tracheal stenosis and intracardiac anomalies. While surgical repair is standardized, coexistent anomalies often determine outcomes. With the paucity of risk stratification, this study aimed to review our experience and stratify risk factors for the surgical outcome of complex pulmonary artery sling repair in the presence of airway or intracardiac lesions. METHODS: Seventy-nine consecutive children with pulmonary artery sling were evaluated retrospectively following surgical repair. Median age at surgery was 5 months (interquartile range 3-9). Surgical approaches included pulmonary artery sling alone (n = 10), pulmonary artery sling with tracheoplasty (n = 41), and pulmonary artery sling with both intracardiac and tracheal surgery (n = 28). RESULTS: There were 7 early (8.8%) deaths. Two patients after left pulmonary artery reimplantation needed revision of the anastomosis. The median intensive care and hospital stay were 11 (interquartile range 9.2-24.8) and 17.9 (interquartile range 4.3-19.8) days, and considerably longer when associated tracheal surgery (p = 0.002). Follow-up was complete in 66/69 and 3 (3.8%) children died late: 2.7, 10.2, and 17 months after surgery. Univariate analysis showed abnormal lung and coexisting structural heart disease as risk factors. Multivariate analysis revealed total cardiopulmonary bypass time as an independent predictor of overall mortality. CONCLUSION: Complex pulmonary artery sling repair can be performed with a good surgical outcomes even when associated with airway malformations or structural heart diseases. Lung abnormality and longer cardiopulmonary bypass time as a surrogate marker of complex surgery, are possible risk factors.

Staging of Surgical Procedures in Comorbid Congenital Tracheal Stenosis and Congenital Cardiovascular Disease.

BACKGROUND: Comorbid long segment congenital tracheal stenosis and congenital cardiovascular abnormalities in children pose significant challenges with regard to repairing these abnormalities simultaneously or in stages. The aim of this study was to explore whether this combination of abnormalities needs a staged approach for surgical repairs. METHODS: All children who underwent both tracheal and cardiac surgical procedures at a tertiary hospital from 1995 to 2018 were analyzed retrospectively for mortality, ventilation days, postoperative intensive care unit days, mediastinitis, and unplanned reoperation by dividing them into simultaneous repairs (group 1), staged repairs within the same admission (group 2), and staged repairs during different admissions (group 3). RESULTS: Of 110 patients included in the study (group 1, 74; group 2. 10; and group 3, 26 patients), there was no significant difference in mortality (P = .85), median ventilation days (P = .99), median intensive care unit days (P = .23), unplanned airway reoperation (P = .36), and unplanned cardiac reoperation (P = .77). There was a significant difference in the rate of mediastinitis (group 1, 3%; group 2, 10%; and group 3, 19%; P = .02). There was no significant difference in 5-year survival (group 1, 86.2%; group 2, 77.8%; and group 3, 85.1%; P = .86). A higher STAT category was identified to be a risk factor for mortality in multivariate Cox regression analysis (relative risk, 5.45). CONCLUSIONS: Combined tracheal and cardiac abnormalities need a stratified approach to facilitate better clinical outcomes. Although the trajectory of care is often based on the clinical presentation, establishing a management protocol will be helpful, for which setting an international database will be useful.

Melody Valve in Aortic Position to Facilitate Mechanical Circulatory Support as a Bridge to Cardiac Transplantation.

We report a patient with congenital heart disease and end stage heart failure after interventions for congenital heart disease resulting in aortic regurgitation that could not be treated with mechanical support to bridge to transplantation. We used a Melody valve (Medtronic Inc, Minneapolis, MN) in aortic position to achieve aortic competence to allow use of a left ventricular assist device to bridge the patient successfully to cardiac transplantation.

Case report on surgical repair of unusual dissection of arterial duct involving main pulmonary artery in a child with vein of Galen malformation.

BACKGROUND: Dissection involving patent arterial duct (PDA) extending to main pulmonary artery (MPA) is an extremely rare condition. CASE SUMMARY: We report a case of complex dissection involving PDA extending to MPA, in a child with vein of Galen malformation. This was surgically corrected under deep hypothermic circulatory arrest, and we describe the pathophysiology and surgical rationale in this report. DISCUSSION: Management of intracardiac lesion in the presence of intracranial arterio-venous malformations such as vein of Galen is very difficult, and employing deep hypothermic circulatory arrest allows one to manage cerebral protection while offering the visualization of intracardiac anatomy more effectively.

Patient-specific simulations for planning treatment in congenital heart disease.

Patient-specific computational models have been extensively developed over the last decades and applied to investigate a wide range of cardiovascular problems. However, translation of these technologies into clinical applications, such as planning of medical procedures, has been limited to a few single case reports. Hence, the use of patient-specific models is still far from becoming a standard of care in clinical practice. The aim of this study is to describe our experience with a modelling framework that allows patient-specific simulations to be used for prediction of clinical outcomes. A cohort of 12 patients with congenital heart disease who were referred for percutaneous pulmonary valve implantation, stenting of aortic coarctation and surgical repair of double-outlet right ventricle was included in this study. Image data routinely acquired for clinical assessment were post-processed to set up patient-specific models and test device implantation and surgery. Finite-element and computational fluid dynamics analyses were run to assess feasibility of each intervention and provide some guidance. Results showed good agreement between simulations and clinical decision including feasibility, device choice and fluid-dynamic parameters. The promising results of this pilot study support translation of computer simulations as tools for personalization of cardiovascular treatments.

Outcome after transcatheter occlusion of patent ductus arteriosus in infants less than 6 kg: A national study from United Kingdom and Ireland.

OBJECTIVES: This study aimed to report our national experience with transcatheter patent ductus arteriosus (PDA) occlusion in infants weighing <6 kg. BACKGROUND: The technique of transcatheter PDA closure has evolved in the past two decades and is increasingly used in smaller patients but data on safety and efficacy are limited. METHODS: Patients weighing < 6 kg in whom transcatheter PDA occlusion was attempted in 13 tertiary paediatric cardiology units in the United Kingdom and Ireland were retrospectively analyzed to review the outcome and complications. RESULTS: A total of 408 patients underwent attempted transcatheter PDA closure between January 2004 and December 2014. The mean weight at catheterization was 4.9 ± 1.0 kg and mean age was 5.7 ± 3.0 months. Successful device implantation was achieved in 374 (92%) patients without major complication and of these, complete occlusion was achieved in 356 (95%) patients at last available follow-up. Device embolization occurred in 20 cases (5%). The incidence of device related obstruction to the left pulmonary artery or aorta and access related peripheral vascular injury were low. There were no deaths related to the procedure. CONCLUSIONS: Transcatheter closure of PDA can be accomplished in selected infants weighing <6 kg despite the manufacturer's recommended weight limit of 6 kg for most ductal occluders. The embolization rate is higher than previously reported in larger patients. Retrievability of the occluder and duct morphology needs careful consideration before deciding whether surgical ligation or transcatheter therapy is the better treatment option.

Piloting the Use of Patient-Specific Cardiac Models as a Novel Tool to Facilitate Communication During Cinical Consultations.

This pilot study aimed to assess the impact of using patient-specific three-dimensional (3D) models of congenital heart disease (CHD) during consultations with adolescent patients. Adolescent CHD patients (n = 20, age 15-18 years, 15 male) were asked to complete two questionnaires during a cardiology transition clinic at a specialist centre. The first questionnaire was completed just before routine consultation with the cardiologist, the second just after the consultation. During the consultation, each patient was presented with a 3D full heart model realised from their medical imaging data. The model was used by the cardiologist to point to main features of the CHD. Outcome measures included rating of health status, confidence in explaining their condition to others, name and features of their CHD (as a surrogate for CHD knowledge), impact of CHD on their lifestyle, satisfaction with previous/current visits, positive/negative features of the 3D model, and open-ended feedback. Significant improvements were registered in confidence in explaining their condition to others (p = 0.008), knowledge of CHD (p < 0.001) and patients' satisfaction (p = 0.005). Descriptions of CHD and impact on lifestyle were more eloquent after seeing a 3D model. The majority of participants reported that models helped their understanding and improved their visit, with a non-negligible 30% of participants indicating that the model made them feel more anxious about their condition. Content analysis of open-ended feedback revealed an overall positive attitude of the participants toward 3D models. Clinical translation of 3D models of CHD for communication purposes warrants further exploration in larger studies.

Can finite element models of ballooning procedures yield mechanical response of the cardiovascular site to overexpansion?

Patient-specific numerical models could aid the decision-making process for percutaneous valve selection; in order to be fully informative, they should include patient-specific data of both anatomy and mechanics of the implantation site. This information can be derived from routine clinical imaging during the cardiac cycle, but data on the implantation site mechanical response to device expansion are not routinely available. We aim to derive the implantation site response to overexpansion by monitoring pressure/dimensional changes during balloon sizing procedures and by applying a reverse engineering approach using a validated computational balloon model. This study presents the proof of concept for such computational framework tested in-vitro. A finite element (FE) model of a PTS-X405 sizing balloon (NuMed, Inc., USA) was created and validated against bench tests carried out on an ad hoc experimental apparatus: first on the balloon alone to replicate free expansion; second on the inflation of the balloon in a rapid prototyped cylinder with material deemed suitable for replicating pulmonary arteries in order to validate balloon/implantation site interaction algorithm. Finally, the balloon was inflated inside a compliant rapid prototyped patient-specific right ventricular outflow tract to test the validity of the approach. The corresponding FE simulation was set up to iteratively infer the mechanical response of the anatomical model. The test in this simplified condition confirmed the feasibility of the proposed approach and the potential for this methodology to provide patient-specific information on mechanical response of the implantation site when overexpanded, ultimately for more realistic computational simulations in patient-specific settings.

Doubly committed subarterial ventricular septal defect: A risk factor for aortic distortion in patients considered for percutaneous pulmonary valve implantation?

Percutaneous pulmonary valve implantation has gradually become the first line strategy for re-intervention for right ventricular outflow tract dysfunction during long-term follow-up after congenital cardiac surgery in many centers. We describe a case of a patient with double outlet right ventricle (Fallot's type) with a doubly committed subarterial ventricular septal defect, where the unique anatomy precluded percutaneous pulmonary valve implantation. © 2016 Wiley Periodicals, Inc.

Patient-specific computational models to support interventional procedures: a case study of complex aortic re-coarctation.

AIMS: We report the application of patient-specific computational models to plan the treatment of complex aortic re-coarctation (rCoA) with a proximal aberrant right subclavian artery in a patient who had previously undergone bare metal stenting. METHODS AND RESULTS: Clinically acquired images were used to set up patient-specific computational models for finite element (FE) and fluid dynamics (CFD) analyses. The 3D geometry was reconstructed from computed tomography and echocardiography images. Computer-generated deployment of a CP covered stent (NuMED, Hopkinton, NY, USA) at different diameters was tested using FE simulations. CFD analyses based on preoperative magnetic resonance flow measurements allowed assessment of rCoA pressure relief and right subclavian artery perfusion in the different scenarios. The simulations suggested an expansion diameter for the CP stent (8 zigs, length=28 mm) of between 16 and 18 mm to relieve the obstruction, cover the aneurysm and maintain satisfactory flow to the right subclavian artery. Following the modelling study, a 16 mm CP covered stent was successfully implanted. CONCLUSIONS: Patient-specific models can be successfully used to plan re-stenting of complex rCoA, showing the benefits of integrating computational techniques into patient management.

3D-manufactured patient-specific models of congenital heart defects for communication in clinical practice: feasibility and acceptability.

OBJECTIVES: To assess the communication potential of three-dimensional (3D) patient-specific models of congenital heart defects and their acceptability in clinical practice for cardiology consultations. DESIGN: This was a questionnaire-based study in which participants were randomised into two groups: the 'model group' received a 3D model of the cardiac lesion(s) being discussed during their appointment, while the 'control group' had a routine visit. SETTING: Outpatient clinic, cardiology follow-up visits. PARTICIPANTS: 103 parents of children with congenital heart disease were recruited (parental age: 43±8 years; patient age: 12±6 years). In order to have a 3D model made, patients needed to have a recent cardiac MRI examination; this was the crucial inclusion criterion. INTERVENTIONS: Questionnaires were administered to the participants before and after the visits and an additional questionnaire was administered to the attending cardiologist. MAIN OUTCOME MEASURES: Rating (1-10) for the liking of the 3D model, its usefulness and the clarity of the explanation received were recorded, as well as rating (1-10) of the parental understanding and their engagement according to the cardiologist. Furthermore, parental knowledge was assessed by asking them to mark diagrams, tick keywords and provide free text answers. The duration of consultations was recorded and parent feedback collected. RESULTS: Parents and cardiologists both found the models to be very useful and helpful in engaging the parents in discussing congenital heart defects. Parental knowledge was not associated with their level of education (p=0.2) and did not improve following their visit. Consultations involving 3D models lasted on average 5 min longer (p=0.02). CONCLUSIONS: Patient-specific models can enhance engagement with parents and improve communication between cardiologists and parents, potentially impacting on parent and patient psychological adjustment following treatment. However, in the short-term, parental understanding of their child's condition did not improve.

Patient-specific finite element models to support clinical decisions: A lesson learnt from a case study of percutaneous pulmonary valve implantation.

OBJECTIVES AND BACKGROUND: Patient-specific finite element (FE) simulations were used to assess different transcatheter valve devices and help select the most appropriate treatment strategy for a patient (17-year-old male) with borderline dimensions for Melody® percutaneous pulmonary valve implantation (PPVI). METHODS AND RESULTS: Patient-specific implantation site morphology was derived from cardiovascular magnetic resonance (CMR) images along with the implantation site mechanical behavior by coupling systolic/diastolic dimensions and the pressure gradient in a linear elastic model, and iterative tuning. In this way, the model accounted for the mechanical response not only of the arterial wall, but also of the surrounding tissue. Four stents (2 balloon-expandable including prestenting and 2 self-expandable) were virtually implanted and the stent final configuration, anchoring, migration forces, arterial wall stresses, paravalvular regurgitation, and device mechanical performance were evaluated. A Sapien29 device with prestenting was indicated as the optimal approach for this specific patient as it had a fully open valve, safe anchoring along the entire circumference, low risk of paravalvular leak, and arterial rupture. However, at the time of the PPVI procedure, after balloon sizing, device implantation was suspended due to perceived high risk of device embolization. CONCLUSIONS: FE analysis allows a comparison between different treatment scenarios to add information to the clinical decision making process. However, further studies are required to fully predict patient-specific response to stenting and therefore true clinical outcomes.

A multiscale model for the study of cardiac biomechanics in single-ventricle surgeries: a clinical case.

Complex congenital heart disease characterized by the underdevelopment of one ventricular chamber (single ventricle (SV) circulation) is normally treated with a three-stage surgical repair. This study aims at developing a multiscale computational framework able to couple a patient-specific three-dimensional finite-element model of the SV to a patient-specific lumped parameter (LP) model of the whole circulation, in a closed-loop fashion. A sequential approach was carried out: (i) cardiocirculatory parameters were estimated by using a fully LP model; (ii) ventricular material parameters and unloaded geometry were identified by means of the stand-alone, three-dimensional model of the SV; and (iii) the three-dimensional model of SV was coupled to the LP model of the circulation, thus closing the loop and creating a multiscale model. Once the patient-specific multiscale model was set using pre-operative clinical data, the virtual surgery was performed, and the post-operative conditions were simulated. This approach allows the analysis of local information on ventricular function as well as global parameters of the cardiovascular system. This methodology is generally applicable to patients suffering from SV disease for surgical planning at different stages of treatment. As an example, a clinical case from stage 1 to stage 2 is considered here.