Lifetime Management of Adolescents and Young Adults with Congenital Aortic Valve Disease.

In this invited expert review, we focus on evolving lifetime management strategies for adolescents and young adults with congenital aortic valve disease, acknowledging that these patients often require multiple interventions during their lifetime. Our goal is to preserve the native aortic valve when feasible. Leveraging advanced multimodality imaging, a detailed assessment of the aortic valve and root complex can be obtained, and a surgically approach tailored to an individual patient's anatomy and pathology can be used. In turn, aortic valve repair and reconstruction can be offered to a greater number of patients, either as a definitive strategy or as a component of a staged strategy to delay the need for aortic valve replacement until later in life when more options are available.

The Society of Thoracic Surgeons Clinical Practice Guidelines on the Management of Neonates and Infants with Coarctation.

BACKGROUND: Although coarctation of the aorta without concomitant intracardiac pathology is relatively common, there is lack of guidance regarding aspects of its management in neonates and infants. METHODS: A panel of experienced congenital cardiac surgeons, cardiologists, and intensivists was created, and key questions related to the management of isolated coarctation in neonates and infants were formed using the PICO (Patients/Population, Intervention, Comparison/Control, Outcome) Framework. A literature search was then performed for each question. Practice guidelines were developed with classification of recommendation and level of evidence using a modified Delphi method. RESULTS: For neonates and infants with isolated coarctation, surgery is indicated in the absence of obvious surgical contraindications. For patients with risk factors for surgery, medical management before intervention is reasonable. For those stable off prostaglandin E1, the threshold for intervention remains unclear. Thoracotomy is indicated when arch hypoplasia is not present. Sternotomy is preferable when arch hypoplasia is present that cannot be adequately addressed through a thoracotomy. Sternotomy may also be considered in the presence of a bovine aortic arch. Antegrade cerebral perfusion may be reasonable when the repair is performed through a sternotomy. Extended end-to-end, arch advancement, and patch augmentation are all reasonable techniques. CONCLUSIONS: Surgery remains the standard of care for the management of isolated coarctation in neonates and infants. Depending on degree and location, arch hypoplasia may require a sternotomy approach as opposed to a thoracotomy approach. Significant opportunities remain to better delineate management in these patients.

Perioperative Assessment of the Hemodynamic Ventriculoarterial Junction of the Aortic Root by Three-Dimensional Echocardiography.

Improved strategies in aortic valve-preserving operations appreciate the dynamic, three-dimensional complexity of the aortic root and its valve. This depends not only on detailed four-dimensional imaging of the planar dimensions of the aortic root but also on quantitative assessment of the valvar leaflets and their competency. The zones of apposition and resulting hemodynamic ventriculoarterial junction formed in diastole determine valvar competency. Current understanding and assessment of this junction is limited, often relying on intraoperative direct surgical inspection. However, this direct inspection itself is limited by evaluation in a nonhemodynamic state with limited field of view. In this review, we discuss the anatomy of the aortic root, including its hemodynamic junction. We review current echocardiographic approaches toward interrogating the incompetent aortic valve for presurgical planning. Furthermore, we introduce and standardize a complementary approach to assessing this hemodynamic ventriculoarterial junction by three-dimensional echocardiography to further personalize presurgical planning for aortic valve surgery.

Transcatheter Aortic Valve Replacement in Congenital Heart Disease.

Transcatheter aortic valve replacement is not widely used in patients with congenital heart disease. We describe our single-center experience of transcatheter aortic valve replacement in congenital heart disease, demonstrating short-term feasibility and safety, role in lifetime management of congenital aortic valve disease, and use as a bridge to recovery, future surgery, or transplantation.

The Ozaki Procedure: Standardized Protocol Adoption of a Complex Innovative Procedure.

Background: The Ozaki procedure using autologous pericardium is an interesting but complex alternative for aortic valve replacement. We present a standardized approach to minimize the learning curve and confirm reproducibility. Methods: After careful preparation, from May 2015 to February 2021, an Ozaki procedure was performed on 46 patients age 51 ± 14 years. Seven had unicuspid (15%), 29 bicuspid (63%), and 10 tricuspid (22%) aortic valves, and 2 patients had endocarditis. Endpoints were operative learning curves, perioperative outcomes, intermediate-term valve hemodynamics, reintervention, health-related quality of life (MacNew Heart Disease Health-Related Quality of Life questionnaire), and mortality. Results: Cardiopulmonary bypass and aortic clamp times decreased from 145 to 125 â€‹minutes and 120 to 100 â€‹minutes, respectively, over the first 20 cases, reflecting the learning curve. There was no major perioperative morbidity or mortality. Median postoperative stay was 6.9 days. Aortic regurgitation was mild or less in all but 2 patients who developed moderate aortic regurgitation. Mean aortic valve gradient was 7.9 mmHg postoperatively, 9.2 mmHg by 6 months, and constant thereafter. Left ventricular ejection fraction was 58% preoperatively, 60% at 6 months, and remained stable thereafter. One patient developed infective endocarditis 7 months postoperatively, failed medical management, and underwent valve replacement at 14 months. Two-year survival was 96%, with 1 noncardiac death at 16 months. Health-related quality of life in mental, physical, and emotional domains was better than matched norms, global 6.2 vs. 5.0 (p < 0.0001). Conclusions: Using a well-prepared standardized approach, the Ozaki procedure is reproducible with a short learning curve, excellent hemodynamic performance, and good quality of life.

Multifocal disease progression and subsequent intervention in patients with actin alpha-2 variants: A single-center experience.

OBJECTIVES: To describe patient characteristics and indications for surgical intervention, reoperation, and outcomes in patients with actin alpha-2 (ACTA2) variants. METHODS: A single-center retrospective cohort study with prospective follow-up was performed for 38 patients with an ACTA2 variant. RESULTS: From 1999 to 2020, 26 (70%) patients underwent surgery; 11 remain under surveillance (mean follow-up, 7.5 ± 5 years). Median age at index operation was 42 (range, 10-69) years, with 4 pediatric cases. Thoracic aortic aneurysm was present in 19 (73%) patients (mean adult max diameter, 5.2 ± 0.8 cm; pediatric z score, 10.7 ± 5.4). Aortic dissection was present in 13 (50%) patients, with 4 (15%) having type A dissection. Operations included replacement of the aortic root in 16 (17%), ascending aorta in 20 (77%), and aortic arch in 14 (54%) patients. Four (15%) patients had coronary artery disease, and 2 (7.7%) underwent concomitant coronary artery bypass grafting. There was no operative mortality, stroke, reoperation for bleeding, or dialysis-dependent renal failure; One (3.8%) patient developed acute on chronic kidney injury. Three patients (12%) required prolonged ventilation. Eleven (42%) patients underwent 26 reoperations, median time 45 (range, 4-147) months, including 5 open thoracoabdominal aneurysm repairs. CONCLUSIONS: Patients with ACTA2 variants frequently develop aortic aneurysm and are at risk of aortic dissection and coronary artery disease. However, age at diagnosis and symptoms at presentation are highly variable. Multiple operations are often required for disease management, particularly after dissection. Close monitoring and timely intervention are important in mitigating disease progression and improving outcomes.

Can perioperative electroencephalogram and adverse hemodynamic events predict neurodevelopmental outcomes in infants with congenital heart disease?

OBJECTIVE: The study objective was to characterize preoperative and postoperative continuous electroencephalogram metrics and hemodynamic adverse events as predictors of neurodevelopment in congenital heart disease infants undergoing cardiac surgery. METHODS: From 2010 to 2021, 320 infants underwent congenital heart disease surgery at our institution, of whom 217 had perioperative continuous electroencephalogram monitoring and were included in our study. Neurodevelopment was assessed in 76 patients by the Bayley Scales of Infant and Toddler Development, 3rd edition, consisting of cognitive, communication, and motor scaled scores. Patient and procedural factors, including hemodynamic adverse events, were included by means of the likelihood of covariate selection in our predictive model. Median (25th, 75th percentile) follow-up was 1.03 (0.09, 3.44) years with 3 (1, 6) Bayley Scales of Infant and Toddler Development, 3rd Edition evaluations per patient. RESULTS: Median age at index surgery was 7 (4, 23) days, and 81 (37%) were female. Epileptiform discharges, encephalopathy, and abnormality (lethargy and coma) were more prevalent on postoperative continuous electroencephalograms, compared with preoperative continuous electroencephalograms (P < .005). In 76 patients with Bayley Scales of Infant and Toddler Development, 3rd edition evaluations, patients with diffuse abnormality (P = .009), waveform discontinuity (P = .007), and lack of continuity (P = .037) on preoperative continuous electroencephalogram had lower cognitive scores. Patients with synchrony (P < .005) on preoperative and waveform continuity (P = .009) on postoperative continuous electroencephalogram had higher fine motor scores. Patients with postoperative adverse events had lower cognitive (P < .005) and gross motor scores (P < .005). CONCLUSIONS: Phenotypic patterns of perioperative continuous electroencephalogram metrics are associated with late-term neurologic injury in infants with congenital heart disease requiring surgery. Continuous electroencephalogram metrics can be integrated with hemodynamic adverse events in a predictive algorithm for neurologic impairment.

The Academic Impact of Congenital Heart Surgeons' Society (CHSS) Studies.

PURPOSE: We reviewed all 64 articles ever published by The Congenital Heart Surgeons' Society (CHSS) Data Center to estimate the academic impact of these peer-reviewed articles. MATERIALS AND METHODS: The Congenital Heart Surgeons' Society has performed research based on 12 Diagnostic Inception Cohorts. The first cohort (Transposition) began enrolling patients on January 1, 1985. We queried PubMed to determine the number of publications that referenced each of the 64 journal articles generated by the datasets of the 12 Diagnostic Inception Cohorts that comprise the CHSS Database. Descriptive summaries of the data were tabulated using mean with standard deviation and median with range. RESULTS: Sixty-four peer-reviewed papers have been published based on the CHSS Database. Fifty-nine peer-reviewed articles have been published based on the 12 Diagnostic Inception Cohorts, and five additional articles have been published based on Data Science. Excluding the recently established Diagnostic Inception Cohort for patients with Ebstein malformation of tricuspid valve, the number of papers published per cohort ranged from 1 for coarctation to 11 for transposition of the great arteries. The 11 articles generated from the CHSS Transposition Cohort were referenced by a total of 111 articles (median number of references per journal article = 9 [range = 0-22, mean = 10.1]). Overall, individual articles were cited by an average of 11 (mean), and a maximum of 41 PubMed-listed publications. Overall, these 64 peer-reviewed articles based on the CHSS Database were cited 692 times in PubMed-listed publications. The first CHSS peer-reviewed article was published in 1987, and during the 35 years from 1987 to 2022, inclusive, the annual number of CHSS publications has ranged from 0 to 7, with a mean of 1.8 publications per year (median = 1, mode = 1). CONCLUSION: Congenital Heart Surgeons' Society studies are widely referenced in the pediatric cardiac surgical literature, with over 10 citations per published article. These cohorts provide unique information unavailable in other sources of data. A tool to access this analysis is available at: [https://data-center.chss.org/multimedia/files/2022/CAI.pdf].

Advocacy at the Eighth World Congress of Pediatric Cardiology and Cardiac Surgery.

The Eighth World Congress of Pediatric Cardiology and Cardiac Surgery (WCPCCS) will be held in Washington DC, USA, from Saturday, 26 August, 2023 to Friday, 1 September, 2023, inclusive. The Eighth World Congress of Pediatric Cardiology and Cardiac Surgery will be the largest and most comprehensive scientific meeting dedicated to paediatric and congenital cardiac care ever held. At the time of the writing of this manuscript, The Eighth World Congress of Pediatric Cardiology and Cardiac Surgery has 5,037 registered attendees (and rising) from 117 countries, a truly diverse and international faculty of over 925 individuals from 89 countries, over 2,000 individual abstracts and poster presenters from 101 countries, and a Best Abstract Competition featuring 153 oral abstracts from 34 countries. For information about the Eighth World Congress of Pediatric Cardiology and Cardiac Surgery, please visit the following website: [www.WCPCCS2023.org]. The purpose of this manuscript is to review the activities related to global health and advocacy that will occur at the Eighth World Congress of Pediatric Cardiology and Cardiac Surgery.Acknowledging the need for urgent change, we wanted to take the opportunity to bring a common voice to the global community and issue the Washington DC WCPCCS Call to Action on Addressing the Global Burden of Pediatric and Congenital Heart Diseases. A copy of this Washington DC WCPCCS Call to Action is provided in the Appendix of this manuscript. This Washington DC WCPCCS Call to Action is an initiative aimed at increasing awareness of the global burden, promoting the development of sustainable care systems, and improving access to high quality and equitable healthcare for children with heart disease as well as adults with congenital heart disease worldwide.

Human fitting of pediatric and infant continuous-flow total artificial heart: visual and virtual assessment.

Background: This study aimed to determine the fit of two small-sized (pediatric and infant) continuous-flow total artificial heart pumps (CFTAHs) in congenital heart surgery patients. Methods: This study was approved by Cleveland Clinic Institutional Review Board. Pediatric cardiac surgery patients (n = 40) were evaluated for anatomical and virtual device fitting (3D-printed models of pediatric [P-CFTAH] and infant [I-CFTAH] models). The virtual sub-study consisted of analysis of preoperative thoracic radiographs and computed tomography (n = 3; 4.2, 5.3, and 10.2 kg) imaging data. Results: P-CFTAH pump fit in 21 out of 40 patients (fit group, 52.5%) but did not fit in 19 patients (non-fit group, 47.5%). I-CFTAH pump fit all of the 33 patients evaluated. There were critical differences due to dimensional variation (p < 0.0001) for the P-CFTAH, such as body weight (BW), height (Ht), and body surface area (BSA). The cutoff values were: BW: 5.71 kg, Ht: 59.0 cm, BSA: 0.31 m2. These cutoff values were additionally confirmed to be optimal by CT imaging. Conclusions: This study demonstrated the range of proper fit for the P-CFTAH and I-CFTAH in congenital heart disease patients. These data suggest the feasibility of both devices for fit in the small-patient population.

Multi-modality imaging evaluation and pre-surgical planning for aortic valve-sparing operations in patients with aortic root aneurysm.

Cardiac computed tomography (CT) and magnetic resonance (CMR) supplement echocardiography in the evaluation of those with aortic root and ascending aortic dilation, determining timing for intervention, guiding pre-surgical planning and post-operative surveillance. The dynamic, three-dimensional complexity of the aortic root and how it relates to the base of the left ventricle must be understood in any surgical approach addressing the aneurysmal aortic root. With improved imaging technology and the importance for proper patient counseling, it is no longer acceptable to enter the operating theater without a detailed blue print of what the problem is, and how best to address it. In addition, reliance on surgical expertise alone for intraoperative evaluation and decision making could be suboptimal due to the unloaded condition of the aortic root and the variance of experience of the surgeons to successfully repair the aortic valve. This is exemplified by the selective surgeons and centers who have the ability to tackle these aortic valve and root pathologies, compared to mitral valve repair techniques that have been codified and are generalizable. This review discusses a multimodality imaging approach in the patient with aortic root aneurysm, focusing on the precision added with pre-surgical CT assessment to guide aortic-valve sparing operations. This precision is afforded with a detailed understanding of the anatomy of the aortic root and underlying support, and its accurate evaluation by standard two- and three-dimensional imaging. Furthermore, we describe the evolving ability to predict the location of ventricular components of the atrioventricular conduction axis with further clinical imaging to personalize surgical strategies.

Expert Consensus Statement: Anatomy, Imaging, and Nomenclature of Congenital Aortic Root Malformations.

Over the past 2 decades, several categorizations have been proposed for the abnormalities of the aortic root. These schemes have mostly been devoid of input from specialists of congenital cardiac disease. The aim of this review is to provide a classification, from the perspective of these specialists, based on an understanding of normal and abnormal morphogenesis and anatomy, with emphasis placed on the features of clinical and surgical relevance. We contend that the description of the congenitally malformed aortic root is simplified when approached in a fashion that recognizes the normal root to be made up of 3 leaflets, supported by their own sinuses, with the sinuses themselves separated by the interleaflet triangles. The malformed root, usually found in the setting of 3 sinuses, can also be found with 2 sinuses, and very rarely with 4 sinuses. This permits description of trisinuate, bisinuate, and quadrisinuate variants, respectively. This feature then provides the basis for classification of the anatomical and functional number of leaflets present. By offering standardized terms and definitions, we submit that our classification will be suitable for those working in all cardiac specialties, whether pediatric or adult. It is of equal value in the settings of acquired or congenital cardiac disease. Our recommendations will serve to amend and/or add to the existing International Paediatric and Congenital Cardiac Code, along with the Eleventh iteration of the International Classification of Diseases provided by the World Health Organization.

Surgical strategies to address re-operative complex left ventricular outflow tract and thoracic aortic pathology: Cleveland Clinic children's experience.

BACKGROUND: Complex patients requiring operations on the left ventricular outflow tract, aortic valve, or thoracic aorta after previous repair of aortopathy constitute a challenging group, with limited information guiding decision-making. We aimed to use our institutional experience to highlight management challenges and describe surgical pearls to address them. METHODS: Forty-one complex patients with surgery on the left ventricular outflow tract, aortic valve, or aorta at Cleveland Clinic Children's between 2016 and 2021 following previous repair of aortic pathology were retrospectively reviewed. Patients with known connective tissue disease or single ventricle circulation were excluded. RESULTS: Median age at index procedure was 23 years (range 0.25-48) with median of 2 prior sternotomies. Previous aortic operations included subvalvular (n = 9), valvular (n = 6), supravalvular (n = 13), and multi-level surgeries (n = 13). Four deaths occurred in median follow-up of 2.5 years. Mean left ventricular outflow tract gradients improved significantly for patients with obstruction (34.9 ± 17.5 mmHg versus 12.6 ± 6.0 mmHg; p < 0.001). Technical pearls include the following: 1) liberal use of anterior aortoventriculoplasty with valve replacement; 2) primarily anterior aortoventriculoplasty following the subpulmonary conus in contrast to more vertical incision for post-arterial switch operation patients; 3) pre-operative imaging of mediastinum and peripheral vasculature for cannulation and sternal re-entry; and 4) proactive use of multi-site peripheral cannulation. CONCLUSIONS: Operation to address the left ventricular outflow tract, aortic valve, or aorta following prior congenital aortic repair can be accomplished with excellent outcomes despite high complexity. These procedures commonly include multiple components, including concomitant valve interventions. Cannulation strategies and anterior aortoventriculoplasty in specific patients require modifications.

Characterization of Favorable Right Ventricular Dimensions for Optimal Reverse Remodeling Following Pulmonary Valve Replacement.

We sought to couple current cardiac magnetic resonance (CMR) thresholds of right ventricular (RV) size and function with longitudinal trajectories of RV recovery, after pulmonary valve replacement (PVR). We aimed to identify optimal timing of PVR and couple CMR-based metrics with contemporaneous echocardiographic metrics. From June 2002 to January 2019, 174 patients with severe pulmonary regurgitation and peak RV outflow tract gradient <30 mm Hg underwent PVR at Cleveland Clinic. Mean age was 35 ± 16 years and 60 (34%) had concomitant tricuspid valve surgery. RV end diastolic area index (RVEDAi) and function metrics were measured by offline image review on preoperative and 794 postoperative echocardiograms. Contemporaneous RV end diastolic volume index (RVEDVi) was assessed on CMR and correlated to RVEDAi. Multiphase nonlinear mixed-effects models were used to analyze the longitudinal change in RV size and function after PVR. RVEDAi was correlated with RVEDVi (P < 0.0001, r = 0.59). RVEDAi decreased slowly over 10 years following PVR. An inflection point at 24 cm2/m2 was noted at 1 year post-PVR and was associated with failure of RV reverse remodeling and RVEDVi ≥150 mL/m2. Compared to patients with preoperative RVEDVi ≥150 mL/m2, patients with RVEDVi <150 mL/m2 had accelerated recovery of longitudinal trajectories of RV size and function metrics on echocardiograms. Reverse remodeling of RV following PVR is an ongoing process. Current accepted threshold values for PVR are associated with greatest RV recovery, suggesting that earlier PVR is warranted. Echocardiography can potentially be utilized in lieu of CMR for surveillance and interventional triage.

Development of a Novel Society of Thoracic Surgeons Adult Congenital Mortality Risk Model.

BACKGROUND: Operative mortality risk models for adults with congenital heart disease (ACHD) undergoing cardiac operations are essential, given the growing population of these patients, yet they are currently unavailable. Existing adult Society of Thoracic Surgeons (STS) models exclude congenital procedures, whereas existing congenital models exclude operations for acquired disease. We aimed to develop an STS mortality risk model for ACHD patients undergoing cardiac operations. METHODS: Leveraging a comprehensive list of diagnostic and procedure codes, ACHD patients who underwent cardiac operations were identified from the STS Adult Cardiac Surgery Database (versions: v2.73, v2.81, and v2.9) between 2011 and 2019. The model was developed and validated in the ACHD population using a 60/40 development/validation split. Univariate analyses and clinical expertise informed the addition of ACHD-relevant procedure and diagnosis variables to existing STS adult risk model variables. Model performance was assessed overall and in 38 subgroups based on patient demographics, procedures, and diagnoses. RESULTS: Forty-seven procedure and diagnosis variables relevant to ACHD were added to existing STS adult risk model variables. The derived ACHD model for operative mortality was well calibrated within demographic, procedural, and diagnosis subgroups and the overall ACHD population, and discrimination in the validation cohort was excellent (C statistic, 0.815) compared with the model using only existing STS adult risk model variables (C statistic, 0.79; P < .0001). CONCLUSIONS: A novel, high-performing STS ACHD mortality risk model has been developed on the basis of contemporary patient data. The ACHD risk model represents an important expansion of the STS portfolio. Implementation with an online risk calculator is planned.