The Significance of Congenital Portosystemic Shunts in Congenital Heart Disease and the Bizarre Phenomenon of Alternating Portosystemic and Systemic-Portal Shunting.

Congenital portosystemic shunts (CPSSs) are rare vascular anomalies characterized by abnormal connections between the portal/splanchnic veins and the systemic veins. CPSSs often occur as an isolated congenital anomaly, but they can also coexist with congenital heart disease (CHD). Owing to their myriad consequences on multiple organ systems, familiarity with CPSS is of tremendous importance to the care of patients with CHD. The rationale and timing for interventions to embolize CPSS in this scenario are discussed. Specific shunt embolization techniques are beyond the scope of this article.

Transcatheter Interventions in Adult Congenital Heart Disease.

Congenital heart disease (CHD) is the most common congenital birth defect with an incidence of 1 in 100. Current survival to adulthood is expected in 9 out of 10 children with severe CHD as the diagnostic, interventional, and surgical success improves. The adult CHD (ACHD) population is increasingly diverse, reflecting the broad spectrum of CHD and evolution of surgical techniques to improve survival. Similarly, transcatheter interventions have seen exponential growth and creativity to reduce the need for repeat sternotomies. This article focuses on newer data and evolving techniques for transcatheter interventions specific to certain ACHD populations.

Hybrid Interventions in Congenital Heart Disease.

Hybrid interventions in congenital heart disease (CHD) embody the inherent collaboration between congenital interventional cardiology and cardiothoracic surgery. Hybrid approaches to complex and common lesions provide the opportunity to circumvent the limitations of patient size, vascular access, severity of illness, and anatomy that would otherwise be prohibitive to surgical and percutaneous techniques alone. This review describes several important hybrid approaches to interventions in CHD.

Current Understanding of Indications, Technical Aspects and Outcomes of Fetal Cardiac Interventions.

With the improvement in the detection of congenital heart disease in fetal life, fetal cardiac interventions are pushing the envelope in hopes of either altering the natural history of disease or improving survival in certain high-risk lesions. These interventions include fetal aortic valvuloplasty for evolving hypoplastic left heart syndrome, fetal atrial septoplasty with or without atrial septal stenting for hypoplastic left heart syndrome and variants with intact or severely restrictive atrial septum, and fetal pulmonary valvuloplasty for severe pulmonary stenosis or pulmonary atresia with intact ventricular septum. This review discusses their indications, technical aspects, and outcomes based on available literature.

Pulmonary Artery Stent Implantation.

Pulmonary artery stent implantation has become integral in the treatment of pulmonary artery stenosis and is probably the most efficacious therapy for these lesions. Advancements in technology involving stent design and the equipment used for stent delivery have made this procedure much safer and more effective. Strategies to mitigate and successfully treat adverse events related to pulmonary artery stent implantation are reasonably well-established. Pulmonary artery stent implantation remains one of the most complex and technically challenging of congenital cardiac interventions.

Interventions for Pulmonary Vein Stenosis.

Pulmonary vein stenosis (PVS) is a rare and unique disease of infants and young children. PVS is attended by high morbidity and mortality, and for many decades, effective therapy eluded the practitioner. However, in the most recent era, interventional techniques when employed in combination with systemic (primary) therapy have had a remarkable impact on outcomes in these at-risk children. Despite apparent complete relief of PVS in a discrete region of a pulmonary vein, stenosis reliably recurs and progresses. In this review, we discuss the current state-of-the-art interventional techniques, through the lens of our collective experiences and practices.

Preliminary report of a thoracic duct-to-pulmonary vein lymphovenous anastomosis in swine: A novel technique and potential treatment for lymphatic failure.

OBJECTIVE: The thoracic duct is the largest lymphatic vessel in the body, and carries fluid and nutrients absorbed in abdominal organs to the central venous circulation. Thoracic duct obstruction can cause significant failure of the lymphatic circulation (i.e., protein-losing enteropathy, plastic bronchitis, etc.). Surgical anastomosis between the thoracic duct and central venous circulation has been used to treat thoracic duct obstruction but cannot provide lymphatic decompression in patients with superior vena cava obstruction or chronically elevated central venous pressures (e.g., right heart failure, single ventricle physiology, etc.). Therefore, this preclinical feasibility study sought to develop a novel and optimal surgical technique for creating a thoracic duct-to-pulmonary vein lymphovenous anastomosis (LVA) in swine that could remain patent and preserve unidirectional lymphatic fluid flow into the systemic venous circulation to provide therapeutic decompression of the lymphatic circulation even at high central venous pressures. METHODS: A thoracic duct-to-pulmonary vein LVA was attempted in 10 piglets (median age 80 [IQR 80-83] days; weight 22.5 [IQR 21.4-26.8] kg). After a right thoracotomy, the thoracic duct was mobilized, transected, and anastomosed to the right inferior pulmonary vein. Animals were systemically anticoagulated on post-operative day 1. Lymphangiography was used to evaluate LVA patency up to post-operative day 7. RESULTS: A thoracic duct-to-pulmonary vein LVA was successfully completed in 8/10 (80.0%) piglets, of which 6/8 (75.0%) survived to the intended study endpoint without any complication (median 6 [IQR 4-7] days). Initially, 2/10 (20.0%) LVAs were aborted intraoperatively, and 2/10 (20.0%) animals were euthanized early due to post-operative complications. However, using an optimized surgical technique, the success rate for creating a thoracic duct-to-pulmonary vein LVA in six animals was 100%, all of which survived to their intended study endpoint without any complications (median 6 [IQR 4-7] days). LVAs remained patent for up to seven days. CONCLUSION: A thoracic duct-to-pulmonary vein LVA can be completed safely and remain patent for at least one week with systemic anticoagulation, which provides an important proof-of-concept that this novel intervention could effectively offload the lymphatic circulation in patients with lymphatic failure and elevated central venous pressures.

Impact of maternal body mass index (BMI) and the challenges of fetal echocardiography.

OBJECTIVES: Fetal echocardiography is the gold standard modality to detect suspected congenital heart disease (CHD). Accurate diagnosis and subsequent prognosis is even more challenging in the presence of a raised maternal body mass index (BMI). This retrospective study aimed to gain insight into the prevalence of obesity within the cohort of patients referred for fetal echocardiography. STUDY DESIGN/METHODS: Retrospective analysis of all pregnant patients referred to the Scottish National Fetal Cardiology Service between 2015 and 2021 due to a suspected fetal cardiac abnormality and examining the associated trends in maternal BMI and the Scottish Index of Multiple Deprivation (SIMD). RESULTS: BMI data were available for 962 (96.3%) of the 998 patients referred during the study period. Median BMI during the study period was 31. BMI range in the seven-year period was 16-63. There was no association between BMI group and year (P = 0.889). A median of 58% of patients referred were classified as overweight (BMI > 25 kg/m2), and only 37% were reported to have a BMI within normal limits. Referral BMI was relatively consistent in the seven years with no dramatic increase in the obese categories. Mean BMI in SIMD 5 (lowest level of deprivation), was significantly lower (P = 0.001), than in SIMD 1 (highest deprivation). CONCLUSIONS: People of child bearing age should be aware the potential limitations that a raised BMI may have upon diagnostic/screening accuracy impacting subsequent ability to provide accurate fetal cardiac diagnoses and prognostic fetal cardiac imaging.

NT-proBNP in systemic right ventricles: a new cutoff level for risk stratification?

INTRODUCTION AND OBJECTIVES: The role of N-terminal pro-B-type natriuretic peptide (NT-proBNP) in the risk prediction of patients with systemic right ventricles (sRV) is not well defined. The aim of this study was to analyze the prognostic value of NT-proBNP in patients with an sRV. METHODS: The prognostic value of NT-proBNP was assessed in 98 patients from the SERVE trial. We used an adjusted Cox proportional hazards model, survival analysis, and c-statistics. The composite primary outcome was the occurrence of clinically relevant arrhythmia, heart failure, or death. Correlations between baseline NT-proBNP values and biventricular volumes and function were assessed by adjusted linear regression models. RESULTS: The median age [interquartile range] at baseline was 39 [32-48] years and 32% were women. The median NT-proBNP was 238 [137-429] ng/L. Baseline NT-proBNP concentrations were significantly higher among the 20 (20%) patients developing the combined primary outcome compared with those who did not (816 [194-1094] vs 205 [122-357]; P = .003). In patients with NT-proBNP concentrations > 75th percentile (> 429 ng/L), we found an exponential increase in the sex- and age-adjusted hazard ratio for the primary outcome. The prognostic value of NT-proBNP was comparable to right ventricular ejection fraction and peak oxygen uptake on exercise testing (c-statistic: 0.71, 0.72 and 0.71, respectively). CONCLUSIONS: In patients with sRVs, NT-proBNP concentrations correlate with sRV volumes and function and may serve as a simple tool for predicting adverse outcomes.

Standardisation in Paediatric Echocardiography Reporting and Critical Interpretation of Measurements, Functional Parameters and Prediction Scores. A Clinical Consensus Statement of the European Association of Cardiovascular Imaging (EACVI) of the ESC and the Association for European Paediatric and Congenital Cardiology (AEPC).

This document has been developed to provide a guide for basic and advanced reporting in pediatric echocardiography. Furthermore, it aims to help clinicians in the interpretation of echocardiographic measurements and functional data for estimating the severity of disease in different pediatric age groups. The following topics will be reviewed and discussed in the present document: i) the general principle in constructing a pediatric echocardiography report, ii) the basic elements to be included, iii) the potential and limitation of currently employed tools used for disease severity quantification during paediatric reporting. A guide for the interpretation of Z-scores will be provided. Use and interpretation of parameters employed for quantification of ventricular systolic function will be discussed. Difficulties in the adoption of adult parameters for the study of diastolic function and valve defects at different ages, pressure and loading conditions will be outlined, with pitfalls for the assessment listed. A guide for careful use of prediction scores for complex congenital heart disease will be provided. Examples of basic and advanced (disease specific) formats for reporting in paediatric echocardiography will be provided. This document should serve as a comprehensive guide to i) structure a comprehensive paediatric echocardiographic report, ii) identify the basic morphological details, measures, and functional parameters to be included during echocardiographic reporting, and iii) correctly interpret measurements and functional data for estimating disease severity.

Experiences and Attitudes Toward Telemedicine in an Adult Congenital Heart Disease Clinic: Lessons Learned from the COVID-19 Pandemic.

The coronavirus disease 2019 (COVID-19) pandemic has driven a broader adoption of telemedicine (TM). We aim to describe adult congenital heart disease (ACHD) patient experiences with TM and explore factors associated with positive attitude toward future TM visits. This is a cross-sectional, single-center study in an outpatient ACHD clinic from February to June, 2022. Between-group comparisons were made using Wilcoxon-Rank Sum, Chi-Square, or Fisher-Exact tests. Univariate logistic regression was performed for variables that could correlate with a "positive" attitude toward future TM visits. Significance was determined using an alpha level of 0.05. Of 262 patients (median age 33 years, 55% female, 81% White), 115 (44%) had a prior TM visit and 110 (96%) reported a positive experience. There were 64 (24%) with a positive attitude toward future TM visits. Concerns include lack of cardiac testing and limited quality of visit. Patients with visits every 3-6 months (Odds Ratio [OR] 2.44; p < 0.01) and prior TM visit (OR 1.89; p = 0.03) had higher odds of a positive attitude toward future TM, whereas males had lower odds (OR 0.53; p = 0.04). Age, annual income, disease complexity, distance from clinic, and employment status were not associated. There is high rate of satisfaction with TM among ACHD patients but only one-quarter indicated interest in using TM in the future. Factors associated with interest in TM visits are identified, and together with patient feedback, can be used to understand potential role of TM for the ACHD population in the post-pandemic era.

A Novel Mental Health Curriculum Pilot for Pediatric Cardiology Fellows: Preparing the Subspecialist to Expand the Team.

Mental health (MH) is an important, yet understudied area of care for patients with congenital heart disease (CHD). Pediatric subspecialty fellows believe MH should be within their scope of practice, but few feel confident in their ability to appropriately screen, evaluate, manage, and make treatment referrals for youth with CHD and concurrent MH concerns. A 6-session, didactic-based curriculum was designed by an interprofessional team of experts. It was administered to pediatric cardiology fellows over 9 months during two academic years. Topics included the following: Introduction to MH and CHD, MH Screening and Clinic-Based Interventions, Psychopharmacology, Delirium, Trauma-Informed Care, and Adult CHD and Transitional Care. An investigator-designed survey was developed to assess fellow comfort with MH knowledge, screening, and treatment recommendation domains. Twenty-three fellows participated with 14 completing the pre-post survey (58% participation, 11/17 in year 1, 3/6 in year 2). Most participants were in their first year of training (9/14, 57%). Overall, fellow comfort with MH topics increased significantly (mean score 2.89 ± 0.46 vs. 3.4 ± 0.4, p = 0.0005), with improved comfort in the MH knowledge (p = 0.003) and treatment recommendation domains (p = 0.001). A didactic-based MH curriculum improves the comfort of pediatric cardiology fellows to address the MH concerns of their patients. The topics in this curriculum will continue to be refined over time and could be generalized to training programs, both nationally and across pediatric specialties, to improve MH care for high-risk populations.

Anesthetic Management of an Adult Patient Post a Fontan Procedure in Laparoscopic Surgery: A Case Report.

We report the successful anesthetic management of laparoscopic surgery in a 21-year-old female patient with Fontan circulation. A preoperative careful review of cardiac catheterization results helped assess the risk of the surgery and implement anesthetic management. Intraoperative management focused on minimizing the impact on pulmonary vascular resistance and venous return by optimizing ventilation and applying lower pneumoperitoneum pressure without tilting the position. Milrinone was administered to reduce pulmonary vascular resistance and provide inotropic support with minimally invasive monitoring. The patient remained stable throughout the procedure without complications. This case highlights the importance of thorough preoperative assessment, individualized intraoperative management, and collaboration with the surgical team when caring for adult Fontan patients undergoing laparoscopic surgery.

Diagnostic work-up and current management strategies for infective endocarditis in the pediatric population.

Infective endocarditis (IE) remains a serious disease that is associated with significant morbidity and mortality, and despite the significant advances that have been made in understanding the disease process in past decades, its incidence appears to be on the rise recently. Endocarditis in children is no longer a rare occurrence. This appeared to be related to a combination of the improved survival of children with congenital heart diseases (CHDs), increase use of intracardiac protheses, and catheter-related interventions. The American Heart Association (AHA) 2007 guidelines reduced the recommendations for use of prophylactic antibiotics in those with CHDs which occurred despite the noticeable increase in endocarditis incidence around that time. In general, the recommendations for managing children with IE are derived from the adults' guidelines, and the evidence-base is lacking in many clinical scenarios. Understanding the epidemiology, clinical presentations, microbiology, and outcomes of different management strategies for endocarditis is needed to have a clear and optimal plan for these children. In the current narrative review, we discuss IE in the pediatric population in terms of etiology, predisposing factors, and different treatment strategies for this unique population.

Lymphatic interventions in congenital heart disease.

Congenital heart disease affects 1/100 live births and is one of the most common congenital abnormalities. The relationship between congenital heart disease and lymphatic abnormalities and/or dysfunction is well documented and can be grossly divided into syndromic and non-syndromic etiologies. In patients with genetic syndromes (as examples listed above), there are known primary abnormal lymphatic development leading to a large pleiotropic manifestation of lymphatic dysfunction. Non-syndromic patients, or those without clear genetic etiologies for their lymphatic dysfunction, are often thought to be secondary to physiologic abnormalities as sequelae of congenital heart disease and palliative surgeries. Patients with congenital heart disease and lymphatic dysfunction have a wide variety of clinical manifestations for which there were not many therapeutic interventions available. The development of new imaging techniques allows us to understand better the pathophysiology of these problems and to develop different percutaneous interventions aiming to restore normal lymphatic function.

Maternal Cardiac Disease and Perinatal Outcomes in a Single Tertiary Care Center.

OBJECTIVE: This study aims to compare the perinatal outcomes of pregnant women with heart disease and a healthy pregnant control group, as well as the maternal and newborn outcomes of pregnant women with congenital heart disease and acquired heart disease. MATERIAL METHOD: Pregnant women with heart disease and healthy control pregnant women were included in this retrospective study. Sociodemographic data of all patients included in the study were obtained from electronic records. Perinatal outcomes of all patients were compared. RESULTS: A total of 258 pregnant women were included in the study. While 129 pregnant women were diagnosed with heart disease, 129 patients were low-risk pregnant women. Preeclampsia (p=0.004) and cesarean section (p=0.01) rates were higher in pregnant women with heart disease compared to healthy pregnant women. Compared with healthy pregnant women, pregnant women with heart disease had a lower birth weight (p=0.003), a higher fetal growth restriction (FGR) rate (p=0.036), lower birth percentiles (p=0.002), a lower 5-minute APGAR (p=0.0001), a higher neonatal intensive care unit (NICU) admission rate (p=0.001), and a longer NICU stay rate (p=0.001). The mean gestational age at birth of pregnant women with congenital heart disease was higher than that of those with acquired heart disease (p=0.017). CONCLUSION: It was observed that all maternal heart diseases were associated with adverse perinatal outcomes compared to healthy pregnant women. In this series, perinatal adverse outcomes of pregnant women with congenital and acquired heart disease did not differ.

Pulmonary responses following cardiac rehabilitation and the relationship with functional outcomes in children and young adults with heart disease.

Introduction: Patients with congenital heart disease (CHD) often have pulmonary abnormalities and exercise intolerance following cardiac surgery. Cardiac rehabilitation (CR) improves exercise capacity in patients with CHD, but minimal study has been performed to see if resting and dynamic pulmonary performance improves following CR in those with prior cardiac surgery. Methods: This was a retrospective cohort study of all patients who completed ≥12 weeks of CR from 2018 through 2022. Demographic, cardiopulmonary exercise test (CPET), spirometry, 6-minute walk, functional strength measures, and outcomes data were collected. Data are presented as median[IQR]. A Student's t-test was used for comparisons between groups and serial measurements were measured with a paired t-test. A p < 0.05 was considered significant. Results: There were a total of 37 patients [age 16.7 (14.2-20.1) years; 46% male] included. Patients with prior surgery (n = 26) were more likely to have abnormal spirometry data than those without heart disease (n = 11) (forced vital capacity [FVC] 76.7 [69.1-84.3]% vs. 96.4 [88.1-104.7]%, p = 0.002), but neither group experienced a significant change in spirometry. On CPET, peak oxygen consumption increased but there was no change in other pulmonary measures during exercise. Percent predicted FVC correlated with hand grip strength (r = 0.57, p = 0.0003) and percent predicted oxygen consumption (r = 0.43, p = 0.009). The number of prior sternotomies showed negative associations with both percent predicted FVC (r = -0.43, p = 0.04) and FEV1 (r = -0.47, p = 0.02). Discussion: Youth and young adults with a prior history of cardiac surgery have resting and dynamic pulmonary abnormalities that do not improve following CR. Multiple sternotomies are associated with worse pulmonary function.

Hybrid stage 1 palliation with simultaneous off-pump ventricular assist device placement in neonates with high-risk single ventricle anatomy: Initial experience.

Background: Infants with single ventricle heart disease and severe atrioventricular valve regurgitation have poor outcomes following conventional staged palliation. As such, ventricular assist device (VAD) placement along with hybrid stage 1 palliation has been proposed as a bridge to heart transplant. We present a novel surgical technique for VAD implantation concurrent with hybrid stage 1 that avoids cardiopulmonary bypass. Methods: We performed a retrospective review of our institutional experience with this novel surgical technique. Results: Three patients (weight, 2.7-3.5 kg; age, 3 to 5 days) underwent hybrid stage 1 with VAD placement, consisting of bilateral 3.5-mm expandable polytetrafluoroethylene (PTFE) pulmonary artery bands, a ductal stent, a 6-mm Berlin Heart outflow cannula onto the main pulmonary trunk with a 10-mm graft, a 6-mm Berlin Heart outflow cannula onto the right atrium, and a 10-mL Berlin Heart pump. In patients with severe aortic arch hypoplasia or coarctation, a 4-mm PTFE graft was sewn from the VAD outflow graft to the innominate artery to protect coronary and cerebral perfusion. Procedures were performed off bypass with minimal blood product use. Patients were extubated on postoperative days 2, 2, and 5. There were no procedural complications. All patients were transferred out of the intensive care unit and demonstrated appropriate weight gain. Anticoagulation strategy was bivalirudin and antiplatelet therapy. The patients underwent transplantation after 149 days, 157 days, and 288 days of support. Conclusions: Off-pump single ventricle VAD placement is technically feasible and can be done at the time of hybrid stage 1 palliation with minimal operative morbidity as a bridge to transplant.

Surgery of Neoaortic Pseudoaneurysm with Transsternal Penetration in a HLHS Patient.

Background Neoaortic pseudoaneurysm after previous surgery is rare and life-threatening. Case Description We present a case of a 6th redo surgery in a 23-year-old male patient with a history of hypoplastic left heart syndrome presenting with transsternal penetration of aortic pseudoaneurysm. The previously implanted Dacron prosthesis showed semicircular dehiscence. It was exchanged by a Vascutek prosthesis during circulatory arrest and selective antegrade cerebral perfusion. Conclusion Control imaging at 1 year after surgery was unobtrusive. Three years after surgery, the patient is alive and doing well.

The Prevalence and Spontaneous Closure of Ventricular Septal Defects the First Year of Life.

INTRODUCTION: Ventricular septal defect (VSD) is one of the most common congenital heart defects. We aimed to determine the prevalence of VSD in a population-based cohort of newborns and assess the rate of spontaneous closure during the first 12 months of life. METHODS: The Copenhagen Baby Heart Study (CBHS) is a population-based cohort study, including more than 25,000 newborns born in the greater Copenhagen area. Newborns underwent echocardiography within 60 days of birth. Newborns with VSDs had echocardiographic follow-up after 3, 6, and 12 months. RESULTS: A total of 850 newborns (3.3% of 25.556) with a VSD were identified in the CBHS. Of these, 787 (92.6% [95% CI 90.1-94.2]) were muscular VSDs, 60 (7.0% [95% CI, 5.5-9.0]) were perimembranous, and 3 (0.4% [95% CI, 0.0-1.1]) were subarterial. After 1 year, 83.5% (607 of 727) of all VSDs had closed spontaneously, resulting in a decrease of prevalence from 3.3% at birth to 0.5% in 1-year old children. Muscular VSDs showed significantly higher rate of spontaneous closure compared with perimembranous VSDs (86.9% (582/670) vs. 46.9% (25/54), p < 0.001). Determinants associated with spontaneous closure were smaller size of the VSD (p < 0.001) and the absence of multiple VSDs (p < 0.0025). CONCLUSION: The prevalence of VSDs in unselected newborns was 3.3%. Almost 9/10 of all VSDs identified in newborns, close spontaneously during the first year of life, ultimately resulting in a prevalence of VSD in 1-year-old children of 0.5%. The identified factors associated with spontaneous closure were muscular type, small size, and absence of multiple VSDs.