Position paper concerning the competence, performance and environment required for the practice of ablation in children and in congenital heart disease.

The population of patients with congenital heart disease (CHD) is continuously increasing, and a significant proportion of these patients will experience arrhythmias because of the underlying congenital heart defect itself or as a consequence of interventional or surgical treatment. Arrhythmias are a leading cause of mortality, morbidity and impaired quality of life in adults with CHD. Arrhythmias may also occur in children with or without CHD. In light of the unique issues, challenges and considerations involved in managing arrhythmias in this growing, ageing and heterogeneous patient population and in children, it appears both timely and essential to critically appraise and synthesize optimal treatment strategies. The introduction of catheter ablation techniques has greatly improved the treatment of cardiac arrhythmias. However, catheter ablation in adults or children with CHD and in children without CHD is more technically demanding, potentially causing various complications, and thus requires a high level of expertise to maximize success rates and minimize complication rates. As French recommendations regarding required technical competence and equipment are lacking in this situation, the Working Group of Pacing and Electrophysiology of the French Society of Cardiology and the Affiliate Group of Paediatric and Adult Congenital Cardiology have decided to produce a common position paper compiled from expert opinions from cardiac electrophysiology and paediatric cardiology. The paper details the features of an interventional cardiac electrophysiology centre that are required for ablation procedures in adults with CHD and in children, the importance of being able to diagnose, monitor and manage complications associated with ablations in these patients and the supplemental hospital-based resources required, such as anaesthesia, surgical back-up, intensive care, haemodynamic assistance and imaging. Lastly, the need for quality evaluations and French registries of ablations in these populations is discussed. The purpose of this consensus statement is therefore to define optimal conditions for the delivery of invasive care regarding ablation of arrhythmias in adults with CHD and in children, and to provide expert and - when possible - evidence-based recommendations on best practice for catheter-based ablation procedures in these specific populations.

Regionalization of Congenital Heart Surgery in the United States.

The objective of this study is to simulate regionalization of congenital heart surgery (CHS) in the United States and assess the impact of such a system on travel distance and mortality. Patients ≤18 years of age who underwent CHS were identified in 2012 State Inpatient Databases. Operations were stratified by the Risk Adjustment for Congenital Heart Surgery, version 1 (RACHS-1) method, with high risk defined as RACHS-1 levels 4-6. Regionalization was simulated by progressive closure of hospitals, beginning with the lowest volume hospital. Patients were moved to the next closest hospital. Analyses were conducted (1) maintaining original hospital mortality rates and (2) estimating mortality rates based on predicted surgical volumes after absorbing moved patients. One hundred fifty-three hospitals from 36 states performed 1 or more operation (19,064 operations). With regionalization wherein, all hospitals performed >310 operations, 37 hospitals remained, from 12.5% to 17.4% fewer deaths occurred (83-116/666), and median patient travel distance increased from 38.5 to 69.6 miles (P < 0.01). When only high-risk operations were regionalized, 3.9-5.9% fewer deaths occurred (26-39/666), and the overall mortality rate did not change significantly. Regionalization of CHS in the United States to higher volume centers may reduce mortality with minimal increase in patient travel distance. Much of the mortality reduction may be missed if solely high-risk patients are regionalized.

Management of the adult Fontan patient.

Adults with congenital heart disease who underwent the Fontan operation to palliate single ventricle heart defects-by direct connection of caval venous return to the pulmonary circulation-have improved survival due to advancements in surgical and interventional techniques. However, cardiac and non-cardiac comorbidities often coexist, complicating management, and contributing to premature morbidity and mortality. Cardiac issues include heart failure symptoms related to systolic and diastolic dysfunction, atrial and ventricular arrhythmias and systemic atrioventricular valve regurgitation. Structural issues may be related to obstruction of the Fontan pathway, or to branch pulmonary artery stenosis, both of which exacerbate symptoms. Non-cardiac complications in adults involve hepatic congestion, fibrosis and cirrhosis, hepatocellular carcinoma, chronic kidney disease, stroke, venous stasis, lymphatic issues and involvement of other organ systems. 'Fontan failure' refers to circulatory dysfunction, either cardiac, non-cardiac, or both, diagnosed after exclusion or treatment of reversible contributors such as structural issues and arrhythmias. Counselling about reproductive health, mental health, perioperative management and overall wellness are paramount for patients' well-being. Fontan patients are typically managed in highly specialised adult congenital heart centres, but may present to cardiologists or other practitioners with cardiac and non-cardiac emergencies or urgencies, sometimes after being out of care. In this review, we discuss the management of the adult Fontan patient, including surveillance, cardiac and non-cardiac complications, reproductive health, and advanced therapies.

Establishing Sustainable Cardiothoracic Surgery Programs in Underserved Countries.

Congenital heart disease is the most common birth defect worldwide, and accounts for a high proportion of the world's infant mortality. About 9 of every 10 babies born each year are born in areas without adequate access to heart surgery; overcoming this problem will necessitate addressing the worldwide shortage of an estimated 3,700 pediatric cardiac surgeons. Establishing sustainable heart surgery programs requires more than an investment of money: political, social, and cultural issues unique to each environment need to be addressed. Organizations desiring to help develop cardiac surgical centers need to focus on communication and bidirectional education, and to make a long-term commitment to each site. By identifying and addressing obstacles, success rates are high.

Organisation of care for pregnancy in patients with congenital heart disease.

Improvements in surgery have resulted in more women with repaired congenital heart disease (CHD) surviving to adulthood. Women with CHD, who wish to embark on pregnancy require prepregnancy counselling. This consultation should cover several issues such as the long-term prognosis of the mother, fertility and miscarriage rates, recurrence risk of CHD in the baby, drug therapy during pregnancy, estimated maternal risk and outcome, expected fetal outcomes and plans for pregnancy. Prenatal genetic testing is available for those patients with an identified genetic defect using pregestational diagnosis or prenatal diagnosis chorionic villus sampling or amniocentesis. Centralisation of care is needed for high-risk patients. Finally, currently there are no recommendations addressing the issue of the delivery. It is crucial that a dedicated plan for delivery should be available for all cardiac patients. The maternal mortality in low-income to middle-income countries is 14 times higher than in high-income countries and needs additional aspects and dedicated care.

Utility and Safety of Combined Interventional Catheterization and Electrophysiology Procedures in a Children's Hospital.

BACKGROUND: Interventional cardiac catheterization (cath) and electrophysiology (EP) procedures are not routinely performed together. There are several perceived barriers affecting this practice, though there are also advantages for both the patient and practitioner to a combined approach. METHODS: This was a single-center retrospective study reviewing combined cath and EP procedures with a preprocedural intention to intervene at Texas Children's Hospital from 2001 to 2014. We excluded procedures in which the intended procedure was purely diagnostic in nature. RESULTS: A total of 121 patients requiring 125 procedures were identified, of which 61 patients underwent 62 procedures that met our inclusion criteria. Potential subgroups of interest included adult congenital heart disease patients (26% of cohort), single ventricle anatomy (34%), and heterotaxy (19%) and collectively 58% of procedures involved a patient in one of these groups. The combined nature of the procedure did not preclude a cath or EP intervention in any patient. There were no mortalities. There were three adverse events, affecting 4.8% of procedures. CONCLUSIONS: Combined interventional cardiac cath and EP procedures in pediatric patients and those with congenital heart disease can be performed safely in a high-volume center. These combined procedures save patients the risk and inconvenience of multiple procedures, and further investigation into cost savings is warranted.

Congenital Heart Disease and Reproductive Risk: An Overview for Obstetricians, Cardiologists, and Primary Care Providers.

Patients with congenital heart disease have improved survival rates, and most patients are now expected to survive into adulthood. This improved survival has resulted in increasing numbers of women with congenital heart disease who are of childbearing age. This patient population requires specialized advice on contraception and pregnancy risk. Understanding the unique challenges this population presents is key to providing appropriate care.

Management of supraventricular arrhythmias in adults with congenital heart disease.

Supraventricular arrhythmias are a frequent complication in adults with congenital heart disease (ACHD). The prevalence increases with time since surgery, complexity of the underlying defect, type of repair and older age at surgery. Arrhythmias are the most frequent reason for hospital admission and along with heart failure the leading cause of death. The arrhythmia-associated increase in morbidity and mortality makes their management a key task in patients with ACHD. Intra-atrial re-entry is the most frequent arrhythmia mechanism. Less common arrhythmia mechanisms are supraventricular tachycardias in the presence of an accessory pathway, atrioventricular nodal re-entrant tachycardia or focal tachycardias. Patient management includes stroke prevention, acute termination and prevention of arrhythmia recurrence. Acute treatment depends on patients' symptoms. In cases of haemodynamic instability, immediate cardioversion is warranted. For stable patients, acute treatment includes rate control and termination by antiarrhythmic drugs or electrical cardioversion. Following a symptomatic arrhythmia, catheter ablation or treatment with antiarrhythmic drugs is recommended to prevent recurrences. Advances in mapping and ablation technology are now associated with high success rates of catheter ablation. In patients with a complex substrate recurrence rates of 50% remain high. However, in the presence of side effects and complications associated with long-term antiarrhythmic drug therapy, redo procedures are encouraged by current guidelines.

Harnessing Teams and Technology to Improve Outcomes in Infants With Single Ventricle.

Infants with single ventricle require staged cardiac surgery, with stage I typically performed shortly after birth, stage II at 4 to 6 months of age, and stage III at 3 to 5 years of age. There is a high risk of interstage mortality and morbidity after infants are discharged from the hospital between stages I and II. Traditional home monitoring requires caregivers to record measurements of weight and oxygen saturation into a binder and requires families to assume a surveillance role. We have developed a tablet PC-based solution that provides secure and nearly instantaneous transfer of patient information to a cloud-based server, with the capacity for instant alerts to be sent to the caregiver team. The cloud-based IT infrastructure lends itself well to being able to be scaled to multiple sites while maintaining strict control over the privacy of each site. All transmitted data are transferred to the electronic medical record daily. The system conforms to recently released Food and Drug Administration regulation that pertains to mobile health technologies and devices. Since this platform was developed in March 2014, 30 patients have been monitored. There have been no interstage deaths. The experience of care providers has been unanimously positive. The addition of video has added to the use of the monitoring program. Of 30 families, 23 expressed a preference for the tablet PC over the notebook, 3 had no preference, and 4 preferred the notebook to the tablet PC.

Adult congenital heart disease: A paradigm of epidemiological change.

Increasing survival rates for patients with congenital heart disease (CHD) represent a major achievement of modern medicine. Despite incredible progress been made in diagnosis, follow-up, early treatment and management in adulthood, many patients are faced with long-term complications, such as arrhythmia, thromboembolism, heart failure, pulmonary hypertension, endocarditis and/or the need for reoperation. In parallel, half of the patients are female, most of childbearing age, and, thus warrant specialist reproductive counseling and appropriate obstetric care. It is not surprising therefore, that healthcare utilization has steadily increased for CHD in recent years. Furthermore, cardiology and other medical disciplines are now faced with new challenges, namely the provision of expert care and optimal, lifelong medical surveillance for these patients.

Predictors of outcomes in children awaiting heart transplantation: an experience from a National Paediatric Heart Transplantation Programme.

OBJECTIVES: The risk of mortality while awaiting heart transplantation (HT) may be greater in the setting of a smaller population and lower donor organ rates. Optimizing allocation policy and identifying the most vulnerable patients will help reduce mortality. As such, we aimed to evaluate the predictors of outcomes in patients awaiting HT as part of a National Paediatric HT Programme. METHODS: Between 1988 and 2015, 200 children were listed for HT as part of a National Paediatric HT Programme. We categorized patients as experiencing one of four competing events: (i) transplant, (ii) death, (iii) delisting for clinical deterioration and (iv) delisting for clinical improvement. Comparison was made across three study eras: Era 1: 1988-95; Era 2: 1996-2005; Era 3: 2006-15. A multivariable competing risk regression analysis was performed to determine the independent predictors of transplantation and the composite event of death or delisting for clinical deterioration. RESULTS: Among the 200 patients listed, 60% (119/200) underwent transplantation, 22% (44/200) died on the waiting list, 7% (13/200) were delisted for clinical deterioration and 11% (21/200) were delisted due to clinical improvement, with 2% (3/200) being still active on the waiting list. The mortality-rate for patients who remained on the waiting list decreased from 27% in Era 1 to 18% in Era 3. The survival from wait-listing was 57, 47 and 39% at 5, 10 and 15 years, respectively. On multivariable competing risk regression, older age (HR 1.09, 95% CI 1.01-1.18, P = 0.029) and the absence of inotropic support (HR 2.22, 1.23-4.00, P = 0.0073) were predictors of reaching transplantation. Higher creatinine (per 20 µmol/l, HR 1.42, 1.03-1.95, P = 0.03) was a predictor of the composite endpoint of death or delisting for deterioration. CONCLUSIONS: In this analysis of a National Paediatric HT Programme, waiting-list mortality has decreased over time though it remains higher than countries with higher donor rates. The requirement for inotropic support and worsening renal function were predictors of failure to reach transplantation. These patients are likely to benefit the most from ventricular-assist device therapy and higher priority listing.

Using Data to Improve Quality: the Pediatric Cardiac Care Consortium.

A program to collect and analyze cardiac catheterization, electrophysiologic studies and cardiac operations in children was initiated in 1982. The purpose was to help centers compare their experience and outcomes with a group of centers to determine areas where their performance might improve. Cardiac centers became members of the Pediatric Cardiac Care Consortium and submitted demographic data and copies of procedure reports regularly to a central office. Data were extracted from the reports, coded by trained coders and entered into a computer database. Annually, the data were analyzed to compare the experience of an individual center with that of the entire group of centers. The annual data were adjusted for severity on the basis of eight factors selected after discussion with participants in the Consortium. Adjustment was by multivariate analysis. Reports were prepared for each center and distributed at an annual meeting. The data were used by centers to review operations where the mortality rate exceeded +2 standard deviations of the group. With discussion, the center staff often initiated changes to improve outcome. The outcome could then be monitored by the annual reports. Our data were also utilized in the creation of the Risk Adjustment for Surgery for Congenital Heart Disease (RACHS)-1 categories of disease severity. The mortality rates of our centers were comparable with the combined hospital discharge data from New York, Massachusetts, and California. From 1982 through 2007, the mortality rates of our centers dropped for each RACHS-1 category, falling to less than 1% for categories 1 and 2 for the last 5-year period. During the 25 years, we received data from 52 centers about 137 654 patients who underwent 117 756 cardiac operations.

National database study of survival of pediatric congenital heart disease patients in Taiwan.

BACKGROUND/PURPOSE: The incidence of congenital heart disease (CHD) and severe CHD is 13.08 and 1.51/1000 live births, respectively, in Taiwan, which has had national health insurance since 1995 and child health indices similar to those in the US. This study sought to further elucidate the fatality of CHD patients and their survival from a national database. METHODS: From the national health insurance database 2000-2010, we retrieved data from CHD patients who were diagnosed at age <6 years. The survival status at discharge was ascertained for estimation of survival. RESULTS: In total, 18,843 pediatric CHD patients were identified. The overall prevalence of CHD was 1288 per 100 000 live-births. Severe CHD (tetralogy of Fallot (4.4%), transposition of the great arteries (1.6%) and double outlet right ventricle (1.1%)) accounted for 11.5% of all cases. The 1-month/5-year survival in simple and severe CHD was 99.1%/97.5% and 90.2%/76.4%, respectively (p < 0.0001). The Kaplan-Meier survival at 5 years of age was lowest for hypoplastic left heart syndrome (19.7%), followed by transposition of the great arteries (66.7%), double outlet right ventricle (69.0%), and common ventricle (66.0%). The 5-year survival of the birth cohort in the same study period was 99.3%. CONCLUSION: This national database study revealed that the survival of children with simple CHD was still slightly lower than that of the general population and the survival of severe CHD patients, though only accounting for one-tenth of CHD cases, remained unsatisfactory. Such survival profiles are similar to those from Western reports and warrant a refined and dedicated medical care program for children with CHD.

Cardiac resource utilization in adults at a freestanding children's hospital.

OBJECTIVE: This article aims to give a comprehensive description of cardiac resource utilization in adults at a freestanding children's hospital. DESIGN: Retrospective chart review. SETTING: Large, tertiary, freestanding children's hospital. PATIENTS: Adults (18 years of age and older) seen within the heart center or evaluated by heart center personnel from January 1, 2006 through December 31, 2010. RESULTS: There were 2794 adults that utilized cardiology services during the study period. The mean patient age was 23.5 ± 8.4 years (18-70.5). The 1748 (62.6%) patients had a diagnosis of congenital heart disease. There were a total of 525 catheterization laboratory encounters and 104 cardiac surgical procedures performed on adult patients during the 5-year study period. The in-hospital mortality for all invasive encounters was 0%. The minor adverse event rate for all invasive encounters was 6.7% and 4.8%, respectively. Also, the overall major adverse event rates were 1.9% and 4.8%, respectively. There were 162 cardiology evaluations performed on adults in the emergency room. There were a total of 5489 adult cardiology clinic visits. The most common congenital heart disease diagnoses were: tetralogy of Fallot: 228 (13%), D-transposition of the great arteries: 208 (11.9%), single ventricle physiology: 187 (10.7%), atrial septal defect 128: (7.3%), and ventricular septal defect: 117 (6.7%). Overall, the complexity of congenital heart disease was: simple: 41%, moderate: 37%, and complex: 22%. CONCLUSIONS: A significant and growing number of adult patients are currently being cared for at pediatric institutions. Excellent outcomes have been achieved at these centers with dedicated adult congenital heart disease services, consisting of both cardiologists and other faculty trained in adult medicine. Others with training in adult medicine, whether they be staff or contracted consulting services, are also required to help manage adult comorbidities.

Risk-adjusted prolonged length of stay as an alternative outcome measure for pediatric congenital cardiac surgery.

BACKGROUND: Morbid events after pediatric congenital cardiac surgery are increasingly used for better outcome measurement and quality comparisons. This study was undertaken to evaluate the relationship between a hospital's risk-adjusted prevalence of prolonged postoperative length of stay (PLOS) and its risk-adjusted mortality rate to investigate whether PLOS can serve as an appropriate quality measure for pediatric congenital cardiac surgery. METHODS: Risk-adjusted prevalence of prolonged PLOS for 12 programs in New York State was estimated using data from 4,776 operations in the New York State pediatric Cardiac Surgery Reporting System (2006-2009). We used logistic regression analysis to adjust for case mix and patient risk factors. The hospital-level correlation between risk-adjusted prolonged PLOS and risk-adjusted mortality rates was examined using Spearman correlation coefficient analysis. RESULTS: Risk-adjusted prevalence of prolonged PLOS ranged from 7.48% to 36.52% for pediatric cardiac programs in New York State during the study period. The Spearman correlation test showed a strong positive relationship between a hospital's risk-adjusted prolonged PLOS and mortality rate (r = 0.83; p = 0.0008). CONCLUSIONS: Prolonged PLOS can be used in lieu of risk-adjusted mortality rates when it is not practical to use mortality rates owing to low case volume or decreasing mortality rates of some procedures.

Home monitoring program reduces interstage mortality after the modified Norwood procedure.

BACKGROUND: From 2002 to 2005, the interstage mortality after a modified Norwood procedure was 7% in our program. An interstage home monitoring program (HMP) was established to identify Norwood procedure patients at increased risk of decompensation and to reduce interstage mortality. METHODS: Results of the first 5 years of the Norwood HMP were reviewed retrospectively. Interstage was defined as the time between Norwood hospital discharge and admission for second stage surgical palliation. In the HMP, families documented oxygen saturation, heart rate, weight, and feedings daily. Nurse practitioners called each family at least weekly, and when issues arose, action plans were determined based on symptom severity. RESULTS: Between October 2005 and October 2010 there were 46 Norwood procedure patients who survived to hospital discharge. All were enrolled in the HMP. Forty-five patients had a Norwood procedure with right ventricle to pulmonary artery conduit, and 1 patient had a modified Blalock-Taussig shunt. Interstage survival was 100%. Nineteen patients (41%) were admitted interstage; 5 patients were admitted twice, 1 patient was admitted 4 times. Seventeen patients (37%) required interstage interventions. Eight patients (17%) required major interventions: conduit stenting, aortic arch balloon angioplasty, emergent shunt, or early Glenn surgery. Minor interventions included supplemental oxygen, blood transfusion, intravenous hydration, diuresis, anti-arrhythmic therapy, or feeding adjustments. CONCLUSIONS: In the first 5 years of the HMP, all infants discharged after a modified Norwood procedure survived the interstage period. The HMP altered clinical management in 37% of patients. Home monitoring of oxygen saturation, heart rate, weight, and feedings, along with comprehensive care coordination, allowed timely interventions and reduced interstage mortality from 7% to 0%.

Incidence and treatment of chylothorax after cardiac surgery in children: analysis of a large multi-institution database.

OBJECTIVE: There is limited information regarding the true incidence of and risk factors for chylothorax after pediatric cardiac surgery. The objective of this study was to determine, from a large multi-institution database, incidence, associated factors, and treatment strategy in patients undergoing pediatric cardiac surgery. METHODS: All patients younger than 18 years in the Pediatric Health Information System (PHIS) database who underwent congenital heart surgery or heart transplant from 2004 to 2011 were included. Procedure complexity was assessed by Risk Adjustment for Congenital Heart Surgery-1. RESULTS: In all, 77,777 patients (55% male) of median age 6.7 months were included. Overall incidence of chylothorax was 2.8% (n = 2205), significantly associated with increased procedure complexity, younger age, genetic syndromes, vein thrombosis, and higher annual hospital volume. Patients with multiple congenital procedures had the highest incidence. Incidence increased with time, from 2% in 2004 to 3.7% in 2011 (P < .0001). Chylothorax was associated with longer stay (P < .0001), increased adjusted risk for in-hospital mortality (odds ratio, 2.13; 95% confidence interval, 1.75-2.61), and higher cost (P < .0001), regardless of procedure complexity. Of all patients with chylothorax, 196 (8.9%) underwent thoracic duct ligation or pleurodesis a median of 18 days after surgery. Total parenteral nutrition, medium-chain fatty acid supplementation, and octreotide were used in 56%, 1.7%, and 16% of patients, respectively. CONCLUSIONS: Chylothorax is a significant problem in pediatric cardiac surgery and is associated with increased mortality, cost, and length of stay. Strategies should be developed to improve prevention and treatment.

Cardiac catheterization and postoperative acute kidney failure in congenital heart pediatric patients.

BACKGROUND: Acute renal failure (ARF) is a severe complication of cardiac operations in pediatric patients. Angiography with the exposure to contrast media is a risk factor for ARF. In the present study, we explored the association between timing of angiography, dose of contrast media, and the incidence of ARF after cardiac operations in pediatric patients. METHODS: We performed a retrospective analysis of prospectively collected data. Angiographic data and other covariates were collected in 277 patients aged ≤12 years receiving angiography and cardiac operations during the same hospital stay. Renal outcome was assessed according to the pediatric Risk, Injury, Failure, Loss of function, End stage score (pRIFLE). RESULTS: One hundred seventy-seven (64%) patients suffered some degree of postoperative renal dysfunction, and 55 (20%) had ARF (pRIFLE stage Failure). Patients with ARF received a significantly (P < 0.001) larger dose of iodine contrast media (4.6 ± 2.6 g/kg) with respect to the other patients (2.8 ± 2.2 g/kg), with a relative risk increase for ARF of 31% per each incremental iodine dose of 1 g/kg at the univariate analysis. A multivariable risk model demonstrated that the risk for ARF is 20 times higher in patients aged younger than 2 years and 3 times higher in case of postoperative low cardiac output. Within this model, the iodine dose on angiography is confirmed as an independent risk factor for ARF, with a relative risk increase for ARF of 16% per each incremental iodine dose of 1 g/kg. CONCLUSIONS: Angiography before cardiac surgery is an important risk factor for ARF in pediatric patients. Being a modifiable risk factor, the contrast media dose should be limited to the lowest possible value, avoiding large doses of iodine which, together with other factors (age and postoperative low cardiac output), concur in the determinism of postoperative ARF.