Advances In Pediatric Cardiology Research Articles

From Crafoord’s End-to-End Anastomosis Approach to Percutaneous Interventions: Coarctation of the Aorta Management Strategies and Reinterventions

First described in 1760 by the anatomist Morgagni, coarctation of the aorta (CoA) is a congenital heart defect characterized by narrowing the aorta, typically distal to the left subclavian artery. It accounts for approximately 5–8% of all congenital heart diseases, with an incidence estimated at 4 per 10,000 live births. In 1944, the Swedish surgeon Clarence Crafoord achieved the first successful surgical CoA repair by performing an aortic end-to-end anastomosis on two patients aged 12 and 27 years old. Presently, the most prevalent techniques for surgical repair, particularly in infants and neonates with isolated coarctation, involve resection with end-to-end anastomosis (EEA) and the modified Crafoord technique (extended resection with end-to-end anastomosis (EEEA)). Subclavian flap aortoplasty (SCAP) is an alternative surgical option for CoA repair in patients under two years of age. In cases where the stenosis extends beyond resection and end-to-end anastomosis feasibility, patch aortoplasty (PP) employing a prosthetic patch can augment the stenotic region, especially for older patients. Despite advances in pediatric cardiology and cardiac surgery, recoarctation remains a significant concern after surgical or interventional repair. This comprehensive review aims to provide a thorough analysis of coarctation management, covering the pioneering techniques introduced by Crafoord using end-to-end anastomosis and now extending to the contemporary era marked by percutaneous interventions as well as the recoarctation rate associated with each type.

FONTAN CIRCULATION AND PREGNANCY: IS IT POSSIBLE

More than 85% of congenital heart disease in children survive to adulthood according to recent advances in pediatric cardiology and heart surgery practice. Fifty percent of this population is made up of women, most of whom have reached child bearing age. In this group of patients, pregnancy represents a new challenge, whose natural history has been modified by surgery. In Fontan circulation, the ventricle unable to compensate for the major restriction in the bottleneck and non physiological suction have to pull blood through the lungs, although the ventricles provide the driving force for the circuit. Women with an adequate Fontan circulation can become pregnant without clinically significant long term sequele. Pre-marital and pre-pregnancy consultation is important. A close collaboration between cardiologist, obstetrician and anaesthesiologist is mandatory in order to get the best outcome of pregnancy.

Perioperative Considerations for Pediatric Patients With Congenital Heart Disease Presenting for Noncardiac Procedures: A Scientific Statement From the American Heart Association

Continuous advances in pediatric cardiology, surgery, and critical care have significantly improved survival rates for children and adults with congenital heart disease. Paradoxically, the resulting increase in longevity has expanded the prevalence of both repaired and unrepaired congenital heart disease and has escalated the need for diagnostic and interventional procedures. Because of this expansion in prevalence, anesthesiologists, pediatricians, and other health care professionals increasingly encounter patients with congenital heart disease or other pediatric cardiac diseases who are presenting for surgical treatment of unrelated, noncardiac disease. Patients with congenital heart disease are at high risk for mortality, complications, and reoperation after noncardiac procedures. Rigorous study of risk factors and outcomes has identified subsets of patients with minor, major, and severe congenital heart disease who may have higher-than-baseline risk when undergoing noncardiac procedures, and this has led to the development of risk prediction scores specific to this population. This scientific statement reviews contemporary data on risk from noncardiac procedures, focusing on pediatric patients with congenital heart disease and describing current knowledge on the subject. This scientific statement also addresses preoperative evaluation and testing, perioperative considerations, and postoperative care in this unique patient population and highlights relevant aspects of the pathophysiology of selected conditions that can influence perioperative care and patient management.

Tachyarrhythmias in Congenital Heart Diseases: From Ion Channels to Catheter Ablation.

Major advances in pediatric cardiology in recent decades, especially surgical techniques, have resulted in an increasing number of patients with congenital heart disease (CHD) surviving to adulthood. This has generated new challenges, particularly with regards to the late onset of complex arrhythmias. Abnormal anatomy, surgical scarring, chronic hypoxemia, hemodynamic compromise, neuro-hormonal abnormalities, and genetic factors can all contribute to creating a unique substrate for arrhythmia development. This review attempts to synthesize the current state of knowledge spanning the spectrum from underlying mechanisms of arrhythmias in patients with congenital heart disease to current ablative strategies. We discuss existing knowledge gaps and highlight important areas for future research.

Validity and reliability of the Italian version of the cardiac quality of life questionnaire for pediatric patients with heart disease (PedsQLTM)

BackgroundCongenital heart disease (CHD) accounts for nearly a third of all major congenital anomalies. Advances in pediatric cardiology shifted attention from mortality to morbidity and health-related quality of life (HRQOL) of patients with CHD and impact on their families. The purposes of this study were to assess the validity and reliability of the Italian version of the Pediatric Quality of Life (PedsQL) Cardiac Module and to create normative data for the Italian population.MethodsThis was an observational cross-sectional study of pediatric patients (aged 2–18 years) with congenital or acquired Heart Disease (HD) and their parents. Families were asked to complete the cardiac pediatric health-related quality of life questionnaire (the Italian PedsQL™ 3.0 Cardiac Module) and the generic pediatric health-related quality of life questionnaire (PedsQL™ 4.0 Generic Core Scales). The sequential validation procedure of the original United States version of the PedsQL™ 3.0 Cardiac Module was carried out under the instruction of the MAPI Research Institute.To assess construct validity, Pearson’s correlation coefficients were assessed between scores on the Cardiac Module scales and scores on the scales of the General Module. To determine agreement between patient self-report and parent proxy-report, we used intraclass correlation coefficients (ICCs). To evaluate Internal consistency of items, we used Cronbach’s alpha Coefficient.ResultsThe study enrolled 400 patients. Construct validity is good between PedsQL Cardiac Module total scores and PedsQL total scores (p < 0.001). The recommended standard value of 0.7 was reached on the Cardiac and General Module core scales. Intercorrelations between PedsQL Cardiac module and PedsQL scores revealed medium to large correlations. In general, correlations between Patient self-reports are poorer than Parent-proxy ones.ConclusionsCardiac PedsQL scores are valid and reliable for pediatric patients with congenital and acquired HD and may be useful for future research and clinical management.

The management of adult congenital heart disease in the emergency department. The Task Force from the Italian Society of Pediatric Cardiology and Congenital Heart Disease

Over recent years, thanks to remarkable advances in pediatric cardiology, cardiac surgery and catheter interventions, survival of children with congenital heart disease has significantly increased with the majority of patients surviving into adulthood. Therefore, the prevalence of adult patients with congenital heart disease has dramatically increased, as well as the need for specific and dedicated programs. Acute heart failure, infective endocarditis and arrhythmias represent the most common causes of visit in the emergency department in this population. Our task force aimed at guiding physicians taking care of this peculiar cohort of patients in the emergency department.

Tetralogy of Fallot: cardiac imaging evaluation.

Thanks to advances in pediatric cardiology, most infants with tetralogy of Fallot (TOF) now survive into adulthood. This relatively new population of adult patients may face long-term complications, including pulmonary regurgitation (PR), right ventricular (RV) tract obstruction, residual shunts, RV dysfunction, and arrythmias. They will often need to undergo pulmonary valve (PV) replacement and other invasive re-interventions. However, the optimal timing for these procedures is challenging, largely due to the complexity of evaluating RV volume and function. The options for the follow-up of these patients have rapidly evolved from an angiography-based approach to the surge of advanced imaging techniques, mainly echocardiography, cardiac magnetic resonance (CMR), and computer tomography (CT). In this review, we outline the indications, strengths and limitations of these modalities in the adult TOF population.

Advances in pediatric cardiology in 2019: a narrative review

Advances in pediatric cardiology in 2019: a narrative review

Promoting Resilience in Families with Children with Congenital Heart Disease

Congenital heart disease (CHD) is the most common birth defect, affecting approximately 40,000 infants annually in the US [1]. CHD involves a variety of heart defects, with a wide spectrum from simple to moderate to complex. Due to advances in pediatric cardiology and cardiac surgery, life expectancy in these children has increased drastically over the past decades. Now 90% of infants diagnosed with CHD live well into adulthood [2]. Due to the increase in survival rates, attention has shifted towards the impact of CHD on psychological and cognitive functioning [3]. Children with CHD are at risk for neurodevelopmental and psychosocial problems related to operative factors. Children with CHD display lower cognitive functioning, and higher rates in behavioral, attentional and emotional problems. Increased parental stress also adds to the psychosocial issues experienced by these children. For this reason, psychological care needed to promote resiliency in this population. Incorporating psychological services into treatment protocols with children with CHD is beneficial and will improve behavioral and emotional functioning across their lifespan [4]. This paper will review the definitions of resiliency and Congenital Heart Disease, as well as the professional roles of people treating infants with CHD, the risk factors deterring resiliency, and the developmental outcomes in children with CHD. It will then examine some of the most common psychological interventions for children with CHD and chronic illness, including CBT and psychoeducational interventions, and family-based interventions that also include a psychoeducational component. I will conclude by discussing the value of this multidimensional approach and why it is the most effective intervention strategy to promote resiliency in children with CHD.

Advances in pediatric cardiology 2018

Advances in pediatric cardiology 2018

Eisenmenger syndrome: current perspectives

Eisenmenger syndrome: current perspectives Heba Nashat,1–3 Aleksander Kempny,1–3 Colm McCabe,1 Laura C Price,1–3 Carl Harries,1 Rafael Alonso-Gonzalez,1–3 Michael A Gatzoulis,1–3 Stephen J Wort,1–3 Konstantinos Dimopoulos1–3 1Adult Congenital Heart Centre and National Centre for Pulmonary Hypertension, Royal Brompton Hospital, 2National Heart and Lung Institute (NHLI), Imperial College, 3National Institute for Health Research (NIHR) Cardiovascular Biomedical Research Unit, Royal Brompton Hospital and National Heart and Lung Institute, Imperial College, London, UK Abstract: Eisenmenger syndrome (ES) is the most severe form of pulmonary arterial hypertension (PAH) related to congenital heart disease (CHD). It results from a cardiac defect allowing significant systemic-to-pulmonary (left-to-right) shunting, which triggers the development of pulmonary vascular disease (PVD) if the defect is not repaired in a timely fashion. Once severe PVD has developed, the defect cannot be repaired. With advances in pediatric cardiology and surgery, the prevalence of ES is steadily falling in developed countries; nonetheless, there will always be patients who are unsuitable for repair at the time of diagnosis, or emigrating from countries with less advanced healthcare, who will develop ES. ES is a multisystem disorder causing chronic hypoxemia and reduced cardiac output resulting in significant morbidity and mortality. While lung (plus defect repair) or combined heart and lung transplantation is thought be the definitive treatment for ES, transplant organs are a limited resource and long-term results are still suboptimal. PAH pharmacotherapy was, until quite recently, largely directed at symptomatic relief and had no impact on morbidity and mortality. Targeted PAH therapies have recently been proven to be beneficial in various forms of PAH in terms of functional status, progression of disease, and prognosis. Data on the effect of PAH therapies in the ES cohort remain limited, but available studies demonstrate evidence of improvement in symptoms, exercise capacity, and some evidence of survival benefit. ES patients should be followed in specialized centers, by means of an interdisciplinary approach by clinicians experienced in PAH and CHD. However, local physicians working in cardiology, respiratory medicine, primary care, and emergency services are likely to encounter ES patients and need to be aware of the main issues and pitfalls in their care. The authors present an overview of the management of ES, focusing on the most common issues and complications. Keywords: Eisenmenger syndrome, pulmonary arterial hypertension, congenital heart disease, cyanosis, pulmonary arterial hypertension therapies, hyperviscosity, thrombosis, bleeding, pregnancy, Down syndrome

Cardiac stem cell therapies for congenital heart diseases

During the last 2 decades, stem cell therapies with bone marrow mononuclear cells (BMMNCs) or mesenchymal stem cells (MSCs) to treat ischemic heart disease, including in pre-clinical and clinical trials, have demonstrated the ability of stem cells to improve cardiac function, infarct size, and cardiac remodeling in adult patients. In recent years, endogenous cardiac stem cells (CSCs) derived from heart tissue have been identified. CSCs have been shown to have superior regenerative potential over other types of stem cells in terms of cardiovascular-lineage differentiation, paracrine factor secretion, and functional improvement after cell transplantation. Cardiac stem cell therapy to regenerate damaged myocardium after chronic infarction has been reported in the SCIPIO and CADUCEUS trials. In contrast, although recent advances in pediatric cardiology, congenital cardiac surgery, and intensive care management have dramatically changed clinical outcomes, there is an increasing recognition of limited therapeutic improvement in children with severe heart failure. Congenital heart failure is a structural heart disease caused by multiple etiologies related to pressure and volume overload, arrhythmia, and myocardial degradation. Stem cell-based strategies to treat heart failure in adults have been investigated; however, little is known about their safety and efficacy in children and planned clinical studies are quite limited. Only case reports have been published and no large clinical trials have been conducted using any type of stem cells. Recently, the TICAP trial has revealed the safety and feasibility of intracoronary infusion of autologous cardiosphere-derived cells (CDCs) in children with hypoplastic left heart syndrome (HLHS). Although this trial had several limitations that required further evaluation, the results from this study provided a foothold for stem cell-based therapeutic strategies in patients with congenital heart disease. Eventually, a new paradigm of stem cell therapy to treat congenital heart failure has started to form. Many important issues including long-term cell engraftment, the mechanism of stem cell recruitment and differentiation, administration route, and appropriate cell types to deliver in situ remain to be investigated. Here, we review the latest research on stem cell therapies for heart failure and discuss the future perspectives on cell-based regenerative strategies to treat patients with congenital heart diseases.

The heart of the matter: from guided microtools to 3-D printing and precision genome editing, promising research could lead to new advances in pediatric cardiology.

The smooth, powerful muscles of a newborn baby?s heart are pulsing normally, squeezing in and letting go rhythmically as a 3-mm-wide catheter-like tube snakes its way through, entering via an artery and being guided slowly by a surgeon. When it reaches its target?a protruding knot of malformed muscle tissue within a ventricle that has been partly blocking the valve?the tip of the precisely controlled tube whirs into action, with tiny scissor-like rotating blades gently grinding up the excess tissue as those pieces are sucked back into the device, leaving no floating particles that could lead to a blockage elsewhere. The defect is fully removed, and the heart?s function is restored to normal, leaving the child with the prospect of a normal life. The whole minimally invasive process takes place inside a beating heart and would otherwise have required open-heart surgery, with the heart stopped for a cardiopulmonary bypass.

Stepwise Approach Using Combined Management in Patients with Congenital Heart Disease and Borderline Pulmonary Vascular Disease.

Background and ObjectivesDespite remarkable advances in pediatric cardiology, pulmonary arterial hypertension associated with congenital heart disease remains a major problem. In the past decade new vasodilators have been introduced and appear to be effective in reducing pulmonary vascular resistance (PVR).Subjects and MethodsFrom 2000 to 2011, we retrospectively reviewed the records of 22 patients who had congenital septal defects and borderline pulmonary vascular disease (PVD). The PVR in these patients was from 6 to 16 wood units · m2, and/or the systolic pulmonary arterial pressure was more than 2/3 of the systemic arterial pressure.ResultsThe median age was 16 years (range, 9 months-46 years). The median duration of follow-up was 7.4 years (range, 1.4-11.7 years). According to hemodynamic data and clinical symptoms, the initial management comprised targeted medical therapy in four (18%), complete closure in four (18%), and partial closure in 14 patients (63.6%). In the four patients who had a high PVR and negative vasoreactivity, the PVR decreased and vasoreactivity increased after targeted medical therapy; three of these patients underwent cardiac surgery later. Finally, 11 (50%) received targeted medical therapy and 21 patients (95.4%) underwent cardiac surgery. Complete closure resulted in six patients and partial closure in 17 patients. Mortality was observed in two patients. The other 19 patients (91%) had New York Heart Association functional class I.ConclusionTargeted medical therapy may be effective in reducing PVR in patients with congenital heart disease and borderline PVD. A stepwise approach may help to achieve improved outcomes in these patients.

Long-term outcomes after heart transplantation in adult patients with cardiac congenital heart disease: impact of initial defect and repair: a collaboratve study on 97 patients

Purpose: An increasing number of children with complex congenital heart disease reach adulthood because of the advances in pediatric cardiology and surgery. Residual abnormalities in cardiac structure and function predispose adults with congenital heart disease (ACHD) to late-onset heart failure and its complications. Heart transplantation (HTx) often remains the only option. But ACHD represent a heterogeneous population. We thought to compare outcomes in different defects and in different physiopathological models of repair i.e. univentricular (1V) or biventricular (2V) status. Methods: Out of a multi-institutional (3 centers) series of 2257 HTx from 1988 and 2012, 100 (4.4%) were performed in 97 ACHD aged ≥16 years at the time of operation (65 men). We evaluated clinical data, etiology, surgical history, perioperative issues and outcomes. Results: The mean age at the time of HTx was 29.8±10.5 years (range 16-58). Forty-three patients (44%) had 1V physiology including 16.5% Fontan-type circulation and 54 (56%) had 2V physiology including 26% with a systemic right ventricle. Primary diagnosis included single ventricle (27%), D-transposition of the great arteries (20%), tricuspid atresia (13%), tetralogy of Fallot (12%), congenital valvular heart diseases (11%), corrected transposition of the great arteries (7%), and other anomalies (9%). Eighty-seven patients (90%) had undergone ≥1 prior cardiac surgery before HTx.An additional surgical procedure during the HTx, like pulmonary artery reconstruction, was performed in 22% patients. Twenty-four patients (25%) required reintervention for post-operative hemorrhage. In-hospital mortality was high (34%). Multivariable factors associated with increased in-hospital mortality included female gender of the donor and tricuspid atresia. The 1V patients were more often younger (26.9 vs 32.1 years, p=0.014), had undergone more surgeries (2.9 vs 1.9 prior surgeries, p=0.002), had a lower requirement for ventricular assist device implantation before HTx (4.7% vs 20.4%, p=0.02), and had longer ischemic (229 vs 182min, p<0.001) and cardiopulmonary bypass times (240 vs 163min, p<0.001). Long-term survival was significantly higher in 2V patients (p=0.023). Conclusion: HTx of ACHD poses several surgical challenges due to complex anatomy, specific physiology and prior surgical interventions. 1V physiology is associated with higher in-hospital and long-term mortality, in comparison to 2V physiology. Further studies are warranted to identify whether earlier HTx of these patients may improve these outcomes.

Boston Circulatory Arrest Study at 16 Years

Caring for the adolescent with surgically repaired D-transposition of the great arteries can be one of the most satisfying experiences in congenital cardiology. The operative mortality for the arterial switch procedure is now <3%.1 Few of these children require cardiac medications; exercise limitations are minimal; and very rarely do the patients require hospitalization. In many respects, advances in pediatric cardiology and cardiac surgery have succeeded in curing this once lethal condition. Article see p 1361 As in the past, however, the data cultivated from the Boston Circulatory Arrest Study (BCAS) mandate that we consider these surgical successes in the context of the larger spheres of school performance and social functioning. One might think that the story had been already told by the time this unique cohort reached 8 years of age. After all, cognitive intelligence is largely determined by early school years. However, the study by Bellinger and colleagues2 in this issue of Circulation shows convincingly that there is much to be learned by reexamining this study population in adolescence. Interestingly, the original hypothesis that a strategy of predominantly low-flow cardiopulmonary bypass compared with predominant hypothermic circulatory arrest might improve neurocognitive outcomes nowadays seems less intriguing. Recent data from the Society of Thoracic Surgeons suggest …

From Numbers to Guidelines

Advances in pediatric cardiology and cardiac surgery have resulted in a change in the mortality, prevalence, and age distribution of patients with congenital heart disease (CHD). In this chapter, we review the changing epidemiology of CHD and the impact of these trends on health services utilization and delivery in this population. We demonstrate not only that adult CHD (ACHD) patients have high utilization rates but also that care gaps exist where disease is expected to be lifelong. We outline the components of quality improvement for the care of ACHD patients. We review the newly published guidelines for the management of CHD conditions in adults, anchoring them to structure, process, and outcome indexes of quality of care. We highlight the manpower needs and the importance of an appropriately trained cardiology workforce to provide ACHD care. Finally, we review recommended health care systems ideally suited to deliver care to this population.

Changing demographics of pulmonary arterial hypertension in congenital heart disease

Pulmonary arterial hypertension (PAH) is a serious complication of congenital heart disease (CHD). Without early surgical repair, around one-third of paediatric CHD patients develop significant PAH. Recent data from the Netherlands suggest that >4% of adult CHD patients have PAH, with higher rates in those with septal defects. A spectrum of cardiac defects is associated with PAH-CHD, although most cases develop as a consequence of large systemic-to-pulmonary shunts. Eisenmenger's syndrome, characterised by reversed pulmonary-to-systemic (right-to-left) shunt, represents the most advanced form of PAH-CHD and affects as many as 50% of those with PAH and left-to-right shunts. It is associated with the poorest outcome among patients with PAH-CHD. 40 yrs ago, ∼50% of children with CHD requiring intervention died within the first year, and <15% survived to adulthood. Subsequent advances in paediatric cardiology have seen most patients with CHD survive to adulthood, with resulting shifts in the demographics of CHD and PAH-CHD. The number of adults presenting with CHD is increasing and, although mortality is decreasing, morbidity is increasing as older patients are at increased risk of arrhythmia, heart failure, valve regurgitation and PAH. Data show that probability of PAH increases with age in patients with cardiac defects.

How Many Patients? How Many Doctors?

One of the fastest growing fields in medicine is the care of adults with congenital heart disease (adult CHD or ACHD). This is mainly a consequence of much longer survival due to advances in pediatric cardiology, interventional catheterization, cardiac surgery, and intensive care. Fifty years ago we could diagnose most forms of moderate and severe CHD, but some anomalies were untreatable and others had relatively low survival after treatment. Today, in-hospital mortality is low for almost all forms of CHD, and most of the survivors can be expected to reach adult life. In addition, a medical system with greater focus on CHD now detects many lesions such as coarctation of the aorta and atrial septal defects that previously went unnoticed. Patients with ACHD need adequate resources, facilities and manpower to care for them, and we in turn need accurate estimates of how many patients have each type and severity of ACHD in order to plan effectively. These estimates are moving targets, because improvements in preoperative, intraoperative, and postoperative care occur continuously. What was good yesterday may not be adequate today. Articles see pp 2254 and 2264 The classic dilemma in studying this problem is how to obtain accurate data on a large number of patients. Studies based on registries showing the incidence of CHD at birth and mortality from CHD may show the big picture, but the details are inaccurate.1 On the other hand, following a smaller population intensively gives accurate data but runs the risk of being unrepresentative of the population as whole as well as risking excessive stochastic variation, especially for complex anomalies with a low birth incidence. Several attempts have been made to derive these data. A landmark report of Task Force I of the 32nd Bethesda Conference set up by the American College of …

Canadian Cardiovascular Society 2009 Consensus Conference on the management of adults with congenital heart disease: Introduction

With advances in pediatric cardiology and cardiac surgery, the population of adults with congenital heart disease (CHD) has increased. In the current era, there are more adults with CHD than children. This population has many unique issues and needs. Since the 2001 Canadian Cardiovascular Society consensus conference report on the management of adults with CHD, there have been significant advances in the field of adult CHD. Therefore, new clinical guidelines have been written by Canadian adult CHD physicians in collaboration with an international panel of experts in the field. The present introductory section is a summary of the epidemiology and scope of adult CHD in Canada, the structure of the Canadian health care system and adult congenital cardiac health services in Canada. The recommendations for antibiotic prophylaxis and genetic evaluation in this population are included. The complete document consists of four manuscripts, which are published online in the present issue of The Canadian Journal of Cardiology, including sections on genetics, outcomes, diagnostic workups, surgical and interventional options, treatment of arrhythmias, assessment of pregnancy and contraception risks, and follow-up recommendations. The complete document and references can also be found at www.ccs.ca or www.cachnet.org.