Persistent patency of the arterial duct represents one of the most common lesions in the field of congenital cardiac disease. The strategies for management continue to evolve. In this review, we focus on management beyond the neonatal period. We review the temporal evolution of strategies for management, illustrate the currently available the techniques for permanent closure of the patent arterial duct, review the expected outcomes after closure, discuss the current controversy over the appropriate treatment of the so-called “silent” duct, and provide recommendations for the current state of management of patients with persistent patency of the arterial duct outside of the neonatal period.

At the Congenital Heart Institute of Florida, we now recommend closure of all patent arterial ducts, regardless of their size. Before selecting and performing the type of procedure, we explain the natural history of the persistently patent arterial duct to the parents or legal guardian of the child. Particular emphasis is placed on the risks of endocarditis, including the recognition that many cases of endocarditis may not be preventable.

The devastating effects of endocarditis, coupled with the perception of more anecdotal reports of endocarditis with the silent duct, as well as the low risk of interventions, has led us to recommend closure of the patent arterial duct in these situations. We now recommend intervention, after informed consent, for all patients with a patent arterial duct regardless of size, including those in which the patent duct is “silent”. We recognize, however, that this remains a controversial topic, especially given the new recommendations for endocarditis prophylaxis from American Heart Association.

Since 2003, our strategy for closure of the patent arterial duct has changed subsequent to the availability of the Amplatzer occluder. This new device has allowed significantly larger patent arterial ducts to be closed with interventional catheterization procedures that in the past would have been closed at surgery. During the interval between 2002 and 2006 inclusive, the overall surgical volume at our Institute has been stable. Over this period, the number of patients undergoing surgical ligation of the patent arterial duct has decreased, with this decline in volume most notable for the subgroup of patients weighing more than five kilograms. This decrease has been especially notable in thoracoscopic procedures and is attributable to the increased ability to close larger ducts using the Amplatzer occluder.

For infants with symptomatic pulmonary overcirculation weighing less than 5 kilograms, our preference is for the surgical approach. For patients who have ductal calcification, significant pleural scarring, or “window-like” arterial ducts, video-assisted ligation is not an option and open surgical techniques are used. When video-assisted ligation is possible, the approach is based on family and surgeon preference. When open thoracotomy is selected, we usually use a muscle-sparing left posterolateral thoracotomy.

For patients weighing more than 5 kilograms, we currently recommend percutaneous closure for all patent arterial ducts as the first intervention, reserving surgical treatment for those cases that are not amenable to the percutaneous approach. For symptomatic infants weighing greater than 5 kilogram with large ducts, we prefer to use the Amplatzer occluder. In rare instances, the size of the required ductal occluder is so large that either encroachment into the aorta or pulmonary arteries is noted, and the device is removed. The child is then referred for surgical closure. We can now often predict via echocardiography that a duct is too large for transcatheter closure, even with the Amplatzer occluder, and refer these patients directly to surgery.

For patients with an asymptomatic patent arterial duct, we prefer to wait until the weight is from 10 to 12 kilograms, or they are closer to 2 years of age. If the patent arterial duct is greater than 2.0 to 2.5 millimetres in diameter, our preference is to use the Amplatzer occluder. For smaller ducts, we typically use stainless steel coils. Using this strategy, we close all patent arterial ducts, regardless of their size.

This review includes a brief discussion, from the perspective of cardiac surgeons, of the rationale for creation and maintenance of multi-institutional databases of outcomes of congenital heart surgery, together with a history of the evolution of such databases, a description of the current state of the art, and a discussion of areas for improvement and future expansion of the concept. Five fundamental areas are reviewed: nomenclature, mechanism of data collection and storage, mechanisms for the evaluation and comparison of the complexity of operations and stratification of risk, mechanisms to ensure the completeness and accuracy of the data, and mechanisms for expansion of the current capabilities of databases to include comparison and sharing of data between medical subspecialties. This review briefly describes several European and North American initiatives related to databases for pediatric and congenital cardiac surgery the Congenital Database of The European Association for Cardio-Thoracic Surgery, the Congenital Database of The Society of Thoracic Surgeons, the Pediatric Cardiac Care Consortium, and the Central Cardiac Audit Database in the United Kingdom. Potential means of approaching the ultimate goal of acquisition of long-term follow-up data, and input of this data over the life of the patient, are also considered.

Most patients with the Mustard procedure are now adults. To date, however, there have been few reports on resting and exercise hemodynamics in a large population of adults with this circulation. The aim of this study is to describe such parameters in one of the largest and oldest populations of adults with the Mustard procedure. The database of the University of Toronto Congenital Cardiac Centre for Adults was examined to identify 84 adults with the Mustard procedure who have undergone cardiopulmonary exercise tests. Magnetic resonance imaging and echocardiography studies were obtained in order to assess right ventricular size, function and baseline hemodynamics. Patients achieved lower maximum uptake of oxygen, maximal heart rate, forced vital capacity, forced expiratory volume in 1 second, and oxygen saturations at maximal exercise compared to a healthy population. Magnetic resonance imaging showed significantly different right ventricular ejection fractions between patients and controls. There were no effects of operative variables or preoperative hemodynamics on current exercise capacity. Patients after the Mustard procedure have subnormal exercise capacities. Factors such as chronotropic incompetence, peripheral deconditioning, and impaired lung function may be responsible for these results.

In patients with cyanotic congenital heart disease, chronic hypoxaemia leads to important changes in blood vessel function and structure. Some of these alterations are maladaptive and probably contribute to impaired cardiopulmonary performance and an increased incidence of thrombotic and embolic events. Recent evidence suggests that deranged endothelial function, a sequel of chronic cyanosis, could be an important factor in the pathogenesis of cyanosis-associated cardiovascular risk. In this article, we discuss the physiological and mechanical consequences of compensatory erythrocytosis and possible pathophysiological mechanisms of vascular dysfunction in chronic cyanosis.

This review includes a brief discussion, from the perspective of the pediatric cardiologist, of the rationale for creation and maintenance of multi-institutional databases of outcomes of the treatment of patients with congenital and paediatric cardiac disease, together with a history of the evolution of such databases, and a description of the current state of the art. A number of projects designed to have broad-based impact are currently in the design phase, or have already been implemented. Not surprisingly, most of the efforts thus far have focused on catheterization procedures and interventions, although some work examining other aspects of paediatric cardiology practice is also beginning. This review briefly describes several European and North American initiatives related to databases for pediatric and congenital cardiology including the Central Cardiac Audit Database of the United Kingdom, national database initiatives for pediatric cardiology in Switzerland and Germany, various database initiatives under the leadership of the Working Groups of The Association for European Paediatric Cardiology, the IMPACT Registry™ (IMproving Pediatric and Adult Congenital Treatment) of the National Cardiovascular Data Registry® of The American College of Cardiology Foundation® and The Society for Cardiovascular Angiography and Interventions (SCAI), the Mid-Atlantic Group of Interventional Cardiology (MAGIC) Catheterization Outcomes Project, the Congenital Cardiac Catheterization Project on Outcomes (C3PO), the Congenital Cardiovascular Interventional Study Consortium (CCISC), and the Joint Council on Congenital Heart Disease (JCCHD) National Quality Improvement Initiative. These projects, each leveraging multicentre data and collaboration, demonstrate the enormous progress that has occurred over the last several years to improve the quality and consistency of information about nonsurgical treatment for congenital cardiac disease. The paediatric cardiology field is well-poised to move quickly beyond outcome assessment and benchmarking, to collaborative quality improvement.

The past two decades have witnessed a major shift towards repair of most congenital cardiac malformations during the neonatal or infant periods of life.1 Early anatomic correction or palliation, dramatic improvements in survival, and reduced morbidity due to improvements in perioperative and long-term medical management, have resulted in new populations of children that have reaped the benefits of the best care currently available for treatment of congenital cardiac disease. The impact of the congenital cardiac malformations, however, extends far beyond the walls of the hospital or clinic where we diagnose, treat, and follow our patients. The breakthrough of achieving predictable results with repair or palliation of most lesions during the neonatal and infant periods mandates us to look beyond survival, and to examine the lives our patients lead when they are outside of our care. Our purpose in this review is to discuss the measures of psychosocial outcome that are appropriate for exploration in those neonates and infants who survive cardiac surgery, to explore what is known about the psychosocial outcomes and quality of life for these patients, and what needs exist for future research.

Over a 25-year period, we encountered 12 patients who died suddenly with coronary arterial lesions due to Kawasaki disease. We report their clinical course, and analyze the happenings of their deaths. Of the 12 patients, 10 were dead on arrival at hospital. Their age at death ranged from 13 months to 27 years, with a median of 16 years, and the interval from the onset of Kawasaki disease to death ranged from 2 months to 24 years. In 4 patients, death was found to be due to myocardial infarction, while in the remaining 8, it could not be determined. In 7 patients, coronary angiograms obtained less than 4 months after the acute onset of Kawasaki disease showed lesions bilaterally, most being giant aneurysms. Myocardial infarction had occurred in 6 patients prior to their death. In 1 patient of the late 1970s, who collapsed after running, cardiac sequels had not been suspected prior to autopsy. During the 1980s, 3 infants with bilateral giant aneurysms died within a year of the initial onset of Kawasaki disease, with acute myocardial infarction being the cause in 2 of them. In the late 1990s, and the 2000s, 5 patients died suddenly with left ventricular dysfunction, their ejection fractions being less than 40 percent more than 20 years after the initial onset of Kawasaki disease. Prior to their sudden deaths, they had had no cardiac events for many years, but had suffered previous myocardial infarctions. Multifocal premature ventricular contractions, and non-sustained ventricular tachycardia, are probable risk factors in such patients. Careful follow-up, checking for ventricular arrhythmia, is needed to prevent sudden death in patients suffering left ventricular dysfunction in the setting of Kawasaki disease.

Introduction: There is limited data on medium to long-term outcome, and the morbidity and mortality associated with the implantation, of pulmonary arterial stents. Purpose: To assess changes in morbidity and mortality over the last two decades. Methods: Retrospective analysis of all patients stented between September, 1989 and July, 2001. Results: We implanted 664 Palmaz stents in 338 patients. The overall number included 229 patients who had undergone repair of tetralogy of Fallot, in whom 468 stents were implanted, 61 patients with congenital stenosis of the branches of the pulmonary trunk, in whom we placed 115 stents, 16 patients after an arterial switch operation who had 38 stents, and 32 patients after the Fontan operation who had 43 stents implanted. The mean age was 12.2 years, and the mean weight was 38 kg. The mean systolic pressure gradient decreased from 41 to 8.7 mmHg, the mean diameter of the stented vessel increased from 5.4 to 11.2 mm, and the ratio of right ventricular to femoral arterial pressure decreased from 0.66 to 0.45, each of these being significant at the level of p being less than 0.01. At a mean follow-up of 5.6 years, the mean gradient was 20 mmHg, the mean ratio of pressure between right ventricle and femoral artery was 0.5, and mean luminal diameter was 9.3 mm. Complications included migration of the stent in 8 patients, and pulmonary edema, hemoptysis and death in 5 patients each. There has been no mortality or morbidity since July of 1997. Technical changes include conservative serial dilations in congenital pulmonary arterial stenosis, avoidance of over-dilation, and simultaneous implantation of stents in the right and left pulmonary arteries in those with systemic pulmonary arterial pressure. Technological advances included shorter stents, improved balloon profiles, and central inflation of the stents. Conclusions: Modification of stenting practices, and increased experience of the operators over the last two decades, has virtually abolished any morbidity or mortality associated with the implantation of stents for congenital or postoperative pulmonary arterial stenoses.

In about 1967, a new clinical entity in infants and children was described in Japan. It was an acute febrile illness associated with engorgement of the conjunctivas, labial erythema, and swelling of the deep cervical lymph nodes without suppuration. This disease is usually called the acute febrile mucocutaneous lymph node syndrome or, more simply, Kawasaki disease.

Aims: To compare the rates of employment, and advice offered concerning careers, in adults with congenital heart disease and controls. To assess the impact of the severity of the congenital cardiac malformation on the chances for employment. Methods: We solicited responses from 299 adults with congenitally malformed hearts, asking them to answer questions from a questionnaire posed at interview by a trained nurse. The adults were asked to give an identical questionnaire to a friend to act as a control. We received responses from 177 of the controls. Results: The responses showed that 51 of 156 (33 per cent) adults with congenital heart disease were unemployed, and 37 of 151 (25 per cent) had been unemployed for more than a year. This is significantly more than 25 of 156 (16 per cent) matched controls unemployed, and 5 of 151 (3 per cent) controls unemployed for more than a year. Almost one-fifth of the adults with congenital heart disease (19 per cent) had received advice regarding their career which they found helpful, which is significantly fewer than the 31 per cent of controls. More (42 per cent) had been given advice against certain occupations than controls (11 per cent). Receiving career advice was significantly associated with employment in the population with congenitally malformed hearts, with almost three-quarters (73 per cent) of those given advice being employed compared to 46 per cent of those not given advice. This pattern was not seen in controls. The severity of the congenital cardiac malformation did not significantly affect the rates of unemployment. Conclusions: Whatever the severity of their disease, adults with congenitally malformed hearts are more likely to be unemployed than matched controls. They are less likely to receive useful advice regarding potential careers, and find the advice given less helpful, than controls, although receiving suitable advice is associated with being employed in the population with congenital cardiac disease.

In 2018, the Neurodevelopmental and Psychosocial Interventions Working Group of the Cardiac Neurodevelopmental Outcome Collaborative convened through support from an R13 grant from the National Heart, Lung, and Blood Institute to survey the state of neurodevelopmental and psychosocial intervention research in CHD and to propose a slate of critical questions and investigations required to improve outcomes for this growing population of survivors and their families. Prior research, although limited, suggests that individualised developmental care interventions delivered early in life are beneficial for improving a range of outcomes including feeding, motor and cognitive development, and physiological regulation. Interventions to address self-regulatory, cognitive, and social-emotional challenges have shown promise in other medical populations, yet their applicability and effectiveness for use in individuals with CHD have not been examined. To move this field of research forward, we must strive to better understand the impact of neurodevelopmental and psychosocial intervention within the CHD population including adapting existing interventions for individuals with CHD. We must examine the ways in which dedicated cardiac neurodevelopmental follow-up programmes bolster resilience and support children and families through the myriad transitions inherent to the experience of living with CHD. And, we must ensure that interventions are person-/family-centred, inclusive of individuals from diverse cultural backgrounds as well as those with genetic/medical comorbidities, and proactive in their efforts to include individuals who are at highest risk but who may be traditionally less likely to participate in intervention trials.

Objective: To assess the incidence of depression, and the ability to interact socially, in adult patients with chronic cyanosis and congenital cardiac malformations. Design: Prospective study of consecutive patients. Setting: Single institution, tertiary referral centre. Patients: Between 1993 and 2000, we assessed 76 patients with congenital cardiac malformations and persistent cyanosis, having a median age of 36.5 years, with a range from 19 to 64 years, at the time of referral. Female patients accounted for just under half (48.6%) of the sample. Just under two-fifths of the cohort (39.5%) had functionally univentricular cardiac anatomy, while 14.8% had tetralogy of Fallot with pulmonary atresia and aorto-pulmonary collateral arteries, and 17% had the Eisenmenger syndrome. During the period of follow-up, 17 (22.4%) of the patients died. Assessment: We used clinical interviews and non-invasive assessment, employing Zung's questionnaire which provides a scale for the self-rating of depression. On this scale, a score above 50 points is indicative of depression. Results: Of the survivors, 32 (54%) completed the self-rating questionnaires. Of these, 20 responders (63%) considered that they lead full lives, including sexual activities, while 26 (81%) had never harboured suicidal thoughts. Depression was diagnosed in 11 responders (34%), with a mean score of 66.9, standard deviation of 8.7, and a range from 53 to 89. The remaining 21 patients (66%) were without signs of depression, scoring a mean of 41.5, with standard deviation of 5.5, and a range from 35 to 46. Depression was associated with older age (40.5 years versus 33.5 years, p =0.01), worse functional state in the classification of the New York Heart Association (2.95 versus 2.48, p =0.03), and unemployment (p < 0.0001), but independent from the severity of cyanosis, the level of the haematocrit, the saturation of oxygen, or previous surgical treatment. Conclusions: To our knowledge, this is the first evidence suggesting a relatively high incidence of depression in adults with congenital cardiac malformations and persistent cyanosis. Larger, multi-centric studies will be needed to confirm or refute these findings.

The aim of this review is to describe the current state of knowledge related to neurodevelopmental outcomes and quality of life for children with hypoplastic left heart syndrome and to explore future questions to be answered for this group of children.

An estimated 15 million children die or are crippled annually by treatable or preventable heart disease in low- and middle-income countries. Global efforts to reduce under-5 mortality have focused on reducing death from communicable diseases in low- and middle-income countries with little to no attention focusing on paediatric CHD and acquired heart disease. Lack of awareness of CHD and acquired heart disease, access to care, poor healthcare infrastructure, competing health priorities, and a critical shortage of specialists are important reasons why paediatric heart disease has not been addressed in low resourced settings. Non-governmental organisations have taken the lead to address these challenges. This review describes the global burden of paediatric heart disease and strategies to improve the quality of care for paediatric heart disease. These strategies would improve outcomes for children with heart disease.

Objectives: To determine the anatomical characteristics of systemic venous collaterals formed after the Fontan operation, and the efficacy of a transcatheter strategy for management. Methods: We reviewed retrospectively the data from cardiac catherization of 50 persistently cyanotic patients after the Fontan operation. Results: A total of 54 transcatheter interventions were performed, at a mean age of 6.3 ± 3.5 years, a mean interval of 2.7 ± 2.9 years from completion of the Fontan circulation. Of 38 patients who had fenestration of the baffle at the time of surgery, 25 had patency of the fenestration, and 24 had the fenestration occluded with a device at the time of interventional treatment for associated venous collaterals. We identified a total of 68 systemic venous collateral channels, of which 36 (53%) were supracardiac, 12 (18%) cardiac, and 20 (29%) infracardiac in origin. The most common site of origin was the brachiocephalic vein (44%), followed by the left phrenic vein (25%). A longer time from surgery, at 3.3 ± 3.4 years, was associated with the identification of collaterals having a diameter larger than 4 mm (p < 0.01). The mean pulmonary arterial pressure was higher in those with larger compared to those with smaller collaterals (13.3 ± 2.8 versus 11.1 ± 2.0 mmHg, p < 0.01). Coils were used for occlusion of 61 vessels, and a Rashkind™ occluder for the remaining 7. After exclusion of the patients undergoing simultaneous closure of their fenestration, systemic saturation of oxygen increased from 89 ± 6% to 95 ± 3% (p < 0.01). Conclusion: Venous collateral channels are common in patients suffering progressive cyanosis in the setting of the Fontan circulation. The collaterals increase in size with time, and are associated with higher pulmonary arterial pressures. Transcatheter treatment is feasible, and results in resolution of cyanosis. Only continuing follow-up will show whether further collateralization occurs in time.