Multiple recurrent supraventricular tachycardia in infantile tuberous sclerosis complex: management requiring triple-drug therapy.

We report the case of a female neonate admitted to the neonatal ICU with a rapid, narrow-complex tachyarrhythmia determined to be supraventricular tachycardia. Multimodality imaging and genetic testing confirmed a diagnosis of tuberous sclerosis complex with multiple cardiac rhabdomyomas. At 13 days of age, the patient was readmitted, exhibiting recurrent supraventricular tachycardia non-responsive to first-line treatment. Management required triple-drug therapy, whereafter the patient remained stable without recurrences. This is a rare report of supraventricular tachycardia in a functionally normal heart with the occurrence of supraventricular tachycardia due to structural abnormalities, with the possibility of multiple concealed accessory pathways.

Mitral annular disjunction and mitral valve prolapse extrapolating adult data to an adolescent cohort?

PURPOSE OF REVIEW: The aim of this study is to provide an update on mitral valve prolapse (MVP) and mitral annular disjunction (MAD) and who may be at risk for ventricular arrhythmias and sudden cardiac death. RECENT FINDINGS: MVP is generally considered a benign condition. However, a small subset of patients may be at risk for life-threatening ventricular arrhythmias. Among the risk factors identified in adults include patients with bileaflet mitral valves, myxomatous changes, myocardial fibrosis, and the presence of MAD. Advances in multimodal imaging have allowed for improved identification of fibrosis, anatomical valve derangements, and the amount of MAD. Recent guidelines have suggested that patients with MVP with or without MAD may be at risk for life-threatening arrhythmias if they have had prior ventricular arrhythmias, ventricular dysfunction, or unexplained syncope. Yet, extrapolation of adult data to a pediatric cohort with similar MVP and MAD at this juncture is challenging. There is, however, early evidence that some pediatric patients with significant myocardial fibrosis or abnormal tissue Doppler may be at risk for ventricular tachycardia. SUMMARY: Mitral valve prolapse and mitral annular disjunction at times coexist and at other times can be seen as isolated entities. While the incidence of arrhythmic MVP is quite rare, there is increasing evidence that certain select adults with MVP may be at risk for ventricular tachycardia and sudden cardiac death. Future multicenter studies are needed to better understand the natural history of arrhythmic mitral valve disease and how early disease manifestation in children may impact findings now being reported in young adults.

Recommendations for Centers Performing Pediatric Heart Surgery in the United States.

Care and outcomes for the more than 40,000 patients undergoing pediatric and congenital heart surgery in the United States annually are known to vary widely. While consensus recommendations have been published across numerous fields as one mechanism to promote a high level of care delivery across centers, it has been more than two decades since the last pediatric heart surgery recommendations were published in the United States. More recent guidance is lacking, and collaborative efforts involving the many disciplines engaged in caring for these children have not been undertaken to date. The present initiative brings together professional societies spanning numerous care domains and congenital cardiac surgeons, pediatric cardiologists, nursing, and other healthcare professionals from diverse programs around the country to develop consensus recommendations for United States centers. The focus of this initial work is on pediatric heart surgery, and it is recommended that future efforts focus in detail on the adult congenital population. We describe the background, rationale, and methodology related to this collaborative effort, and recommendations put forth for Essential Care Centers (essential services necessary for any program), and Comprehensive Care Centers (services to optimize comprehensive and high-complexity care), encompassing structure, process, and outcome metrics across 14 domains.

Recommendations for centers performing pediatric heart surgery in the United States.

Care and outcomes for the more than 40,000 patients undergoing pediatric and congenital heart surgery in the United States annually are known to vary widely. While consensus recommendations have been published across numerous fields as one mechanism to promote a high level of care delivery across centers, it has been more than two decades since the last pediatric heart surgery recommendations were published in the United States. More recent guidance is lacking, and collaborative efforts involving the many disciplines engaged in caring for these children have not been undertaken to date. The present initiative brings together professional societies spanning numerous care domains and congenital cardiac surgeons, pediatric cardiologists, nursing, and other healthcare professionals from diverse programs around the country to develop consensus recommendations for United States centers. The focus of this initial work is on pediatric heart surgery, and it is recommended that future efforts focus in detail on the adult congenital population. We describe the background, rationale, and methodology related to this collaborative effort, and recommendations put forth for Essential Care Centers (essential services necessary for any program), and Comprehensive Care Centers (services to optimize comprehensive and high-complexity care), encompassing structure, process, and outcome metrics across 14 domains.

Recommendations for Centers Performing Pediatric Heart Surgery in the United States.

Care and outcomes for the more than 40,000 patients undergoing pediatric and congenital heart surgery in the United States annually are known to vary widely. While consensus recommendations have been published across numerous fields as one mechanism to promote a high level of care delivery across centers, it has been more than two decades since the last pediatric heart surgery recommendations were published in the United States. More recent guidance is lacking, and collaborative efforts involving the many disciplines engaged in caring for these children have not been undertaken to date. The present initiative brings together professional societies spanning numerous care domains and congenital cardiac surgeons, pediatric cardiologists, nursing, and other healthcare professionals from diverse programs around the country to develop consensus recommendations for United States centers. The focus of this initial work is on pediatric heart surgery, and it is recommended that future efforts focus in detail on the adult congenital population. We describe the background, rationale, and methodology related to this collaborative effort, and recommendations put forth for Essential Care Centers (essential services necessary for any program), and Comprehensive Care Centers (services to optimize comprehensive and high-complexity care), encompassing structure, process, and outcome metrics across 14 domains.

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

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

Preliminary assessment of paediatric electrophysiology cardiac implantable electronic device resources around the world.

BACKGROUND: The Pediatric and Congenital Electrophysiology Society (PACES) is a global organisation committed to the care of children and adults with CHD and arrhythmias. OBJECTIVE: To evaluate the global needs and potential inequities as it relates to cardiac implantable electronic devices. METHODS: ARROW (Assessment of Rhythm Resources arOund the World) is an online survey about cardiac implantable electronic devices, sent electronically to physicians within the field of Cardiology, Pediatric Cardiology, Electrophysiology and Pediatric Electrophysiology. RESULTS: ARROW received 42 responders from 28 countries, 50% from low-/middle-income regions. The main differences between low-/middle- and high-income regions include availability of expertise on paediatric electrophysiology (50% versus 93%, p < 00.5) and possibility to perform invasive procedures (35% versus 93%, p < 0.005). Implant of devices in low-income areas relies significantly on patient's resources (71%). The follow-up of the devices is on the hands of paediatric cardiologist/electrophysiologist in higher resources centres (93% versus 50%, p < 0.05). CONCLUSIONS: The ARROW survey represents an initial assessment of the geographical characteristics in the field of Pediatric Electrophysiology. The next step is to make this "state of the art" more extensive to other aspects of the expertise. The relevance of collecting this data before the World Congress of Pediatric Cardiology and Cardiac Surgery (WCPCCS) in 2023 in Washington DC was emphasised in order to share the resulting information with the international community and set a plan of action to assist the development of arrhythmia services for children within developing regions of the world.

Arrhythmogenic right ventricular cardiomyopathy in the pediatric population.

PURPOSE OF REVIEW: Review the current state of the art of arrhythmogenic right ventricular cardiomyopathy (ARVC) diagnosis and risk stratification in the pediatric population. RECENT FINDINGS: ARVC is an inherited cardiomyopathy characterized by progressive myocyte loss and fibrofatty replacement of predominantly the right ventricle and high risk of ventricular arrhythmias and sudden cardiac death (SCD). ARVC is one of the leading causes of arrhythmic cardiac arrest in young people. Early diagnosis and accurate risk assessment are challenging, especially in children who often exhibit little to no phenotype, even if genotype positive. Multimodal imaging provides more detailed assessment of the right ventricle and has been shown in pediatric patients to identify earlier preclinical disease expression. Identification of patients with ARVC allows the clinician to intervene early with appropriate exercise restrictions, even if genotype positive only without phenotypic expression. Emphasis should be placed on stratifying the patient's risk of ventricular arrhythmias and SCD. SUMMARY: ARVC is a challenging diagnosis to make in adolescents who often do not exhibit clinical symptoms. Newer multimodal imaging techniques and improvements in genetic testing and biomarkers should help improve early diagnosis. Exercise restriction for children with ARVC has been shown to reduce disease advancement and decreases the risk of a life-threatening event.

Challenge of managing opposing rhythms in a mother and fetus.

INTRODUCTION: We report a case of a fetus with complex congenital heart disease and supraventricular tachycardia in the setting of maternal high grade atrioventricular block at 26 weeks' gestation. METHODS AND RESULTS: Electroanatomic mapping allowed successful implantation of a permanent pacemaker to provide adequate back-up pacing in the mother with zero radiation exposure, thus allowing safe delivery of transplacental anti-arrhythmic medications to reduce the fetal arrhythmia burden and optimize the fetal ventricular rate. CONCLUSION: This is the first reported case of using electroanatomic mapping, with zero fluoroscopy use, for pacemaker lead placement and for a novel indication.

An unusual presentation of a large cardiac mass.

We present a case of a large left ventricular capillary haemangioma incidentally discovered in a pre-adolescent patient.

Thoracoscopic Cardiac Sympathetic Denervation: Adjunct Therapy for Secondary Prevention of Life-Threatening Ventricular Arrhythmias in Children.

BACKGROUND: Cardiac sympathetic denervation (CSD) is a surgical option for patients with life-threatening ventricular arrhythmias. Previously described cohorts included populations in which CSD was performed for primary and secondary prevention. We report the efficacy of CSD as adjunct therapy in children with medically refractory life-threatening arrhythmias. MATERIALS AND METHODS: Retrospective review of patients undergoing thoracoscopic CSD at one institution between January 2008 and July 2017. Patient demographics, indications, procedural details, complications, length of stay, and effectiveness were evaluated. RESULTS: Ten thoracoscopic CSD procedures were performed in 8 patients. Mean age was 8.2 years (8 days-19 years); mean weight was 32.6 kg (2.7-57 kg); and 50% were female. Four had long QT syndrome, 3 catecholaminergic polymorphic ventricular tachycardia, and 1 short QT syndrome. All patients had at least two (2 to >40) episodes of resuscitated ventricular arrhythmia and were maximized on medical therapy. Six patients had implantable cardioverter-defibrillators (ICD) with a mean of 11.9 appropriate discharges (1-40) before CSD. All patients underwent left CSD; 2 subsequently required right CSD. Four of the 6 ICD patients experienced dramatic improvement (total 48 ICD discharges pre-CSD; 3 post-CSD). Two patients noncompliant with medical therapy had no significant improvement (24 ICD discharges pre-CSD; 23 post-CSD) and also underwent right CSD, again with no improvement (23 discharges pre-right CSD; 28 post-right CSD). CONCLUSIONS: Thoracoscopic CSD can be safely performed in the neonate and pediatric populations. When utilized with medication therapy, CSD is an effective adjunct in reducing ICD discharges and arrhythmias.

Challenges of healthcare administration: optimizing quality and value at an affordable cost in pediatric cardiology.

PURPOSE OF REVIEW: The purpose of this review is to explore the paradigm shift in healthcare delivery that will need to take place over the next few years away from an emphasis on supply-driven health care to better quality transparent-driven health care whose focus is on the consumer's best interest. RECENT FINDINGS: The current healthcare system is fragmented and costs continue to rise. The best way to contain costs is to improve quality to the consumer, the patient. Physicians and hospitals need to align in a team-based approach that allows physicians to understand current costs and how to strive toward a focus on healthcare outcomes. Pediatric cardiology is a unique discipline that cares for patients with complex congenital conditions that will span their lifetime and also involves not just cardiology but surgery, intensive care, anesthesia, nursing, and a host of inpatient and ambulatory services. Understanding what matters to the patient and his/her family and presenting quality outcomes in a transparent fashion will gradually allow a shift to take place away from physician visits, tests ordered, and procedures performed. This can only be achieved with physicians, given the appropriate tools to understand costs, value, and outcomes and models where the hospitals and physicians are aligned. SUMMARY: The transformation to a value-based healthcare system is beginning and pediatric cardiologists need to be educated, given the appropriate resources, receive appropriate feedback, and patients need to be part of the solution so that care providers can understand what matters most to them.

The 2017 Seventh World Congress of Paediatric Cardiology and Cardiac Surgery: "The Olympics of our Profession".

The 1st World Congress of Paediatric Cardiology was held in London, United Kingdom, in 1980, organised by Dr Jane Somerville and Prof. Fergus Macartney. The idea was that of Jane Somerville, who worked with enormous energy and enthusiasm to bring together paediatric cardiologists and surgeons from around the world. The 2nd World Congress of Paediatric Cardiology took place in New York in 1985, organised by Bill Rashkind, Mary Ellen Engle, and Eugene Doyle. The 3rd World Congress of Paediatric Cardiology was held in Bangkok, Thailand, in 1989, organised by Chompol Vongraprateep. Although cardiac surgeons were heavily involved in these early meetings, a separate World Congress of Paediatric Cardiac Surgery was held in Bergamo, Italy, in 1988, organised by Lucio Parenzan. Thereafter, it was recognised that surgeons and cardiologists working on the same problems and driven by a desire to help children would really rather meet together. A momentous decision was taken to initiate a Joint World Congress of Paediatric Cardiology and Cardiac Surgery. A steering committee was established with membership comprising the main organisers of the four separate previous Congresses and additional members were recruited in an effort to achieve numerical equality of cardiologists and surgeons and a broad geographical representation. The historic 1st "World Congress of Paediatric Cardiology and Cardiac Surgery" took place in Paris in June, 1993, organised by Jean Kachaner. The next was to be held in Japan, but the catastrophic Kobe earthquake in 1995 forced relocation to Hawaii in 1997. Then followed Toronto, Canada, 2001, organised by Bill Williams and Lee Benson; Buenos Aires, Argentina, 2005, organised by Horatio Capelli and Guillermo Kreutzer; Cairns, Australia, 2009, organised by Jim Wilkinson; Cape Town, South Africa, 2013, organised by Christopher Hugo-Hamman; and Barcelona, Spain, 2017, organised by Sertac Cicek. With stops in Europe (1993), Asia-Pacific (1997), North America (2001), South America (2005), Australia (2009), Africa (2013), and Europe again (2017), in 2021, The World Congress of Paediatric Cardiology and Cardiac Surgery will be held for the first time in the continental United States. 1 The 8th World Congress of Paediatric Cardiology and Cardiac Surgery will be held in Washington DC, United States of America, 19-24 September, 2021, and will be organised by Jeffrey P. Jacobs and Gil Wernovsky. Mitchell I. Cohen served as the Scientific Program Co-Chair for the 2017 World Congress of Paediatric Cardiology and Cardiac Surgery, and he will again serve as the Scientific Program Co-Chair for the 2021 World Congress of Paediatric Cardiology and Cardiac Surgery along with Kathyrn Dodds RN, MSN, CRNP. Information about the upcoming 8th World Congress of Paediatric Cardiology and Cardiac Surgery can be found at www.WCPCCS2021.org.

Heart Failure Summit Review: cardiac re-synchronisation therapy in the failing heart.

Extrapolating cardiac resynchronization therapy (CRT) to pediatric patients with heart failure has at times been difficult given the heterogeneity of pediatric cardiomyopathies, varying congenital heart disease (CHD) substrates, and the fact that most pediatric heart failure patients have right bundle branch block (RBBB) as opposed to LBBB. Yet, despite these limitations a number of multi-center retrospective studies in North America and Europe have identified some data to suggest that certain sub-populations tend to respond positively to CRT. In order to address some of the heterogeneity it is helpful to subdivide pediatric and young adult patients with CHD into four potential groups: (1) CRT for chronic RV pacing, (2) dilated cardiomyopathies, (3) pulmonary right ventricles, and (4) systemic right ventricles. The chronic RV paced group, especially long-standing RV apical pacing, with ventricular dyssynchrony has consistently shown to be the group that best responds to a proactive resynchronization course. CRT therapy in pulmonary right ventricles such as post-op tetralogy of Fallot have shown some promise and may be considered especially if there is evidence of concomitant left ventricular dysfunction with an electrical dyssynchrony. Patients with systemic right ventricles such as post-atrial baffle surgery or congenitally corrected transposition reportedly do well with CRT in the presence of both inter-ventricular and intra-ventricular dyssynchrony. There is little doubt that moving forward to best way to identify which pediatric patients with heart failure will respond to CRT, will require a collaborative effort between the electrophysiologist and the echocardiographer to identify appropriate candidates with electrical and mechanical dyssynchrony.

Hybrid procedure as treatment for large obstructive left ventricular rhabdomyoma.

We present one of the first reported cases of a neonate with a prenatal diagnosis of large left ventricular rhabdomyoma obstructing the outflow tract that underwent a hybrid procedure with stenting of the patent duct arteriosus and bilateral pulmonary artery banding.

Controversies, genetics, diagnostic assessment, and outcomes relating to the heterotaxy syndrome.

How best to analyse and describe the features of the situation commonly known as "visceral heterotaxy" remains controversial. Much of the disagreement devolves on how to deal with the concept of isomerism. In the opinion of some, the concept of bilateral right-sidedness and bilateral left-sidedness, while useful in helping to remember which abnormalities are likely to occur in asplenia or polysplenia, should not be granted the status of a specific "situs", since there are numerous examples of exceptions to these patterns. On the other hand, those who favour the concept of isomerism point out that, when describing only the heart, and taking the structure of the atrial appendages as the starting point for analysis, basing this on the extent of the pectinate muscles relative to the atrioventricular junctions, then the only possible arrangements for the appendages are the usual one, its mirror-image, and the two situations in which appendages of comparable morphology are found on both sides of the heart, these being the arrangements of right or left isomerism. It is certainly the case that the arrangement of the organs is not always in harmony with the arrangement of the atrial appendages, but those circumstances, in which there is disharmony, can readily be described by paying specific attention to each series of organs. On this basis, in this review, we describe the approach to heterotaxy, and isomerism of the atrial appendages, in terms of the genetic background, the diagnosis, and outcomes after cardiac surgery. Attention is given to the various diagnostic modalities, including fetal and postnatal echocardiography, recent tomographic and magnetic resonance imaging techniques, and the time-honoured approach using angiography.

Postpericardiotomy syndrome after permanent pacemaker implantation in children and young adults.

BACKGROUND: Postpericardiotomy syndrome (PPS) occurs in 10% to 50% of pediatric patients after cardiac surgery. The incidence and outcome of PPS after permanent pacemaker implantation in children is not described. METHODS: A retrospective analysis was performed of all pediatric patients who underwent isolated placement of a pacemaker between January 1984 and December 2002. Patients who underwent congenital heart surgery at the time of pacemaker implantation were excluded. PPS was diagnosed on the basis of clinical symptoms with echocardiographic confirmation of a pericardial effusion. RESULTS: Four hundred and forty-three pacemakers (237 epicardial, 206 transvenous) were implanted in 370 patients (median age 10 years, range 2 months to 24 years). Eight (2%) episodes of PPS (6 epicardial, 2 transvenous) occurred in 7 patients. The median time from implantation to PPS was 12.5 days (range 8 to 22 days). Six (75%) episodes followed primary pacemaker implantation, two occurred after subsequent lead revision. Three patients were initially treated with medical therapy (1 nonsteroidal agents, 2 steroids), and 1 required subsequent pericardiocentesis. Five patients underwent initial pericardiocentesis followed by medication. One patient had echocardiographic recurrence of a pericardial effusion 3 weeks after a nonsteroidal taper, with resolution after nonsteroidal agents were reinitiated. One patient required a pericardial window for a persistent effusion. No pacemaker was explanted. CONCLUSIONS: PPS occurred in 2% of children undergoing isolated pacemaker implantation of both epicardial and transvenous systems. PPS is usually managed successfully with medical therapy. Patients with medical treatment failure were successfully treated with pericardiocentesis or the surgical creation of a pericardial window.