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.

The WHF Roadmap for Reducing CV Morbidity and Mortality Through Prevention and Control of RHD.

Rheumatic heart disease (RHD) is a preventable non-communicable condition that disproportionately affects the world's poorest and most vulnerable. The World Heart Federation Roadmap for improved RHD control is a resource designed to help a variety of stakeholders raise the profile of RHD nationally and globally, and provide a framework to guide and support the strengthening of national, regional and global RHD control efforts. The Roadmap identifies the barriers that limit access to and uptake of proven interventions for the prevention and control of RHD. It also highlights a variety of established and promising solutions that may be used to overcome these barriers. As a general guide, the Roadmap is meant to serve as the foundation for the development of tailored plans of action to improve RHD control in specific contexts.

Lutembacher syndrome variant: Rheumatic heart disease involving all four valves and associated with an atrial septal defect in a child.

Rheumatic heart disease (RHD) is the most common cause of acquired heart disease in children and young adults in developing countries. It results from throat infection with group A beta hemolytic streptococcus that proceeds to acute rheumatic fever (ARF). We report a 13 years old girl from Darfur presenting with recurrent acute rheumatic fever for 4 years that led to affection of all her heart valves with severe mitral and tricuspid regurgitation together with moderate pulmonary and mild aortic valve regurgitation. There was an associated atrial septal defect (Lutembacher syndrome variant). The disease was severe and led to cardiogenic shock and death while awaiting surgery. The case highlights the impact of RHD on young people and the need to implement control programs for RHD in Sudan.

Rationale and design of the African group A streptococcal infection registry: the AFROStrep study.

INTRODUCTION: Group A ß-haemolytic Streptococcus (GAS), a Gram-positive bacterium, also known as Streptococcus pyogenes, causes pyoderma, pharyngitis and invasive disease. Repeated GAS infections may lead to autoimmune diseases such as acute post-streptococcal glomerulonephritis, acute rheumatic fever (ARF) and rheumatic heart disease (RHD). Invasive GAS (iGAS) disease is an important cause of mortality and morbidity worldwide. The burden of GAS infections is, however, unknown in Africa because of lack of surveillance systems. METHODS AND ANALYSIS: The African group A streptococcal infection registry (the AFROStrep study) is a collaborative multicentre study of clinical, microbiological, epidemiological and molecular characteristics for GAS infection in Africa. The AFROStrep registry comprises two components: (1) active surveillance of GAS pharyngitis cases from sentinel primary care centres (non-iGAS) and (2) passive surveillance of iGAS disease from microbiology laboratories. Isolates will also be subjected to DNA isolation to allow for characterisation by molecular methods and cryopreservation for long-term storage. The AFROStrep study seeks to collect comprehensive data on GAS isolates in Africa. The biorepository will serve as a platform for vaccine development in Africa. ETHICS AND DISSEMINATION: Ethics approval for the AFROStrep registry has been obtained from the Human Research Ethics Committee at the University of Cape Town (HREC/REF: R006/2015). Each recruiting site will seek ethics approval from their local ethics' committee. All participants will be required to provide consent for inclusion into the registry as well as for the storage of isolates and molecular investigations to be conducted thereon. Strict confidentiality will be applied throughout. Findings and updates will be disseminated to collaborators, researchers, health planners and colleagues through peer-reviewed journal articles, conference publications and proceedings.

Noncompaction cardiomyopathy: A new mechanism for mitral regurgitation with distinct clinical, echocardiographic features and pathological correlations.

UNLABELLED: Noncompaction cardiomyopathy (NCCM) is a primary, genetic cardiomyopathy with variable clinical manifestations that include mitral regurgitation (MR). METHODS: This study comprised patients diagnosed with NCCM and MR in two cardiac centers (King Abdul-Aziz Cardiac Center, Riyadh, Kingdom of Saudi Arabia and Sudan Heart Institute, Khartoum, Sudan), and seen in the period between 2002 and 2013. The study describes follow up, clinical, echocardiographic, and histopathological findings. RESULTS: Nineteen cases (85% females) were identified. Ten percent of the cases had relapses and remissions of heart failure. Echocardiographic features included leaflet retraction in all patients, characteristic malcoaptation, and a zigzag deformity of anterior leaflet in 57% of patients. Ruptured chordae were found in 15% of the patients. One patient had pathological examination of the mitral valve which showed myxomatous degeneration, and sclerotic and calcific changes. CONCLUSION: We describe and discuss a new mechanism for MR caused by NCCM with identifiable clinical and echocardiographic features, and pathological correlations.

Evaluation of physicians' knowledge about prevention of rheumatic fever and rheumatic heart disease before and after a teaching session.

Rheumatic fever (RF) and rheumatic heart disease RHD remain as one of the major cardiovascular problems in Sudanese children. The cornerstones for control of RF and RHD are primary and secondary preventions as adopted by Sudan's programme. This study aimed to describe and raise the paediatric doctors' awareness about prevention of RF and RHD using lectures. It was a prospective, cross-sectional, hospital based study, conducted in Khartoum. The study populations were paediatric doctors including house officers, medical officers and registrars. Data were collected through self-administered questionnaire, which was constructed to assess the doctor's awareness about RF and RHD prevention before and after attending lectures. Eighty seven doctors participated in the study. The results showed that the overall doctors' awareness about prevention of rheumatic fever and rheumatic heart disease was at average level. It was raised by intervention through lectures to good level. It is recommended to introduce training programs for physicians in order to improve doctors' awareness about prevention of RF and RHD. Such activities need to be conducted at regular intervals.

Isolated subpulmonic fibrous ring, mirror-image dextrocardia and situs solitus in a young lady unreported and a near miss.

Congenital diseases causing obstruction of the right ventricular outflow tract (RVOT) are common, but the isolated subpulmonary membrane/ring is extremely rare and can be difficult to diagnose precisely, especially in adults. We report a case of surgically resected isolated subpulmonic fibrous ring in a lady with mirror-image dextrocardia and abdominal situs solitus that was misdiagnosed by echocardiography as a subaortic membrane.

Endomyocardial fibrosis: an under-diagnosed cause of cardiomyopathy in Sudanese children.

Endomyocardial fibrosis (EMF) is a tropical cardiomyopathy reported in many tropical countries. Patients were seen at the Children's Hospital-Khartoum, Sudan during September 2007-08 where cardiac evaluation was done. Six patients were identified (18% of all children with cardiomyopathy); all were males aged 8-17 years. All patients presented with abdominal distention, stunted growth, raised jugular venous pressure, ascites and hepatosplenomegaly. Electrocardiogram abnormalities included atrial fibrillation, first and third degree AV block and tall P wave. Echocardiography revealed huge right atrium dilatation and right ventricle apex obliteration by fibrous tissue. One patient had left-side affection with moderate mitral regurgitation. Eosinophilia was present in three patients. Two patients received antituberculosis drugs with no improvement, two had history of treated bilharziasis and two had been labeled as having Ebstein disease. EMF is an important cause of cardiomyopathy in Sudan that is often misdiagnosed. Clinical and echocardiographic examinations can lead to the correct diagnosis.

Ventricular noncompaction: Over or under diagnosis?

Ventricular noncompaction (VNC) describes a cardiomyopathy characterized by excessive myocardial trabeculations and deep intertrabecular recesses. Detection rates are increasingly rising and the frequency of NVC has changed from a disease that is thought to be rare to that described as 'relatively common'. However, there had been a number of un-answered questions regarding the clinical and echocardiographic features of VNC. In this review we discuss the frequency of VNC and emphasize its distinctive clinical features and associations.

Unique features of non-compaction of the ventricular myocardium in Arab and African patients.

Non-compaction of the ventricular myocardium (NCVM) is an under-diagnosed cardiomyopathy. Patients diagnosed with NCVM at the King Abdulaziz Cardiac Centre, Riyadh, KSA from January 2000 to July 2004 and at the Sudan Heart Centre from August 2004 to July 2007 were included. Fifty-two patients with NCVM were identified (22 per 10 000 echocardiograms). Patients were divided into three groups, namely, group 1: isolated NCVM (21 patients), group 2: NCVM associated with congenital heart disease (CHD) (26 patients), and group 3: NCVM associated with mitral regurgitation (MR) (seven patients). Group 1 included 14 females and four males. Five patients (27%) had a positive family history with a lethal outcome in five other siblings; 14 patients (76%) presented with myocardial dysfunction and two had left ventricle thrombus. Group 2 included CHD; the most common pathologies were ventricular septal defects (VSD), pulmonary and tricuspid atresia and hypoplastic left heart syndrome. Sixteen patients (61%) had myocardial dysfunction, seven had surgical repair/palliation, and four (80%) developed serious post-operative complications. Group 3 included seven patients with MR associated with deformity of the anterior mitral leaflet and malcoaptation. Myocardial function was preserved in all patients with this pathology. In four patients of the whole cohort there was clinical as well as echocardiographic improvement. In two patients, left ventricular hypertrophy was noted. There were significantly more females in the group with isolated NCVM than in the group with associated CHD (p = 0.03, odds ratio = 4.2, 95% CI = 0.529-16.1). We presented the largest series of NCVM in our area and found it to be not as rare as was thought, with females being more affected. Spontaneous improvement and left ventricular hypertrophy were unique features, and mitral valve deformity leading to MR was an established association.

Clinical and echocardiographic features of Ebstein's malformation in Sudanese patients.

UNLABELLED: Ebstein's malformation is a rare congenital cardiac malformation, accounting for about 0.5% of all congenital cardiac lesions. We report our experience with the anomaly as encountered at the Sudan Heart Centre from July 2004 to April 2005. Diagnosis was based on the echocardiographic demonstration of displacement of the septal leaflet of the tricuspid valve towards the ventricular apex by greater than 8 millimetres per metre squared. RESULTS: In a period of 10 months, we identified 12 patients with the malformation, this number constituting 2% of all patients seen with congenital cardiac disease. The age ranged from 2 weeks to 35 years. Of the patients, half were asymptomatic, while the other half presented with congestive cardiac failure, 4 of these having cyanosis in addition to heart failure. Associated diseases included chronic renal failure, impaired hearing, stunted growth, and developmental delay. Electrocardiographic abnormalities included peaked and tall P waves, seen in four-fifths, an Rsr pattern, first degree atrioventricular block, atrial fibrillation, and Wolf-Parkinson-White syndrome, including the Mahaim pattern of pre-excitation. Associated abnormalities diagnosed echocardiographically included atrial septal defects, prolapse of the leaflets of the mitral valve, left ventricular dysfunction, atrial septal aneurysm, pulmonary valvar stenosis, and pericardial effusion. CONCLUSIONS: Ebstein's malformation was seen four times more frequently at the Sudan Heart Centre when compared to the average frequency reported in the Western literature. Many of the associated diseases encountered in Sudan had not previously been reported.

Renal calculi complicating short-term furosemide therapy after congenital heart surgery.

BACKGROUND: Renal calcification is a known complication of Furosemide therapy. METHODS: We describe 3 children who were diagnosed with renal calculi, not nephrocalcinosis, following the use of Furosemide for 4 weeks. All the infants (24, 18, and 8 months) had successful repair/palliation of congenital heart disease. The dose of Furosemide was 1-2 mg/kg/day and the duration between starting treatment and development of hematuria was 4 weeks. RESULTS: In all 3 patients renal ultrasound confirmed the presence of renal calculi with no nephrocalcinosis. On follow-up, hematuria improved after 3-4 months. CONCLUSION: Renal calculi can complicate short-term Furosemide treatment in children after repair of congenital heart disease. Patients on Furosemide should be closely monitored by urine analysis and/or urine calcium/creatinine ratio for early detection of this complication.

The variable clinical presentation of, and outcome for, noncompaction of the ventricular myocardium in infants and children, an under-diagnosed cardiomyopathy.

Noncompaction of the ventricular myocardium is increasingly recognized as an important cause of cardiomyopathy. Its echocardiographic definition, however, is not yet clearly refined, and differentiation from other conditions with hypertrabeculation can be difficult. We report a prospective short-term follow-up of 15 children with noncompaction, excluding those with associated complex congenital cardiac disease. The clinical presentation and outcome were variable, with 2 patients being asymptomatic. For 5 patients, presentation was with cardiac failure due to depressed myocardial function. The function deteriorated in two, remained the same in two, and improved in the other patient. Cardiac failure due to mitral regurgitation was the mode of presentation in 2 patients with preserved myocardial function, one of whom needed replacement of the mitral valve. In 6 patients (40%), symptoms of cardiac failure were due to noncomplex congenital cardiac disease. All of them had ventricular septal defects. In addition, two had cleft mitral valves, and one had a large persistently patent arterial duct. The diagnosis of noncompaction was initially missed on more than one echocardiographic study in one-third of our patients. We conclude that noncompaction is under-diagnosed, and is not as rare as is thought. In children, it is often associated with other cardiac lesions that can cause cardiac failure in the presence of preserved myocardial function.