Objective: At present, the haemodynamic diagnosis of orthostatic intolerance is based mainly on the head-up tilt table test, which is sometimes risky for patients. Thus, it is important to find objective and safe methods to differentiate haemodynamic patterns of orthostatic intolerance cases. Methods: In all, 629 children with orthostatic intolerance, either vasovagal syncope or postural orthostatic tachycardia syndrome, were included in the multi-centre clinical study. We analysed the association between the clinical manifestation and haemodynamic patterns of the patients. Results: Syncope after motion with a prodrome of chest distress or palpitations and the concomitant symptom(s) after a syncopal attack, with debilitation, dizziness or headache, were the most important variables in predicting the diagnosis of vasovagal syncope. The overall diagnostic accuracy was 71.5%. Conclusion: Complaint of syncope after motion with prodromal chest distress or palpitation and the concomitant symptom after a syncopal attack, with subsequent debilitation, dizziness or headache, were the most important variables in the diagnosis of vasovagal syncope in children with orthostatic intolerance.

Aim: To investigate the previously unknown birth incidence, treatment, and mortality of children with congenital heart disease in Tunisia. Methods: We undertook a retrospective review of medical records of all patients who were born in 2010 and 2011, and were diagnosed in Sfax (Tunisia) with congenital heart defect. Results: Among 37,294 births, 255 children were detected to have congenital heart disease, yielding a birth incidence of 6.8 per 1000. The most frequently occurring conditions were ventricular septal defects (31%), ostium secundum atrial septal defects (12.9%), and pulmonary valve abnormalities (12%). Coarctation of the aorta, tetralogy of Fallot, univentricular physiology, pulmonary atresia with ventricular septal defect, and transposition of the great arteries were found in 4.3%, 6.2%, 3.4%, 2.7%, and 2.7%, respectively. During the follow-up of 1 year, 23% of the children died. About three-quarters of those deaths happened before surgery. Conclusion: The present study is in line with the general estimates in the world. It has revealed a high case of mortality among the patients awaiting corrective surgery. These children need more facilities.

Background: Myocardial injury in previously healthy children is rare, with a wide range of aetiologies. It is increasingly being identified on the basis of elevated troponin levels during routine evaluation of cardiorespiratory symptoms. Establishing the aetiology remains challenging because of the lack of an accepted work-up algorithm. Our objective was to delineate the contribution of diagnostic modalities and troponin patterns towards the final diagnosis. Methods: A retrospective chart review of previously healthy patients admitted to the Pediatric Cardiology Service with myocardial injury was carried out. Data analysed included echocardiograms, electrocardiograms, cardiac catheterisations, magnetic resonance imaging, drug screen tests, troponin values, and final diagnosis. Results: A total of 32 patients were identified. The diagnoses were: myocarditis in 16 patients, vasospasm due to drug use in seven, myopericarditis in six, anomalous coronary artery origins in two, and Prinzmetal's angina in one patient. The electrocardiograms were abnormal in 27 of the 32 patients (84%), echocardiograms in 18 of the 32 patients (56%), cardiac magnetic resonance imaging in two of the four patients (50%), urine drug screen in five of the 25 patients (20%), and cardiac catheterisations in two of the 15 patients (13%). Conclusions: Myocarditis is the most common aetiology of myocardial injury in children. Clinical history remains the basic screening tool; drug screens help identify coronary vasospasms secondary to drug use (22% of our cohort). Patients with anomalous coronaries had exertional symptoms. Initial troponin levels and progression were not diagnostic or prognostic. Catheterisation is of limited value and did not change management. Magnetic resonance imaging with gadolinium enhancement is probably the most useful test when initial evaluation is not diagnostic.

Background: The aim of this study was to evaluate our experience in central extracorporeal life support with an integrated left ventricular vent in children with cardiac failure. Methods: Eight children acquired extracorporeal life support with a left ventricular vent, either after cardiac surgery (n = 4) or during an acute cardiac illness (n = 4). The ascending aorta and right atrium were cannulated. The left ventricular vent was inserted through the right superior pulmonary vein and connected to the venous line on the extracorporeal life support such that active left heart decompression was achieved. Results: No patient died while on support, seven patients were successfully weaned from it and one patient was transitioned to a biventricular assist device. The median length of support was 6 days (range 5–10 days). One patient died while in the hospital, despite successful weaning from extracorporeal life support. No intra-cardiac thrombus or embolic stroke was observed. No patient developed relevant intracranial bleeding resulting in neurological dysfunction during and after extracorporeal life support. Conclusions: In case of a low cardiac output and an insufficient inter-atrial shunt, additional left ventricular decompression via a vent could help avoid left heart distension and might promote myocardial recovery. In pulmonary dysfunction, separate blood gas analyses from the venous cannula and the left ventricular vent help detect possible coronary hypoxia when the left ventricle begins to recover. We recommend the use of central extracorporeal life support with an integrated left ventricular vent in children with intractable cardiac failure.

Transcatheter pulmonary valve insertion is the most important advance in congenital interventional cardiology since atrial septal defect devices became commonly available 15 years ago. It has changed the way we look at a number of diverse diagnoses and changes how we plan, diagnose, operate, and follow-up patients. It has changed how we counsel families expecting a child that may benefit from it. Expanded use of the Melody® valve, outside its United States Food and Drug Administration approved indications, has helped numerous additional patients. The use of transcatheter pulmonary valve insertion in selected patients following surgical Gore-tex® bileaflet in valve right ventricular outflow tract reconstruction and those with a history of prior small homograft conduits will be discussed.

Hypertension is a risk factor in many cardiovascular or cerebrovascular diseases. Adolescent hypertension may develop into adult hypertension. Examining the aetiologic factors of adolescent hypertension is crucial in its prevention and in managing its possible complications. The predominant view of aetiologic factors includes heredity, overweight/obesity, insulin resistance, gestational hypertension, diet, and intrauterine growth retardation. This article reported that children's academic grades in schools were positively correlated with adolescent blood pressure.

Objective: During the course of acute rheumatic fever, some electrocardiographic changes are seen. First-degree atrioventricular block is the most common electrocardiographic abnormality. Second- and third-degree atrioventricular block, ventricular tachycardia, and junctional acceleration are also seen. In the present study, the specificity of accelerated junctional rhythm to acute rheumatic fever was investigated. Methods: The study included patients with acute rheumatic fever (Group 1), healthy children who had suffered from recent group A β-haemolytic streptococcal upper respiratory tract infection but did not develop acute rheumatic fever (Group 2), and patients who had other diseases that may affect the joints and/or heart (Group 3). Results: Accelerated junctional rhythm was detected in 10 patients in Group 1, but in none of the patients from Group 2 or 3. Specificity of accelerated junctional rhythm for acute rheumatic fever was 100% and the positive predictive value was 100%. Conclusion: Accelerated junctional rhythm is specific to acute rheumatic fever. Although its frequency is low, it seems that it can be used in the differential diagnosis of acute rheumatic fever, especially in patients with isolated polyarthritis.

Pulmonary valve replacement in patients with congenital cardiac disease is now being performed with more liberal indications in light of the data that chronic pulmonary insufficiency is not a benign lesion. The beneficial effects of valve replacement with low operative mortality and morbidity support this approach. Many options exist for a pulmonary valve prosthesis, which underscores the fact that there is no ideal valve available. Our efforts are focussed around a synthetic valve that avoids the bio-degeneration of a bio-prosthesis and avoids the need for life-long coumadin. We developed a bicuspid (bileaflet) polytetrafluoroethylene valve design, which has now gone through three major revisions in >200 patients over 14 years. We began the experience utilising a polytetrafluoroethylene hand-sewn bicuspid valve in the right ventricular outflow tract, initially using 0.6 mm and more recently 0.1 mm polytetrafluoroethylene. The 0.1 mm thickness material functions well as a leaflet, maintaining a relatively thin and flexible nature. It does not calcify or initiate thromboses at least for the first several years. We identified issues with dehiscence of the leaflet from the right ventricular outflow tract muscle, especially in the larger, potentially expansive right ventricular outflow tracts, and this prompted our latest design change to place the valve within a polytetrafluoroethylene tube. This current version of the polytetrafluoroethylene valve conduit has excellent short-to-intermediate-term function. Further follow-up is necessary to determine late durability and life-long valve-related procedural risk for our patients.

Objective: Despite the broadened indications for Fontan procedure, there are patients who could not proceed to Fontan procedure because of the strict Fontan criteria during the early period. Some patients suffer from post-Glenn complications such as hypoxia, arrhythmia, or fatigue with exertion long after the Glenn procedure. We explored the possibility of Fontan completion for those patients. Methods: Between 2004 and 2010, five consecutive patients aged between 13 and 31 years (median 21) underwent Fontan completion. These patients had been followed up for more than 10 years (10 to 13, median 11) after Glenn procedure as non-Fontan candidates. We summarise these patients retrospectively in terms of their pre-operative physiological condition, surgical strategy, and problems that these patients hold. Results: Pre-operative catheterisation showed pulmonary vascular resistance ranging from 0.9 to 3.7 (median 2.2), pulmonary to systemic flow ratio of 0.3 to 1.6 (median 0.9), and two patients had significant aortopulmonary collaterals. Extracardiac total cavopulmonary connections were performed in three patients, lateral tunnel total cavopulmonary connection in one patient, and intracardiac total cavopulmonary connection in one patient, without a surgical fenestration. Concomitant surgeries were required including valve surgeries – atrioventricular valve plasty in three patients and tricuspid valve replacement in one patient; systemic outflow tract obstruction release – Damus–Kaye–Stansel procedure in two patients and subaortic stenosis resection in one patient; and anti-arrhythmic therapies – maze procedure in two patients, cryoablation in two patients, and pacemaker implantation in two patients. All patients are now in New York Heart Association category I. Conclusion: Patients often suffer from post-Glenn complications. Of those, if they are re-examined carefully, some may have a chance to undergo Fontan completion and benefit from it. Multiple lesions such as atrioventricular valve regurgitation, systemic outflow obstruction, or arrhythmia should be surgically repaired concomitantly.

Objective: To evaluate heart rate variability by Holter monitoring in type 1 diabetic children compared with a healthy control group and determine the factors modifying heart rate variability. Methods: This was designed as a prospective study comparing 28 patients, diagnosed with type 1 diabetes and under follow-up, with 27 healthy control group subjects. Results: The patients were aged 9.9 ± 4.2 years in the diabetic group, including 13 (46.5%) girls and 15 (53.5%) boys. The healthy control group comprised 20 (74%) girls and seven boys (26%) with an average age of 8.6 ± 3.7 years. The search for factors modifying heart rate variability yielded the following correlations: for the time-dependent variables, negative between age and both average and maximal heart rate (r = −0.263 and −0.460, respectively), negative between haemoglobin A1c and percentage of differences between adjacent RR intervals >50 ms, positive between diabetes duration and square root of the mean of the sum of squares of differences between adjacent NN intervals. The average heart rate and percentage of differences between adjacent RR intervals >50 ms was significantly higher in the girls than the boys in all groups. With regard to the frequency-dependent factors affecting heart rate variability, correlations were found between haemoglobin A1c level and both total power and very low frequency (r = −0.751 and −0.644) and between very low frequency and diabetes duration. Conclusion: A reduction in heart rate variability parameters was observed in type 1 diabetes mellitus patients who had a long disease duration or were poorly controlled, as compared with healthy controls.

Purpose: The aim of this study was to compare associations between generic versus disease-specific functional health status assessments and patient and clinical characteristics for patients with severe congenital heart disease. Methods: This was a cross-sectional observational study involving 325 single ventricle patients, aged 10–18 years, after Fontan procedure. Enrolled patients underwent a medical history review, laboratory testing, and assessment of the functional health status by completion of the generic Child Report Child Health Questionnaire and the disease-specific Congenital Heart Adolescent and Teenage questionnaire. Correlated conceptually equivalent domains from both questionnaires were identified and their associations with patient and clinical variables were compared. Results: From the generic assessment, patients perceived marginally lower physical functioning (p = 0.05) but greater freedom from bodily pain compared with a normal population (p < 0.001). The equivalent physical functioning/limitations domain of the generic instrument, compared with the disease-specific instrument, had similar associations (higher multi-variable model R2) with medical history variables (R2 = 0.14 versus R2 = 0.12, respectively) and stronger associations with exercise testing variables (R2 = 0.22 versus R2 = 0.06). Similarly, the corresponding freedom from bodily pain/symptoms domains from both questionnaires showed a greater association for the generic instrument with medical history variables (R2 = 0.15 versus R2 = 0.09, respectively) and non-cardiac conditions (R2 = 0.13 versus R2 = 0.06). The associations of each questionnaire with echocardiographic results, cardiac magnetic resonance imaging results, and serum brain natriuretic peptide levels were uniformly weak (R2 range <0.01 to 0.04). Conclusions: Assessment of the physical functional health status using generic and disease-specific instruments yields few differences with regard to associations between conceptually similar domains and patient and clinical characteristics for adolescents after Fontan procedure.

Aim: This study aimed to compare the effects of dexmedetomidine–propofol and ketamine–propofol sedation on haemodynamic stability, immobility, and recovery time in children who underwent transcatheter closure of atrial septal defects. Methods: In all, 46 children scheduled for transcatheter closure of atrial septal defects (n = 46) were included. The dexmedetomidine–propofol group (n = 23) received dexmedetomidine (1 μg/kg) and propofol (1 mg/kg) for induction, followed by dexmedetomidine (0.5 μg/kg/hour) and propofol (100 μg/kg/minute) for maintenance. The ketamine–propofol group (n = 23) received ketamine (1 mg/kg) and propofol (1 mg/kg) for induction, followed by ketamine (1 mg/kg) and propofol (100 μg/kg/minute) for maintenance. Results: In all, 11 patients in the dexmedetomidine group (47.8%) and one patient (4.3%) in the ketamine group demonstrated a decrease ≥20% from the baseline in mean arterial pressure (p = 0.01). Heart rates decreased ≥20% from the baseline value in 10 patients (43.4%) in the dexmedetomidine group and three patients (13%) in the ketamine group (p = 0.047). Heart rate values were observed to be lower in the dexmedetomidine group throughout the procedure after the first 10 minutes. The number of patients requiring additional propofol was higher in the dexmedetomidine group (p = 0.01). The recovery times were similar in the two groups – 15.86 ± 6.50 minutes in the dexmedetomidine group and 19.65 ± 8.19 minutes in the ketamine group; p = 0.09. Conclusion: The ketamine–propofol combination was less likely to induce haemodynamic instability, with no significant change in recovery times, compared with the dexmedetomidine–propofol combination. The ketamine–propofol combination provided good conditions for the intervention.

Objective: Medline classifies publications as clinical trials, randomised control trials, meta-analyses, practice guidelines, reviews, case reports, editorials, and letters. We tested the hypothesis that cardiology-related publications have increased with a shift in the type of publications over the past 10 years by age category. Methods: To retrieve from Medline the cardiology articles, we used the keyword “heart disease”, but limited the search to articles in English from 2000 to 2009. We repeated the search using one limit according to the publication type and using age tags. We used regression analysis to determine the effect of the year of publication on the number of publications of each type. Results: During the 10-year period, Medline registered 152,849 cardiology articles, doubling from 10,452 in 2000 to 20,841 in 2009, of which 8.5% were tagged as both paediatric and adult. There was a linear increase in the number over the study period in the total number of publications and in all categories, except for practice guidelines. There was almost a twofold increase in adult and neonatal articles, but ∼70% in paediatric articles. The rate of increase was 66% for randomised control trials, 73% for clinical trials, 124% for meta-analyses, 117% for editorials, 36% for reviews, and 103% for case reports. Practice guidelines remained very low, increasing significantly for paediatric and neonatal articles. Conclusions: There was a substantial increase in cardiology articles over the past 10 years, being greater for adult and neonatal articles compared with paediatric articles. The increase varied according to the type of article.

Isolation of the subclavian artery is associated with “steal” of blood from the carotid circulation to the arm, through the circle of Willis and the vertebral artery. When associated with a patent arterial duct, there is an additional “steal” of blood from the arm to the lungs, through the arterial duct because of the lower pulmonary vascular resistance. When this combination manifests clinically with arm ischaemia on the side of the isolated subclavian artery, closure of the arterial duct will prevent the “steal” of blood from the subclavian artery to the pulmonary artery and may improve the blood flow to the arm. We report three patients with this unusual combination of the “steal” phenomenon that improved after interventional closure of the arterial duct. This report discusses the embryological basis of the defect, clinical and echocardiographic clues to diagnose this unusual anomaly, angiographic findings, and transcatheter management options.

This manuscript presents a technically straightforward technique to allow for mitral valve repair in the patient with rheumatic mitral stenosis. This non-resection technique allows for the correction of both mitral stenosis and regurgitation without requiring complex subvalvar procedures and eliminates the concerns for postoperative systolic anterior motion. The authors feel this three-part technique of bi-commissural release, anterior leaflet augmentation, and oversized annuloplasty may allow for a more reproducible approach to repair of the rheumatic mitral valve.

Objectives: Our aim was to evaluate patients who were treated by percutaneous stent implantation. Methods: Patients with aortic coarctation (n = 35) who had been treated with 38 stents – 12 bare and 26 covered – were evaluated. The demographics and procedural and follow-up data were recorded from hospital registers and compared according to patient specifications, for example, weight and coarctation nature. Results: The procedure was successful in all patients. There was a statistically significant difference between the patients with native coarctation (n = 17) and those with recurrent coarctation (n = 18) in terms of pre-procedural blood pressures, systolic gradients, coarctation diameters, and the ratio of the coarctation site diameter to the descending aorta diameter. Although all patients received antihypertensive drugs before the procedure, the drug was discontinued in 26 patients during follow-up (p < 0.001). Stent migration was observed in four patients with recurrent coarctation (11.4%), and peripheral arterial injury was seen in three patients (8.5%). The mean follow-up time was 34 ± 16 months. On average, 21 (6–42) months after the procedure, six patients underwent cardiac catheterisation. At least 2 years after the procedure, tomography was performed in 20 patients (57.2%). Patients who were evaluated by multi-slice computerised tomography revealed no pathologies. There was no statistically significant difference between the five patients weighing less than 20 kg and the other 30 patients in terms of demographic and procedural characteristics, procedure success and complication rates, and follow-up data. Conclusion: Stent implantation for aortic coarctation is a method yielding satisfactory results in reducing coarctation gradients, efficient enlargement of the lesion area, and resolution of hypertension for children, including those weighing less than 20 kg.

Between January, 2009 and December, 2013, 84 patients were identified who underwent isolated mitral valve surgery in Jamaica at The University Hospital of the West Indies and The Bustamante Hospital for Children. The most common pathology requiring surgery was rheumatic heart disease, accounting for 84% of the procedures performed. The majority of patients had regurgitation of the mitral valve (67%), stenosis of the mitral valve (22%), and mixed mitral valve disease (11%). The most common procedure performed was replacement of the mitral valve (69%), followed by mitral valve repair (29%). Among the patients, one underwent closed mitral commissurotomy. The choice of procedure differed between age groups. In the paediatric population (<18 years of age), the majority of patients underwent repair of the mitral valve (89%). In the adult population (18 years and above), the majority of patients underwent mitral valve replacement (93%). Overall, of all the patients undergoing replacement of the mitral valve, 89% received a mechanical valve prosthesis, whereas 11% received a bioprosthetic valve prosthesis. Of the group of patients who underwent mitral valve repair for rheumatic heart disease, 19% required re-operation. The average time between initial surgery and re-operation was 1.2 years. Rheumatic fever and rheumatic heart disease remain significant public health challenges in Jamaica and other developing countries. Focus must remain on primary and secondary prevention strategies in order to limit the burden of rheumatic valvulopathies. Attention should also be directed towards improving access to surgical treatment for young adults.