Introduction: The diagnosis of acute rheumatic fever (RF) is based on clinical findings. However, during the chronic phase of the disease, the clinical approach is not sufficient for the follow-up of the patients and the Doppler echocardiography is a tool for the diagnosis of cardiac involvement. Prognostic variables that influence long-term outcomes are not well known. Methods: 462 patients with RF according to Jones criteria were studied, and followed-up from the initial attack to 13.6 ± 4.6 years. All patients underwent clinical assessment and Doppler echocardiography for the detection of heart valve involvement in the acute and chronic phases. Multivariate logistic regression analysis was used to identify the factors influencing long-term heart valve disease. Results: Carditis occurred in 55.8% and subclinical valvulitis in 35.3% patients. In the chronic phase, 33% of the patients had significant valvular heart disease. No normal Doppler echocardiography exam was observed on patients who had severe valvulitis, although heart auscultation had become normal in 13% of these. In the multivariate analysis, only the severity of carditis and the mitral and/or aortic valvulitis were associated with significant valvular heart disease. Chorea or arthritis were protective factors for significant valvular heart disease, odds ratio 0.41 (95% C.I. 0.22 - 0.77) and 0.43 (95% C.I. 0.23 - 0.82), respectively. Conclusions: Our study suggests that the use of Doppler echocardiography during RF helps to identify prognostic factors regarding the development of significant valvular heart disease. Initial severe carditis is an important factor in the long-term prognosis of chronic RHD, whereas arthritis and chore during the initial episode of RF appears to be protective. Strict secondary prophylaxis should be mandatory in high risk patients.

Introduction: Coronary artery involvement is the most dreaded long-term complication of Kawasaki disease. Our aim was to look at the pattern of cardiovascular involvement in Pakistani children admitted with Kawasaki disease. Methods: This study included children admitted with Kawasaki disease at the Aga Khan University Hospital Karachi over a period of 14 years from January 1997 to December 2010. Information gathered included patient demographics, clinical features, investigations, echocardiographic findings, treatment and follow-up. Those with coronary artery involvement on initial echocardiogram remained on long-term follow-up with clinical examination and echocardiogram. Results: A total of 56 patients were admitted. (Mean age at diagnosis 33 ± 30 months, age range 2 months to 9 years). 18% of patients had incomplete features. Twenty-five percent (14/56) patients presented after 10 days of fever. Cardiac examination was normal except for tachycardia. Abnormal coronary arteries were seen in 23 patients (41%) - left main coronary artery in 23 (41%), left anterior descending and right main coronary artery in 20 (36%), circumflex branch in 17 (30%). Risk factors for cardiac involvement were male sex, fever >10 days duration at the time of initial presentation and neutrophil percentage >75% in the initial white blood cell counts. Fifty four of 56 cases received intravenous immunoglobulin (IVIG), Seventy-five percent of the patients received IVIG within 10 days of illness. Mean duration of follow-up was 2.5 years. Eight percent of the patients still continue to have abnormal coronaries. There was no mortality. Conclusions: A higher incidence of coronary artery involvement was found in our study. Presentation after 10 days of illness increases the risk of coronary artery involvement. High index of suspicion among the general pediatricians about the disease can possibly be helpful for early referral and treatment.

Objective: To assess the efficacy and safety of transcatheter closure (TCC) of patent ductus arteriosus (PDA) with severe pulmonary arterial hypertension (PHT). Background: TCC of small and moderate-sized PDAs is well established. However, there is a paucity of data on TCC of large PDA with severe PHT. Methods: This is a retrospective observational study of 76 patients with large PDA and severe PHT who were referred for TCC. Multiple clinical and investigational parameters were evaluated to decide the reversibility of PHT. Following the TCC, patients were serially followed up to assess the efficacy and safety of closure and its impact on PHT. Results: Of 76 patients, nine were found to have PDA with Eisenmenger's syndrome. Of remaining 67, two were thought to have irreversible PHT based on hemodynamic data obtained after balloon occlusion of the duct. Sixty five patients, who eventually underwent TCC of PDA, had a median age of 9.1 years (range 1 month to 40 years). The weight ranged between 2.5 to 62 kg (median 14 kg). The PDA size was 9.1 ± 4.6 mm. The mean systolic pulmonary artery pressure was 66.9 ± 15.3 mm Hg. Duct occluder was used in 63 and muscular ventricular septal defect closure device in 2. The follow up was available in 56 (86%) with a mean follow up period of 65 ± 34 months. All the patients had complete closure of the PDA at 6 months follow up. Mild obstruction of left pulmonary artery (n=3) and aortic isthmus flow (n=6) was noted at the time of discharge. During the follow up, partial or complete resolution of PHT was observed in all the patients in whom Doppler-derived right ventricular systolic pressure was recorded (available in 40 of 56 patients). Conclusions: TCC of large PDA with severe PHT and significant left to right shunt was found to be effective and safe in the short and intermediate term.

Background: In selected neonates and infants, primary palliative stent implantation may be indicated for coarctation of the aorta. We describe our experience with this approach in five consecutive patients. Methods: Five neonates and infants (age range 6 to 68 days, gestation 33 to 38 weeks, weight range at procedure of between 1650 to 4000 g) underwent palliative stent implantation as primary therapy for coarctation of the aorta. Indications for primary stent implantation were varied. All procedures were performed by elective surgical cut down of the axillary artery. Standard coronary stents (diameter 4.5 to 5 mm, length 12 to 16 mm) were delivered via a 4F sheath. The axillary artery was repaired after removal of the sheath. Results: All procedures were acutely successful, and without procedural complications. All patients survived to hospital discharge. Four patients have subsequently undergone elective stent removal and surgical repair of the arch, at between 38 and 83 days following stent implantation. Complete stent removal was achieved in three patients. Over a follow-up ranging between 8 weeks and 36 months, none of the patients has had any further complications. Conclusions: This palliative approach is warranted in carefully selected patients. Long-term follow-up is required.

Background: In repaired tetralogy of Fallot (TOF), exercise test parameters like peak oxygen uptake and ventilatory efficiency predict mortality. Studies have also suggested cardiac magnetic resonance (CMR)-derived right ventricular (RV) size threshold values for pulmonary valve replacement in repaired TOF. However, effects of proposed RV size on exercise capacity and morbidity are not known. Methods: The relationship between CMR-derived ventricular size, function, and pulmonary regurgitation (PR) and NYHA class, exercise performance, and electrocardiogram (ECG) was studied in patients of repaired TOF with pure PR in a retrospective review of records. Results: 46 patients (22 females), mean age 14 years (8−30.8), were studied. There was no relationship between CMR-derived ventricular size, function, or PR and exercise test parameters, or NYHA class. RV end systolic and end diastolic volume correlated positively with the degree of PR. QRS duration on ECG correlated positively with RV end-diastolic volume (P < 0.01, r² = 0.34) and PR (P < 0.01, r² = 0.52). Conclusions: In repaired TOF and pure PR, there is no correlation between ventricular size or function and exercise performance. RV size increases with increasing PR. Timing of pulmonary valve replacement in TOF with pure PR needs further prospective evaluation for its effect on morbidity and mortality.

Background: Fetal echocardiography is a well established sensitive tool to diagnose congenital heart disease (CHD) in utero. One of the determinants of effective utilization of fetal echocardiography is its awareness in the general population. The present hospital based study was undertaken to assess the awareness of the need for fetal echocardiography amongst Indian parents. Methods: One thousand one hundred and thirty eight consecutive parents who visited the pediatric cardiology outpatient department of a tertiary care centre over a period of two months were asked to fill up a questionnaire that included their demographic data, educational status, history of CHD in children, awareness of fetal echocardiography and source of information and timing of fetal echocardiogram if performed. The data was categorized and awareness was noted in different groups. Results: The awareness in the study population was 2.2%. Awareness was found to be similar across the study population irrespective of the demographics and high risk status of the parents. Conclusions: The awareness of fetal echocardiography, an important tool in reducing the incidence of complex CHD, thereby impacting public health, is alarmingly low in the population studied. Appropriate action to increase awareness of fetal echocardiography needs to be looked into.

Objective: To assess endothelial dysfunction and the risk for coronary atherosclerosis in children with irreversible pulmonary hypertension due to congenital heart disease (CHD). Methods: The study included 18 cyanotic patients (the mean age was 12.28 ± 3.26 years) who developed irreversible pulmonary hypertension due to cyanotic and acyanotic CHDs, and 18 control patients (the mean age was 11.78 ± 3.00 years). Study groups were compared for flow-mediated dilatation (FMD), carotid intima media thickness (CIMT) and atherosclerotic risk factors. Results: Compared to the control group, the mean FMD was significantly reduced in the cyanotic group (5.26 ± 2.42% and 9.48 ± 2.60%, respectively; P-value < 0.001). No significant difference was observed between the groups in CIMT (0.41 ± 0.08 mm and 0.39 ± 0.06 mm, respectively; P-value = 0.299). The levels of total cholesterol, low-density lipoprotein-cholesterol and very low-density lipoprotein-cholesterol were statistically significantly lower compared tothe control group (P-value = 0.001, 0.006 and 0.014, respectively), whereas no statistically significant difference was found in the levels of high-density lipoprotein-cholesterol and triglycerides (P-value = 0.113 and 0.975, respectively). Conclusions: Systemic endothelial dysfunction in children with irreversible pulmonary hypertension due to CHD was noted but there was no increased risk for atherosclerosis.

Background: Outreach echocardiographic services led by cardiac sonographers may help district level hospitals in the management of patients suspected to have cardiac anomalies. However, the safety and utility of such an approach is not tested. Methods: We retrospectively reviewed our experience of patients seen in the outreach visits by the echocardiographers alone and subsequently reviewed in the pediatric cardiology clinic. Comparison between the diagnosis made by the echocardiographer and the consultant pediatric cardiologist were done. We defined safety as no change in patient management plan between the outreach evaluation and the pediatric cardiology clinic evaluation, and we defined usefulness as being beneficial, serviceable and of practical use. Results: Two senior echocardiographic technicians did 41 clinic visits and over a period of 17 months, 623 patients were seen. Patients less than 3 months of age constitute 63% of the total patients seen. Normal echocardiographic examinations were found in 342 (55%) of patients. These patients were not seen in our cardiology clinic. Abnormal echocardiographic examinations were found in 281 (45%) of patients. Among the 281 patients with abnormal echos in the outreach visits, 251 patients (89.3%) were seen in the pediatric cardiology clinic. Comparing the results of the outreach clinic evaluation to that of the pediatric cardiology clinic, 73 patients (29%) diagnosed to have a minor CHD turned to have normal echocardiographic examinations. In all patients seen in both the outreach clinics and the pediatric tertiary cardiac clinics there was no change in patient's management plan. Conclusions: Outreach clinic conducted by pediatric echo sonographers could be useful and safe. It may help in reducing unnecessary visits to pediatric cardiology clinics, provide parental reassurance, and help in narrowing the differential diagnosis in critically ill patient unable to be transferred to tertiary cardiac centers provided it is done by experienced echosonographers.

Despite identifying that rheumatic fever (RF) is the result of an immunological reaction following group-A beta-hemolytic streptococcal infection, the pathogenesis remains elusive. RF has been incorrectly designated as causing pancarditis, since it does not cause myocarditis. Research directed toward myocarditis, targeting myosin to unravel the pathogenesis has not succeeded in more than 60 years. RF causes permanent damage to cardiac valves. The mitral valve (MV), derived from the wall of the left ventricle, is composed of a central core of connective tissue, covered on both sides by endothelium. The left ventricle does not have either myocardial or intermyocardial connective tissue involvement in RF. By exclusion, therefore, the primary site of RF damage appears to be the endothelium. Evaluation of the histopathology and immunopathology indicates that RF is a disease of the valvular and vascular endothelium. It is not a connective tissue disorder. Research to identify pathogenesis needs to be focused toward valvular endothelium.

Z-scores are a means of expressing the deviation of a given measurement from the size or age specific population mean. By taking account of growth or age, Z-scores are an excellent means of charting serial measurements in paediatric cardiological practice. They can be applied to echocardiographic measurements, blood pressure and patient growth, and thus may assist in clinical decision-making.

Right aortic arch (RAA) associated with isolated atrial septal defect (ASD) is very rare. We report successful closure of ASD associated with RAA using a 26-mm atrial septal occluder in a 30-year-old male patient. The impingement of right descending aorta in RAA caused malposition of the device in the left atrium. Deployment of the device through the right upper pulmonary vein successfully closed the defect. Follow-up evaluation by computerized tomography scan and echocardiogram showed no pulmonary venous obstruction.

Most patients with total anomalous pulmonary venous connection have a set pattern of pulmonary venous drainage and predictable sites of obstruction. However, uncommon variations do exist and delineating the entire course is more important than just knowing the site of drainage. Azygos vein involvement in the circuit is nearly always associated with a complicated course, as was seen in our patient. This report reviews the drainage patterns when azygos vein forms a part of the circuit.

Persistent fifth aortic arch (PFAA), also known as congenital double-lumen aortic arch, is a rare developmental anomaly of the aortic arch. A 5-month-old baby who presented with congestive cardiac failure was diagnosed to have a large patent ductus arteriosus with PFAA. Although this arch anomaly had no hemodynamic relevance in this case, preoperative diagnosis prevented surprises "on table."

Myocardial ischemia following arterial switch operation (ASO) usually occurs due to coronary ostial narrowing that might result from technical failure in translocation of the coronary arteries to the neoaorta. We present an unusual case report of neonatal myocardial ischemia caused by coronary steal secondary to aortopulmonary collaterals, following ASO in transposition of great arteries.

A 12-year-old boy with coarctation of aorta developed infective endarteritis and mycotic aneurysm at the site distal to coarctation. The computed tomography angiogram was very helpful in the diagnosis. Medical management and early surgical intervention was curative. Infective endarteritis in coarctation may be underdiagnosed.

Isolated annular dilatation is an extremely uncommon cause of congenital mitral regurgitation. We report a case of a 5-year-old child with idiopathic isolated annular dilatation causing severe congenital mitral regurgitation.

Manipulation of a pacemaker with consequent malfunction of the device has been called Twiddler's syndrome. Use of active-fixation leads and subpectoral pacemaker pockets has been considered to help in avoiding this problem. We describe a child in whom twiddling was not prevented despite implantation of a lumenless atrial lead and insertion of the pacemaker generator in a subpectoral pocket.

We present the case report of a child with Factor V Leiden mutation who underwent Fontan procedure. Thromboembolism is a widely recognized complication of the Fontan procedure and its modifications. Factor V Leiden mutation, being a hypercoagulable state, posed a higher risk for thromboembolism in this child. Appropriate measures taken before and after surgery prevented postoperative coagulopathy.

A 19 year old man presented with dizziness that was exacerbated while using left arm. On investigation, subclavian steal syndrome (SSS) was diagnosed. He underwent left carotid to subclavian bypass with relief of symptoms. Subclavian steal syndrome, although rare, should be considered in the differential diagnosis in patients with the history of subclavian artery manipulation in the past in proper settings.

We report a case of 3-year-old girl who had persistence of large residual flow following transcatheter closure of a 6 mm 'Type A' patent ductus arteriosus using a 12 × 10 mm duct occluder. Angiography revealed a large left-to-right shunt coursing through and exiting around the implanted device. Near total abolition of the residual shunt was achieved by initial implantation of an embolization coil within the duct occluder and subsequently an Amplatzer duct occluder (ADO II) adjacent to the duct occluder. This challenging case describes an additional technique of abolishing a large residual flow in and around a Nitinol duct occluder device.

Congenital absence of pericardium is rarely seen, often diagnosed intraoperatively during cardiac and thoracic surgeries. Left-sided pericardial defects are more common than right-sided ones. We present a case of an incidentally detected congenital absence of right pericardium with herniation of part of the right lung during ventricular septal defect closure surgery in a male child aged 4 years.

Congenital left atrial appendage aneurysm is very rare. We describe a giant left atrial appendage aneurysm with a pinball-like mobile thrombus in a 2-year-old child with cardioembolic stroke. Patient underwent successful surgical resection of the aneurysm.