Background: Aorto-left ventricular tunnel (ALVT) is a rare congenital extracardiac channel with progressive left ventricular dilatation needs early correction. Materials and Methods: This is a report of diagnosis and management of aorto-left ventricular tunnel (ALVT) over a period of 11 years from a single institution. Seven patients (age range: 7 days-45 years) presented with heart failure. The diagnosis of ALVT was made by transthoracic echocardiogram in all cases. Results: Treatment was refused by two patients who died during follow-up. Surgical closure of the tunnel was done in four cases, of which one needed Bentall procedure. Two patients had residual leak after the surgery. Transcatheter closure using Amplatzer muscular device was performed in two cases (for postoperative residual leak in one and primary procedure in the other). Significant hemolysis developed in one of them, necessitating the removal of the device and closed surgically. This child underwent aortic valve replacement two years later. All the remaining patients were doing well during the median follow-up of 30 months (range: 1.5-9 years). Conclusion: ALVT is a rare and potentially fatal anomaly that is ideally managed surgically. Catheter closure has a limited role.

Aim: Absent pulmonary valve syndrome (APVS) is found in 3-6% of patients with Tetralogy of Fallot (TOF). Along with findings of TOF, absence of pulmonary valve tissue results in aneurysmal dilatation of the main and branch pulmonary arteries compressing the trachea, main-stem, and intrapulmonary bronchi leading to obstructive airways disease. Our objective was to review pulmonary function tests (PFT) in TOF-APVS patients. Materials and Methods: Eight PFT were performed on five mechanically ventilated TOF-APVS patients in the intensive care unit. Tidal volume, forced vital capacity (FVC), maximal expiratory flow 25%, resistance and compliance of the respiratory system were measured. Results: Pre-operative PFTs showed markedly elevated airways resistance (R _RS ) (median 0.45 cmH ₂ O/mL/sec, range 0.17-0.66) and marked variability of the static compliance of the respiratory system (C _RS ) (median 0.6 mL/cmH ₂ O/kg, range 0.25-2.6). Flow-volume loops measured by forced deflation showed flow limitation within the medium to small airways. Post-operative FVC was reduced in four of the five patients (median 46 mL/kg, IQR 42.9 - 48.8 mL/kg). Patients studied with various levels of positive end expiratory pressure (PEEP) showed improvement in tidal volume and reduced obstruction with PEEP greater than 10 cmH ₂ O. For three patients with pre-operative data available, surgical correction resulted in near-normal post-operative C _RS and improved, but still elevated R _RS (median 0.14 cmH ₂ O/mL/sec, interquartile range [IQR] 0.11-0.31). Conclusion: For our patients with TOF-APVS, airway resistance was elevated. Flow limitation was seen in the medium to small airways with a mild reduction of FVC. PFTs may help guide management of mechanical ventilation for TOF-APVS patients.

Objectives: To validate the physical and psychological effectiveness of cardiac pacing in pediatric patients with breath-holding spells (BHS) and prolonged asystole. Materials and Methods: The records and clinical data of all the patients with BHS who presented to our center in the period of 2001-2013 were reviewed. All patients who received cardiac pacemaker implantation for prolonged asystole during BHS were included. In addition, the parents were asked to fill out a standardized quality of life (QOL) questionnaire. Results: Seven patients were identified. The mean onset of symptoms was 7 month (1-12 months) of age, documented asystole was 12-21 seconds, and a permanent cardiac pacemaker device was implanted at a mean age of 23 months (8 months-3.9 years). No pacemaker related adverse events were recorded. Follow up showed immediate resolution from spells in four cases (4/7). Two patients (2/7) showed significant reduction of frequency and severity of spells, with complete elimination of loss of consciousness (LOC). One patient (1/7) with an additional neurologic disorder continued to have minor pallid BHS and eventually switched from pallid to cyanotic spells without further detection of bradycardia or asystole in holter examination. QOL questionnaire revealed significant reduction in subjective stress levels of patients (P = 0.012) and parents (P = 0.007) after pacemaker implantation. Conclusion: Cardiac pacing using appropriate pacemaker settings seems effective in the prevention of LOC and reduction of the frequency of BHS. Our results imply a reduction of subjective stress levels of patients and parents as well as an increased quality of everyday life. After all, randomized controlled trials of the influence of cardiac pacemaker implantation on subjective stress levels in patients with BHS are needed.

Screening for rheumatic heart disease (RHD) requires workers skilled in echocardiography, which typically involves prolonged, specialized training. Task shifting echocardiographic screening to nonexpert health workers may be a solution in settings with limited human resources. An 8-week training program was designed to train health workers without any prior experience in focused echocardiography for RHD screening. Seven health workers participated. At the completion of training, the health workers performed unsupervised echocardiography on 16 volunteer children with known RHD status. A pediatric cardiologist assessed image quality. Participants provided qualitative feedback. The quality of echocardiograms were high at completion of training (55 of 56 were adequate for diagnosis) and all cases of RHD were identified. Feedback was strongly positive. Training health workers to perform focused echocardiography for RHD screening is feasible. After systematic testing for accuracy, this training program could be adapted in other settings seeking to expand echocardiographic capabilities.

The history of surgery for transposition of great arteries (TGA) has paralleled the history of cardiac surgery. In fact, it began before the birth of open heart surgery when the palliative Blalock-Hanlon septectomy was first performed in 1948. The atrial switch, which was an attempt to correct the physiology of transposition, had significant shortcomings. The arterial switch sought to address them. This has emerged as an anatomically as well as physiologically appropriate solution. Today we continue to pursue technical refinements as well as try to expand the indications of the arterial switch. This review traces the various milestones in this perpetual journey.

Sri Lanka is an island nation in Indian Ocean that provides free healthcare to all citizens through government healthcare system. It has commendable health indices in the region. Pediatric cardiac services have rapidly progressed over past few years helping to further bring down infant and under-five mortality rates. Lady Ridgeway Hospital for Children (LRH) is the only tertiary care referral center for children with heart disease in the country. Currently it performs approximately 1,000 cardiac catheterizations and 1,000 cardiac surgeries every year. Target is to double the surgical output to treat all children with heart diseases in a timely and appropriate manner. Being a middle-income country, this is not an easy task. Technology used in diagnosis and treatment of congenital heart diseases is rapidly advancing with its price tag. In such a setting, it is challenging to proceed to achieve this target in a resource-limited environment.

Sudden cardiac death (SCD) most commonly results from previously undiagnosed congenital, acquired, or hereditary cardiac diseases. Congenital aortic valvular, subvalvular, and supravalvular disease with left ventricular outflow tract obstruction is an important preventable cause of sudden death. This report documents sudden death presumably due to acute myocardial ischemia in a young male with an undiagnosed supravalvular aortic stenosis (SVAS) due to a rare association of isolation of coronary sinuses of Valsalva. Congenital supravalvular pulmonary stenosis and mitral valvular dysplasia were also present.

Pseudoaneurysms of the ventricle are an uncommon occurrence in children. They may be secondary to previous cardiac surgery or infection of the pericardial space. Infective pseudoaneurysms require urgent surgery because rupture of the pseudoaneurysm may have catastrophic consequences. The outcome with surgery is excellent. We report a toddler with ruptured pseudoaneurysm secondary to purulent pericarditis who recovered with an emergency surgical closure of the opening of the pseudo-aneurysm.

A 3-day-old neonate presented with features suggestive of coarctation of aorta. Echocardiography showed a large organized thrombus in the transverse arch causing obstruction to theaortic arch and carotids with partial recanalization. Patient underwent surgical thromboendarterectomy with arch reconstruction. The evaluation did not reveal any hematological abnormalities and suspected to be due to fetal thromboembolism. Patient improved well, and no neurological deficits were observed during follow-up.

The patient presented with flash pulmonary edema related to severe left ventricular diastolic dysfunction several weeks after arterial switch operation (ASO) for D-transposition of the great arteries. Long segment, critical left main coronary artery stenosis in this 3.6 kilogram infant was successfully stented and resulted in resolution of the clinical findings. At 15-month follow-up, the patient remains asymptomatic and thriving.

Coronary artery stenosis is a rare phenomenon in children. Coronary stent implantation is generally not considered a standard treatment option due to technical difficulties and potential complications in this group of patients. Nevertheless, several pediatric cases reporting successful implantation with acceptable short-term experiences have been described. The following case presents a successful stent implantation for left main coronary artery (LMCA) stenosis early after surgery for anomalous left coronary artery from pulmonary artery (ALCAPA) at the age of 6 months. The excellent mid-term results and notably the procedure's potential as a long-term treatment in small children are highlighted. A 6-month-old infant underwent surgery for ALCAPA. Due to sudden postoperative deterioration, cardiac catheterization was performed. Coronary angiography revealed severe (90%) ostial LMCA stenosis. A PROMUS drug-eluting stent (Promus Element AL3.0 Χ 8 mm, Boston Scientific, Natick, Massachusetts, USA) was implanted. The procedure was performed without complications. Antiplatelet therapy with acetylsalicylic acid and clopidogrel was initiated. Subsequently, cardiac function improved slowly. Cardiac catheterization 3 years 8 months after stent implantation showed no restenosis with a proximal LMCA diameter still at the 50 ^th percentile for age. Neither were signs of heart failure reported at the last follow-up at 7 years of age. Presupposing normal growth, the implanted stent would thus provide sufficient myocardial perfusion with a LMCA lumen at the 40 ^th percentile at the age of 16 years. In selected cases, coronary stent implantation may be an effective mid- to long-term treatment of coronary artery stenosis even in very young children.

Multimodality image overlay is increasingly used for complex interventional procedures in the cardiac catheterization lab. We report a case in which three-dimensional magnetic resonance imaging (3D MRI) overlay onto live fluoroscopic imaging was utilized to safely obtain transhepatic access in a 12-year-old patient with prune belly syndrome, complex and distorted abdominal anatomy, and a vascular mass within the liver.

Mutations in PRKAG2 gene that regulates the γ2 subunit of the adenosine monophosphate (AMP) dependent protein kinase have been associated with the development of atrioventricular (AV) accessory pathways, cardiac hypertrophy, and conduction system abnormalities. These patients can potentially be misdiagnosed as hypertrophic cardiomyopathy (HOCM) and/or Wolf-Parkinson White (WPW) syndrome due to similar clinical phenotype. Early recognition of this disease entity is very important as ablation of suspected accessory pathways is not effective and the natural history of the disease is very different from HOCM and WPW syndrome.

Rubinstein-Taybi syndrome is a well-characterized condition causing distinctive physical characteristics, intellectual disability, and multiple congenital malformations. Cardiac abnormalities are found in a third of individuals with this condition and usually consist of isolated septal defects or patent ductus arteriosus, although more complex congenital lesions have been described. We present the first reported case of tricuspid atresia and pulmonary atresia with hypoplasia of the right ventricle in the setting of Rubenstein-Taybi syndrome.

Isolation of the right subclavian artery (RSCA) is rare, and this finding in association with d-transposition of the great arteries (d-TGA) is extremely unusual. We present a case of an isolated RSCA in a newborn with d-TGA in whom the clinical presentation was diagnostic. We discuss the imaging modalities used to confirm the diagnosis, the embryological basis of the finding, and the surgical repair.

Cardiac malignancies presenting in infancy are rare. Desmoplastic small round cell tumor (DSRCT) is a rare occurrence in this age group. No case of intrapericardial DSRCT has been reported in the literature in infants.

Surgicel® is bioabsorbable hemostatic mesh, frequently packed around oozing surgical bed. We report two morbidities due to it. Following transannular patch repair for Fallot's tetralogy, Surgicel® was packed around distal main pulmonary artery. Echocardiography in the intensive care unit (ICU) showed right ventricular dysfunction due to extrinsic obstruction and complete occlusion of left pulmonary artery (LPA) flows. Another patient with arterial switch operation had postoperative fibrillatory cardiac arrest, needing resuscitation with internal cardiac massage. The arrest was triggered by coronary ischemia due to periaortic compression. Both instances were caused by hygroscopic nature of Surgicel®, which absorbed blood, swelled, and compressed the luminal tissues.

A coronary cameral fistula (CCF) involves a sizable communication between a coronary artery and a cardiac chamber. We present a case of giant coronary cameral fistula associated with coarctation of aorta in a 13 days old neonate.