Congenitally corrected transposition of the great arteries (ccTGA) is a complex cardiac anomaly with an unfavorable natural history. Surgical treatment has been available for over 50 years. Initial procedures used for ccTGA did not correct atrio-ventricular discordance, leaving the right ventricle in systemic position. In the past two decades anatomic repair has been considered to be a better option. Many cases subjected to anatomic repairs would also be suitable for the Fontan strategy, which probably has a lower initial risk. The rationale for use of the Fontan operation in management of congenitally corrected transposition is discussed in this review, with comparisons to other strategies.

Numerous articles have been published linking consanguineous marriage to an elevated prevalence of congenital heart disease, with ventricular septal defects and atrial septal defects the most commonly cited disorders. While initially persuasive, on closer examination many of these studies have fundamental shortcomings in their design and in the recruitment of study subjects and controls. Improved matching of cases and controls, to include recognition of the long-established community boundaries within which most marriages are contracted, and the assessment of consanguinity within specific levels and types of marital union would improve and help to focus the study outcomes. At the same time, major discrepancies between studies in their reported prevalence and types of congenital heart disease suggest an urgent need for greater standardization in the classification and reporting of these disorders.

Introduction: Congenital heart disease (CHD) is associated with multiple risk factors, consanguinity may be one such significant factor. The role of consanguinity in the etiology of CHD is supported by inbreeding studies, which demonstrate an autosomal recessive pattern of inheritance of some congenital heart defects. This study was done to find out the risk factors for CHD. Methods: A case-control study was done on pediatric patients at a tertiary care hospital, Aga Khan University Hospital, located in Karachi, Pakistan. A total of 500 patients, 250 cases and 250 controls were included in the study. Results: Amongst the 250 cases (i.e. those diagnosed with CHD), 122 patients (48.8%) were born of consanguineous marriages while in the controls (i.e. non-CHD) only 72 patients (28.9%) showed a consanguinity amongst parents. On multivariate analysis, consanguinity emerged as an independent risk factor for CHD; adjusted odds ratio 2.59 (95% C. I. 1.73 - 3.87). Other risk factors included low birth weight, maternal co-morbidities, family history of CHD and first born child. On the other hand, medications used by the mother during the index pregnancy, maternal age and gender of the child did not significantly increase the risk of developing CHD. Conclusions: Analyses of our results show that parental consanguinity, family history of CHD, maternal co-morbidities, first born child and low birth weight are independent risk factors for CHD.

Introduction and Aim : Focussed cardiac intensive care is known to produce better outcomes. We have evaluated the benefits of a dedicated Pediatric Cardiac Intensive Care Unit (PCICU) in the early postoperative outcomes of patients undergoing surgery for congenital heart disease. Methods : Prospectively collected data of 634 consecutive patients who underwent congenital heart surgery from September 2008 to September 2009 were analyzed. Midway through this period a dedicated PCICU was started. The patients who were treated in this new PCICU formed the study group (Group B, n = 318). The patients who were treated in a common postoperative cardiac surgery ICU formed the control group (Group A, n = 316). Early postoperative outcomes between the two groups were compared. Results : The two groups were comparable with respect to demographic data and intraoperative variables. The duration of mechanical ventilation in the dedicated pediatric cardiac ICU group (32.22 ± 52.02 hours) was lower when compared with the combined adult and pediatric surgery ICU group (42.92 ± 74.24 hours, P= 0.04). There was a shorter duration of ICU stay in the dedicated pediatric cardiac ICU group (2.69 ± 2.9 days vs. 3.43 ± 3.80 days, P = 0.001). The study group also showed a shorter duration of inotropic support and duration of invasive lines. The incidence of blood stream infections was also lower in the dedicated pediatric ICU group (5.03 vs. 9.18%, P = 0.04). A subgroup analysis of neonates and infants <1 year showed that the advantages of a dedicated pediatric intensive care unit were more pronounced in this group of patients. Conclusions : Establishment of a dedicated pediatric cardiac intensive care unit has shown better outcomes in terms of earlier extubation, de-intensification, and discharge from the ICU. Blood stream infections were also reduced.

Background : Tissue Doppler Imaging (TDI) is a sensitive index of myocardial function. Its role in the fetus has not been extensively evaluated. Objective: To compare myocardial tissue Doppler velocities in fetuses with hypoplastic left heart syndrome (HLHS) to those of normal fetuses (matched for gestational age.) Methods: Cross-sectional retrospective study conducted at 2 large perinatal centers (2003-2007). Fetuses with HLHS ( n = 13) were compared with normal fetuses ( n = 207) in 5 gestational age groups. TDI data included peak systolic (s'), peak early (e'), and late diastolic velocities (a'). Linear regression was used to compare TDI parameters in fetuses with HLHS to normal fetuses matched for gestational age. Results: Fetuses with HLHS had significantly reduced lateral tricuspid annular e' as compared to normal fetuses. Both normal fetuses and those with HLHS had linear increase in TDI velocities with advancing gestational age. Conclusions: TDI velocities are abnormal in fetuses with HLHS. TDI can be useful in serial follow-up of cardiac function in fetuses with HLHS.

Introduction: The ideal management strategy for patients presenting late with transposition of great arteries (TGA), intact ventricular septum (IVS), and regressed left ventricle (LV) is not clear. Primary switch, two-stage switch, and Senning operation are the options. Left ventricular retraining prior to arterial switch by ductal stenting may be effective, but the experience is very limited. Methods: Five of six children aged 3-6 months with TGA-IVS and regressed LV underwent recanalization and transcatheter stenting of ductus arteriosus. The ductal stent was removed during arterial switch surgery. Results: The procedure was successful in 5/6 patients. All the patients had totally occluded ductus and needed recanalization with coronary total occlusion hardware. The ductus was dilated and stented with coronary stents. In all the patients, there was significant luminal narrowing despite adequate stent placement and deployment. Two patients needed reintervention for abrupt closure of the stent. Ductal stenting resulted in left ventricular preparedness within 7-14 days. One patient died of progressive sepsis after 14 days of stenting, even though the LV was prepared. Four patients underwent successful uneventful arterial switch surgery. During surgery, it was observed that the mucosal folds of duct were protruding through the struts of the stent in one patient. Conclusions: Ductal stenting is a good alternative strategy for left ventricular retraining in TGA with regressed LV even in patients with occluded ducts.

Objective: To evaluate the effect of percutaneous closure of patent ductus arteriosus (PDA) on left ventricular (LV) systolic and diastolic function in children. Background: Limited studies are available on alteration in LV hemodynamics, especially diastolic function, after PDA closure. Methods: Thirty-two consecutive children with isolated PDA treated by trans-catheter closure were studied. The LV systolic and diastolic function were assessed by two-dimensional (2D) echocardiography and tissue Doppler imaging 1 day before the PDA closure, on day 1, and on follow-up. Results: At baseline, none of the patients had LV systolic dysfunction. On day 1 post-PDA closure, 8 (25%) children developed LV systolic dysfunction. The baseline LV ejection fraction (LVEF), LV end-systolic dimension (LVESD), and PDA diastolic gradient predicted the post-closure LVEF. Patients who developed post-closure LV systolic dysfunction had poorer LV diastolic function than those who did not. LV diastolic properties improved after PDA closure; however, the improvement in LV diastolic properties lagged behind the improvement in the LV systolic function. All children were asymptomatic and had normal LVEF on follow up of >3 months. Conclusions: Percutaneous closure of PDA is associated with the reversible LV systolic dysfunction. Improvement in the LV diastolic function lags behind that in the LV systolic function.

A small number of nonrandomized and retrospective studies have compared outcomes of classical Norwood procedures for hypoplasticleft-heart syndrome and single ventricle lesions involving a Blalock-Taussig (BT) shunt to the modified procedure using a right ventricle to pulmonary artery conduit. Some of these studies reported data for the same outcomes and a meta-analysis was done to analyze pooled outcomes comparing in-hospital mortality, interstage mortality, cardiopulmonary bypass time, systolic and diastolic blood pressures 24 h postoperatively, length of intensive care and hospital stay, and need for postoperative extracorporeal membrane oxygenation. Right ventricle to pulmonary artery conduit was associated with an insignificant reduction of in-hospital mortality (odds ratio, 0.674, 95% confidence interval, 0.367 to 1.238), and in the length of hospital stay. There were significant reductions in cardiopulmonary bypass time, length of intensive care unit stay, and need for postoperative extracorporeal membrane oxygenation, postoperative ventilation times, and interstage mortality (odds ratio, 0.191, confidence interval, 0.0620 to 0.587). There was a significant increase in diastolic blood pressure and an insignificant increase in systolic blood pressure 24 h postoperatively. This pooled analysis demonstrates potential advantages associated with the right ventricle to pulmonary artery conduit when compared to the modified BT shunt in palliation and demonstrates the need for large randomized controlled trials that compare a number of outcomes in both procedures.

Hypocalcemia is a less known but treatable cause for dilated cardiomyopathy, leading to severe heart failure in children. Cardiogenic shock related to hypocalcemic cardiomyopathy is a rare event. We describe 5 infants presenting with cardiogenic shock over 3 years, who were found to have severe hypocalcemia as a sole cause of myocardial dysfunction. The patients responded to calcium and vitamin D supplementation promptly and left ventricular systolic function normalized within months of treatment. In any case of cardiogenic shock, hypocalcemia should be included in the differential diagnosis and must be investigated.

Objective: Acute rheumatic fever (ARF) is an endemic disease observed in children of developing countries. The purpose of this study was to test if it was possible to identify myocardial involvement in cases with rheumatic carditis by the measurement of serum cardiac TnT. Methods: 30 patients diagnosed as ARF underwent echocardiography and their cardiac troponin T (cTnT) serum levels were measured. Patients were divided into group 1: Arthritis alone, group 2: carditis, and group 3 carditis with congestive heart failure (CHF). Results: cTnT serum levels were normal in all except one patient with in group 3. Two patients in carditis (group 2) and three patients in CHF (group 3) had dilation in left ventricular end diastolic diameter. Conclusions: Normal cTnT levels in our patient group suggests that inflammation rather than myocardial necrosis is predominant in ARF carditis.

Four neonates presented within 24 hours of birth with stridor, respiratory distress and a weak cry. Clinical examination of the cardiovascular system revealed no abnormality. The transthoracic echocardiogram showed large aneurysm of ductus arteriosus at the aortic isthmus, tapering to a small tortuous channel at the site of pulmonary artery insertion. Computerized tomography scan performed in two of the neonates demonstrated considerable compression of adjacent thoracic structures. One required surgical excision due to persistence of symptoms. Serial echocardiograms in the remaining three babies showed transition through various stages of resolution over a period of 6 weeks to 3 months, resulting in the obliteration of the aneurysm. All babies are doing well during the follow-up.

Atrial pacing in the neonate is rarely done due to constraints of equipment, technical knowhow, and risk of complications. We describe a novel method of atrial pacing in the new born using equipment readily available in any intensive care unit. Atrial pacing can be achieved using an adult bipolar pacing wire directly introduced through the umbilical venous route. We have used this method in 5 neonates, and achieved atrial capture in 4 patients. Temporary atrial pacing is a therapeutic option in a few neonatal arrhythmias, such as atrial flutter, a few types of re-entrant tachycardias, such as accessory pathway mediated tachycardia and junctional reciprocating tachycardia, and has a limited role in cardiopulmonary resuscitation where sinoatrial node dysfunction is suspected.

Cardiovascular risk factors in children are increasing at an alarming rate in the western world. However, there is limited information regarding these in the South Asian children. This review attempts at summarizing such evidence. South Asians are remarkable for the earlier onset of adult cardiovascular disease (CVD) by almost a decade compared to the Caucasians. We identified published literature, mainly on PubMed, Embase and Cochrane library using specific search terms such as lipid abnormalities, high blood pressure, hyperglycemia, tobacco use, obesity, physical inactivity, and unhealthy dietary practices. Atherosclerotic CVD processes begin early in childhood and are influenced over the life course by genetic and potentially modifiable risk factors and environmental exposure. 80% of adult CVD burden will fall on the developing nations by 2020. The concept of primordial prevention is fast emerging as a necessary prevention tool to curb adult CVD epidemic. Established guidelines and proven preventive strategies on cardiovascular health exist; however, are always implemented half-heartedly. Composite screening and prediction tools for adults can be adapted and validated in children tailored to South Asian population. South Asian children could be at a greater risk of developing cardiovascular risk factors at an earlier stage, thus, timely interventions are imperative.

Advances in the fetal magnetic resonance imaging (MRI) over the last few years have resulted in the exploring the use of fetal MRI to detect congenital cardiac anomalies. Early detection of congenital cardiac anomalies can help more appropriately manage the infant's delivery and neonatal management. MRI offers anatomical and functional studies and is a safe adjunct that can help more fully understand a fetus' cardiac anatomy. It is important for the obstetricians and pediatric cardiologists to be aware of the recent advancements in fetal MRI and it`s potential utility in diagnosing congenital cardiac anomalies.

Complex congenital heart diseases are often associated with complex alterations in hemodynamics. Understanding these key hemodynamic changes is critical to making management decisions including surgery and postoperative management. Existing tools for imaging and hemodynamic assessment like echocardiography, computed tomography and cardiac catheterization have inherent limitations. Cardiac magnetic resonance imaging (MRI) is emerging as a powerful bouquet of tools that allow not only excellent imaging, but also a unique insight into hemodynamics. This article introduces the reader to cardiac MRI and its utility through the clinical example of a child with a complex congenital cyanotic heart disease.

Levoatriocardinal vein (LACV) is characterized by an abnormal connection between pulmonary and systemic venous return. This extremely rare cardiac malformation is usually associated with left-sided obstructive lesions including mitral atresia, hypoplastic left-heart syndrome, and abnormal pulmonary venous connection. Patients may have low systemic cardiac output and pulmonary venous obstruction symptoms. In this manuscript, we report a case with LACV and normal pulmonary venous return with absence of any intracardiac pathology. LACV was demonstrated with echocardiography, angiography, and computed tomography. Surgical correction was made successfully.

Aetiology of pericardial calcifications can be multifactorial. Tuberculosis has been reported as the most common cause. Other known causes include uraemia, asbestosis, post-traumatic or postoperative. We report a rare case of pericardial calcification seen in a patient with established acute intermittent porphyria. A direct causal relationship cannot be established between porphyria and pericardial calcification, but it may be due to deposition of the porphyrin in the pericardium.

This report describes the findings and management of a young male who presented with an acute ST-segment elevation myocardial infarction due to compression of the circumflex coronary artery by a large aneurysm of left sinus of Valsalva.

Percutaneous closure of an atrial septal defect (ASD) has been established as a safe and effective alternative to surgical management. We describe a case of a 41-year-old patient in whom an Amplatzer septal occluder device was used to close a moderately large ASD and who subsequently developed incessant intra-atrial macro-reenterant tachycardia. The tachycardia was terminated by radiofrequency ablation guided by electroanatomical mapping.

Pseudoaneurysm of ascending aorta is an infrequent but well-recognized and potentially fatal complication after cardiac surgeries. The complication can develop early, delayed or late, and the presentation is also varied. We are presenting here two cases of pseudoaneurysm of ascending aorta following cardiac surgery that were successfully managed by the transcatheter method. The first one occurred following coronary artery bypass surgery and the second one occurred following double-valve replacement surgery. The aortic openings of these aneurysms were occluded with 12 mm and 10 mm atrial septal occluders, respectively, with a good outcome. An immediate postprocedure angiogram showed no residual flow into the sac. Six months of follow-up of both cases also showed excellent results.

Coexistence of divided left atrium with tetralogy of Fallot is rare. Preoperative diagnosis of this rare association is difficult. We here report preoperative diagnosis of this rare combination. In addition, the patient also had coronary to left ventricle fistula.

Presence of coronary to pulmonary artery fistula is generally a feature of pulmonary atresia with ventricular septal defect. We present a rare case of left anterior descending coronary artery to pulmonary artery fistula in a patient of tetralogy of Fallot.

Hutchinson-Gilford progeria syndrome (HGPS) is a rare premature aging syndrome that results from mutation in the Laminin A gene. This case report of a 12-year-old girl with HGPS is presented for the rarity of the syndrome and the classical clinical features that were observed in the patient. All patients with this condition should undergo early and periodic evaluation for cardiovascular diseases. However, the prognosis is poor and management is mainly conservative. There is no proven therapy available. Mortality in this uniformly fatal condition is primarily due to myocardial infarction, strokes or congestive cardiac failure between ages 7 and 21 years due to the rapidly progressive arteriosclerosis involving the large vessels.

A 2-month-old child was referred as a case of dilated cardiomyopathy with cardiogenic shock. On evaluation, hypocalcemia secondary to severe vitamin D deficiency was found. There were no clinical or radiological features of rickets. The child had developed multiorgan failure due to cardiogenic shock at the time of admission and could not be saved despite adequate ventilatory and pharmacologic support. Hypocalcemia should be considered as an important differential diagnosis in cases of dilated cardiomyopathy in infants.

Kingella kingae , a HACEK (Haemophilus parainfluenzae, Aggregatibacter actinomycetemcomitans, Aggregatibacter aphrophilus, Cardiobacterium hominis, Eikenella corrodens, Kingella kingae) organism, is a common resident of the upper airway in children; it has been associated with endocarditis in children with pre-existing heart conditions. This case report describes K. kingae endocarditis leading to valvular damage in a previously healthy 18-month-old child. Our patient developed a K. kingae bacteremia that was later complicated by meningitis, septic embolic stroke, and endocarditis of the mitral valve, leading to perforation of the posterolateral leaflet. The patient was initially treated conservatively with cefotaxime but, subsequently, required a mitral valve repair with a pericardial patch and annuloplasty. This report draws attention to the need for clinicians to be aware of the potentially serious complications of K. kingae infection in young children. If K. kingae infection is suspected then therapy should be initiated promptly with a β-lactam, followed by early echocardiographic assessment. This case also highlights the lack of specific guidelines available for K. kingae endocarditis.

We report supravalvular aortic stenosis in a 12 year old patient who presented with mental retardation, malformed teeth, broad lower lips, pectus carinatum, clinodactyly, kyphoscoliosis with symptoms of shortness of breath. On examination presence of better volume pulse in right radial artery with ejection systolic murmur best heard in right 2^nd intercostal space were noted. Patient was diagnosed as having William's syndrome with investigations demonstrating Supravalvular aortic stenosis with a gradient of 170 mm Hg.

A 4 year old boy was referred for evaluation of failure to thrive and mild cyanosis. He was found to have a structurally normal heart with evidence of microscopic pulmonary arterio-venous (AV) fistulae. Later, he was diagnosed to have congenital porto-systemic shunt, a very rare cause of pulmonary AV fistula.