India faces a huge burden of pediatric and adult congenital heart diseases (CHDs). Many acquired valvar, myocardial, and vascular diseases also need treatment in childhood and adolescence. The emergence of pediatric cardiology as an independent specialty has been a relatively recent development. A few centers of excellence in pediatric cardiology have developed. However, the requirement of pediatric cardiac care and pediatric cardiologists is far in excess of what is available. There are no guidelines at present in India for uniform training in pediatric cardiology. Many training programs are nonstructured and do not focus on the regional needs. Both core training and advanced training programs are essential to provide adequate numbers of community-level pediatric cardiologists and academic leaders respectively. This article proposes a detailed plan and curriculum for comprehensive training of future pediatric cardiologists in India.

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Background: Over the past two decades, it has been observed that hypertension shows an increasing trend in children and adolescents. Various factors are contributing to this upward trend, and they primarily include changes in lifestyle and dietary habits. Objectives: The aim of this study was to evaluate the prevalence of hypertension in school going adolescent children and to study the associated risk factors. Materials and Methods: This prospective cross-sectional observational study was conducted over a period of one year on apparently healthy adolescents of randomly selected urban schools of Bhopal district of Madhya Pradesh, Central India. A pretested and prevalidated questionnaire was used to collect the details including present or past history of illness, family history of hypertension, socioeconomic status, and sleep pattern and birth weight of the children. This was followed by anthropometric and blood pressure (BP) measurements and thorough systemic examination. Results: Out of 1221 children recruited in the study, 618 were boys, and 603 were girls. 22.7%, body mass index (BMI) of majority (85%) of the students was between 5 ^th and 84 ^th percentile, 5.65% were obese (BMI ≥95 ^th ) and 9.18% children were overweight (85 ^th -95 ^th percentile). Systolic and diastolic hypertension (BP >95 ^th percentile) was seen in 61 (4.1%) and 48 (3.9%) participants, respectively. Both systolic and diastolic hypertension was seen in 30 (2.45%) participants. Systolic and diastolic prehypertension (BP 90 ^th to <95 ^th percentile) was seen in 88 (7.3%) and 68 (5.6%) participants, respectively. A highly significant association (P < 0.01) of sex, BMI, systolic BP, family history of hypertension, and birth weight with diastolic BP was seen. Conclusion: There is a significant positive correlation of BMI with both systolic and diastolic BP. The family history of hypertension appears to be an important risk factor for the increase in both systolic and diastolic BP. Low birth weight and male sex seem to be risk factors for diastolic hypertension.

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Cor-triatriatum dexter is an extremely rare congenital heart defect in which there is complete persistence of the right valve of embryonic sinus venosus that results in partitioning of the right atrium into a smooth and trabeculated portion. The smooth portion receives venous blood from inferior vena cava, superior vena cava, and coronary sinus while the trabeculated portion contains the right atrial appendage and the opening of tricuspid valve. We report a 1-week-old child who presented with intermittent episodes of central cyanosis. Echocardiography, established, and bubble contrast study confirmed the diagnosis of an isolated cor-triatriatum dexter. The baby initially underwent an intervention by cardiac catheterization, which was unsuccessful in disrupting the membrane and re-direct the systemic venous flow to the right heart chambers. She subsequently had the cor-triatriatum dexter membrane resected via an uncomplicated open-heart surgery.

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Context: Levosimendan is a new generation inotrope with calcium sensitizing properties and proven benefits in adults. Aims: This study investigates the use of levosimendan as a first line inotrope in congenital heart surgery. Settings and Design: Prospective, observational study in a tertiary care center. Materials and Methods: One hundred and ten patients undergoing congenital cardiac surgery received levosimendan at a loading dose of 12 mcg/kg during rewarming on cardiopulmonary bypass followed by continuous infusion of 0.1 mcg/kg/min for 48 h. Hemodynamic parameters were recorded at the time of admission to Intensive Care Unit, and at 3 h, 6 h, 12 h, 24 h, and 48 h thereafter. Statistical Analysis: Categorical variables were compared using Chi-square test. Non-normally distributed quantitative variables were compared between groups using Kruskal-Wallis test. Results: At discharge from operating room (OR), 36 (32.7%) patients required levosimendan alone to maintain optimum cardiac output, 59 (53.6%) patients required the addition of low-dose adrenaline (<0.1 mcg/kg/min) and 15 (13.6%) patients required either increment in adrenaline to high-dose (≥0.1 mcg/kg/min) or starting another inotrope/vasoactive agent. Overall, there were five mortalities. Hypotension leading to discontinuation of levosimendan was not found in any patient. Arrhythmias were observed in three patients. Fifty-four patients were extubated in the OR. Conclusions: Levosimendan-based inotropic regime offers optimized cardiac output with a well-controlled heart rate and a low incidence of arrhythmias in patients undergoing all categories of congenital heart surgeries.

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We report a rare case of ruptured right sinus of valsalva into the left ventricle (LV). Transthoracic echocardiography showed a marked turbulent flow from the right aortic sinus to the LV. We describe a novel technique of closure of this defect with duct occluder, involving the formation of an arterio-arterial loop, without resorting to the usual arteriovenous loop.

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The artery allegedly forming in the fifth pharyngeal arch has increasingly been implicated as responsible for various vascular malformations in patients with congenitally malformed hearts. Observations from studies on developing embryos, however, have failed to provide support to substantiate several of these inferences such that the very existence of the fifth arch artery remains debatable. To the best of our knowledge, in only a solitary human embryo has a vascular channel been found that truly resembled the artery of the fifth arch. Despite the meager evidence to support its existence, the fifth arch artery has been invoked to explain the morphogenesis of double-barreled aorta, some unusual forms of aortopulmonary communications, and abnormalities of the brachiocephalic arteries. In most of these instances, the interpretations have proved fallible when examined in the light of existing knowledge of cardiac development. In our opinion, there are more plausible alternative explanations for the majority of these descriptions. Double-barreled aorta is more likely to result from retention of the recently identified dorsal collateral channels while abnormalities of brachiocephalic arteries are better explained on the basis of extensive remodeling of aortic arches during fetal development. Some examples of aortopulmonary communications, nonetheless, may well represent persistence of the developing artery of the fifth pharyngeal arch. We here present one such case - a patient with tetralogy of Fallot and pulmonary atresia, in whom the fifth arch artery provided a necessary communication between the ascending aorta and the pulmonary arteries. In this light, we discuss the features we consider to be essential before attaching the tag of "fifth arch artery" to a candidate vascular channel.

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Background: Cardiac catheterizations expose both the patient and staff to the risks of ionizing radiation. Studies using the "air gap" technique (AGT) in various radiological procedures indicate that its use leads to reduction in radiation exposure but there are no data on its use for pediatric cardiac catheterization. The aim of this study was to retrospectively review the radiation exposure data for children weighing <20 kg during cardiac catheterizations using AGT and an "as low as reasonably achievable (ALARA)" radiation reduction protocol. Patients and Methods: All patients weighing <20 kg who underwent cardiac catheterization at the Children's Hospital at Montefiore (CHAM), New York, the United States from 05/2011 to 10/2013 were included. Transplant patients who underwent routine endomyocardial biopsy and those who had surgical procedures at the time of the catheterizations were excluded. The ALARA protocol was used in concert with AGT with the flat panel detector positioned 110 cm from the patient. Demographics, procedural data, and patient radiation exposure levels were collected and analyzed. Results: One-hundred and twenty-seven patients underwent 151 procedures within the study period. The median age was 1.2 years (range: 1 day to 7.9 years) and median weight was 8.8 kg (range: 1.9-19.7). Eighty-nine (59%) of the procedures were interventional. The median total fluoro time was 13 min [interquartile range (IQR) 7.3-21.8]. The median total air Kerma (K) product was 55.6 mGy (IQR 17.6-94.2) and dose area product (DAP) was 189 Gym2 (IQR 62.6-425.5). Conclusion: Use of a novel ALARA and AGT protocol for cardiac catheterizations in children markedly reduced radiation exposure to levels far below recently reported values.

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Few reports on co-occurrence of Takayasu's arteritis (TA) and tuberculosis (Tb) have been published in childhood. A 12-year-old girl presented with 4-month's history of a dry cough, persistent fever, marked weakness, and weight loss. Physical examination revealed impalpable peripheral pulses and unrecordable blood pressure (BP) on upper limbs. In lower limbs, peripheral pulses were normal and BP reached respectively 160/90 and 140/87 mmHg. Laboratory investigations showed an erythrocyte sedimentation rate at 140 mm in the 1 ^st h and microcytic anemia (8.6 g/dl). Doppler ultrasound and computed tomography angiography revealed significant thickening of the aortic-arch and both common carotid arteries wall, with luminal narrowing of the right common carotid and its branches and severe stenosis of the left subclavian artery. Simultaneously, the diagnosis of active pulmonary Tb was achieved based on radiological data, positive Mantoux test and successful response to antitubercular drugs. During follow-up, corticostetroids and methotrexate were required to control TA relapses.

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Background: Cirrhotic cardiomyopathy (CCM) is described as the presence of cardiac dysfunction in cirrhotic patients. In children with chronic liver disease, CCM has been very rarely investigated. The Aim of the Study: Is to evaluate the cardiac function of cirrhotic children to identify those with CCM. Patients and Methods: Fifty-two cirrhotic patients and 53 age and sex matched controls were assessed using serum brain-type natriuretic peptide (BNP), conventional echocardiography, and tissue Doppler imaging. Results: Patients' mean ages were 7.66 ± 4.16 years (vs. 6.88 ± 3.04 years for the controls). The study included 27 males and 25 females (28 and 25 respectively for the controls). Patients had larger left atrium and right ventricle (RV) (P value 0.05) and increased LV posterior wall thickness than controls (P value 0.04). They had higher late atrial diastolic filling velocity (A) of tricuspid valve (TV) inflow (0.59 ± 0.17 vs. 0.5 ± 0.1 m/s P < 0.001) and lower ratios between the early diastolic filling velocity (E) and A wave velocity (E/A) of both mitral valve and TV inflow (1.7 ± 0.35 vs. 1.87 ± 0.34 and 1.3 ± 0.3 vs. 1.5 ± 0.3 P < 0.005 and 0.0008, respectively). Patients had significantly longer isovolumic relaxation time of LV (45.5 ± 11.1 vs. 40.5 ± 7.7 ms P0.008), higher late diastolic peak myocardial velocity (AͲ) (11.8 ± 3.6 vs. 9.5 ± 2.7 ms P 0.0003) and systolic velocity (SͲ) of the RV (14.5 ± 2.7 vs. 13.2 ± 2.9, P 0.01) and significantly higher myocardial performance index of both LV and RV (P 0.001 and 0.01). BNP levels were significantly higher in cases than controls (5.25 ng/l vs. 3.75 ng/l, P < 0.04) and was correlated with the E wave velocity of the TV (r 0.004) and the E/EͲ ratio of the RV (r 0.001). None of the clinical or laboratory data were correlated with the BNP level. Conclusion: Cirrhotic children have cardiac dysfunction mainly in the form of diastolic dysfunction. There is a need that CCM be more accurately described in children.

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Aortic complications occur rarely after pediatric orthotopic heart transplantation, but are typically accompanied by catastrophic events. We describe the three cases of major aortic complications in our experience of 329 pediatric heart transplants. This case series and review highlight the important risk factors for aortic complications after heart transplantation.

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Test occlusion with a balloon is done to predict operability of large hypertensive patent ductus arteriosus (PDA). If the fall in the pulmonary artery pressures is inadequate, a complete closure is not desired. To create a predictable premeasured fenestration in a nitinol occluder device used for closing hypertensive PDA. A large nitinol occluder device was punctured with an 18G needle to advance a 0.035½ stiff guide wire through the occluder before loading it into the delivery system. The occluder with the guidewire was then deployed across the PDA. A coronary guide catheter was later threaded through the guidewire into the fabric of the device, which was still held by the delivery cable. A coronary stent was deployed across the fenestration in the occluder to keep it patent. An 8-year-old boy with Down syndrome and hypertensive PDA was hemodynamically assessed. Even though there was a fall in the pulmonary vascular resistance index and pressures on test occlusion, the pulmonary artery pressures were labile with fluctuations. A customized fenestration was made in a 16 mm muscular ventricular septal defect occluder (MVSO) with a 4.5 mm bare-metal coronary stent. The pulmonary artery pressures remained at half of the aortic pressures after the procedure. This fenestration model precisely and predictably fenestrated a large occluder device used to close a hypertensive large PDA. Long-term patency of these fenestrations has to be assessed on the follow-up, and may be improved through larger fenestrations, systemic anticoagulation and use of covered stents.

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"Legal highs" such as K2, which typically contain synthetic cannabinoids, are increasingly popular with adolescents around the world. We have limited knowledge concerning their toxicity or adverse effects and their mechanism of action is poorly understood. While synthetic cannabinoids have been linked to adverse cardiovascular effects, cases of ST-elevation myocardial infarction (STEMI) associated with K2 use are exceedingly rare. We report a case of a 14-year-old boy who suffered an STEMI after smoking K2. To our knowledge, this is not only the youngest case of an STEMI associated with K2 use, but also the first case to be reported outside of the United States of America. Pediatricians worldwide must be aware of the clinical significance and potential harm associated with the use of synthetic cannabinoids, to better educate patients and their families regarding the dangers of using such "legal" substances.

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Arterial desaturation may occur after the Kawashima procedure and, in the absence of venovenous collaterals is usually due to pulmonary arteriovenous malformations. Stenting of the pulmonary arteries, oxygen therapy, and pulmonary vasodilators such as sildenafil have not been able to resolve the arterial desaturation and the only way to do this has been Fontan completion. The time course of the formation of these malformations after the Kawashima and the progression of cyanosis and its resolution after the Fontan has only been demonstrated in case reports and small case series. We pool the available data to model arterial saturations in patients with pulmonary arteriovenous malformations after the Kawashima procedure.

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A 6-year- old child developed constrictive pericarditis 2 years after undergoing an open-heart surgery for a congenital cardiac disorder. No other cause of pericarditis was identified. The clinical condition improved after pericardiectomy. The case is reported for its rarity.

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Kawasaki disease is a common nonspecific vasculitis seen in childhood. The most significant long-term sequela is coronary artery aneurysm. However, the spectrum of complications involves not only the heart, but also other organs such as the eyes, skin, kidneys, gallbladder, liver, and central nervous system. Sensorineural hearing loss (SNHL) is a relatively unrecognized complication of the disease. Although most of the complications (except coronary artery aneurysm) are self-limiting, SNHL can be persistent. It is, especially important in infants and young children who might not be able to report the hearing deficits and are most likely to have cognitive and speech delays if this hearing loss is not addressed in a timely manner. We report a child with Kawasaki disease who had SNHL during the 2 ^nd week of the illness. The aim of this article is to briefly review the pathophysiology behind this hearing loss and strongly emphasize the importance of universal hearing evaluation in all children diagnosed with Kawasaki disease. This screening in children with Kawasaki disease may provide some timely intervention if needed. Since most Kawasaki disease patients will be seen by cardiologists, we hope to create more awareness about this complication to the cardiology community as well.

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The Nitinol coil system was recently developed by "PFM" specifically for the transcatheter occlusion of ventricular septal defects (VSD). The device consists of a coil fitted with polyester fibers designated for the closure of perimembranous defects with an aneurysmal septum and some muscular VSDs. We report a case of fatal acute infective endocarditis 10 days following the procedure.

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Extracorporeal membrane oxygenation (ECMO) with a centrifugal pump requires a certain flow rate; therefore, its application for low body weight infants is frequently accompanied by oxygenator membrane malfunction and/or inadequate perfusion. To prevent low-flow associated complications, we report a case in which a novel system of dual roller pumps was used. A baby girl with a body mass index 0.25 m ² , who experienced difficulty weaning from cardiopulmonary bypass after a Norwood-like operation, required an ECMO. Concerns for the tube lifespan reduction due to roller pump friction led to the use of a double roller pump circulation. The termination of ECMO during tube exchange is not needed, because circulation is maintained by another roller pump. The novel strategy of ECMO with double roller pumps will allow low perfusion rate to provide adequate circulatory support for low body weight patients.

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In a post operative tetralogy of fallot (TOF) physiology patient, abnormal right ventricular (RV) function remains the greatest matter of concern. Due to restrictive RV diastolic dysfunction, there is detectable antegrade diastolic flow in the pulmonary artery during atrial systole. We report a case of 21 year old male patient with total correction done in infancy using right ventricle to pulmonary artery conduit. He was relatively asymptomatic with a unique pattern of antegrade diastolic flow in both early and late diastolic phases (pan diastolic). This physiology was supportive and made him relatively asymptomatic. We discuss the physiology and clinical implication of the same.

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Radiofrequency (RF) assisted perforation of the atrial septum was performed successfully in three infants using a 0.035΂ RF wire deployed through a Williams right posterior catheter. Balloon atrial septoplasty was performed over the 0.035΂ RF wire in two of them, shortening the procedural time.

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Mostly ingested button batteries passed through the gastrointestinal tract without any adverse effects. But button battery can lead to hazardous complications including tracheoesophageal fistula (TEF), especially when the battery is impacted in the esophagus. Urgent esophagoscopic removal of the battery is essential in all cases. Once the TEF is identified, conservative management is the initial treatment of choice. Delayed primary repair can be tried if spontaneous closure does not occur. Here in we want to report a rare case of air leak syndrome, pneumo-pericardium secondary to the corrosive effect of a button battery and child recovered completely with conservative management.

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Management of long standing left to right shunt lesion resulting in elevated pulmonary vascular resistance (PVR) is challenging. Limited surgical options are further complicated by an unpredictable postoperative period. Unidirectional valve patch (UVP) closure has shown to be useful in cases of the large ventricular septal defect (VSD) who present late. We report a case of large aortopulmonary window coexisting with a large VSD with severe pulmonary artery hypertension and significantly elevated PVR that was managed surgically by closure of the window by sandwich technique and closure of the septal defect with a UVP. This report emphasizes the importance of UVP in the management of such patients.

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A 6-year-old male child born with hypoplastic left heart syndrome (HLHS) was palliated with an extracardiac nonfenestrated Fontan procedure (18-mm Gore-Tex tube graft). He developed low-pressure (mean Fontan pressure 10 mmHg) protein-losing enteropathy 6 months after Fontan palliation. After initially responding to medical therapy and transcatheter pulmonary artery stent implantation, he developed medically refractory protein-losing enteropathy. At this time, his transthoracic echocardiogram showed new restriction across his native atrial septum with an 8 mmHg mean gradient. Cardiac catheterization now showed high-pressure (mean Fontan pressure 18-20 mmHg) protein-losing enteropathy and a new 6 mmHg mean gradient across the atrial septum. To avoid cardiopulmonary bypass, he underwent successful transcatheter relief of atrial septal restriction and creation of a fenestration with rapid clinical and biochemical improvement of his protein-losing enteropathy.

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