Background: Epidemiological transition with increasing burden of cardiovascular risk factors is evident not only in adults but also in children. The data on the prevalence of prehypertension and hypertension in children show large regional differences in India and such data are not available from Central India. We, therefore, conducted a large cross-sectional study in Indore to determine the distribution of blood pressure (BP) and the prevalence of hypertension and prehypertension among schoolchildren. Methods: A total of 11,312 children (5305 girls, 6007 boys) aged 5–15 years, drawn from 80 government and private schools in equal proportion, were evaluated. Anthropometric measurements were obtained and BPs were measured using The Fourth Report on The Diagnosis, Evaluation, and Treatment of High Blood Pressure in Children and Adolescents as reference standard. BP ≥90^th to <95^th percentile for given percentile of height was considered as prehypertension, whereas any BP ≥95^th percentile was defined as hypertension. Multiple linear regression analysis was used to find out the determinants of hypertension in these children. Results: Prehypertension was detected in 6.9% and 6.5% and hypertension was found in 6.8% and 7.0% of boys and girls, respectively. Height and weight were found to be a significant predictor of systolic and diastolic BP among both boys and girls. Conclusions: Our results show a high prevalence of prehypertension and hypertension in Indore schoolchildren with age and height being significant determinants. This highlights the need for routine BP measurements in children by pediatricians when they treat them for intercurrent illnesses or vaccinate them. It should also be mandatory as a part of school health checkup programs to detect childhood hypertension for further counseling and therapy.

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Computed tomography has an established role in the evaluation of a variety of cardiac disorders, including congenital heart diseases. The current generation of high-speed scanners produces volumetric data at low doses of radiation. The interpretation of cardiac anatomy, however, is generally limited to multiplanar assessment of two-dimensional images. The volume rendering technique provides an excellent three-dimensional demonstration of external morphology but offers little information about the intracardiac anatomy. The alternative approach of virtual cardiac dissection, which is a modification of volume rendering, on the other hand, provides crucial insights regarding the intracardiac anatomy. At present, virtual cardiac dissection requires expensive software packages. These software packages are not available in all countries, thus limiting its use in routine clinical care. We present here the details of a newly developed technique that permits virtual cardiac dissection using a personal computer and open-source software. Our technique involves no additional cost and can be achieved in the comfort of the office or operating room of the cardiologist, radiologist, or cardiac surgeon. This enhanced three-dimensional visualization of intracardiac anatomy will surely improve the understanding of the morphological details of both normal and malformed hearts. In addition, by permitting assessment in projections with which modern-day cardiologists and cardiac surgeons are conversant, it is likely to improve clinical decision-making. We illustrate here its potential utility in the morphologic assessment of the atrial septum and its deficiencies, along with malformations of the ventricular outflow tracts, including common arterial trunk.

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Background: Severe pectus excavatum in children may result in cardiorespiratory functional impairment; therefore, we evaluated cardiopulmonary response to exercise before and after the Nuss procedure. Methods: Twenty-four physically active pediatric patients aged 9–18 years with severe pectus excavatum (Haller index >3.25) were included in the study. Cardiopulmonary exercise testing using treadmill and modified Bruce protocol was performed before and after the Nuss procedure. Results: Maximal oxygen uptake and oxygen pulse improved by 40.6% (32 ± 13–45 ± 10 ml/kg/min; P = 0.0001) and 44.4% (9 ± 4–13 ± 5 ml/beat; P = 0.03), respectively, after surgical correction of pectus excavatum by Nuss procedure. Significant improvement in maximum voluntary ventilation and minute ventilation after Nuss procedure was also noted. Conclusions: We found that, after repair of pectus excavatum by Nuss procedure, the exercise capacity as measured by maximal oxygen consumption improved significantly primarily due to increase in oxygen pulse, an indirect measurement of stroke volume.

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Background: Complete atrioventricular septal defect (CAVSD) in association with tetralogy of Fallot is a rare and complex disease that makes its repair more difficult than repair of either lesion alone. We reviewed retrospectively our experience in managing this lesion. Patients and Methods: Between February 2006 and May 2017, 16 patients who underwent repair of CAVSD/tetralogy of Fallot (TOF) were reviewed retrospectively. Fifteen patients had trisomy 21. Five patients underwent primary repair while eleven patients went for staged repair in the form of right ventricular outflow tract (RVOT) stenting (n = 9) or systemic to pulmonary (S-P) surgical shunt (n = 2). RVOT stenting has replaced surgical shunt since 2012 in our center. Early presentation with cyanosis was the main determinant factor for staged versus primary repair. Results: The median age at first palliation was 46 days (range 15–99 days). The median age at total repair for both groups was 6 months (range 3–18 months); the median age for the palliated patients was 6.5 months (range 5–18 months) while the median age for primary repaired patients was 5 months (range 3–11 months). The median weight at final repair was 6.9 kg (3.7–8.2 kg). The pulmonary valve was preserved in five patients (31%), four of them had no prior palliation. Chylothorax occurred in 50% of the patients. One late mortality occurred after final repair due to sepsis. Conclusion: CAVSD/TOF can be repaired with low mortality and morbidity. The use of RVOT stent has replaced the surgical (S-P) shunt with acceptable results in our center.

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Unbalance in atrioventricular septal defect can be found in more than one anatomic level and in different degrees at each level. The definition of “unbalance” has historically been focused in comparing the dimensions of main cardiac structures, such as the atrioventricular valve and the ventricles. However, the hemodynamic aspects of unbalance need to be considered as having, at least, similar relevance. New concepts and already described parameters must be combined and understood as a whole to help the surgical decision-making process.

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Background: Dyslipidemias have increased during the last decades in children. Aim: The objective of this study was to analyze the influence of different polymorphisms in plasma triglyceride levels of children following a dietary treatment with plant sterols. Design: A randomized, double-blind, crossover, controlled clinical trial was carried out in 26 children (16 women). Materials and Methods: Commercial milk, with 2.24 g sterols, was ingested daily during 3 weeks , and the same amount of skimmed milk without sterols, during the 3 week placebo phase. Both phases were separated by a washout period of 2 weeks. At the beginning and end of each phase, blood draws were performed. Results: Apolipoprotein A5 Ser19Trp (P = 0.002), peroxisome proliferator-activated receptor-alpha L162V (P = 0.003), APOE APOE2/3/4 (P = 0.012), and APOE APOE2,3,4 (P = 0.025) show statistically significant differences between their haplotypes in plasma triglyceride levels. Other genes did not show statistically significant differences. Conclusions: Further studies are needed to establish which genotype combinations would be the most protective against hypertriglyceridemia.

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Objective: The objective of the study is to report the experience with acetaminophen in low doses as an alternative to the treatment of the ductus arteriosus of the preterm newborn. Materials and Methods: Retrospective study including preterm newborns with patent ductus arteriosus who received oral acetaminophen because treatment with indomethacin had failed or is contraindicated. A dosage consisted of a first dose of 25 mg/kg and maintenance doses of 30 mg/kg/day, for 3 to 7 days. A second cycle was administered in cases of reopening of the ductus arteriosus. The rates of ductal closure and surgery were calculated. Patients were categorized into responder and nonresponder groups for acetaminophen, and the average values of ductal diameter, weight, gestational age, and postnatal age were compared. Results: Eighty-seven preterm newborns, with a postnatal age from 3 to 27 days, with average values of ductus arteriosus equal to 2.5 ± 0.8 mm/kg, gestational age 27.2 ± 1.9 weeks, and birth weight 888.9 ± 241 g, received acetaminophen for 3 to 7 days. A second cycle was administered in 15 preterm newborns. The ductus closure rate, after one or two cycles, was 74.7%, and the recommendations for surgical closure were progressively reduced from 50% in the 1 st year to 6.2% in the past year. Lower ductal closure rate occurred in the group of newborns with the lowest average weight (P = 0.018), the highest average ductal diameter (P = 0.002), and the lowest average gestational age (P = 0.09). Postnatal age at the start of acetaminophen use was shown to be irrelevant regarding the treatment (P = 0.591). Conclusions: Acetaminophen in low doses showed to be an effective alternative for the closure of the ductus arteriosus for preterm newborns in whom treatment with indomethacin or ibuprofen failed or was contraindicated.

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Isolation of the left brachiocephalic artery (ILBA) is an extremely rare anomaly of aortic arch with diverse manifestations in the neurologic system, heart, and left upper arm. This anomaly is defined as the absence of connection of the left brachiocephalic artery (LBA) to aortic arch and connection of LBA to pulmonary artery (PA) through a patent arterial duct (PAD). However, this definition is not inclusive of all cases. Not only are there inconsistencies in the definition and terminology of this aortic arch anomaly but also there is no classification for this anomaly despite its heterogeneous nature in terms of anatomy, clinical presentation and prognosis. We performed a 52-year comprehensive literature review in the period between 1966 and 2018. Our inclusion criteria were any manuscript that included a case report or case series, with confirmed diagnosis of ILBA. All quantitative data were analyzed using descriptive analysis by SPSS version 21 (IBM SPSS Statistics, USA). Results were presented as mean ± standard deviation and median. Based on the presence or absence of connection of LBA to PA and the number of sources of steal from the LBA, we classified ILBA into three types: single-steal type with no connection of LBA to PA and single source of blood flow steal from LBA through the left subclavian artery (LSCA), double-steal type with connection of LBA to PA through PAD and two sources of steal through LSCA and arterial duct (AD), and triple-steal type with bilateral PADs and therefore, three sources of blood flow steal from LBA including the LSCA and the double ADs. Patients with single-steal type have the best prognosis and present latest with symptoms of cerebrovascular insufficiency or left arm claudication. The oldest reported patient was 69 years of age with symptoms of dizziness and near syncope. No death was reported in these patients. Double-steal type is the most common type and is often associated with genetic syndromes and/or extracardiac anomalies. Triple-steal type is the rarest type with the earliest presentation and worst prognosis. The oldest reported patient was 60 days of age. All reported cases had cardiac symptoms, pulmonary overcirculation, pulmonary hypertension, and fatal outcome.

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Left subclavian artery (LSA) stenosis causing limb length shortening or vascular compromise or significant pulse volume and blood pressure differences between two upper limbs are rarely described in pediatric patients. Here, we are presenting three such cases, two of them were congenital and another one acquired in origin. All of them were successfully treated with transcatheter implantation of stent in LSA. Normalization of limb length was detected on follow-up.

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We report the use of the Abbott three-dimensional mapping system (EnSite Precision) for the placement of an atrial pacemaker lead in a patient after modified Fontan procedure. The mapping system was used for the identification of a promising pacing site in the complexly modified right atrium and to visualize the successful placement of an atrial lead at the same spot.

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A 6-month-old infant with moderate-sized patent ductus arteriosus (PDA) and inadequate weight gain underwent closure of the duct using Amplatzer Ductal Occluder II (ADO II). She developed severe progressive left pulmonary artery (LPA) stenosis due to protrusion of the disc at the pulmonary end of the ADO II. She was subjected to balloon angioplasty of the LPA stenosis with suboptimal result. Hence, she was subjected to stenting of the LPA using a Formula stent which could be subsequently postdilated to keep up with the growth of the child. Immediate and short-term results were excellent anatomically as well as physiologically.

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Cerebral oximeter is a noninvasive device which provides continuous monitoring of the regional cerebral saturation using near-infrared spectroscopy (NIRS). After gaining popularity as an intraoperative monitoring tool, use of NIRS monitoring has also expanded to postoperative period of congenital heart diseases now. Shunt underflow is a known complication after Blalock–Taussig (BT) shunt, which is conventionally detected by a drop in oxygen saturation and metabolic acidosis. We report a case where cerebral regional saturation monitoring by NIRS helped in early detection of low pulmonary flow state during postoperative period of neonatal BT shunt. We observed that the drop in regional cerebral oxygen saturation preceded fall in peripheral oxygen saturation during shunt underflow.

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An unguarded atrioventricular orifice is an extremely rare congenital anomaly characterized by the absence of the atrioventricular valve in varying proportions. While atresia of the mitral or aortic valves are usually described as causes for hypoplastic left heart, our case highlights the role of free atrioventricular valve regurgitation and consequent volume loss of the left heart, giving rise to a small left ventricle. There was an associated double-outlet right ventricle and Type B aortic interruption. While we have attempted to discuss the complex management options in this scenario, the parents decided to withdraw further care.

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Transseptal puncture is a routine technique to access the left atrium during catheter ablation, but carries a small risk of perforation into the pericardium or aorta. When conservative management fails, surgical repair is usually needed. We report an 81-year-old male in whom aortic root perforation occurred after transseptal puncture and was treated successfully by percutaneous deployment of an Amplatzer Duct Occluder II device.

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Transcatheter therapy for partial anomalous pulmonary venous connection with dual drainage is unique and rarely reported. We report a 69-year-old female with recurrent brain abscess and partial anomalous connection of the left upper pulmonary vein with dual drainage to the vertical vein (VV) and left atrium (LA). Transcatheter occlusion of the VV was done using an 18-mm St. Jude Amplatzer Vascular Plug II, thus redirecting the left-sided pulmonary venous drainage to LA. Careful evaluation of partial anomalous pulmonary venous drainage with cross-sectional imaging is essential to allow the delineation of dual connections, enabling a less invasive transcatheter treatment approach.

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We report two cases of agenesis of the right pulmonary veins (PVs) associated with sequestration of the right lung with systemic to pulmonary collateral. Both the children were referred for evaluation of recurrent lower respiratory tract infections. Transthoracic echocardiographic evaluation showed reversal of flow in the right pulmonary artery (PA) and absent right PVs. Cardiac catheterization confirmed the diagnosis of agenesis of the right PVs. Transcatheter closure of collaterals was performed in view of significant shunt from systemic to PA. Both the patients are asymptomatic on follow-up.

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Coarctation of the aorta (CoA) represents 5%–8% of congenital heart disease patients and is one of the most common causes of neonatal surgical intervention. These patients require close lifelong follow-up due to frequent long-term complications. Although transthoracic echocardiography is the first-line technique for its diagnosis and follow-up, cross-sectional imaging with cardiovascular magnetic resonance (CMR) gives excellent anatomical and functional information, especially in complex CoA. We present the case of a 17-year-old patient who underwent complicated neonatal CoA repair and demonstrate how CMR and thorough operative records helped to define the exact anatomy of repair many years after surgery. Furthermore, we conclude that keeping surgical drawings in the patient records can be of great importance, especially in complicated cases.

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Isolation of the left common carotid artery (LCCA) is a very rare congenital aortic arch anomaly. We present this finding in a female infant with pulmonary atresia and intact ventricular septum, with a clinical diagnosis of CHARGE syndrome. Cardiac catheterization revealed an anomalous origin of the LCCA from the pulmonary trunk, with retrograde filling of the pulmonary trunk seen during left subclavian artery injection. The LCCA was ligated during central shunt placement.

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The usual sources of pulmonary blood flow in pulmonary atresia (PA) with(VSD) are patent ductus arteriosus and aortopulmonary collaterals. However, rarely fistulous collaterals may also arise from the coronary arteries which usually open into the main pulmonary trunk or branch pulmonary arteries. In such cases, selective coronary angiogram may be required for the demonstration of pulmonary arterial anatomy. A case of PA with VSD with failure to demonstrate pulmonary arteries on routine catheterization study (ventricular, aortic root, and descending aortic angiograms) is being presented here. A coronary artery-to-pulmonary artery fistula was suspected in view of dilated left main coronary artery, and pulmonary arteries were well demonstrated with selective coronary angiogram.

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Intrapericardial teratoma is a germ-cell tumor that typically arises from the base of the heart and usually diagnosed in the fetal or neonatal period. Although benign, these tumors can be massive in size causing direct compression of the heart. Life-threatening complications such as fetal hydrops, cardiac failure, superior vena cava syndrome, and cardiac tamponade caused by these teratomas have been reported. Early surgical excision is curative. We present the images of a mature intrapericardial teratoma diagnosed in an asymptomatic neonate. The neonate was managed successfully by elective surgical excision.

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Kawasaki disease (KD) is a systemic vasculitis commonly associated with coronary artery aneurysms. Small-sized and medium-sized systemic arterial aneurysms have also been described, particularly in infants and patients with resistant KD. This case illustrates the presentation of a systemic arterial aneurysm complicated by arterial thrombosis and successful interventional management in a young infant.

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Persistence of the embryological fifth aortic arch in postnatal life has been a subject of debate for over more than a century. We describe a patient with congenital kyphoscoliosis and variant anatomy in the aortic arch and brachiocephalic trunk which could be possibly explained by the persistence of bilateral fifth aortic arches, a pattern which has never been previously reported in literature.

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AMPLATZER™ muscular ventricular septal defect occluder is used commonly for off-label purposes. We describe an unusual case of a patient with tetralogy of Fallot who underwent repair with a right ventricle to pulmonary artery homograft due to abnormal coronary artery pattern. During the initial surgery, the native right ventricular outflow tract was left open. At 30 years of age, he was symptomatic due to severe native right ventricular outflow tract insufficiency. Cardiac MRI confirmed a dilated right ventricle and pulmonary insufficiency through the native right ventricular outflow tract, despite no significant homograft insufficiency due to previous Melody^® valve placement. The right ventricular outflow tract was closed successfully using an 18 mm AMPLATZER™ muscular ventricular septal defect occluder. At 5-year follow-up, there is no native right ventricular outflow tract insufficiency and no additional arrhythmia. We suggest that percutaneous closure of the insufficient; native right ventricular outflow tract using a septal occluder is an alternative to surgical management.

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We report a unique case of dilated aortic root and pulmonary artery in an infant with clinical features consistent with Beals syndrome confirmed to have fibrillin-2 mutation. This case highlights a novel finding of main pulmonary artery dilatation that has not been previously reported with Beals syndrome or fibrillin-2 mutation. In addition, the importance of serial echocardiography and consideration of medical management is discussed.

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