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Standard treatment of acute Kawasaki disease involves giving 2 grams per kilogram of immunoglobulin intravenously along with aspirin. More than half of the patients with acute Kawasaki disease, nonetheless, can be cured by giving only 1 gram per kilogram of immunoglobulin, thus reducing this aspect of the cost of treatment by half. Our purpose was to predict those patients with acute Kawasaki disease who would respond to treatment with 1 gram per kilogram of immunoglobulin given intravenously on the basis of their clinical profiles and laboratory findings prior to the initial treatment. We performed a retrospective review of the clinical records of consecutive patients with acute Kawasaki disease treated in our hospital with intravenous immunoglobulin from January, 2001, to December, 2005.
During this period, we treated in this fashion 98 patients with acute Kawasaki disease. 65% of these needing immunoglobulin therapy were cured by giving 1 gram per kilogram. The neutrophil count and the percentage of white blood cells representing neutrophils, along with aspirate aminotransferase, alanine aminotransferase, bilirubin and C reactive protein, were all significantly lower, and sodium was significantly higher, in those responding to 1 gram per kilogram of immunoglobulin when compared to those who did not respond. The days of illness at the first intravenous treatment was later in those responding than in those failing to respond. We generated a score for prediction, assigning a point for each of C reactive protein equal to or greater than 10 mg/dl, sodium equal to or lower than 133 meq/l, alanine aminotransferase equal to or greater than 110 IU/l, and 2 points for the percentage of white blood cells representing neutrophils equal to or greater than 70%. Using a cut-off point of a score less than 2, we were able to identify those responding with 60% sensitivity, and 91% specificity.
Thus, we are now able to predict those patients with acute Kawasaki disease who will respond to immunoglobulin given intravenously at 1 gram per kilogram using laboratory data, with a potential saving in medical costs.
Epicardial pacing is the standard approach for permanent pacing in small children and patients with functionally univentricular physiology. The longevity of epicardial leads, however, is compromised by increased occurrences of exit block and lead fractures. We report our experience with a technique of placing a second ventricular lead, and attaching it to the atrial port of a dual chamber pacemaker to prevent the need for early re-operation in the event of failure of the primary epicardial lead. A retrospective review showed that, over the period from 2001 through 2007, epicardial ventricular pacemakers had been placed in 88 patients. In 6 of these, we had placed 2 ventricular leads, their median weight being 8.0 kilograms, with a range from 4.2 to 31.8 kilograms. Fracture of a lead occurred in 1 of the patients (17%) 8 months after placement, requiring reprogramming to pace from the atrial port. This possibility avoided the need for repeated emergent surgery. At a median follow-up of 1.5 years, with a range from 0.3 to 4.4 years, there have been no complications. During the same time period, overall failure of epicardial leads at our institution was 13%. Placement of a second ventricular epicardial pacing lead, attached to the atrial port of a dual chamber pacemaker, therefore, may provide a safe and effective means of ventricular pacing in the setting of epicardial lead failure, and may obviate the need for repeat, potentially urgent, pacemaker surgery.
The Sardinian Hypertensive Adolescents Research Programme Study, which for the sake of simplicity we will describe as SHARP, was aimed at detecting the prevalence of hypertension in a number of Southern Italian students, using a process of longitudinal screening lasting 3 years, hoping to answer the question whether it is better to use tables charting values established in the United States of America, or to use charts specific for the Italian population.
In all, we studied 839 children, of whom 52.6% were male. We defined hypertension as the average blood pressure exceeding the 95th percentile according to previous tables prepared by the United States Task Force, and previous Italian references.
Use of the American tables identified very high proportions of hypertensive subjects if compared with the distribution curves from our own study (p less than 0.00001), albeit that our findings correlated well with previous Italian charts as reference (no statistical significance).
In short, notwithstanding a little difference in millimetres of mercury about the same percentiles, the tables prepared in the United States of America overestimate the prevalence of hypertension, specific Italian material being more suitable for our needs. Our study emphasises the need to integrate these standards with more up-to-date and representative reports on Italian children, as is done periodically in the United States of America. Even using the Italian specific charts, nonetheless, hypertension was more common in Sardinian children than would be expected from international studies, with one-tenth of the sample being hypertensive.
Our aim in this part of the Sardinian Hypertensive Adolescents Research Programme, also known as the SHARP study, was to use longitudinal screening over a period of 3 years to search for any relationship between hypertension and excessive weight in a number of Southern Italian students. We also sought to establish if this correlation can change according to the criterion used to define children considered to be overweight.
We studied 839 children, of whom 52.6% were male, defining hypertension as an average blood pressure exceeding the 95th percentile according to previous Italian reference tables. We defined those overweight using different criterions, first those with a body mass index exceeding 25 kilograms per square metres, second those with the index exceeding the 85th percentile, third using the references established by Rolland-Cachera, and fourth according to the relative body weight. The different methods used in defining overweight produced very different estimates, ranging from 8.9% to 26.4%. Our novel findings were that systolic hypertension was present in all children deemed overweight using any of the criterions, but only use of the second and third criterions produced results related significantly to both systolic and diastolic hypertension. In short, excessive weight is strongly associated with systolic hypertension in adolescence. Definition of those being overweight on the basis of a body mass index exceeding the 85th percentile, or using the references established by Rolland-Cachera, proved to be best related with both systolic and diastolic hypertension.
Neonatal aortic coarctation presenting with multiorgan failure is a life threatening condition.
Our aim was to investigate whether emergency balloon dilation improved the prognosis of neonates with aortic coarctation who present with multiorgan failure.
We studied all neonates referred over a period of 6 years with aortic coarctation and left ventricular dysfunction, with or without multiorgan failure. During the first half of the period, from January, 2001 to December, 2003, patients were treated surgically after regression of the multiorgan failure. During the second half, from January, 2004, to January, 2007, patients not responding to medical management were referred for balloon dilation of the coarcted segment.
Among 113 and 181 neonates, respectively, treated during the 2 periods, 40 and 36 had left ventricular dysfunction (p less than 0.01), and 11 and 23 had multiorgan failure (p less than 0.01). Prior to 2004, 6 deaths occurred before surgery. After 2004, 3 out of 14 patients (21%) treated by balloon angioplasty died in consequence of the procedure.
During the period of the study, the global mortality of patients with left ventricular dysfunction diminished from 17.5 to 13.8%, this difference not being statistically significant. Mortality due to multiorgan failure, however, diminished from 54 to 13% (p less than 0.01). Multiorgan failure was reversed under medical treatment in 45% of patients, but in 87% under the regime of medical combined with interventional treatment (p less than 0.01).
To the best of our knowledge, this is the first series of neonates with aortic coarctation and multiorgan failure receiving a palliative treatment by balloon dilation. Although the prognosis of this condition remains severe, emergency balloon dilation can diminish mortality, providing a bridge to surgery in severely ill patients.
The purposes of our study were, first, to determine the prevalence of dental caries in young patients with congenital cardiac disease, second, to determine the effect of oral health in their quality of life, third, to examine parental knowledge about associations between oral health and cardiac health, and fourth, to examine parental perceptions regarding access to dental care. A standardized questionnaire was given to a convenience sample of parents of 43 children with cardiac disease and 43 healthy controls from 12 to 71 months of age. In every patient, we performed a dental examination. Descriptive statistics, the two-tailed t-test, and Fisher’s exact test were used for statistical analysis. Of the children with cardiac disease, 17% had caries, compared to 13% of the control group. Almost half of those with cardiac disease had never seen a dentist, compared to 35% of the control subjects. Of the parents of those with cardiac disease, one-fifth did not know whether oral health was important for the heart, compared to one-tenth of controls. Insurance and access to care were not barriers to obtain dental treatment. Statistically significant differences were, first, parents of children with congenitally malformed hearts felt more guilt about their child’s oral health than control parents (p = 0.026), second, they were more upset about the dental problems and/or treatments of their children (p = 0.012), and third, they thought that dental problems and/or dental treatment made their children more irritable (p = 0.012). Our findings indicate that it is of paramount importance that cardiologists and their associated staff educate patients and families about oral health and other issues associated with congenital cardiac disease.
Familial hypercholesterolemia is a monogenic, autosomal dominant disorder caused by mutations in the LDL receptor gene. Familial homozygous hypercholesterolemia results when both the alleles have the defective mutation. It is characterized by cutaneous and tendinous xanthomas, premature corneal arcing, and is associated with an increased risk of coronary arterial disease. It is also seriously underdiagnosed, resulting in delayed treatment.
We present a cross-sectional study of 5 patients with familial homozygous hypercholesterolemia who presented to the department of cardiology at Sri Jayadeva Institute of Cardiology, Bangalore, India. All of them underwent coronary angiography as part of the investigation of their angina.
All 5 patients were in 2nd or 3rd decade of life, 4 being male, and 4 presenting with effort angina, the other having unstable angina. All had multiple tendinous xanthomas. The majority had significant high grade coronary arterial stenosis. Coronary arterial bypass grafting was necessary in 3, with the others undergoing percutaneous insertion of coronary arterial stents.
Familial homozygous hypercholesterolemia is a potentially dangerous risk factor that can result in premature coronary arterial disease in children and young adults. This can result in severe morbidity and premature death in young individuals. We also emphasise the need to screen first-degree relatives and extended family members, this playing an important role in early detection and treatment. Despite recent advances in treatment using lipid lowering agents, the disease remains a significant challenge.
Patients with Turner’s syndrome are at risk of aortic dilation and dissection. Currently, it is not known whether such dilation is related to associated cardiovascular abnormalities, or to the genetic anomaly itself.
We studied echocardiographically 107 patients with genetically proven Turner’s syndrome, with heterogeneous underlying karyotypes, and without associated cardiac lesions. Their average age was 19.6 plus or minus 8.4 years. We compared the finding with those from 71 age-matched healthy female volunteers. The diameter of the aorta was measured at the level of the basal attachments of the aortic valvar leaflets, the sinuses of Valsalva, the sinutubular junction, and its ascending component.
Compared to control subjects, the patients with Turner’s syndrome had larger diameters of the aorta at the level of the sinuses of Valsalva, at 23.4+/−4.8 versus 25.5+/−4.1 millimetres (p = 0.0014), the sinutubular junction, at 19.9+/−3.8 versus 23.3+/−4.1 millimetres (p < 0.0001), and the ascending aorta, at 22.3+/−4.9 versus 24.6+/−4.4millimetres (p = 0.0011). Dilation of the sinutubular junction, found in just over one-quarter of the patients, was more common than dilation of the ascending aorta, the latter found in less than one-tenth. The patients with Turner’s syndrome, therefore, presented with remodelling of the aortic root, with relative dilation of the sinutubular junction. The underlying karyotype influenced both the dimensions of the sinutubular junction (p = 0.0054), and the ascending aorta (p = 0.0064), so that patients with the karyotype 45X had larger aortas. The karyotype was the strongest predictor by multivariate analysis for dilation at both these sites (p = 0.0138 and 0.0085, respectively).
Dilation at the sinutubular junction is frequent in patients with Turner’s syndrome, and is more common than dilation of the ascending aorta. The syndrome is associated with a remodelling of the aortic root, with prominent dilation of the sinutubular junction. There seems to be a relation between aortic dilation and the underlying genotype.
There is a high incidence of scoliosis in patients who have undergone cardiothoracic surgery for correction of congenital cardiac disease, this risk being 10 times higher than in the general population.
Materials and methods
So as to analyse the surgical and postoperative complications, we designed a retrospective study to include every child who underwent spinal orthopaedic surgery, and who had previously undergone cardiothoracic surgery because of a congenital cardiac malformation. We excluded those patients who had syndromes associated with the development of scoliosis.
We identified 18 patients with surgically treated congenital cardiac disease who had undergone surgery for scoliosis over a period of 7 years. This group came from a total number of 87 patients undergoing spinal fusion over the same period. Of those with congenitally malformed hearts, 61% had acyanotic lesions, with ventricular septal defect being the most frequent single lesion, present in 40%. All the patients needed blood transfusions during the surgery, with aprotinin used in 73% to reduce the bleeding, and inotropes needed for 4 children. During the immediate postoperative period, 1 patient died in the first 24 hours, while 7 (39%) had different complications, pneumonia in 4, pleural effusions in 2, and rhabdomyolysis in the other, as opposed to a rate of complications of 27% in patients without heart disease.
The surgical and postoperative complications in these patients depend on the specific cardiac lesion. A multidisciplinary team with experience in the treatment of congenitally malformed hearts is essential for appropriate management of these patients.
To report our experience with balloon dilation of critical aortic valvar stenosis in neonates via the umbilical artery using currently available catheters.
There is no agreement regarding the optimal vascular approach for balloon dilation of critical aortic valvar stenosis in neonates.
Since June of 2005, we have attempted to obtain umbilical arterial access within the first week after birth in all neonates with critical aortic valvar stenosis. In patients in whom umbilical artery access was obtained, we proceeded with an attempt at balloon dilation.
We were presented with 5 patients with critical aortic valvar stenosis within the first week after birth, and the umbilical arterial approach was obtained in all, with effective relief of the stenosis achieved in 4.
The umbilical arterial approach should always be considered for balloon dilation of neonatal critical aortic valvar stenosis. Using currently available catheters, the procedure is safe, simple, and effective even in patients weighing less than 2.5 kilograms. Further experience using this approach is warranted.
to analyse retrospectively the data of fetuses diagnosed with isolated complete atrioventricular block and efficacy of treatment of the fetus by maternal therapy.
Between 1992 and 2004, we diagnosed complete atrioventricular block in 26 singleton and 2 twins fetuses of 27 pregnant women known to have anti Ro/La antibodies, 11 with autoimmune disease, one patient analysed in 2 pregnancies. At presentation, 20 of the fetuses were compensated and non-hydropic, while 8 had hydrops. Twenty patients were treated with dexamethasone, 2 with associated salbutamol and one mother with isoproterenol.
Age at presentation was not different between the hydropic and non-hydropic fetuses. The fetuses with hydrops, however, had a lower mean heart rate at presentation, 48.5 ± 9.25 with a range from 32 to 60, compared to 59.95 ± 7.9 beats per minute, with a range from 50 to 80, in the non-hydropic fetuses (p less than 0.002). Equally, after birth the mean heart rate in hydropic fetuses was 42.6 ± 5.1, with a range from 38 to50, as opposed to 56.05 ± 11.8 beats per minute, with a range from 29 to 110, in the non-hydropic fetuses (p less than 0.015), The hydropic fetuses were delivered at 31.7 ± 3.8 weeks’ gestation, with a range from 29 to 38 weeks (p less than 0.003) compared to 35.5 weeks’ gestation ±2.04, with a range from 31 to 38, in the non-hydropic fetuses. Mortality was 37.5% in the hydropic fetuses, versus 5% of those without hydrops (p less than 0.02). Pacemakers were implanted in 22 of 26 infants born alive, at a median of 45 days, with a range from 1 day to 5 years, in those without hydrops during fetal life, and 3 days, with a range from 1 day to 8 months in those afflicted by hydrops, of whom 2 died despite the implant of the pacemaker. The presence and degree of hydrops had a significantly negative predictive value. No significant differences were observed between the treated and non treated cases, albeit that administration of steroids ameliorated rapidly the hydrops in 3 of 5 cases.
The outcome in our cases was mainly dependent on the presence and degree of fetal cardiac failure. Treatment of the fetus by maternal administration of steroids did not result in any regression of the conduction disorder, but had a favourable effect on fetal hydrops.
Atrioventricular block is an important complication after percutaneous closure of perimembranous ventricular septal defects. In the majority of cases, it appears in early or in the midterm of closure. We present a patient who developed symptomatic Mobitz 2 second-degree atrioventricular block 3 years following percutaneous closure of such a defect.
We describe an atypical myxoma in the right ventricular outflow tract of a 12-year old girl. After identification using echocardiography, we assessed its precise location and attachments by magnetic resonance imaging. Subsequent to surgical removal, histology confirmed the myxomatous nature of the tumour.
We describe the findings in a neonate with aortic atresia, interruption of the aortic arch between the carotid arteries, and aortopulmonary window. This unusual association changes the anticipated physiology, and helps in the survival. We emphasize the fact that this combination simulates common arterial trunk, both clinically and on echocardiography.