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Cryptogenic cerebral ischemia in young patients is commonly ascribed to paradoxical embolism. We report the clinical case of a young patient with cryptogenic stroke and a patent foramen ovale, undergoing percutaneous closure of atrial septal defect. Contrast transoesophageal echocardiography at the end of the procedure demonstrated massive late residual right-to-left shunt, due to the coexistence of pulmonary arteriovenous fistulas that were subsequently closed. Routinary adoption of contrast transoesophageal echocardiography at the end of patent foramen ovale closure interventions may be useful to detect early and late residual shunts. Late residual shunts may be due to pulmonary fistulas, a well-known risk factor for recurrent thromboembolic events.
Anthracycline cardiotoxicity is an important side-effect in long-term childhood cancer survivors. We evaluated the incidence of and factors associated with anthracycline cardiotoxicity in a population of patients diagnosed with bone or soft tissue sarcoma.
Materials and methods
We retrospectively enrolled patients diagnosed with bone or soft tissue sarcoma, from 1995 to 2011, treated with anthracycline chemotherapy at our Centre and with a follow-up echocardiography carried out ⩾3 years from cardiotoxic therapy completion. Cardiac toxicity was graded using Common Terminology Criteria for Adverse Events version 4.0.
A total of 82 patients were eligible. The median age at treatment was 11.9 years (1.44–18). We evaluated the median cumulative anthracycline dose, age at treatment, sex, thoracic radiotherapy, hematopoietic stem cell transplantation, and high-dose cyclophosphamide treatment as possible risk factors for cardiotoxicity. The median cumulative anthracycline dose was 390.75 mg/m2 (80–580). Of the 82 patients, 12 (14.6%) developed cardiotoxicity with grade ⩾2 ejection fraction decline: four patients were asymptomatic and did not receive any treatment; six patients were treated with pharmacological heart failure therapy; one patient with severe cardiomyopathy underwent heart transplantation and did not need any further treatment; and one patient died while waiting for heart transplantation. The median time at cardiac toxicity, from the end of anthracycline frontline chemotherapy, was 4.2 years (0.05–9.6). Cumulative anthracycline dose ⩾300 mg/m2 (p 0.04) was the only risk factor for cardiotoxicity on statistical analyses.
In our population, the cumulative incidence of cardiotoxicity is comparable to rates in the literature. This underlines the need for primary prevention and lifelong cardiac toxicity surveillance programmes in long-term childhood cancer survivors.
Data regarding long-term outcome after percutaneous closure of left superior caval vein draining into the left atrium are lacking. The aim of the present study was to report the long-term follow-up by using contrast-enhanced CT.
In all, three patients underwent percutaneous closure of left superior caval vein draining into the left atrium between 2005 and 2015. All of them were evaluated clinically and underwent contrast-enhanced CT.
In one patient, the Amplatzer® Septal Occluder was used. In two patients, the Amplatzer® Vascular Plug type-1 was preferred: the device size/LSVC diameter ratio was 1.7 in the child and 1.2 in the adult. There were no early-onset or long-term onset complications. CT was performed 1, 2, and 10 years after the procedure, respectively. Complete occlusion of the vessel was documented in all. After 10 years since the procedure, CT revealed a persistent trivial residual shunt through the accessory hemiazygos vein in one patient, in whom the device was implanted above its drainage into the left superior caval vein. When an Amplatzer® Vascular Plug type-1 is oversized compared with the venous vessel diameter, it immediately assumes a dog-bone shape that disappears early to regain its shape memory and nominal size.
Percutaneous occlusion of left superior caval vein draining into the left atrium has excellent early and long-term outcomes. The optimal implantation of the device is below the drainage of the accessory hemiazygos vein, when present. The device might be oversized compared with the left superior caval vein diameter according to the age of the patient.
We report the case of a young boy with a history of total occlusion of the superior caval vein, diagnosed early after complex neonatal cardiac surgery, who developed severe protein-losing enteropathy. Protein-losing enteropathy was precipitated by a relatively mild stenosis at the junction of the inferior caval vein with the right atrium. Percutaneous stent dilation of the veno-atrial junction definitively relieved the stenosis, and the protein-losing enteropathy subsequently resolved.
We report two cases with levoatriocardinal vein and partial anomalous pulmonary venous drainage in left-sided obstructive lesions. This association may be difficult to recognise by echocardiography. Cardiac CT and MRI were crucial to define the diagnosis and to tailor the best therapeutic option.
We present the case of a 15-year-old boy who underwent arterial switch operation due to transposition of the great arteries with severe scoliosis, obstruction of the right coronary ostium, and severe stenosis of the pulmonary trunk. Balloon angioplasty caused a large aortopulmonary shunt provoking myocardial ischaemia and pulmonary hypertension. The traumatic “aortopulmonary window” was percutaneously occluded using an Amplatzer Septal Occluder device as a bridge to surgical repair.
The aim of our study was to establish the prevalence and the prognostic value of haematological abnormalities in children with cardiac failure.
Patients and methods
A series of 218 consecutive children with a first diagnosis of idiopathic dilated cardiomyopathy were retrospectively examined. Haematological evaluation was performed at first diagnosis. Death or cardiac transplantation was the main outcome measure.
The median age was 0.6 years, ranging from 1 day to 15.8 years and median follow-up was 2.65 years, ranging from 0 to 17.2 years. After a median interval of 0.2 years, ranging from 0 to 8.7 years, 56 patients died and 25 were transplanted. Event-free survival at 1 and 5 years was 68% (95% confidence interval, 63–75%) and 62% (95% confidence interval, 56–69%). Blood levels of haemoglobin less than 10 grams per decilitre, urea over 8 millimoles per litre, and C-reactive protein over 10 milligrams per litre were found in 24%, 20%, and 24% of patients, respectively. The log-rank test showed that haemoglobin (p = 0.000) and C-reactive protein (p = 0.021) were predictors of death or transplantation. In the multivariate Cox model, haemoglobin (hazard ratio = 0.735; confidence interval = 0.636–0.849; p = 0.000) and urea (hazard ratio = 1.083; confidence interval = 1:002–1:171; p = 0.045) were predictive of poor outcome. Cubic spline functions showed that the positive role of haemoglobin on survival was linear for values less than 12 grams per decilitre and null for values more than 12 grams per decilitre. Adaptive index models for risk stratification and Classification and Regression Tree analysis allowed to identify the cut-off values for haemoglobin (less than 10.2 grams per decilitre) and urea (more than 8.8 millimoles per litre), as well as to derive a predictor model.
In children with idiopathic dilated cardiomyopathy, anaemia is the strongest independent prognostic factor of early death or transplantation.
We report the case of a child with severe and atypical stenosis of the left main coronary artery, which occurred late after arterial switch operation for transposition of the great arteries. Cardiac computed tomography accurately defined the lesion, showing the presence of post-stenotic dilation, guided the surgical approach and assessed coronary patency after revascularisation surgery.
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.
Recent evidence has suggested that persistence of the left superior caval vein is associated with a high incidence of obstructive lesions of the left heart. To shed more light on this issue 1085 patients with congenital heart disease were studied retrospectively, with the aim of estimating the prevalence of a persistent left superior caval vein and its associated anomalies, focusing attention on obstructive lesions in the left and right ventricles. Patients with isomerism of the atrial appendages, or hypoplastic left heart syndrome, were excluded. A persisting left superior caval vein was present in 57 patients (5.2%). The overall incidence of obstructive lesions of the left heart was higher in patients with than in those without a persistent left superior caval vein (31.6 versus 7.8%, p < 0.001). Relative hypoplasia of the left ventricle was also higher in patients with persistent left superior caval vein (14 versus 0.8%, p < 0.001). The obstructive lesions found in the left heart, compared with the number in those without a left caval vein, were: mitral stenosis, 5.2 versus 0.7%; subaortic stenosis, 5.3 versus 0.9%; aortic coarctation, 17.5 versus 5.8% (p < 0.01); all of these in association, 3.5 versus 0.4%. In contrast, the incidence of obstructive lesions of the right heart was similar in the two groups of patients. It is concluded that persistence of the left superior caval vein can perturb the normal development of the left ventricle, being strongly associated with obstructions to left ventricular inflow and outflow.
We report the multicentric French experience with transcatheter closure in children weighing 15 kilograms or less, with the aim of assessing the efficacy of the procedure in this age group.
We included all children weighing 15 kilograms or less, and seen between January, 1997, and June, 2004, who had successful transcatheter closure of an interatrial communication within the oval fossa.
Transcatheter closure was performed in 35 patients weighing 15 kilograms or less, of whom 14 were male and 21 female. The procedures were undertaken in 8 different centres, the patients having a median age of 3 years, with a range from zero to 6.2 years, and a mean weight of 13 kilograms, with a range from 3.6 to 15 kilograms. All the patients were symptomatic, with associated cardiac malformations present in 4 cases, and extracardiac anomalies in 4 patients, including Down’s syndrome in 3, and Adams Oliver syndrome in the other case. In 1 patient, emergency cardiac surgery was needed 24 h after the procedure to correct a previously undiagnosed divided right atrium. No other complication occurred. After a median follow-up of 2 years, with a range from 0.5 to 5.2 years, all the patients are asymptomatic, except for one long-standing patient with bronchodysplasia. In 1 other patient, a small residual bidirectional shunt was detected by echocardiography. No patient presented significant arrhythmia. In the patients followed-up for more than 12 months, we found a significant gain in weight gain.
Transcatheter closure of an interatrial communication within the oval fossa is efficient in children weighing 15 kilograms or less, and can be proposed as a first line of treatment in symptomatic patients. Children with retarded growth tend to have complete recovery within one year of closure.
Balloon angioplasty is now the elective technique for relief of aortic recoarctation, since it has low mortality, low morbidity, and good results at follow-up. Some concern exists concerning the possible increased risk in young children. To investigate such age-related aspects, we examined 58 children undergoing consecutive balloon angioplasty for postsurgical recoarctation. Of the children, 30 were younger and 28 older than 1 year. Recoarctation was more frequent with ventricular septal defect or other cardiac anomalies (p < 0.001). Systolic hypertension was present in 70% of children aged less than 1 year, but in only 32% of those older than 1 year (p < 0.001). The initial gradient was inversely related with the age at dilation (r = −0.28, p < 0.05), and correlated with systolic blood pressure (r = 0.81, p < 0.01). The procedure was successful in 87% of children older than, and 86% of those under 1 year. Age was not related with residual gradient, nor with the percentage increase of diameter of the site of stenosis. After balloon angioplasty, systolic hypertension was still present in 37% of children younger than 1 year, and in 25% of those older than 1 year (p < 0.05). Three complications occurred in children younger than 1 year, and 6 in those older (p < 0.05). Our results show, first, that recoarctation occurs earlier in the setting of complex disease, second that children suffering recoarctation at a younger age are more likely to be hypertensive, either before or after balloon angioplasty, third, that balloon angioplasty has the same rate of success in children below or above the age of one year, although the procedure still carries a not negligible risk. Finally, the procedure does not carry a higher risk for those children below the age of 1 year.
Objectives: To identify predictive factors of complications occurring during paediatric interventional catheterisation. Background: Interventional paediatric catheterisation is still burdened by a substantial risk. Risk factors, however, have rarely been investigated. Methods: We analysed prospectively 1,022 interventional procedures performed over a period of 8 years, excluding 260 procedures for atrial septostomy. We considered several patient-related variables, specifically age, weight, and gender, type of procedure, times required for fluoroscopy and the overall procedure, technical challenge, and the severity of the clinical condition. We also analysed variables linked to the environment, specifically the date of the examination, whether the operator remained in training, the novelty of the material, any breakdown in the installation, and errors made by the operator. We classified complications as those without clinical consequence, those which proved lethal, those requiring cardiopulmonary resuscitation, elective or emergency surgery, hospitalisation in the intensive care unit, and those leading to recatheterisation. Results: Our average incidence of complications was 4.1 per cent, which did not change significantly during the period of study. Of the patients, 4 died, 7 needed urgent surgery, 5 elective surgery, 3 hospitalisation in intensive care unit, and 8 recatheterisation. Independent risk factors for complications were technical challenge, critical clinical condition, operator in training, operator error, and breakdown of the installation. Young age was not associated with a higher risk of complications. Patients in whom no cause for complication could be found, either related to their own features or the environment, had a risk of complication of 1.4 per cent (95 per cent confidence intervals from 0.7 to 2.5 per cent). Conclusions: Our data show that variables relating either to the patient or the environment of catheterisation are associated with an increased risk of procedural complications. Knowledge of the risk factors can improve the odds of paediatric interventional catheterisation.
Objective: Primary implantation of stents is an accepted technique for treating aortic recoarctation, albeit that the effects of stenting on pressure profiles, carotid mechanical properties, intimal medial thickness, and reflection of the pulse wave have not been systematically investigated. Methods: Over the period from 1 January, 1999, to 31 December, 2002, we implanted stents to relieve aortic recoarctation in 15 patients, with a median age of 17 years, and a range from 7 to 29 years, with a median weight of 56 kilograms, ranging from 20 to 96 kilograms. Indications were a gradient of 20 millimetres of mercury or more measured in all, systemic hypertension at rest in 8, and systemic hypertension at exercise in all. Of the patients, 5 were receiving anti-hypertensive treatment. Before implantation of the stents, and after a mean follow-up of 22 months, all patients underwent an exercise test, vascular echography, and examination of the common carotid artery so as to determine its cross sectional compliance and distensibility, and the augmentation index. Results: The stents were implanted successfully in all patients. The mean gradient was reduced from 27 to 4 millimetres of mercury (p < 0.001). Systolic blood pressure at rest diminished from 140 to 131 millimetres of mercury (p = 0.04), while hypertension at rest regressed in 4 patients. Systolic blood pressure at exercise diminished from 245 to 222 millimetres of mercury (p = 0.018), and hypertension at exercise regressed in 1 patient. Anti-hypertensive treatment is still required for 4 patients. A correlation was found between systolic blood pressure at rest and initial peak-to-peak gradient (r = 0.8), and between initial gradient and percentage reduction of systolic blood pressure at rest at follow-up (r = −0.73). Compliance and distensibility of the common carotid artery were not significantly modified, albeit that the intimal medial thickness diminished from 0.64 to 0.57 millimetres (p = 0.04), and the augmentation index decreased from 5 to −1 (p = 0.012). Conclusions: Primary implantation of stents is effective in mid-term repair of aortic recoarctation. Although there is an improvement in systemic hypertension, the tensional profile and vascular sonography are not normalized. At long term follow-up, the suppression of an early reflection site of the pulse wave could decrease the wall stress of the great elastic vessels, reducing the thickness of the arterial walls.
An 18-month-old boy, referred because of an infection of the airways, was found to have a right-sided heart, a hypoplastic right lung, absence of the right pulmonary artery, and persistence of the hepatic venous plexus. The benign association of this unusual variant of the scimitar syndrome and persistence of the hepatic venous plexus needs to be recognised in order to avoid extensive investigations and surgery involving the inferior caval vein.
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