Cardiac tumours in children

Cardiac tumours are benign or malignant neoplasms arising primarily in the inner lining, muscle layer, or the surrounding pericardium of the heart. They can be primary or metastatic. Primary cardiac tumours are rare in paediatric practice with a prevalence of 0.0017 to 0.28 in autopsy series. In contrast, the incidence of cardiac tumours during foetal life has been reported to be approximately 0.14%. The vast majority of primary cardiac tumours in children are benign, whilst approximately 10% are malignant. Secondary malignant tumours are 10–20 times more prevalent than primary malignant tumours. Rhabdomyoma is the most common cardiac tumour during foetal life and childhood. It accounts for more than 60% of all primary cardiac tumours. The frequency and type of cardiac tumours in adults differ from those in children with 75% being benign and 25% being malignant. Myxomas are the most common primary tumours in adults constituting 40% of benign tumours. Sarcomas make up 75% of malignant cardiac masses. Echocardiography, Computing Tomography (CT) and Magnetic Resonance Imaging (MRI) of the heart are the main non-invasive diagnostic tools. Cardiac catheterisation is seldom necessary. Tumour biopsy with histological assessment remains the gold standard for confirmation of the diagnosis. Surgical resection of primary cardiac tumours should be considered to relieve symptoms and mechanical obstruction to blood flow. The outcome of surgical resection in symptomatic, non-myxomatous benign cardiac tumours is favourable. Patients with primary cardiac malignancies may benefit from palliative surgery but this approach should not be recommended for patients with metastatic cardiac tumours. Surgery, chemotherapy and radiotherapy may prolong survival. The prognosis for malignant primary cardiac tumours is generally extremely poor

Effect of sitagliptin on cardiovascular outcomes in type 2 diabetes

BACKGROUND: Data are lacking on the long-term effect on cardiovascular events of adding sitagliptin, a dipeptidyl peptidase 4 inhibitor, to usual care in patients with type 2 diabetes and cardiovascular disease. METHODS: In this randomized, double-blind study, we assigned 14,671 patients to add either sitagliptin or placebo to their existing therapy. Open-label use of antihyperglycemic therapy was encouraged as required, aimed at reaching individually appropriate glycemic targets in all patients. To determine whether sitagliptin was noninferior to placebo, we used a relative risk of 1.3 as the marginal upper boundary. The primary cardiovascular outcome was a composite of cardiovascular death, nonfatal myocardial infarction, nonfatal stroke, or hospitalization for unstable angina. RESULTS: During a median follow-up of 3.0 years, there was a small difference in glycated hemoglobin levels (least-squares mean difference for sitagliptin vs. placebo, -0.29 percentage points; 95% confidence interval [CI], -0.32 to -0.27). Overall, the primary outcome occurred in 839 patients in the sitagliptin group (11.4%; 4.06 per 100 person-years) and 851 patients in the placebo group (11.6%; 4.17 per 100 person-years). Sitagliptin was noninferior to placebo for the primary composite cardiovascular outcome (hazard ratio, 0.98; 95% CI, 0.88 to 1.09; P<0.001). Rates of hospitalization for heart failure did not differ between the two groups (hazard ratio, 1.00; 95% CI, 0.83 to 1.20; P = 0.98). There were no significant between-group differences in rates of acute pancreatitis (P = 0.07) or pancreatic cancer (P = 0.32). CONCLUSIONS: Among patients with type 2 diabetes and established cardiovascular disease, adding sitagliptin to usual care did not appear to increase the risk of major adverse cardiovascular events, hospitalization for heart failure, or other adverse events

Epidemiology and outcome of tachyarrhythmias in tertiary pediatric cardiac centers

Introduction: Little is known about the real importance of pediatric arrhythmias. Methods: We analyzed the epidemiology, presentation and outcome of all clinically relevant tachyarrhythmias followed up in our pediatric institutions from 1995 to 2006. Results: A total of 250 cases were identified. The mean age ± SD at diagnosis was 4.7 ± 5.3 years, 45 cases were neonatal (18%). Supraventricular arrhythmias were noted in 210 children (84%), ventricular arrhythmias in 40 (16%). The most frequent symptoms were palpitations (n = 71) and syncope (n = 48) in older children, as well as monitoring of diseases (n = 62) and heart failure (n = 49) in younger patients. Recurrence was noted under or after therapy in 75 cases, mostly in cases diagnosed beyond infancy. At long-term follow-up, 169 patients have no recurrence without treatment (of whom 34 had required catheter ablation), 71 are under therapy and 10 died. Conclusion: Supraventricular arrhythmias in younger children are often an incidental diagnosis, respond to antiarrhythmic therapy and have a high incidence of resolution. In older children with supraventricular arrhythmias and in those with ventricular arrhythmias, delayed diagnosis or misdiagnosis is not rare, the arrhythmias are unlikely to resolve spontaneously and long-term antiarrhythmic treatment or catheter ablation is necessary. Copyright © 2008 S. Karger AG.SCOPUS: ar.jinfo:eu-repo/semantics/publishe

Left-ventricular inflow obstruction due to a dilated coronary sinus mimicking cor triatriatum

Persistence of the left superior vena cava with drainage to the coronary sinus is a common congenital anomaly. We report an infant with such a malformation associated with marked enlargement of the coronary sinus, which produced partial supramitral obstruction and consequently impairment to the left-ventricular inflow. The patient pre-sented with cardiac failure in infancy and features mimicking cor triatriatum. Surgical relief of the supramitral obstruction resulted in immediate reversal of the pulmonary hypertension, with clinical improvement. This rare entity, only once previously reported, is an unusual cause of pulmonary hypertension in infancy.SCOPUS: ar.jinfo:eu-repo/semantics/publishe

Cardiac syncope in pediatric patients

Objective: To assess the epidemiology of cardiac syncope in children and evaluate the guidelines on its management. Material and methods: We analyzed the etiology to syncope and diagnostic workup in consecutive pediatric patients presenting with syncope in our emergency departments or cardiac outpatient clinics between 1997 and 2005, and who were subsequently diagnosed as having cardiac syncope. Results: A primary cardiac cause was identified in 11 syncopal patients presenting to the emergency room and 14 patients to the cardiac clinic: supraventricular tachyarrhythmia in 9, ventricular tachyarrhythmia in 10, pacemaker dysfunction in 2, and isolated cases of sick sinus syndrome, hypoxic spell, hypertrophic cardiomyopathy, and primary pulmonary hypertension. Some elements suggested potential cardiac disease as a cause of syncope in all cases. The resting electrocardiogram and the echocardiogram were interpreted as positive and relevant to the diagnosis in 17 and 3 patients, respectively. Exercise electrocardiogram and Holter recording provided diagnostic information previously not seen on the resting electrocardiogram in six and three patients, respectively. Three children have died and one child has neurological sequelae following resuscitation. Conclusion: Our data support the premise that careful history taking with special focus on the events leading up to syncope, as well as a complete physical examination, can guide practitioners in discerning which syncopal children need further cardiac investigations. © 2007 Wiley Periodicals, Inc.SCOPUS: ar.jinfo:eu-repo/semantics/publishe

Congenital heart disease in 111 225 births in Belgium: birth prevalence, treatment and survival in the 21st century.

AIM: To investigate the birth prevalence, treatment modalities and short-term survival of children with congenital heart disease who were born in 2002. METHODS: We undertook a retrospective review of medical records of all patients who were born in 2002, and were diagnosed, treated and/or followed-up in one of the seven-paediatric cardiology programmes in Belgium. RESULTS: In 111 225 births, 921 children with congenital heart disease were detected, yielding a birth prevalence of 8.3 per 1000. The most frequently occurring conditions were ventricular septal defects (VSDs) (33%), ostium secundum atrial septal defects (18%) and pulmonary valve abnormalities (10%). Thirty-nine percent of the children either had a cardiosurgical operation or catheter intervention. In this study, 4% of the children died. The actuarial survival at 6 months and 1 year of age was 97% and 96%, respectively and remained stable after then. Compared to other heart defects, mortality was higher in univentricular physiology, pulmonary atresia with VSD, left ventricle outflow obstruction and tetralogy of Fallot. CONCLUSION: Survival of congenital heart disease is excellent and continued to improve in the early 21st century. New therapeutic options are increasingly used. This study provides baseline data for the longitudinal follow-up of this cohort

QT interval prolongation in future SIDS victims: a polysomnographic study.: QT intervals in future SIDS victims

International audienceOBJECTIVE: Previous data have suggested that a prolonged QTc interval during the first days of life can be associated with some cases of sudden infant death syndrome (SIDS). Analysis of heart rate variability during sleep in future SIDS victims has shown findings compatible with an imbalance in autonomic tone. We hypothesized that some future SIDS infants could have longer QTc intervals during sleep, compared with healthy control infants, and that this difference would correlate with the autonomic imbalance already found in these infants. METHODS: QTc intervals and a heart rate autoregressive power spectral analysis were calculated during the same periods in the polysomnographic sleep recordings of 18 infants who eventually died of SIDS and of 18 control infants. The control infants were matched for sex, gestational age, postnatal age, birth weight, and sleep position. The median postnatal age was 8 weeks. RESULTS: Compared with control infants, future SIDS victims were characterized by having longer QTc intervals during total sleep (P = 0.019), rapid eye movement sleep (P = 0.045) and non-rapid eye movement sleep (P = 0.029). When the night was divided into 3 equal parts, this difference was always present but was most marked during the last part of the night. There was, respectively, a negative and a positive correlation between parasympathetic activity and sympathovagal balance and median and maximum QTc interval values. CONCLUSION: Compared with QTc intervals in matched control infants, QTc intervals were increased in future SIDS victims. Such a prolongation could be related to the autonomic dysfunction already reported in these patients

QT interval prolongation in future SIDS victims: A polysomnographic study

SCOPUS: ar.jinfo:eu-repo/semantics/publishe

Early and late outcomes after surgical management of congenital vascular rings

Persistent respiratory or feeding problems in children may be associated with a congenital vascular ring. Surgical management is fairly standardized, but long-term outcomes are not well described. This study aims to investigate clinical presentation, surgical treatment, and risk factors for early mortality and late outcome. Our database revealed 62 surgically treated vascular ring patients between 1993 and 2014. Double aortic arch was the most common diagnosis (53%). Median age at operation was 1 year. Symptoms were mainly respiratory (89%) and feeding problems (32%). Median extubation time and hospital stay were 4 h and 5 days. Mean follow-up was 7.8 +/- 5.8 years. Early mortality was 8% and was related to anatomical diagnosis, concomitant anomalies, and a need for preoperative intubation. Freedom from residual symptoms at 1 and 6 months was 63 and 82%, respectively. Freedom from inhalation therapy at the last follow-up was 82% and was influenced by a type of vascular ring and preoperative ventilation. Dysphagia symptoms always disappeared. Conclusion: Surgical relief of tracheoesophageal compression is commonly effective in vascular ring anomalies. Respiratory symptoms necessitating chronic inhalation therapy only persist in a minority of children. Patients with double aortic arch are at increased risk to remain symptomatic, particularly with infectious exacerbations