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.
Objective-To assess the possibility of maintaining ductal patency in neonates with complex pulmonary atresia by percutaneous implantation of balloon expandable stents.Patients-Two duct-dependent neonates with long segment pulmonary atresia, right sided aortic arch, and left sided arterial duct.Results-Stents with final diameter of 3 5 or 4 mm and initial length of 7 or 15 mm were successfully positioned in the arterial duct. Two stents were required in one child and four in the other in order to stent the entire length of the duct. After dependent congenital heart disease has not, however, been attempted. We report the successful maintenance of ductal patency by implantation of expandable stainless steel stents. The technical procedure (performed in each case under general anaesthesia), its potential complications, and its very considerable future promise in the palliation of duct dependent congenital heart disease are discussed. Patients and methods CASE 1A 2-6 kg boy presented with cyanosis and a soft continuous murmur when he was four hours old. He had an extensive cleft lip and palate and a bifid right thumb. Echocardiography and later cardiac catheterisation and angiography showed severe hypoplasia of the right ventricle and tricuspid valve with an intact ventricular septum, right ventricular sinusoids, long segment pulmonary atresia, small but confluent pulmonary arteries, a right sided aortic arch, and a left sided arterial duct arising at the origin of the left subclavian artery. He was maintained on an infusion of prostaglandin E and at the age of five days underwent a Waterston aortopulmonary anastomosis. The right pulmonary artery was found to be approximately 2-5 mm in diameter behind the ascending aorta. Postoperatively the child remained duct dependent. Angiography showed that the shunt was completely occluded. In view of the failed attempt at surgical palliation and with informed consent from the child's parents, we believed the alternative approach of stenting the arterial duct was justified.
We conducted a genome-wide association study to search for risk alleles associated with Tetralogy of Fallot (TOF), using a northern European discovery set of 835 cases and 5159 controls. A region on chromosome 12q24 was associated (P = 1.4 × 10−7) and replicated convincingly (P = 3.9 × 10−5) in 798 cases and 2931 controls [per allele odds ratio (OR) = 1.27 in replication cohort, P = 7.7 × 10−11 in combined populations]. Single nucleotide polymorphisms in the glypican 5 gene on chromosome 13q32 were also associated (P = 1.7 × 10−7) and replicated convincingly (P = 1.2 × 10−5) in 789 cases and 2927 controls (per allele OR = 1.31 in replication cohort, P = 3.03 × 10−11 in combined populations). Four additional regions on chromosomes 10, 15 and 16 showed suggestive association accompanied by nominal replication. This study, the first genome-wide association study of a congenital heart malformation phenotype, provides evidence that common genetic variation influences the risk of TOF.
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