1/16. Left superior vena cava connection to unroofed coronary sinus associated with positional cyanosis: successful transcatheter treatment using Gianturco-Grifka vascular occlusion device.A persistent left superior vena cava connection to an unroofed coronary sinus is a rare cardiac anomaly that is associated with a variable degree of cyanosis. We report an infant with this condition and the unusual feature of cyanosis dependent on head position. When the patient's head was rotated to the left, he developed severe stenosis of the left internal jugular vein, enlarged cervical collateral veins that connected to the right superior vena cava and had an oxygen saturation 95%. When the patient's head was rotated to the right, the left internal jugular vein was widely patent and systemic oxygen saturation decreased to 87%. There was no right ventricular volume overload. Temporary occlusion of the left superior vena cava documented tolerable proximal venous pressure. cyanosis was relieved by transcatheter closure of the left superior vena cava with a Gianturco-Grifka vascular occlusion device. Cathet. Cardiovasc. Intervent. 48:369-373, 1999.- - - - - - - - - - ranking = 1keywords = closure (Clic here for more details about this article) |
2/16. Device closure of an atrial septal defect following successful balloon valvuloplasty in a neonate with critical pulmonary valve stenosis and persistent cyanosis.Persistent cyanosis after successful balloon valvuloplasty for neonatal critical pulmonary valve stenosis is often related to poor right ventricular compliance and right-to-left shunting at the atrial level. A successful catheter closure of an atrial septal defect was performed with a dramatic increase in systemic oxygen saturation alleviating the need for a surgical systemic-to-pulmonary artery shunt.- - - - - - - - - - ranking = 5keywords = closure (Clic here for more details about this article) |
3/16. Postsurgical use of amplatzer septal occluder in cyanotic patients with pulmonary atresia/intact ventricular septum: significance of cor triatriatum dexter and dilated right atrium.Percutaneous closure of secundum atrial septal defects (ASDs) has been shown to be safe and effective. However, its role after surgery in patients with cyanotic congenital heart disease who may have associated cor triatriatum dexter and a dilated right atrium has not been established. This article reports on successful closure in such patients, including precautions and results.- - - - - - - - - - ranking = 2keywords = closure (Clic here for more details about this article) |
4/16. Transcatheter closure of large persistent left superior vena cava causing cyanosis in two patients post-Fontan operation utilizing the Gianturco Grifka vascular occlusion device.We report the successful transcatheter closure of a large persistent left superior vena cava draining into the pulmonary venous circulation causing cyanosis in two patients who had previously undergone the Fontan operation utilizing the Gianturco Grifka vascular occlusion device. Cathet Cardiovasc Intervent 2001;53:398-404.- - - - - - - - - - ranking = 5keywords = closure (Clic here for more details about this article) |
5/16. Ebstein's anomaly: one and a half ventricular repair.patients with Ebstein's anomaly can present after childhood or adolescence with cyanosis, arrhythmias, severe right ventricular dysfunction and frequently with left ventricular dysfunction secondary to the prolonged cyanosis and to the right ventricular interference. At this point conventional repair is accompanied by elevated mortality and morbidity and poor functional results. We report our experience with three patients (8, 16 and 35 years of age) with Ebstein's anomaly, very dilated right atrium, severe tricuspid valve regurgitation (4/4), bi-directional shunt through an atrial septal defect and reduced left ventricular function (mean ejection fraction = 58%, mean shortening fraction = 25%). All underwent one and a half ventricular repair consisting of closure of the atrial septal defect, tricuspid repair with reduction of the atrialised portion of the right ventricle and end-to-side anastomosis of the superior vena cava to the right pulmonary artery. All patients survived, with a mean follow-up of 33 months. In all there was complete regression of the cyanosis and of the signs of heart failure. Postoperative echocardiography showed reduced degree of tricuspid regurgitation (2/4) and improvement of the left ventricular function (mean ejection fraction = 77%, mean shortening fraction = 40%). In patients with Ebstein's anomaly referred late for surgery with severely compromised right ventricular function or even with reduced biventricular function, the presence of a relatively hypoplastic and/or malfunctioning right ventricular chamber inadequate to sustain the entire systemic venous return but capable of managing part of the systemic venous return, permits a one and a half ventricular repair with good functional results.- - - - - - - - - - ranking = 1keywords = closure (Clic here for more details about this article) |
6/16. Transcatheter coil closure of pulmonary arteriovenous malformations in children.Pulmonary arteriovenous fistula (PAVF) is a rare vascular malformation of the lung that may lead to cyanosis, epistaxis, hemoptysis, and neurological deficits or cerebral abscess. The purpose of this study is to assess the effectiveness of percutaneous transcatheter embolization of PAVF in pediatric patients. Transcatheter embolization of PAVF using spring coils was performed in three patients (two males and one female) who presented between 1989 and 1999. The age at presentation ranged from 8 months to 3 years (mean 19.6 months). All patients had cyanosis and clubbing. Neurological, dermatological, or other cardiac manifestations were absent. The arterial oxygen saturations at presentation ranged from 60 to 72% (mean 64%). During eight procedures, multiple coils (total of 41 coils, average 14 coils per patient) were delivered to occlude the fistulas successfully. There was complete occlusion of the fistulas in all patients after the multiple interventions. The aortic saturations increased from a mean of 66% to a mean of 95%. Chest radiographs demonstrated dramatic regression of the pulmonary shadows in all three patients. There were no complications encountered during the procedures or during follow-up. Transcatheter coil embolization of PAVF is a safe and effective method of treatment in the pediatric age group. patients may require multiple procedures to completely occlude the fistulas. Long-term follow-up is essential to ensure absence of recurrence due to recanalization.- - - - - - - - - - ranking = 4keywords = closure (Clic here for more details about this article) |
7/16. Surgical treatment for isolated hypoplasia of the right ventricle.Four patients with isolated hypoplasia of the right ventricle who underwent intracardiac repair are described. An atrial septal defect (ASD) was successfully closed in 3 patients with mild hypoplasia of the right ventricle. The remaining patients underwent ASD closure and right atrium-to-pulmonary artery connection with a conduit with a bovine pericardial valve because of severe hypoplasia of the right ventricle and tricuspid valve. Postoperative angiocardiogram in the last patient demonstrated two-channel pathways from the right atrium to the pulmonary artery via both the right ventricle and external conduit. At long-term follow-up studies, 3 patients had developed arrhythmias and/or sinus node dysfunction, and one had died, probably because of arrhythmia, 13 years postoperatively. The right ventricular ejection fractions were below normal levels at 11 and 12 years postoperatively in 2 patients who underwent closure of ASD. It was concluded that the mild form of hypoplasia of the right ventricle should be surgically corrected by ASD closure, while right atrium-to-pulmonary artery connection or Fontan's operation should be chosen for cases of severe hypoplasia. Arrhythmias and hypofunction of the right ventricle are long-term problems after intracardiac repair of this anomaly.- - - - - - - - - - ranking = 3keywords = closure (Clic here for more details about this article) |
8/16. Central cyanosis due to prominence of the eustachian and thebesian valves.A 9-year-old female child with serious central cyanosis was found to have right to left shunt across a large secundum atrial septal defect despite normal right-sided pressures. Preoperative cross-sectional echocardiography suggested the presence of large sinus venosus eustachian and thebesian valves as the mechanism responsible for diversion of the inferior caval and coronary sinus venous return to the left atrium across the interatrial secundum defect. Surgical excision of the unduly prominent sinus venosus valve and patch closure of the atrial septal defect resulted in complete disappearance of the cyanosis and physiological and clinical cure.- - - - - - - - - - ranking = 1keywords = closure (Clic here for more details about this article) |
9/16. Anomalous hepatic venous drainage.We present a 3-year-old boy born with anomalous hepatic venous drainage into the left atrium and a small sinus venosus atrial septum defect, in whom pulmonary arteriovenous malformations developed with progressive cyanosis. Surgical redirection of the hepatic venous drainage to the right atrium and closure of the atrial septal defect led to regression of the pulmonary arteriovenous malformations. However, in contrast to other reports, progressive pulmonary hypertension developed postoperatively.- - - - - - - - - - ranking = 1keywords = closure (Clic here for more details about this article) |
10/16. Dysphagia as a risk factor for sudden unexplained death in infancy.The TRIAD of encephalopathy, subdural haemorrhages, and retinal haemorrhages is commonly considered diagnostic of shaken baby syndrome, but the original paper describes a statistically linked QUADRAD of features, the fourth of which is a previous history of feeding difficulties (dysphagia). Recent reviews of giving pacifiers (dummies) to infants during sleeping periods have found a significant reduction in the incidence of sudden infant death Syndrome. Stimulation of swallowing is a possible connection with dysphagia, which is examined here, illustrated by a well documented case. Although amniotic fluid passes freely through the larynx of fetal mammals during fetal breathing, application of pure water to the laryngeal epithelium in infants causes choking and laryngeal closure. "water sensors" in the surface respond to lack of chloride ions and adapt very slowly or not at all. Others have found in puppies that following application of pure water only 32% resume breathing in less than 30-40s. The rest needed at least one saline flush, and some required artificial ventilation in addition. These receptors also respond to high potassium concentrations and acid or alkaline solutions. Normally, airway closure during swallowing or vomiting prevents entry of feed or oesophageal reflux, but in some forms of dysphagia leakage can occur, causing paroxysmal coughing, reflex laryngeal closure, and so prolonged apnoea. Recently, it has been realised that the TRIAD injuries can also result from high intracranial vascular pressures transmitted from intra-thoracic pressure surges during paroxysmal coughing, choking, etc. Triggering of such pressure surges by dysphagic accidents provides a physiological link to injuries commonly considered diagnostic of shaken baby syndrome, completing the statistically identified QUADRAD of features. Further dysphagic research might reveal predictive factors, and preventative measures such as feeds of optimal pH.- - - - - - - - - - ranking = 3keywords = closure (Clic here for more details about this article) |
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