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1/608. The diagnosis of cor triatriatum sinistrum in children: a continuing dilemma.

    cor triatriatum (CT) is a rare congenital cardiac anomaly. The salient clinical, roentgenographic, electrocardiographic, echocardiographic, and hemodynamic findings are presented in two asymptomatic children and one with nonspecific dyspnea on exertion. Two male children had a classical form of cor triatriatum with normal physical and inconsequential roentgenographic and electrocardiographic findings. One of the male patients had surgery for a large atrial septal defect ostium secundum (ASD 2 degrees) and pulmonary hypertension in infancy. The female patient had CT with a communicating accessory chamber to right atrium and a rare patent foramen ovale. Her clinical findings confirmed an atrial level shunt. All patients had excision of the fibromuscular membrane from the right and left atrial transseptal approach with excellent results and with no recurrence during 2 to 4 year follow-up. We report the dilemma encountered in the clinical diagnosis of CT in children and the pivotal role played by echocardiography in the diagnosis of this anomaly.
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keywords = cardiac
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2/608. Complete atrioventricular septal defect and Ebstein's anomaly.

    A case child with complete atrioventricular septal defect (AVSD) and Ebstein's anomaly underwent surgical treatment at 3 months of age. She died on the third postoperative day. Postmortem examination showed complete AVSD, downward displacement of the right atrioventricular valve, left ventricular outflow tract obstruction, and hypertensive pulmonary vascular disease. association of complete AVSD and Ebstein's anomaly is a rare cardiac anomaly for which no attempt at surgical repair has previously been made. This report deals with our experience and also with the morphological features of this anomaly.
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keywords = cardiac
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3/608. Autosomal dominant secundum atrial septal defect with various cardiac and noncardiac defects: a new midline disorder.

    We report on a Lebanese family in which 12 persons had an atrial septal defect and various cardiac and noncardiac anomalies. Cardiac anomalies are left axis deviation of QRS, right bundle branch block, atrial fibrillation, wolff-parkinson-white syndrome, nodal atrioventricular rhythm, aortic stenosis, pulmonic valve stenosis, mitral stenosis (lutembacher syndrome), and low implantation of the tricuspid valve (Ebstein disease). Noncardiac abnormalities consisted specially of the presence of hypertelorism, cleft lip, and pectus excavatum. This combination appears to constitute a hitherto undescribed autosomal dominant midline disorder of the heart and upper half of the body with almost full penetrance and variable expressivity. The mutation does not map to any known locus involved in atrial septal defect or conduction block.
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ranking = 11.000024596136
keywords = cardiac, heart
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4/608. A new low profile balloon atrial septostomy catheter: initial animal and clinical experience.

    OBJECTIVE: To evaluate the safety and efficacy of a new low profile balloon septostomy catheter in neonatal animals as well as in one newborn infant. BACKGROUND: Balloon atrial septostomy remains one of the most commonly performed palliative procedures in pediatric cardiology. The currently available septostomy catheter requires a large introducer sheath (6 or 7F), does not have an end hole for confirmation of position or pressure measurement and is limited in patients with a small left atrium due to its large balloon inflated diameter. methods: Four neonatal piglets (average weight 3.9 kg) underwent percutaneous balloon atrial septostomy using the new balloon catheter inflated to 1 cc via a 5F sheath in the femoral vein. Two other piglets (average weight 4.9 kg) underwent septostomy with the conventional catheter inflated to 3.5 cc via a 6 or 7F sheath in the femoral vein. All animals underwent transthoracic echocardiography pre and post septostomy. All animals were sacrificed after the procedure and the size of the atrial defect created was measured. One neonate with Taussig-Bing anomaly underwent septostomy with the new balloon catheter. RESULTS: The left atrium was entered in all piglets. It was easier to enter the left atrium with an end hole catheter which was exchanged over a wire with the septostomy catheter. Septostomy was performed with the new or conventional catheters without complications. echocardiography demonstrated a very small patent foramen ovale prior to the procedure and a large atrial defect after septostomy. The average size of the defect created by the new catheter was 11.3 x 10 mm in diameter and 11 x 10 mm using the conventional catheter. A 10 x 10 mm atrial communication was created in the neonate. CONCLUSIONS: This study demonstrates the safety and efficacy of this new catheter. This catheter will be of potential importance in patients with a small left atrium and in small neonates with congenital heart disease requiring septostomy.
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ranking = 2.4596136460672E-5
keywords = heart
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5/608. Pitfalls of undetected patent foramen ovale in off-pump cases.

    We describe pitfalls of a hitherto undetected patent foramen ovale during the conduct of an off-bypass coronary revascularization. Manipulation of the heart resulted in right-to-left shunt and severe desaturation requiring institution of cardiopulmonary bypass to close the patent foramen ovale and complete the revascularization.
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ranking = 2.4596136460672E-5
keywords = heart
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6/608. Hypoxemia after prior cardiac surgery due to interatrial shunting and its treatment with a novel transcatheter occlusion device.

    We describe two unusual cases of hypoxemia after cardiac surgery due to intracardiac right-to-left shunting through a patent foramen ovale or atrial septal defect. The interatrial defects were successfully occluded by placement of a novel, transcather device, the Angelwings Atrial Septal Defect Occluder Device, with resolution of hypoxemia.
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ranking = 6
keywords = cardiac
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7/608. Use of the laryngeal mask airway during repair of atrial septal defect in children.

    We describe the elective use of the laryngeal mask airway in two children undergoing cardiopulmonary bypass for repair of an atrial septal defect. Total surgical time was short and cardiopulmonary bypass performed at normothermia allowing removal of the laryngeal mask airway on the operating table on completion of surgery. We were able to adequately oxygenate and ventilate the children throughout the procedure using positive pressure ventilation and spontaneous ventilation. The use of caudal fentanyl and rectal diclofenac aided postoperative pain management. Atrial septal defect repair has become one of the more straightforward cardiac operations partly as a result of new cardiopulmonary bypass techniques. Avoidance of intubation and postoperative ventilation in appropriate patients would make this procedure ideal for 'fast tracking' and offers the potential advantages of decreased length of stay in hospital and reduction in overall costs.
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keywords = cardiac
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8/608. Simultaneous surgical treatment of atrial septal defect and atrial flutter using a simple modification of the atrial incision.

    The reentrant circuit of common atrial flutter is known to be located in the right atrium between two anatomical barriers. Recent electrophysiologic studies have defined the tricuspid annulus as the anterior barrier, and the terminal crest and its continuation as the eustachian ridge as the posterior barrier. Construction of a bidirectional block to conduction between these two barriers by means of lesions created with radiofrequency current have been shown to be effective in ablating the flutter. We now find that surgical creation of such a block to conduction between the barriers by a simple modification of the atrial incision line is equally effective. In a 6-year-old boy, who was admitted to our hospital for closure of an atrial septal defect and treatment of sustained atrial flutter, the atriotomy was performed perpendicular to the terminal groove and extended towards the tricuspid annulus, placing some cryothermal lesions between the end of the incision and the annulus. The septal defect was closed using a Dacron patch. The child was free of arrhythmia both during the postoperative stay and over the initial three months of follow-up. We conclude that this simple modification of the atrial incision line provides cure of atrial flutter in children who require atriotomy for repair of congenital cardiac anomalies. It may also be beneficial in preventing 'incisional' reentrant tachycardia.
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keywords = cardiac
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9/608. Atrioventricular septal defect with separate right and left atrioventricular valvar orifices in a patient with foetal hydantoin syndrome.

    The teratogenic properties of phenytoin, including cardiac malformations, have been previously documented. We report one patient with foetal hydantoin syndrome and atrioventricular septal defect with common atrioventricular junction but separate right and left atrioventricular valves, an association that has not been described, to the best of our knowledge.
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ranking = 1
keywords = cardiac
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10/608. Right-to-left interatrial shunt in ARDS: dramatic improvement in prone position.

    The mechanisms leading to shunting through a patent foramen ovale include high right-sided cardiac pressures and respiratory factors due to mechanical ventilation and also anatomical changes in the right atrium as described in the platypnea-orthodeoxia syndrome. We report a patient with the adult respiratory distress syndrome (ARDS) who had a right-to-left atrial shunt which decreased in the prone position, after which oxygenation improved. The patient was admitted to the intensive care unit because of ARDS due to an invasive fungal infection. He had a history of chronic lymphocytic leukemia and paradoxical embolisms through a patent foramen ovale. Despite mechanical ventilation and antifungal treatment he developed severe ARDS. He was therefore turned to the prone position. blood gas values improved dramatically (arterial oxygen tension/fractional inspired oxygen ratio increasing from 59 to 278 torr). Transcranial Doppler sonography was performed with bubble study, which confirmed a massive right-to-left shunt in the supine position and which instantaneously decreased in the prone position. This case suggests that a decrease in right-to-left shunt in patients who have a patent foramen ovale could partly explain the improvement in hypoxemia in the prone position.
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ranking = 1
keywords = cardiac
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