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1/1168. 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. ( info)

2/1168. 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. ( info)

3/1168. 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. ( info)

4/1168. Aortic dissection in an elderly patient with atrial septal defect.

    We report a case of acute aortic dissection that occurred in the late course of surgically untreated atrial septal defect. A 60-year-old man with acute aortic dissection and atrial septal defect was operated on successfully, and we discuss the causal relationship between these two unusual conditions. ( info)

5/1168. Multiple secundum type atrial septal defects: identification by transthoracic color Doppler echocardiography.

    This case illustrates the utility of two-dimensional color Doppler echocardiography in detecting and localizing multiple atrial septal defects not consistently demonstrated by standard two-dimensional echocardiography. ( info)

6/1168. 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. ( info)

7/1168. Unilateral total and contralateral partial pulmonary agenesis associated with total anomalous pulmonary venous drainage.

    A term newborn presented with total right and partial left lung agenesis associated with total anomalous obstructed left pulmonary venous drainage. This unique association with a dramatic prognosis is thought to be due to a developmental insult early in the embryonic formation of the lung and its supporting vasculature. ( info)

8/1168. 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. ( info)

9/1168. Ebstein's anomaly associated with severe valvar pulmonary stenosis: successful palliation with balloon pulmonary valvuloplasty in an adult.

    We report a 52-year-old adult with Ebstein's anomaly associated with severe valvar pulmonary stenosis, secundum atrial septal defect, polycythemia, hypoxemia, and severe exertional limitations. Balloon pulmonary valvuloplasty relieved the valvar obstruction, reduced the degree of tricuspid regurgitation and polycythemia, and improved systemic oxygenation and exercise abilities. Use of this technique in patients with this combination of lesions can postpone the need for surgical intervention. ( info)

10/1168. 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. ( info)
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