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1/60. 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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2/60. An unusual tethering of the bridging leaflets in atrioventricular septal defect producing a communication from left atrium to right ventricle.

    We describe a 39-year-old woman who was diagnosed as having an unusual atrioventricular septal defect with a communication from left atrium to right ventricle. A common atrioventricular junction, with partially separated right and left atrioventricular orifices, was found at transoesophageal ultrasonic examination. Both bridging leaflets were attached to the underside of the atrial septum, which was grossly malaligned relative to the ventricular septum. The shunt was exclusively from left atrium to right ventricle because of the overriding of the left atrioventricular valve, with the left component of the inferior bridging leaflet firmly fused to the ventricular septal crest.
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3/60. Transseptal approach in children after patch occlusion of atrial septal defect: first experience with the cutting balloon.

    Two children required a transseptal approach to the left heart for endovascular stent redilation late after pericardial patch closure of atrial septal defects performed at the time of their initial surgical intervention. Following perforation of thickened interatrial patches in both patients, cutting balloons were used to create adequate interatrial communications. Cathet. Cardiovasc. Intervent. 48:378-381, 1999.
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4/60. Platypnoea-orthodeoxia syndrome.

    PLATYPNOEA: orthodeoxia is a rare syndrome of postural hypoxaemia accompanied by breathlessness. The predominant symptom, dyspnoea induced by upright posture, can be debilitating and difficult to discern without thorough evaluation of the patient's pattern of dyspnoea. The precise cause of the syndrome is unclear but patients develop right to left intracardiac shunting in the presence of normal right sided cardiac pressures. Initially, patients should have confirmation of orthostatic desaturation by erect and supine pulse oximetry. However, definitive diagnosis of an orthostatic intracardiac shunt is most readily established by echocardiography. The use of echocontrast with postural manoeuvres may facilitate the diagnosis. The treatment of choice is surgical closure of the intracardiac (usually interatrial) communication, which may result in dramatic symptomatic and haemodynamic improvement. Three cases (a 27 year old man and two women aged 63 and 72 years) are described that exemplify the presentation of this syndrome, and reflect the varied management strategies and outcomes of this condition.
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5/60. Familial occurrence of isolated right ventricular hypoplasia.

    Isolated right ventricular hypoplasia is a rare congenital anomaly. This condition is usually associated with a communication between the atria in the form of a patent foramen ovale or secondum atrial septal defect. We describe a familial occurrence of this rare disease. A 1-day-old male child and his 34-year-old father were found to have isolated right ventricular hypoplasia with atrial septal defect. An autosomal dominant mode of inheritance is likely for this rare congenital anomaly.
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6/60. dilatation of a restrictive interatrial communication using a balloon angioplasty catheter.

    Balloon atrioseptostomy is a life-saving procedure palliating certain congenital heart defects like transposition of the great arteries, right or left atrioventricular valve atresia, hypoplastic left heart syndrome, and pulmonary hypertension. Occasionally the Rashkind balloon septostomy technique may be ineffective in creating an adequate interatrial communication. We performed balloon dilatation of a restricted atrial septal defect using a balloon angioplasty catheter in a three-month-old infant.
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7/60. Late cardiac tamponade after transcatheter closure of atrial septal defect with Cardioseal device.

    cardiac tamponade occurring late after interventional closure of defects within the oval fossa is a very rare but life-threatening complication. We describe such an occurrence after use of a Cardioseal device to close an interatrial communication. Two arms of the device had perforated left atrial wall. The device was removed at surgery, and the defect closed uneventfully. All available means should be used to identify this complication.
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8/60. A rare form of atrioventricular septal defect with severe subaortic stenosis.

    An unusual form of atrioventricular septal defect associated with severe subaortic obstruction is reported in a neonate who presented with intractable cardiac failure. The baby had a large defect in the atrioventricular septum allowing communication from the left ventricle to the right atrium, without interatrial or interventricular communication, and a cleft anterior mitral leaflet. The baby expired despite palliative surgery performed to bypass the subaortic stenosis.
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9/60. Patent foramen ovale and brain microembolization during scoliosis surgery in adolescents.

    STUDY DESIGN: A case series is reported. OBJECTIVE: To improve understanding of the potential mechanisms associated with cerebral microemboli during scoliosis surgery in adolescents. SUMMARY OF BACKGROUND DATA: Paradoxical cerebral fat microembolization during scoliosis surgery has been associated with right-to-left shunting through an undetected patent foramen ovale. The prevalence of this cardiac defect in the adult population may be as high as 15% to 25%. Although the clinical relevance of this embolic phenomenon during scoliosis surgery has not been investigated, a few reports have documented its fatal consequences. It has been suspected in some patients with postoperative reduced visual function, particularly after complex instrumented fusions. methods: Bilateral transcranial Doppler (2 MHz) was used to monitor cerebral microemboli in the right and left middle cerebral arteries during spinal instrumentation and fusion in four patients. Additionally, transthoracic or transesophageal echocardiography incorporating pulse and color Doppler (3.5-5 MHz) and a contrast test was used in these patients to detect atrial shunts. RESULTS: Intraoperative transcranial Doppler monitoring detected high rates of cerebral microemboli associated with the presence of an atrial communication in two adolescents. In two additional patients with no detected brain microembolization, echocardiographic examination indicated the absence of an atrial shunt. CONCLUSION: The study findings suggest that unrecognized atrial communications in adolescents undergoing scoliosis surgery contribute to higher rates of Doppler-detected cerebral microemboli than those found in adolescents without this congenital defect.
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10/60. Transcutaneous-PO2 monitoring for detection of exercise-induced right-to-left shunts in children with congenital heart defects: a case report.

    A 2-y-old boy with scimitar syndrome underwent surgery involving the redirection of partial anomalous pulmonary venous return to the left atrium. Heart catheterization after the operation did not reveal any significant intra-cardiac shunts. An exercise test performed at the age of 10 y revealed a reduction in working capacity. At the age of 12 y, the patient became unconscious and experienced seizures during heavy physical exercise. EEG and Holter ECG examinations were normal. In a second exercise test, a fall in transcutaneous PO2 was demonstrated at the start of the test. A new heart catheterization revealed communication between the inferior vena cava and the left atrium owing to a misplaced patch. No right-to-left shunt was found at rest, probably as a result of drainage of the inferior vena cava to the superior vena cava by the azygous vein. An exercise test after re-operation revealed normal conditions. CONCLUSION: Haemodynamic studies during heart catheterization in children are usually performed at rest. This could result in exercise-induced right-to-left-shunts being overlooked. The use of PtcO2 monitoring during exercise tests is a non-invasive means of exposing these shunts.
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