11/1168. Cutaneous telangiectasias, sparse hair, and type I membranoproliferative glomerulonephritis. We report the unusual association of normocomplementemic type I membranoproliferative glomerulonephritis in a 10-year-old girl with sparse red hair, absent eyebrows and eyelashes, cutaneous telangiectasias, and an atrial septal defect. ( info) |
12/1168. 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. ( info) |
13/1168. 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. ( info) |
14/1168. Infective endocarditis on an occluder closing an atrial septal defect. Closure of atrial septal defects be means of intravenous catheterisation has been undertaken using a variety of devices as an alternative to surgical closure. We describe the first case, to the best of our knowledge, of infective endocarditis complicating a successful transcatheter closure. This highlights the potential risk of this procedure, and emphasises the need for appropriate antibiotic prophylaxis until complete endothelialization of the device has occurred. ( info) |
15/1168. 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. ( info) |
16/1168. 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. ( info) |
17/1168. 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. ( info) |
18/1168. Chiari network entanglement and herniation into the left atrium by an atrial septal defect occluder device. The Chiari network is a fenestrated membrane consisting of threads and strands in the right atrium. First described in 1897 by anatomist Hans Chiari, it is a congenital remnant of embryonic development resulting from incomplete resorption of the right valve of the sinus venosus. Found in 2% to 3% of the population, it is generally not of clinical importance. Rarely, however, the network may be associated with serious complications such as thrombus formation, embolus entrapment, arrhythmia, tumor development, and catheter entrapment. We report the entanglement of an Amplatzer septal occluder device catheter in a prominent Chiari network that was herniated into the left atrium. Transesophageal echocardiographic recognition of this before deployment and guidance during disentanglement is described below. ( info) |
19/1168. Fatal pulmonary embolism after atrial septal defect closure in a paediatric patient. A four-year-old girl died of massive acute bilateral pulmonary embolism 11 days after direct closure of a secundum atrial septal defect (ASD II), despite postoperative anticoagulation until the patient was ambulatory. An autopsy showed thrombotic deposits on the suture line of the ASD closure, bilateral 90% occlusion of the pulmonary arteries, and haemorrhagic ulcerative ischaemic colitis of the descending colon and the sigmoid. ( info) |
| A 30-year-old Japanese woman was admitted to hospital for dyspnea. She had a history of corrective surgery for a large atrial septal defect and partial anomalous pulmonary venous drainage, which had produced cyanosis in her infancy. However, her cyanosis continued postoperatively. angiography revealed a double inferior vena cava (IVC), with the left IVC connected with the hemiazygos vein and the right IVC with the left atrium through a very small orifice. Most of the blood from the 2 IVCs flowed into the superior vena cava via the distended azygos and hemiazygos veins. Pulmonary arteriography revealed no abnormal structures. Pulmonary arterial pressure was normal. There was marked pulmonary venous oxygen desaturation. perfusion lung scintigraphy revealed multiple segmental perfusion defects. These findings suggested the presence of diffuse microscopic pulmonary arteriovenous fistulas bilaterally in the lungs. The patient appears to be the first reported adult case of microscopic and diffuse arteriovenous fistulas. Neither resection of the arteriovenous fistulas nor corrective surgery for the diversion was indicated, and heart-lung transplantation might be the only treatment able to relieve her dyspnea. ( info) |