Catheter-based closure of atrial septal defects in the oval fossa with the Amplatzer device in patients in their first or second year of life.

OBJECTIVE: To assess feasibility, safety, and efficacy of the use of Amplatzer occluders in closure of atrial septal defects (ASD) in children in the first 2 years of life. BACKGROUND: Although scattered reports on such closure have been published, no agreement consists on the policy. METHODS: In 654 patients of all ages, closure was achieved in 632 (96.6%). Data were analyzed retrospectively in all 71 children where an attempt had been made to close the ASD before their second birthday. RESULTS: Median age and body weight were 17.2 months (range 3.9-23.8) and 10.0 kg (range 3.8-14.5), respectively. Median fluoroscopy time was 13.6 min and median device size 15 mm. Successful closure was achieved in 68 children (95.8%). Three times the procedure was aborted: in one, the device repeatedly straddled the septum; in the other two, a small left atrium restricted the movement of the left-sided disc. One device embolized and was reimplanted after retrieval. One infant with multiple disorders died 6 days after closure from acute sepsis probably unrelated to the procedure. No other complications occurred. Only trivial shunts closing with time were registered during follow-up. Symptomatic patients profited markedly from closure. CONCLUSION: The results and complications of ASD closure with the Amplatzer device in patients in their first 2 years of life compare favorably with procedures in older patients, provided that the size of the septum and the dimensions in the left atrium are taken into consideration when selecting the size of the device.

[Catheter closure of open ductus arteriosus--the first 100 patients]. / Kateterbasert lukking av åpen ductus arteriosus--de første 100 pasienter.

BACKGROUND: Patent ductus arteriosus is increasingly treated with catheter based techniques. We present our results as by the end of 1998 of the first 100 patients given such treatment. MATERIAL AND METHODS: The 100 patients between 0.6 and 31.4 years were initially treated with one of these devices: The Rashkind umbrella (60 patients), Cook PDA coils (31 patients) or Amplatzer ductal occluders (ten patients). Seven umbrella patients were treated twice, one with an additional umbrella, six with coils; two coil patients twice, one with another coil and one--after embolisation--with a peg. RESULTS: The overall complete closure rate for all groups was 90% after nine patients had been treated a second time. The primary complete closure rate in pegs was 100%, after reintervention 85% in umbrella and 97% in coil patients. Neither umbrellas nor pegs have embolised. One coil embolised in the course of implantation of 40 coils (2.5%). No other complication has occurred. INTERPRETATION: The closure of the arterial duct with catheter techniques compares favorably with surgery and is now established as the method of choice. Following the investigational introduction of pegs there is no longer an upper limit in the size of ducts suitable for such treatment.

Transcatheter closure as standard treatment for most interatrial defects: experience in 200 patients treated with the Amplatzer Septal Occluder.

To judge whether an Amplatzer Septal Occluder can be used as standard therapy instead of surgery for closure of atrial septal defects we report our experiences in 200 patients. Of these patients, 127 had an atrial septal defect with haemodynamically significant left-to-right shunt, 68 patients a persistent oval foramen after presumed paradoxical embolism, and 5 had a fenestration after Fontan-repair. Mean age was 29.8 years (0.8 to 77.7 years). Body weight ranged from 6.9 to 120.0 kg (mean 51.5 kg). After diagnostic cardiac catheterization, and balloon-sizing of the defect, we implanted Amplatzer Septal Occluders with stents of 4 to 28 mm diameter. Follow-up studies were obtained after 48 hours, and one, six, and twelve months. Transcatheter closure of the atrial septal defect proved successful in all without any relevant residual shunts. In particular, complete closure was achieved in all patients after presumed paradoxical embolism. The mean period of follow-up is 9.5 months, with a range from 0.4 to 23.5 months, giving a total of 1898 patient months. The occlusion rate after three month was 98.1%. A trivial haemodynamically insignificant residual shunt remained in 1.9% of the patients. Fluoroscopy times ranged from 0 to 43.5 minutes, with a median of 8.7 minutes. The excellent results in the short and medium term in children and adults have resulted in using this device routinely at the present time for closure of central atrial septal defects up to a diameter of 28 mm. Final judgement, however, is only possible after long-term follow-up.

Truncus arteriosus with anomalous pulmonary venous connection.

The anatomic, diagnostic, and management findings of 6 patients with truncus arteriosus and anomalous pulmonary venous connections are described. Additional risk factors indicative of poor prognosis were found in 3 of 4 patients with truncus arteriosus and totally anomalous pulmonary venous connection and in 1 patient with partially anomalous pulmonary venous connection.

[Catheter treatment of congenital heart defects. Arterial septal defects and open ductus arteriosus]. / Kateterbasert behandling av medfødte hjertefeil. Atrieseptumdefekter og åpen ductus arteriosus.

The results of new methods for catheter treatment of congenital heart defects are presented. Between 1989 and 1996 closure of a patent ductus arteriosus was performed in 66 instances on 63 patients, eight of which were with coils. Three patients were treated twice, one with an additional umbrella, two with coils. The overall complete closure rate for umbrellas was 75%, after two ducts, which were initially totally occluded, recanalized. In six more patients the procedure was either aborted or indication was not present. All six ducts treated with coils as the first procedure were completely closed. One of two patients who had residual leak after previous umbrella treatment achieved complete closure after subsequent coil implantation. Closure of atrial septal defects in the oval fossa was performed using the Amplatzer septal occluder in seven children. Complete closure was achieved in all of them. There have been no complications, in particular there have been no cases of embolization in any of the groups. The results seem to indicate that coil occlusion of a persistently patent duct may be at least as good as the umbrella in terms of complete closure. So far both methods have been safe, but experience with coils is limited. The closure of atrial septal defects shows encouraging results. We will continue to offer this treatment as an alternative to open heart surgery in carefully selected patients.

Cardiac findings in generalized lipodystrophy.

The cardiac findings in eight patients, two of whom were female, with total lipodystrophy (Berardinelli-Seip's disease) are reported. One of them had the acquired form of the disease. Four patients died at a mean age of 32 years. As far as we know, at least three of them most likely died for cardiac reasons, one shortly after recovering from an attempted suicide. All eight patients had hypertrophic hearts, mostly with deranged diastolic, but also systolic, function. One had pulmonary hypertension. We conclude that generalized lipodystrophy is a serious disease with cardiac affection leading to cardiac dysfunction and early death. There is no specific cardiac treatment, and the treatment should be according to the general guidelines for patients with hypertrophic, dysfunctioning hearts.

Wire trapping in Rashkind's patent ductus arteriosus occluder: its etiology and how it may be avoided.

Release of the Rashkind ductus umbrella may be complicated in that the central wire of the delivery catheter may become trapped within the foam of the umbrella. This problem occurs only if the central retaining wire is advanced when trying to disengage the catheter and umbrella. We propose a simple solution to the problem: Retract the sleeve instead of advancing the retaining wire.

[The "umbrella closure" of the ductus arteriosus]. / Paraplylukking av ductus arteriosus.

The technical details of the method are outlined, and the Norwegian experience reported: From September 1988 to January 1994 we attempted to close open ducts with the Rashkind technique in 26 patients. Two ducts were too small to accept the device, and in one case the implanted device could not be released. This patient was referred to surgery. In the remaining 23 patients the device was left in place. The mean X-ray fluoroscopy time was 15 minutes. No complications occurred. In eight patients (35%) a residual shunt could be detected by ultrasonic Doppler technique, but in seven of them haemodynamical significance was lacking. The umbrella method is a good alternative to traditional treatment.

[Williams-Beuren syndrome in Norway]. / Williams-Beurens syndrom i Norge.

The author describes the Williams-Beuren syndrome and the cases of 57 patients in an attempt to increase awareness of the syndrome, which probably is underdiagnosed. Its characteristic features are a combination of a special facial appearance and a mental condition: The face typically shows a broad, flattened upper lip, small chin, and hypoplasia of teeth, often strabism, flattened nasal bridge and fullness around the eyes. Mentally the patients appear to be happy, talkative, pleasant children, who are indiscriminatingly friendly and have serious difficulties in concentrating. Only 53% of the patients have an affected heart, of whom 93% have supravalvular aortic stenosis, peripheral pulmonic stenoses or combinations of the two. Nine patients have had heart operations. The facial features in children of different age groups and races are depicted.

[Hypoplastic left heart syndrome. Present experiences and future possibilities]. / Hypoplastisk venstre hjerte-syndrom. Erfaringer hittil og mulige veier videre.

The authors report experiences from Norwood's staged palliation for hypoplastic left heart syndrome performed in Philadelphia on Norwegian children. Although there is no universal agreement that these children should be operated at all, the Norwegian health authorities have decided that all expenses for transport, examination and treatment shall be covered by the National Health Insurance Scheme. Since 1987 a total of 21 children have been transported to USA for operation. Eight of these children have completed all the planned stages of the operative treatment. Two are waiting for stage 3. 11 children have died. The authors discuss how to proceed with regard to treatment of this complicated patient group in future.

Mental health and psychosocial functioning in adolescents with congenital heart disease. A comparison between adolescents born with severe heart defect and atrial septal defect.

Twenty-six adolescents, aged 13-18 years, with severe congenital heart disease were matched for sex, age and living area with 26 adolescents with repaired atrial septal defect and regarded as physically fit. These two groups were compared according to somatic condition, psychopathology, psychosocial functioning and chronic family difficulties. A higher rate of psychiatric problems in the complex group, an association between psychosocial functioning and physical capacity, as well as an association between psychosocial functioning and chronic family difficulties were observed. These findings suggest that physical capacity is of crucial importance for mental health and functioning of adolescents with congenital heart disease. The association with chronic family difficulties also suggests that a comprehensive biopsychosocial approach is necessary in the treatment and rehabilitation of these patients.

Balloon debanding of the pulmonary artery.

Clinical balloon debanding of the pulmonary artery is reported in two patients, 6 months and 4 years of age. The band was made of Dacron, and was closed with one single polypropylene suture. This suture was subsequently ruptured with a balloon inflated within the pulmonary artery at the site of the band. Balloon debanding relieved the gradient almost completely in the two patients. No problems occurred during the procedure. In the future this may prove to be an efficient and reliable way to remove the effect of a pulmonary artery banding.

[Percutaneous closure of ductus arteriosus using the umbrella technic]. / Perkutan lukning av ductus arteriosus med paraplyteknikk.

In four patients between three and five years of age we performed closure of open ductus arteriosus with the Rashkind umbrella technique after percutaneous approach from the right groin. The smallest internal ductus diameter measured between 2 and 4 mm, and pulmonary blood flow was from 1.4 to 2.0 times systemic flow. In all four patients, pulmonary artery pressure was normal. In three patients the ductus arteriosus was completely closed, in the fourth a small residual shunt persisted. There were no complications. We describe the method in detail.

[Proton spin tomography in the diagnosis of aortic isthmus stenosis in infancy]. / Kernspintomographie in der Diagnostik der Aortenisthmusstenose im Säuglingsalter.

We present the use of nuclear magnetic resonance imaging in the diagnostic work-up of coarctation in infancy. The diagnosis may be difficult with usual non-invasive methods, especially when shunts disguise the typical findings. Although magnetic resonance will certainly not be the method of choice in all cases, it should be seriously considered in infants when other non-invasive methods do not give a definite diagnosis.

Senning operation for transposition of the great arteries in the first month of life.

Twelve patients with uncomplicated transposition of the great arteries were operated upon in the first month of life following an initial Rashkind procedure. They were all in a clinically unacceptable condition and were mostly acidotic or bad mixers without acidosis. Two patients died postoperatively from a cerebral haemorrhage not discovered prior to operation. One patient died 5 months postoperatively from bronchiolitis. At postoperative follow-up, all patients were asymptomatic. Cardiac catheterization showed that 1 patient had a significant upper caval vein stenosis, 2 patients had small atrial shunts, and 1 patient had an unimportant pulmonary stenosis. Innocent atrial rhythm disturbances were encountered in 1 case only, whereas 9 patients had normal sinus rhythm at the last examination. The Senning operation remains a good alternative for transposition repair in the neonate.