Use of the Amplatzer muscular ventricular septal defect occluder for closure of perimembranous ventricular septal defects.

BACKGROUND: Transcatheter closure of a perimembranous ventricular septal defect (PmVSD) is usually performed with an asymmetric Amplatzer occluder, which is not an ideal device. Experience with the use of the Amplatzer muscular ventricular septal defect occluder (MVSO) to close selected PmVSDs is presented. SETTING: Two tertiary referral centres for paediatric cardiology in two countries. OBJECTIVE: To look at the safety and efficacy of the application of the MVSO in patients with appropriate PmVSD anatomy. PATIENTS AND INTERVENTION: The procedure was performed in 10 patients aged 3.2-40 (mean 12.5) years. All had a PmVSD with a mean diameter of 5.4 (range 4-11) mm, with an extension towards the muscular septum. The mean distance of the defect from the aortic valve was 5.4 (range 4-6) mm. In all but one patient, the MVSO was introduced in routine antegrade transvenous fashion (4-mm device in one patient, 6-mm device in five, 8 mm in two, 10 mm in one, and 12 mm in one). In one patient, the device was deployed by retrograde implantation. RESULTS: All procedures except one were performed without complications, and complete closure of the VSD was achieved. One patient with a residual shunt developed haemolysis, which resolved over 10 days. In three patients, trivial, non-progressive tricuspid regurgitation appeared after the procedure. No other complications were observed over 1.7 (range 0.2-3.5) years of follow-up. CONCLUSION: Application of the MVSO for closure of selected PmVSDs seems to be a safe and effective treatment option.

Atrioventricular block after transcatheter closure of perimembranous ventricular septal defects.

OBJECTIVE: To look at the presentation, treatment and outcome of patients who developed atrioventricular block after transcatheter closure of a perimembranous ventricular septal defect (PMVSD) with the Amplatzer PMVSD device. SETTING: Three tertiary referral centres for paediatric cardiology in two countries. RESULTS: All three patients presented within 10 days of the procedure. All three patients were treated with intravenous steroids. A permanent pacemaker was inserted in all patients but no pacemaker required activation after two months. CONCLUSION: Complete atrioventricular block occurring in the weeks after device occlusion of a PMVSD appears to resolve quickly. Continued involvement in multicentre device databases is required to monitor safety.

Postinfarction ventricular septal defect closure with Amplatzer occluders.

OBJECTIVE: Postinfarction ventricular septal defect (PIVSD) is a rare and life-threatening complication with high risk of both surgical and medical treatment. Another option available now is transcatheter closure. The purpose of this paper is to report the results of such treatment with Amplatzer occluders. METHOD: Seven patients aged from 51 to 71 years were included. The procedure was performed between 2 and 10 weeks after myocardial infarction. One patient had double residual VSD (2 months after previous surgery) and another, coexisting critical stenosis of right coronary artery (RCA). All patients were in III/IV NYHA class, on intropes, one patient on aortic balloon counterpulsation. Venous jugular approach was used to close the VSD in six patients, venous transfemoral in one patient. Implantation of six Ampaltzer atrial septal occluders (ASO) and one muscular Amplatzer VSD occluder (VSO) were performed. RESULTS: All procedures but two were finished successfully. In one patient, the defect could not be entered neither from the venous nor the arterial side due to unusual oblique course (which was confirmed during subsequent operation). In the second patient (2 weeks after MI), the reason was unstable position of 24 mm ASO (probably due to necrotic borders of VSD). Immediate significant clinical improvement was achieved in all patients, in whom PIVSD was closed with Amplatzer occluders. In one postsurgical patient, two ASO were used; in another patient, prior to VSD closure, PTCA and stent implantation to RCA was performed. The stretched diameter of PIVSD ranged from 8 to 22 mm, the size of implanted Amplatzer occluders from 12 to 24 mm. Fluoroscopy time was 60 min (18-120). During the procedure, ventricular fibrillation requiring defibrillation was observed in three patients. One patient died 1 week after the procedure because of multiorgan failure and increasing mitral incompetence (MI). CONCLUSIONS: Despite some technical problems, implantation of Amplatzer occluders, is an attractive option of treatment of patients with subacute PIVSD.

Major intrahepatic veno-venous fistula after modified Fontan operation treated by transcatheter implantation of amplatzer septal occluder.

Patients with complex congenital cardiac malformations who have been converted to the Fontan circulation with partial exclusion of the hepatic veins may develop progressive cyanosis because of formation of intrahepatic veno-venous malformations. We describe transcatheter closure of a major intrahepatic fistula in such a setting using an Amplatzer septal occluder delivered by the left jugular venous approach in a 5 year old boy.

[Transcatheter closure of atrial septal defects in adults with the Amplatzer atrial septal occluder]. / Przezcewnikowe zamykanie ubytku miedzyprzedsionkowego u doroslych za pomoca korka Amplatzer.

UNLABELLED: In many centres the Amplatzer Septal Occluder (ASO) (AGA Med. Corp. Minnesota, USA) has become the device of choice for secundum atrial septal defect (ASD) closure in children. Current trend towards transcatheter closure of ASD in children could be translated to adults and many patients (pts) may avoid the need of open heart surgery. Assessment the efficacy and complication of device occlusion of ASD in adults, using ASO. Between October 1997 and April 2001 transcatheter closure of ASD was attempted in 51 pts who fulfilled the inclusion criteria--significant shunt with sufficient rims of interatrial septum. Mean age of pts was 29 (16-63) y, mean ASD diameter assessed by transesophageal echocardiography (TEE) was 14.7 (7-24) mm, assessed during catheterization by balloon sizing (stretched diameter) was 20.2 (8-36) mm. There were 9 pts with multiple ASDs, 2 pts with aneurysm of interatrial septum and 2--after previous surgery (recanalization of ASD). The ASO devices were successfully implanted in all, but one pt. In one patient because of unstable position of ASO (floppy rims), device was removed and bigger one was applied during next session. In one case early embolization to abdominal aorta occurred, ASO was translocated to aortic arch with Dotter basket and removed from aorta during simultaneous surgical closure of ASD. Mean fluoroscopy time was 15 (4-50) min. The occlusion rate after 24 h was 90%, after 1 month (m) 92%, after 3 m 93.5%, after 1 year (y) 93.3% and after 2 y 93.3%. All residual shunts were trivial. There were no late complication. CONCLUSIONS: The excellent results of ASD closure with ASO in adults indicate this treatment as a method of choice in selected patients, but long term follow-up is necessary to state final judgement.

Coarctation of the aorta with right aortic arch. A rare case and atypical clinical picture.

We report the case of a 13-year-old girl with a diffusely hypoplastic right aortic arch, anomalous origin of the left subclavian artery, and a small, insignificant ventricular septal defect. The patient's pulse was forceful at the carotid arteries, but it was markedly weaker at all extremities. Catheterization revealed that both common carotid arteries arose from the dilated ascending aorta; the right subclavian and vertebral arteries arose from the hypoplastic posterior segment of the aortic arch, and the left subclavian artery arose from the normally developed descending aorta. The pressure gradient between the ascending and descending aorta was 80 mmHg. A bypass grafting procedure was performed to connect the ascending and the upper abdominal aorta. No pressure gradient remained after the operation, nor was a gradient detected during 2 years of follow-up. The origin of both subclavian arteries distal to the area of coarctation resulted in an atypical clinical picture and delayed diagnosis. Ten previously reported cases of coarctation of the aorta with right aortic arch are reviewed.

Stent implantation for post-Mustard systemic venous obstruction.

In this paper, we report on the use of stents in the treatment of late-onset post-Mustard systemic venous obstruction in three patients with clinical signs of obstructive caval syndrome. After unsuccessful balloon dilation, Palmaz-Schatz stents were implanted at the veno-atrial junction. Further dilation has been achieved using high-pressure balloons. Vessel diameter increased from 4.4+/-1.8 to 13+/-1.7 mm (P < 0.05) and the trans-stenotic pressure gradient dropped from 8+/-6 to 0 mmHg (P < 0.01), with clinical improvement. After 26+/-4 months of non-invasive follow-up, no signs of recurrent stenosis were observed. Stent implantation is effective in the treatment of systemic venous obstruction after Mustard operation.

[The role of the stent in non-coronary cardiopathies]. / Papel del stent en las cardiopatías no coronarias.

INTRODUCTION: The stent has demonstrated to be a useful device in the prevention of postangioplasty coronary restenosis and it is expected to have a favourable effect as an alternative or complementary treatment of stenotic lesions in arteries or veins associated with congenital defects. The aim of this study is to analyze our experience in this setting. MATERIAL AND METHODS: From February 1992 to March 1996, 28 stenting procedures were performed in 26 patients (mean age: 8.6 +/- 0.7 years; mean weight: 26.2 +/- 3 kg). In 12 patients, stenting was single, and a iliac Palmaz stents were always used. Stenting location was: pulmonary artery branches in 17 patients, right ventricular outflow in 2 patients, in the junction of right atrium with pulmonary artery in 2 patients, systemic veins in 2 patients and in post Mustard intratrial channel stenosis in 2 patients. 25 patients had previously undergone at least one surgical procedure. RESULTS: The stenotic diameter of the treated lesions increased significantly after the procedure (4.4 +/- 0.3 mm before stenting vs 11.6 +/- 0.3 mm after stenting, p < 0.0001) and the transtenotic gradient decreased from 38.1 +/- 5 to 12 +/- 3.8 mmHg. Those changes were associated with a diminution of right ventricular pressure (81.6 +/- 3 vs 56.7 +/- 6 mmHg, p < 0.0001) in patients with pulmonary branch stenosis without septal defects. There was no mortality among the percutaneously treated patients and only one patient needed surgery. Nevertheless, one patient died after bilateral intraoperative stenting. CONCLUSION: The treatment of proximal or distal stenotic lesions in the pulmonary tree, systemic veins, and obstructed intraatrial channels with stents, can replace or complement conventional balloon angioplasty. It also offers a useful and effective alternative to surgery, when it is impossible or carries a risk.

Cellular adhesion molecules changes in myocardium during first year post heart transplant.

The increased presence of intercellular adhesion molecule-1 (ICAM-1) and vascular adhesion molecule-1 (VCAM-1) in the myocardium after orthotropic heart transplantation (OHT) has been implicated in early and late organ rejection. The aim of this study was to investigate the changes in ICAM-1 and VCAM-1 during the first years post-OHT. Accordingly, we studied multiple endomyocardial biopsy specimens collected from 11 randomly selected patients (all males, mean age 46 +/- 11 years). Qualification criteria for OHT included: ischemic cardiomyopathy in 6 pts (55%) and idiopathic dilated cardiomyopathy in 5 pts (45%). All patients were receiving triple immunosuppressive regimen. The therapy consisted of cyclosporine, azathioprine, and prednisolone. Multiple endomyocardial biopsy specimens were collected at 7, 30, 90 and 360 days post OHT from all patients (pts), and examined routinely for histologic signs of organ rejection. To assess levels of adhesion molecules we used monoclonal antibodies (murine anti-human ICAM-1 and VCAM-1) on frozen sections. Immunoreactivity (IR) was detected using a commercially available kit. Intensity of IR was assessed based on a semiquantitative scoring system. In this study, IR scores > or = 2 + were considered positive for ICAM-1, and scores > or = 1 + were considered positive for VCAM-1. IR scores in specimens obtained from consecutive biopsies were compared with the initial biopsy collected at the day 7. The results were analyzed using nonparametric statistics. The routine evaluation revealed histological signs of organ rejection (> or = 2) in 2 pts at 7 days, in 5 pts at 30 days, in 3 pts at 90 days, and in 1 patient at 360 days. On the other hand, ICAM-1 and VCAM-1 expression were absent in the majority of patients at 7, 30 and 90 days, but their presence was significantly increased at 360 days (p < 0.05). Absence of the early expression of ICAM-1 and VCAM-1 may be related to the protective effect of triple immunosuppressive therapy in these patients. The expression of ICAM-1 and VCAM-1 strongly emerging at 1 year post OHT may reflect chronic rejection in myocardium. In conclusion, the immunohistological monitoring of the adhesion molecules in biopsy specimens during routine biopsy schedule may be helpful for the discovery of chronic rejection.

[Glucose metabolism in children undergoing extracorporeal circulation: its correlation with weight and the degree of hypothermia]. / Metabolismo de la glucosa en niños sometidos a circulación extracorpórea: correlación con peso y con grado de hipotermia.

INTRODUCTION: A prospective study has been carried out in 33 children with congenital heart disease, aged 5 days to 16 years, operated on with the aid of cardiopulmonary bypass (CPB), in order to evaluate intra and postoperative glycemia in relation to children's weight and intra-CPB perfusion temperature. MATERIAL AND METHODS: Plasmatic levels of glucose, lactate, insulin, C-peptide, growth hormone (GH) and cortisol were measured at five different times, from pre-CPB up to 18 hours post-CPB. Thirteen children weighing less than 10 kilograms (group I) were compared to 20 children weighing more than 10 kilograms (group II), and 15 children operated on with perfusion temperature below 27 degrees C (group A) were compared to 18 with perfusion temperature above 27 degrees C. RESULTS: All the cases showed intraoperative hyperglycemia. In relation to weight, evolution of glycemia was similar in both groups but anaerobic glycolysis was significantly higher in group I. In relation to temperature, glycemia, lactic acidemia intra- and post-CPB and serum C-peptide post-CPB were significantly higher in group A. Intraoperative insulin was decreased and GH serum levels were also higher in group I and A, but fell drastically post-CPB in all groups. CONCLUSIONS: Perioperative control of glycemia is important in children operated on with the aid of CPB. Control of lactic acidemia is also expedient. Carbohydrates metabolism and its hormonal regulation are more disturbed in infants and in children operated on with low perfusion temperature.

[Non-surgical extraction of intravascular foreign bodies in children. Experience with 8 cases]. / Extracción no quirúrgica de cuerpos extraños intravasculares en niños. Experiencia en 8 casos.

BACKGROUND AND OBJECTIVES: Foreign body retrieval in the catheter room is a useful procedure at any age, but, although its interest, few reports of such technique have been reported in children. METHODS: We review and present our experience in 8 children, aged 5 days to 11 years, five of them having congenital hearts defects. RESULTS: We retrieved 4 catheter fragments, 2 endocardial electrode catheter tips, 1 Rashkind 12 mm. PDA umbrella, and 1 detachable Jackson coil. All of them were placed in systemic veins, right heart chambers or pulmonary arteries. We used biplane fluoroscopy and percutaneous right femoral vein puncture in all cases. Goose-Neck (Microvena Corporation) snares were used in 5 patients, hand made snares in 2 and a Swan-Ganz catheter in one. In 4 cases, the snare was introduced trough a Mullins long sheath and the foreign body pulled into its distal end, in order to bring it out of the femoral vein. Six foreign bodies came off the femoral vein: 4 trough the puncture site and 2 needing a venous cut-down. The two remaining foreign bodies, stopped while pulling at the common iliac vein and a minor surgical procedure was needed for final extraction. CONCLUSIONS: Therapeutic catheterization is the technique of choice for intravascular foreign body retrieval in children.

[Selected indices in perinatal evaluation of the newborn with respiratory disturbances. I. Usefulness in differential diagnosis]. / Wybrane wskazniki okoloporodowej oceny noworodków z zaburzeniami oddechowymi. I. Przydatnosc w diagnostyce róznicowej.

There are several maternal and fetal factors, which may influence the development of respiratory disturbances in the newborn. Certain, indices of perinatal evaluation (delivery route, gestational age, birth weight, maturity degree, sex and Apgar score) in two groups of patients were compared. The first group compressed 36 newborns with Hyaline Membrane Disease (HMD), the second called aspiration--pneumonia (AP) group-44 newborns with other kinds of respiratory disturbances: 18 with pneumonia, 12 with fetal liquid aspiration syndrome, 8 with meconium aspiration syndromes and 6 with transient tachypnea. The diagnosis of each kind of respiratory disorder was based on the criteria of Hjalmarson. In both groups high percentage of newborn babies born by cesarean section (higher in AP group) was found. Hence the conclusion that the decision to perform this procedure should consider the risk of neonatal pulmonary complications. In the HMD group lower gestational age was found and in the AP group more babies were born with intrauterine dystrophy. In both groups prevalence of the male sex and low Apgar score was found.

[Selected indices in perinatal evaluation of the newborn with respiratory disturbances. II. Prognostic value]. / Wybrane wskazniki okoloporodowej oceny noworodków z zaburzeniami oddechowymi. II. Przydatnosc rokownicza.

Respiratory disturbances are the most frequent causes of life-threatening situations in the newborn. In this paper evaluation indices of the newborn were compared in 2 groups of patients. The first comprised 36 babies with Hyaline Membrane Disease (HMD) with good or fatal outcome, in the second 44 newborns with Aspiration-Pneumonia (AP) syndromes were included. Prognostic value of following indices was considered: delivery route, gestational age, birth weight, maturity degree, sex and Apgar score. Bad prognostic value of low birth weight was found in the HMD group and low Apgar score and intrauterine dystrophy in the AP group. Mortality was similar in children delivered vaginally and by cesarean section in both groups of patients.

[Anatomical correction of transposition of the great arteries in 7 newborn infants]. / Korekcja anatomiczna przelozenia wielkich pni tetniczych u siedmiu noworodków.

In the presented article, the course and results of anatomical correction of transposition of great arteries (TGA) in 7 neonates (2 females and 5 males) with mean body mass of 3250 g and 2 to 5 days old (mean 3 days) are reviewed. Surgery was performed in moderate hypothermia. St. Thomas cold cardioplegia was used. Mean aortic clamping time was 70 min (55-115), and the time of extracorporeal circulation was 165 min (117-210). Low cardiac output in all patients in the postoperative period required prolonged mechanical ventilation and positive inotropic drugs. Out of 7 patients operated, two died (29%). The cause of death in both cases was myocardial ischemia of right ventricle. The other 5 patients were discharged after healing of operational wound. In the control echocardiographic examination performed 3 to 12 months postoperatively, apart from one case of moderate pulmonary artery stenosis, no other haemodynamically significant complications were noted.

[Rheumatic cardiopathy in children younger than 6 years of age]. / Cardiopatía reumática en niños menores de 6 años.

UNLABELLED: Most of the published papers on Rheumatic Fever (RF) have not included the younger population. We selected 211 cases of children with RF younger than 6 years of age from 9,471 clinical files from 1944 to 1982. These were followed retrospectively to identify the presence of rheumatic activity, subsequent attacks and penicillin profilaxis. From de 211 cases, 209 had carditis; 57% of them were girls and 43% boys. There were no previous infections of the upper respiratory tract in 36% of the patients. The number of cases with RF increased abruptly after 3 years of age and continued increasing until 5 years of age when 70.5% of the population had there first clinically recognized attack. Lesions were present in the mitral valve in 80% of the cases, in the aortic valve in 12%, in the tricuspid in 5% and in the pulmonary valve in 3%. The death rate during the first attack was 20% being refractory heart failure the main cause of death. Thirteen cases suffered rheumatic pneumonia, 9 of whom died (69.2%). CONCLUSIONS: 1) The incidence of acute rheumatic fever in children under 6 years of age has decreased with time. 2) The death rate as well as the valvular damage decreased with the parents cooperation with the treatment. 3) The changes in the clinical picture and the severity of valve sequelea may be due to penicillin profilaxis and the better understanding of the disease.

[Clinical forms of respiration disorders in newborn infants]. / Kliniczne postacie zaburzen oddechowych u noworodków.

Ventilatory disturbances are the most often cause of death in neonates. The study was carried out on 80 neonates, treated in the intensive care unit. In all during the first 48 hours the following symptoms occurred: tachypnoea (above 60 per minute), cyanosis, retraction of the intercostal muscles, expiratory disturbances. Diagnosis of clinical forms of ventilatory disturbances was made basing on Hjalmarson's criteria. The results of this study imply that the most often seen form of ventilatory disturbances in neonates in the hyaline membrane disease--found in 45% of the cases. The highest mortality (75%) was seen in the meconium aspiration syndrome.

[Long term results of treatment with cytostatic drugs on respiratory function tests in children]. / Odlegly wplyw leczenia cytostatykami na wyniki badan czynnosci pluc u dzieci.

In 47 children after a 5 year remission of treated acute lymphoblastic leukemia respiratory function was assessed. In 11 a decrease of VC was found, while in 12 signs of obturation of lower airways were present. These results imply the presence of restrictive changes suggesting an interstitial lung disease. These children are at risk of developing a chronic pneumopathy.