[Interventional occlusion of persistent ductus arteriosus Botalli with Gianturco spirals]. / Interventioneller Verschluss des persistierenden Ductus arteriosus Botalli mit Gianturco-Spiralen.

Interventional duct occlusion is increasingly performed with detachable coils. In a series of 33 patients (34 procedures), we consistently used Gianturco-coils which were the initially employed coils. They are not securable but can be implanted in a simple manner. The rate of embolization was high (28%) in the first 14 procedures; however, it declined to 5% in the last 20 patients. We modified the technique by partially blocking the duct, when necessary, with a catheter which was advanced from the pulmonary artery and by implanting up to three coils. There was only one embolization and one small residual shunt in the last 20 patients. The mean fluoroscopy time in these patients was 11.0 +/- 5.8 minutes and decreased to 7.0 +/- 3.2 minutes in the last 10 patients with successful closure. Considering our experience, we continue to recommend the use of Gianturco-coils especially in the small duct which can be closed quickly at low cost.

Congenital aneurysm of the descending aorta in a neonate.

A large aneurysm of the descending aorta of unknown etiology was diagnosed in utero. Medical treatment with propranolol was continued after surgery which was performed at the age of 2 months.

Pulmonary valve annulus grows after balloon dilatation of neonatal critical pulmonary valve stenosis.

BACKGROUND: Neonates with critical pulmonary valve stenosis often demonstrate small or hypoplastic right ventricular structures. Relief of the obstruction enhances forward flow across the right ventricle and reduces its pressure load. Growth of the right ventricle and especially of the pulmonary valve annulus was evaluated after balloon dilatation. METHODS: Ten consecutive neonates with critical pulmonary valve stenosis who underwent balloon valvuloplasty were studied by serial echocardiography to assess growth of right ventricular structures at follow-up. RESULTS: The mean diameter of the pulmonary valve annulus increased from 6.1 +/- 1.4 mm to 12.6 +/- 3.5 mm (z scores from -2.9 +/- 1.0 SD to - 1.3 +/- 1.2 SD, p < 0.0001) after a mean follow-up period of 2.7 +/- 2.0 years. The mean diameter of the tricuspid valve annulus increased from 12.9 +/- 3.8 mm to 19.0 +/- 3.1 mm; however, the respective z score did not change significantly (from 0.5 +/- 2.4 SD to -0.5 +/- 1.0 SD). Right ventricular cavity size was hypoplastic in four patients initially and normal in all patients at latest follow-up. CONCLUSIONS: Balloon dilatation of critical pulmonary valve stenosis encourages catch-up growth of the pulmonary valve, and surgery may be avoided even in a hypoplastic pulmonary valve annulus.

[Recanalization of Botalli's ductus arteriosus after catheter occlusion with a Rashkind double umbrella device]. / Rekanalisation eines Ductus arteriosus Botalli nach Katheterverschluss mit einem Rashkind-Doppelschirmchen.

Interventional duct occlusion with the Rashkind PDA occluder has become a widespread alternative treatment to surgery. Whereas residual shunting is well-known, reopening of a completely occluded duct has been published in only 3 cases. We report another case of a young adult in whom ductal shunting reappeared after he had restarted physical exercise and sports activities soon after the occlusion procedure. Recanalization might have been due to changing of the device position without embolisation. A second Rashkind PDA occluder was successfully implanted 6 months after the first procedure with complete closure of the residual shunt.

Transcatheter closure of atrial septal defects by the "buttoned" device: complications and need for surgical revision.

Immediate and medium-term experience with transcatheter closure of a secundum atrial septal defect by the "buttoned" device in seven patients is reported. Complications occurred in two patients during the procedure. In one patient with complications, the occluder was partly released in the right atrium. All efforts to correct its position were unsuccessful and caused considerable deformation of the device, which had to be removed surgically. In the other patient with complications, disconnection of the occluder and counteroccluder occurred immediately after removal of the loading wire. Both parts were retrieved by catheter. Five patients had uneventful closure of the atrial septal defect. On follow-up, however, displacement of the device towards the mitral valve was observed in two patients, which caused mitral regurgitation. Surgical removal of the device and repair of the mitral valve was necessary in both patients. Two years after the procedure, the atrial septal defect was closed completely in two of the remaining three patients and a small residual defect persisted in one patient.

Pulmonary artery banding with a novel percutaneously, bidirectionally adjustable device.

Pulmonary artery banding is commonly performed as a palliative procedure in complex congenital heart disease, when pulmonary blood flow is increased. However, the hemodynamics may change postoperatively requiring readjustment of the band, which may necessitate a second operation. We report a new system for pulmonary artery banding which allows precise placement of the band intraoperatively, as well as bidirectional percutaneous adjustment of the band postoperatively. Via left lateral thoracothomy the new device was implanted without complications into a neonate with congestive heart failure due to tricuspid atresia (IIc) and coarctation of the aorta. Although optimal placement of the band had been achieved intraoperatively the band had to be tightened 25 h after the operation and released 85 h after the operation in order to optimize hemodynamics. The bidirectionally adjustable device for banding of the pulmonary artery is superior to previously used devices with either no or unidirectional adjustability of the band because it is safe and easy to implant and has the potential to reduce the number of reoperations associated with this type of procedure.

Treatment of critical pulmonary valve stenosis by balloon dilatation in the neonate.

Critical pulmonary valve stenosis represents an emergency, and immediate treatment is mandatory. The purpose of this study was to evaluate the immediate and medium-term results of pulmonary valve dilatation. We report 18 neonates in whom pulmonary valvuloplasty was attempted. The procedure could be accomplished in 14 patients. The angiographically determined diameters of the pulmonary and tricuspid valve at the time of procedure were 5.6 +/- 1.5 mm and 14.0 +/- 5.4 mm. The mean Doppler gradient decreased from 71 +/- 27 mm Hg to 27 +/- 14 mm Hg. Perforation of the right ventricular outflow tract was the major complication in three patients with one fatal event. Infusion of prostaglandin E1 could be discontinued 1 to 5 days after the procedure. On follow-up three children required a second balloon dilatation with good results. Seven patients monitored for more than 9 months with a mean follow-up time of 34.4 +/- 16 months had a residual gradient of 11.6 +/- 6.7 mm Hg. In spite of a hypoplastic pulmonary valve annulus in seven of the patients, results were good and surgery could be avoided.

Surgical commissurotomy of the aortic valve: outcome of open valvotomy in neonates with critical aortic stenosis.

Early intervention is necessary in neonates with critical aortic stenosis. The advent of alternate therapy, particularly balloon aortic valvuloplasty, requires a reappraisal of the traditional surgical approach, including the efficacy of initial transvalvar gradient reduction and freedom from recurrence of obstruction in the longer term. This report describes a series of 33 consecutive infants who underwent surgical aortic valvotomy in the first month of life. The hospital mortality was 18 percent with a 5-year probability of survival of 66 percent (90 percent CI, 50 percent to 79 percent). Fourteen reinterventions, nine reoperations and five balloon dilatations, were required at a median age of 0.8 years (range 9 days to 6 years). Three patients died after reintervention (one early and two late). The median follow-up time was 5.8 years (range 0.2 to 14 years). At last follow-up Doppler investigation the average Doppler mean and peak gradients were 34 mm Hg and 18 mm Hg, respectively. Open valvotomy in neonatal aortic valve stenosis allows the use of an appropriate surgical approach with low initial mortality and satisfactory 5-year freedom from reintervention.

[Comparative studies of hemodynamics under prostacyclin and nifedipine in patients with Eisenmenger syndrome]. / Vergleichende Untersuchungen der Hämodynamik unter Prostazyklin und Nifedipin bei Patienten mit Eisenmenger-Syndrom.

In 10 patients 4-20 years of age with obstructive pulmonary vascular disease after Eisenmenger reaction pulmonary arterial and aortic pressure, pulmonary and systemic flow (Qp, Qs), and pulmonary and systemic vascular resistance (Rp, Rs) were determined before and during stepwise increasing doses of prostacyclin and nifedipine. Prostacyclin caused a significant decrease of pulmonary and systemic vascular resistance and a significant decrease of pressures in the aorta and the pulmonary artery, whereas no significant changes of hemodynamics were realized following nifedipine. The individual hemodynamic changes during prostacyclin were favorable in only two patients who demonstrated a marked decrease of Rp with no substantial change of Rs resulting in an increase in arterial oxygen saturation. In the other patients prostacyclin resulted in an adverse effect with a decrease of Rs exceeding that of Rp in 5 patients, a paradoxical increase of Rp in 1 patient, and a critical decrease of Qs with respective low central venous blood oxygen saturation and consequently low arterial oxygen saturation caused by a small right-to-left shunt in 2 patients. Nifedipine did not bring about a significant general change of hemodynamic parameters. The individual control showed no effect in 5 patients, an unfavorable effect by a decrease of Rs exceeding that of Rp in 2 patients, and a favorable net effect in only 3 patients, induced in 1 patient only after a very high dosage of nifedipine and leading to a certain increase of arterial oxygen saturation in only 1 other patient. From our results a beneficial effect of nifedipine in an average dosage on the hemodynamics of patients with Eisenmenger syndrome cannot be recognized.(ABSTRACT TRUNCATED AT 250 WORDS)

[Sinus node dysfunction in children without heart defect]. / Sinusknotendysfunktion bei Kindern ohne Herzfehler.

Sinus node dysfunction (SND) is a rare cause of bradycardia in children without structural heart disease. The clinical and diagnostic findings in 4 children with this condition are described. Two of them presented with symptoms, in one arrhythmias had been noted before birth, and a routine physical examination had revealed bradycardia in another. Age at onset of either clinical symptoms or bradycardia ranged from 0 to 11 1/2 years. Routine and 24-h-electrocardiograms showed atrioventricular junctional rhythms with minimal rates of 25/min and episodes of asystole with a maximal duration of 10.3 s. Other electrocardiographic abnormalities such as first degree atrioventricular block, ventricular extrasystoles or tachycardia were common findings. Electrophysiological studies were performed in 3 cases and confirmed the diagnosis of SND. A permanent pacemaker was inserted in 2 children; medical treatment did not have any long-term effect. During a follow-up period of 5 to 13 years there were no complications. In summary, SND in childhood can be assessed by Holter monitoring with high reliability. Electrophysiological studies are not necessary and of limited value. Therapeutic policies and prognostic statements are difficult to establish due to the small number of cases so far described. Permanent cardiac pacing, however, is unavoidable in symptomatic children.

Complete heart block in a 9 year old girl caused by borreliosis.

A complete atrioventricular block was seen in a nine year old girl in whom an infection with Borrelia burgdorferi was confirmed by serological testing. There were no other symptoms or cutaneous manifestations of the disease. Though a rash on the right ear was later recalled by her parents. The patient was treated with high dose penicillin and orciprenaline was given intermittently. The complete heart block disappeared within four days.

Oxygen deficit and blood lactate in prepubertal boys during exercise above the anaerobic threshold.

Recent studies have shown that in boys a steady-state of blood lactate is maintained at exercise levels above the anaerobic threshold. Therefore, the explanation hitherto provided for the steeper increase in blood lactate beyond the anaerobic threshold, i.e. the onset of anaerobic metabolism, needs modification. Investigations were carried out in ten boys, aged 11-12 years, during treadmill running. Maximal oxygen uptake (VO2max) and maximal blood lactate were determined during incremental exercise. Subsequently each boy performed four runs at different high constant speeds of 16 min duration, in order to determine maximal steady-state blood lactate. The underlying data also served to estimate roughly the lactate anaerobic threshold. Oxygen uptaken (VO2) was measured at 0.5 min intervals during the initial 7.5 min of each constant-speed run. Maximal steady-state blood lactate was 5.6 mmol/l corresponding to 92% of VO2max. The mean blood lactate at which the anaerobic threshold was reached or just exceeded was 2.7 mmol/l corresponding to 82% of VO2max. Oxygen transport transient kinetics were computed from the mean 0.5 min VO2-values during the constant-speed runs near the maximal steady-state blood lactate and from runs near the anaerobic threshold. Half-times of VO2 response were shorter than values previously reported for adults due to a faster increase in VO2 at the onset of exercise. Half-times increased with increasing work rates as did the oxygen deficit, due to a slower increase in VO2 along with a longer time required to attain a steady-state at higher work rates.(ABSTRACT TRUNCATED AT 250 WORDS)

Anaerobic threshold and maximal steady-state blood lactate in prepubertal boys.

To elucidate further the special nature of anaerobic threshold in children, 11 boys, mean age 12.1 years (range 11.4-12.5 years), were investigated during treadmill running. Oxygen uptake, including maximal oxygen uptake (VO2max), ventilation and the "ventilatory anaerobic threshold" were determined during incremental exercise, with determination of maximal blood lactate following exercise. Within 2 weeks following this test four runs of 16-min duration were performed at a constant speed, starting with a speed corresponding to about 75% of VO2max and increasing it during the next run by 0.5 or 1.0 km.h-1 according to the blood lactate concentrations in the previous run, in order to determine maximal steady-state blood lactate concentration. Blood lactate was determined at the end of every 4-min period. "Anaerobic threshold" was calculated from the increase in concentration of blood lactate obtained at the end of the runs at constant speed. The mean maximal steady-state blood lactate concentration was 5.0 mmol.l-1 corresponding to 88% of the aerobic power, whereas the mean value of the conventional "anaerobic threshold" was only 2.6 mmol.l-1, which corresponded to 78% of the VO2max. The correlations between the parameters of "anaerobic threshold", "ventilatory anaerobic threshold" and maximal steady-state blood lactate were only poor. Our results demonstrated that, in the children tested, the point at which a steeper increase in lactate concentrations during progressive work occurred did not correspond to the true anaerobic threshold, i.e. the exercise intensity above which a continuous increase in lactate concentration occurs at a constant exercise intensity.

Special problems in Fontan-type operations for complex cardiac lesions.

Four patients with complex cardiac lesions, who underwent successfully a Fontan type of operation are presented, each case representing a particular problem in surgical management. The first exhibited displacement of a trileaflet straddling and overriding left atrioventricular valve in addition to tricuspid atresia, while the second one had unilateral lung perfusion. In the third case, a stenosis had developed near the origin of the right pulmonary artery, and pulmonary vascular resistance could not be determined prior to the definite palliation procedure, whereas the fourth patient presented with stenosis of the left atrioventricular valve. Surgery was planned after detailed evaluation of the morphologic and hemodynamic features by means of echocardiography and cardiac catheterization. Our results illustrate the common tendency to extend the limits for modified Fontan procedures, which also includes revision of the original criteria for selection.

Atrioventricular septal defect with double-outlet right atrium.

A rare type of primum atrial septal defect is described in which the atrial septum deviated to the left, resulting in a double-outlet right atrium. The obstruction at the site of the small atrial septum defect led to an elevation of the left atrial mean pressure and to pulmonary arterial hypertension. The presence of mixed blood in the right atrium caused systemic arterial desaturation. A cleft in the septal leaflet of the left atrioventricular (AV) valve caused a left ventricular-right atrial shunt, which-added to the left-to-right atrial shunt--totaled 60% of the pulmonary circulating volume. Preoperative knowledge of the anatomic features facilitated surgical repair, which consisted in resection of the abnormal septum, construction of a new septum with a Dacron patch, and closure of the septal commissure of the left-sided AV valve.

[Modified Blalock-Taussig anastomosis. Its significance within the scope of surgical treatment of tetralogy of Fallot]. / Die modifizierte Blalock-Taussig-Anastomose. Ihre Bedeutung im Rahmen der chirurgischen Behandlung der Fallotschen Tetralogie.

We report our results with 14 cases of tetralogy of Fallot, which were treated with a modified Blalock-Taussig shunt first, total correction having been performed in 9 of them after a mean interval of 15 months. The age of the patients ranged from 12 days to 3.3 years, with a mean of 4.7 months in 12 patients below the age of one year. In 11 cases we used a 5 mm Gore-Tex prosthesis, in 2 a 6 mm prosthesis and in one a 4 mm prosthesis. All children survived the operation. In one case shunt thrombosis occurred after a bout of pneumonia and a second shunt had to be constructed on the other side. Control angiography, performed after a mean period of 15 months in 13 patients revealed a patent shunt, though angulation of the ipsilateral pulmonary artery was noted in one case. The ratio of the diameter of the pulmonary artery to the aorta increased by 20%. The significance of these findings with regard to the question of primary or two-stage repair in infants less than one year old is discussed in view of different risk factors.