[Nonsurgical occlusion of an atrial septal defect in childhood: initial Swiss experiences]. / Nichtchirurgischer Verschluss eines Vorhofseptumdefektes im Kindesalter: erste Schweizer Erfahrungen.

The initial experience with catheter closure of an atrial septal defect (ASD) in children, performed at two Swiss centers is presented. The ASD closures were performed according to international multicenter study protocols. 14 children, aged 3.9-17.5 years underwent closure by catheter. The defect size varied between 12 and 22 mm (balloon sized), the ratio between pulmonary and systemic blood flow showed a mean of 2.2 (1.5-3.5). Catheter closure was done using three different occlusion devices. More recently only the Amplatzer occluder was used at both institutions. In 12 children (86%) defect closure was successful and after a follow-up of 3-32 months (mean 17) only one child had a trivial residual interatrial shunt. In all children, echocardiographic follow-up showed an unchanged and correct device position on both sides of the atrial septum. In two children, a floppy aortic segment of the atrial septal rim led to instable device position: both children underwent surgical defect closure later. The children with successful device closure showed no complications during the catheterization or during follow-up. There were no thrombotic complications on the surfaces of the devices. Catheter closure of an ASD during childhood is a safe and efficient alternative to standard surgical treatment.

Interventional catheterization in surgically treated patients with congenital heart disease.

Interventional catheterization is an alternative to surgery for some congenital heart defects. For other malformations, the surgeon and the interventionist will join in an effort to obtain an optimal result: the typical example is pulmonary atresia with VSD and aortopulmonary collaterals. In other cases, the cardiologist may be called upon to intervene with catheter techniques to correct sequelae or residual lesions after surgical correction, avoiding redo surgery. Most often, the task consists of opening stenoses by balloon dilatation and/or stenting the main targets being pulmonary artery branch stenoses, venous obstructions after Mustard procedure, and recoarctations. Whereas simple balloon dilatation of recoarctation often brings good results, stents are often needed to obtain optimal results in pulmonary branch stenoses. Stenting of pulmonary veins has been disappointing. Closing unwanted vessels and defects is another task for the interventional cardiologist after cardiac surgery. Here, the most frequent procedure is closing aortopulmonary collaterals in pulmonary atresia and VSD after corrective surgery. Advantages and limitations of these procedures are discussed.

Fetal echocardiography in pregnancies of women with congenital heart disease--clinical utility and limitations.

UNLABELLED: Women with important cyanotic or uncyanotic, operated or unoperated congenital heart disease (CHD) have been shown to carry an inherent risk during pregnancy for themselves and for their fetus. Obstetrical and fetal echocardiography has recently been upgraded by new technical developments in ultrasound machines. These improvements have increased the detection rate of congenital malformations and cardiac anomalies which ranged in the past between 4 and 60% for significant anomalies. Obesity or an unfavourable position of the fetus may, however, obscure the imaging quality and cause limitations to visualise the fetal heart from different angles and thus prevent the detection of anomalies. In addition, several cardiac anomalies develop throughout pregnancy and may not yet be present at an early date of screening. While the risk for a congenital cardiac malformation (CCM) in a normal population is 0.8-1%, the recurrence rate for CCM increases to 2 to 3% when a previous child has been affected but will become significantly higher when genetically determined anomalies have affected a family member or when the pregnant woman (5.8%) has CHD. The aim of fetal screening in women with CCM is to ascertain normal intrauterine growth, to exclude fetal CHD and/or to ascertain a malformation or arrhythmia which has been suspected during an obstetrical screening. The acquired detailed echocardiographic knowledge of the malformation or arrhythmia allows the explanation of a CCM to the future parents, to present therapeutic options during pregnancy or after birth and to plan delivery in a tertiary center that provides early cardiovascular and/or catheter interventions and disposes of intensive care facilities for affected newborns. Under certain conditions, termination can be discussed in early pregnancy. Very recent publications have shown how important a prenatal diagnosis can become in a new-born with transposition of the great arteries and a very restrictive foramen ovale (Circulation 1999). Therapeutic measures in the fetus have been attempted with very limited success so far; successful life saving treatment does, however, exist for fetal arrhythmias. IN CONCLUSION: Fetal echocardiography has become an important analytical tool in high-risk pregnancies, especially when parents are affected by a CCM. The examination is safe and can be performed with a high predictive and sensitivity rate.

Local low-dose urokinase treatment of acquired intracardiac thrombi in preterm infants.

We report three cases of intracardiac thrombi in preterm infants of very low birth weight, in whom local low-dose urokinase treatment achieved complete thrombolysis without any signs of systemic fibrinolytic activity or side-effects. This new treatment strategy seems to be safe, requires minimal monitoring of fibrinolytic activity, and may be a new option for thrombolysis in high-risk patients such as premature infants, patients recently operated on, and patients presenting with other contra-indications for systemic fibrinolytic therapy.

Aortic valve repair by cusp extension with the use of fresh autologous pericardium in children with rheumatic aortic insufficiency.

OBJECTIVES: Our goal was to evaluate the midterm results of aortic valve repair by a more sophisticated tailoring of cusp extension-taking into account the dimensions of the native aortic cusps-with the use of fresh autologous pericardium. PATIENTS AND METHODS: Forty-one children who had severe rheumatic aortic insufficiency (mean age 11.5 +/- 2.7 years) underwent aortic valve repair by means of this cusp extension technique over a 5-year period. Twenty-four of them underwent concomitant mitral valve repair for associated rheumatic mitral valve disease. All children were then followed up by transthoracic echocardiography before discharge, at 3 and 6 months after the operation, and at yearly intervals thereafter. RESULTS: Follow-up was complete in all patients and ranged from 3 months to 5 years (median 3 years). No operative and no early postoperative deaths occurred. Only 1 patient died, 9 months after the operation, of septicemia and multiple organ failure. Actuarial survival was 97% at 1 year and has remained unchanged at 3 years. On discharge, the degree of aortic insufficiency was grade 0 for 27 children and grade I for 14. Exacerbation of aortic insufficiency from grade I to grade II was observed in only 1 patient, and none of the children required reoperation for aortic insufficiency during the follow-up period. Mean peak systolic aortic valve gradients at discharge were lower than preoperative values (P =.04), and no significant increase in the peak systolic transvalvular gradient was detected thereafter during the follow-up period. Mean left ventricular dimensions were significantly reduced at discharge when compared with preoperative values (P <.0001). CONCLUSIONS: Functional results of aortic valve repair with cusp extension using fresh pericardium have been satisfactory at medium term, particularly in children with a small aortic anulus at the time of initial repair, because the expansion potential of fresh autologous pericardium is equivalent to that of the growing sinotubular junction and aortic anulus diameters.

Fungal endocarditis in critically ill children.

UNLABELLED: All cases of infective endocarditis occurring from January 1990 to December 1996 at our institution were reviewed, with a special focus on fungal endocarditis. Five critically ill children with fungal endocarditis and eleven children with bacterial endocarditis were recorded. The proportion of fungal endocarditis in our series was 5/16 (31%) and Candida albicans (4/5) was the most common fungal pathogen. Only one patient required heart surgery because of a loose patch but all the others were treated only by medical management for cure. The hospital survival rate was 80% (4/5) and the overall long-term survival rate was 60% (3/5) with only one death directly related to fungal infection. CONCLUSION: Despite the small number of cases, a sole medical approach including amphotericin B and long-term fluconazole prophylaxis for the treatment of fungal endocarditis in critically ill children seems to offer an alternative to surgical treatment which may be kept for failure of medical treatment.

European Study on Maternal and Fetal Management of Fetal Supraventricular Tachyarrhythmia:Proposed Protocol for an International Project

> Objectives: This European protocol is designed to estimate the efficacy of transplacental digoxin administered intravenously to the mother in sustained fetal supraventricular tachyarrhythmias (FSVT) with or without cardiac failure and in intermittent runs of FSVT with cardiac failure. The natural history of FSVT is evaluated in self-limited runs of FSVT without cardiac failure. Study structure: The fetal criteria for inclusion in the study are: gestational age <35 weeks, a normal cardiac anatomy, no severe extracardiac anomalies and a heart rate of the FSVT >200 beats/min. The maternal criteria include no prior treatment with digoxin or drugs effecting fetal heart rate and hemodynamics, and no contraindication to digoxin. Doppler fetal echocardiography is utilized for the diagnosis of the type of FSVT, sequential evaluation, and assessment of fetal hemodynamics. Fetuses are assigned two groups: Group I includes all fetuses with sustained FSVT and those with intermittent runs and cardiac failure; Group II comprises fetuses with intermittent runs and no cardiac failure. Fetuses in Group I will be treated. Group II will receive no treatment initially and will be observed. Treatment is based on a acute loading phase with digoxin administered intravenously to the mother for a period of 7 days. Doses are adjusted to through levels of maternal digoxin, maternal clinical condition, and electrocardiogram. If sinus rhythm is obtained, a maintenance phase follows with oral digoxin. Another drug, according to local experience and preference, is added to oral digoxin if digoxin intravenously fails to restore sinus rhythm and gestational age is <36 weeks. Post natal outcome will be followed by sequential clinical and Holter examinations. The data collected will be reviewed in a blinded fashion. The collection of the data for the protocol requires extensive information on the maternal, fetal, and long-term follow-up studies. They are detailed in the Appendix section. Expected results: We propose to obstetricians and pediatric cardiologists involved in fetal cardiology a rationale for the management and treatment of FSVT. Our expectation is to obtain a controlled study on a large number of cases at an international scale and thus provide a homogeneous maternal and fetal therapeutic strategy for FSVT.

[Short and long term results of pulmonary balloon valvuloplasty in children]. / Résultats à court et long terme de la valvuloplastie pulmonaire au ballonnet chez l'enfant.

Percutaneous balloon valvuloplasty is an alternative to surgical valvotomy for congenital pulmonary valve stenosis. Between 1985 and 1997, 73 valvuloplasties were performed in 70 patients, age range 1 day to 24.7 years. We reviewed the short- and long-term results in regard to transvalvular gradient and pulmonary insufficiency. The gradient was measured pre- and immediately post-valvuloplasty at catheterization and by Doppler echocardiography, and by echocardiography only at follow-up. Valvuloplasty was successful in 61/70 patients whereas surgical valvotomy was necessary in 9/70 because of a very dysplastic valve. A second valvuloplasty was performed in 3 patients presenting with restenosis. A 60% decrease in the gradient was noted immediately after the procedure, from 78.3 +/- 24.5 to 33.7 +/- 19.7 mm Hg (p < 0.05). A follow-up of more than 2 years (4.2 +/- 2.2 years) was obtained in 35 patients. A further decrease in the gradient was noted, to 20 +/- 12 mm Hg, and the degree of pulmonary insufficiency was mild in all patients except 2 with a moderate leak. The mortality was 0% and only 1 patient had a complication (endocarditis). The short- and long-term results of pulmonary percutaneous balloon valvuloplasty are excellent. It can be considered the therapy of choice. The gradient shows a further decrease during follow-up due to regression of the infundibular muscular stenosis. Failure is attributed to very dysplastic valves and these patients may require, in a second stage, a surgical approach (patch enlargement and/or valvulectomy).

Multiple-leaflet mitral valve as a rare cause of congenital mitral insufficiency.

A rare case of congenital mitral insufficiency characterized by six well-demarcated leaflets of mitral valve and annular dilatation is reported. At operation, the mitral valve was successfully repaired by resection of the posterior median leaflet with subsequent annular plication, closure of unusual valvular tissue divisions, and ring annuloplasty.

Dilated cardiomyopathy and thrombo-embolism.

UNLABELLED: The purpose of this study was to investigate the incidence, outcome and prevention of thrombo-embolism in children with dilated cardiomyopathy. From 130 patients with dilated cardiomyopathy, 17 (14%) showed evidence of thrombo-embolism. Seven had initial cardiac thrombus, 7 exhibited initial embolus and in 3 thrombo-embolism was only diagnosed at autopsy. All 17 patients showed seriously impaired systolic function of the left ventricle with fractional shortening (FS) of 10 +/- 3%, range 5%-17%, as compared to those without thrombo-embolism with FS of 17% +/- 6%, range 5%-26% (P < 0.0001). Seven patients were treated with oral anticoagulants once thrombo-embolism had been diagnosed; one of them experienced a further embolic event as opposed to three out of four patients not treated with anticoagulants. CONCLUSION: All children with dilated cardiomyopathy and fractional shortening below 20% should be treated with prophylactic anticoagulative agents.

Hypoplasia of the posterior leaflet as a rare cause of congenital mitral insufficiency.

A rare case of congenital mitral insufficiency characterized by the hypoplasia of the posterior leaflet is reported. At operation, the mitral valve was successfully repaired by a ring annuloplasty, which created a satisfactory surface of coaptation between the anterior leaflet and the bulky posterior muscular structure. The presence of this posterior muscular structure represents a developmental arrest at the stage of conversion from muscular chordae and leaflets to thin connective structures.

Inhaled nitric oxide to test the vasodilator capacity of the pulmonary vascular bed in children with long-standing pulmonary hypertension and congenital heart disease.

Nitric oxide-induced vasodilator capacity greatly varies among children with pulmonary hypertension and elevated vascular resistance. The decline of this selective response seems to parallel the progression of established vascular disease and thus may be helpful for the selection of patients for operation.

Ventricular late potentials and spontaneous ventricular arrhythmias after surgical repair of tetralogy of Fallot: do they have prognostic value?

OBJECTIVE: To determine the prognostic significance of ventricular late potentials and spontaneous ventricular arrhythmias detected early after surgical repair of tetralogy of Fallot. DESIGN: Prospective observational. SETTING: Cardiology department of a teaching hospital. METHODS: Between June 1984 and June 1991, 104 children (63 boys, 41 girls) were studied by signal averaging and 24 hour ambulatory monitoring after surgical repair of tetralogy of Fallot. Mean (SD) age at operation was 6 (3) years, mean interval between operation and evaluation was 8 (25) months, and the follow up after evaluation was one to 88 (mean 30) months. RESULTS: Ventricular late potentials were detected in 24/104 patients (23%) and spontaneous ventricular arrhythmias in 39/96 patients (38%); usually (in 81%) these were unifocal and rare. Patients with ventricular late potentials were older at operation than patients without late potentials (9 (3) v 6 (3) years, P = 0.002). No correlation was found between the presence of ventricular late potentials and the presence or complexity of spontaneous ventricular arrhythmias early after operation. During the mean follow up of 2.5 years no case of sudden death or sustained ventricular tachycardia was found. CONCLUSION: Short-term prognosis after surgical repair of tetralogy of Fallot is good; ventricular late potentials and spontaneous ventricular arrhythmias are often detected shortly after operation, but in the medium term follow up they do not predict sudden death or serious ventricular arrhythmias.

Right atrial thrombosis associated with central venous catheters in children with cancer.

Right atrial thrombosis (RAT) is infrequently diagnosed in children with cancer. Once RAT is documented, medical fibrinolysis or surgical thrombectomy is recommended. A RAT was documented in a child with lymphoma and was successfully lysed with recombinant tissue-type plasminogen activator. The case is presented and therapeutic options reviewed.

Physiological pulmonary branch stenosis in newborns: 2D-echocardiographic and Doppler characteristics and follow up.

Transient systolic murmurs in neonates and premature infants due to mild left (LPA) and right (RPA) pulmonary branch stenosis is recognized but follow up studies are lacking. We studied echocardiographically 21 neonates with murmur and 10 controls. Diameters of the main pulmonary artery (MPA), LPA and RPA were smaller in patients with murmur. Colour-coded Doppler showed turbulent flow in LPA and RPA in 20/21 (95%) patients and flow velocities of both pulmonary branches were significantly higher than in controls. The follow up study at 3 months in 14/21 (67%) patients showed absent or decreased murmur in 9 (64%). Echographically, absolute and relative diameters of LPA and RPA increased whereas the ratio of MPA/aorta did not change suggesting accelerated growth or dilatation of the pulmonary branches. Flow velocities decreased significantly in the branches. Thus, transient systolic murmurs in neonates are associated with temporary relative hypoplasia of the pulmonary branches which showed increased growth leading to disappearance of the murmur in most cases within 3 months of life.

Regression of cardiac insufficiency after ambulatory intravenous deferoxamine in thalassemia major.

A case of severe cardiac failure due to iron overload in a patient with beta-thalassemia major is reported. The patient was successfully treated with high-dose ambulatory intravenous deferoxamine (desferrioxamine). This type of chelation appears to be a valuable alternative to subcutaneous deferoxamine administration in the presence of severe iron overload.

[Closure of patent ductus arteriosus using interventional catheterization]. / Fermeture du canal artériel persistant par cathétérisme interventionnel.

Percutaneous closure of patent ductus arteriosus can be performed with the Rashkind technique. This procedure has been performed in 16 patients in Geneva since 1989. The age of the patients varied from 2 to 44 years (4 boys, 7 girls, and 5 women). Pulmonary hypertension was present in only 1 child. A 12 mm Rashkind umbrella was used in 11 patients with ductus of < or = 4 mm inner diameter, while a 17 mm umbrella was used in 5 cases for larger ducts. Ductus closure was carried out through a transvenous femoral approach in 15 cases, while in 1 patient a transarterial approach was employed because of impossibility of passing the ductus from the pulmonary artery. There was neither mortality nor morbidity in this series. The aortography performed just after device placement showed total ductus occlusion in 8 patients and partial closure in 8 others. The follow-up by color Doppler showed disappearance of the residual shunt in 4 of these 8 patients. Prophylaxis of bacterial endocarditis is therefore no longer necessary in 13 patients with complete ductus closure. Our experience with this technique confirms its efficacy and the possibility it offers of avoiding thoracotomy in the vast majority of patients with a patent ductus arteriosus. Further miniaturization of the device and delivery system should make it applicable in the neonatal period.

[Prenatal diagnosis of arrhythmias and associated congenital cardiac abnormalities using ultrasonography]. / Le diagnostic prénatal des arythmies et des malformations cardiaques congénitales associées par ultrasonographie.

Cardiac arrhythmias are one of the most frequent indications for a 2D and Doppler fetal echocardiographic assessment (DE). If accompanied by a non-immune fetal hydrops, arrhythmias (A) involve an increased risk of intrauterine death. Some A and mainly the bradyarrhythmias (BA) can be the first sign of major cardiac malformation (CM). In a series of 404 pregnancies between week 17 and 40 of gestation (mean 27.6 weeks), a Doppler echocardiography was carried out; in 137 (35%) fetuses (F) this examination was based on the indication of arrhythmia. 33 (24.2%) of these fetuses showed a cardiac malformation. Bradyarrhythmias were found in 20 F; 5 had a complete AV-block including, 4 with an important cardiac abnormality (TU, complete AV-canal, corr. TGV). SVT's and auricular flutters (AF) made transplacental treatment necessary in 4 fetuses (1 AF, 3 SVT); they had no major cardiac malformation. PAC's were present in 59 pregnancies of which several had minor or major anomalies. Doppler echocardiography served to define the arrhythmia and the structural cardiac malformation, but also to follow transplacental treatment. These investigations have allowed us to schedule delivery under neonatal surveillance. After birth, 5 neonates had to be treated for persistent dysrhythmia (5 SVT, 2 complete AB-blocks), and 10 for a major cardiac malformation.