A fetal Wolff-Parkinson-White syndrome diagnosed prenatally by magnetocardiography.

We report a case of fetal Wolff-Parkinson-White (WPW) syndrome diagnosed prenatally by magnetocardiography (MCG). At 32 weeks' gestation, the fetus was diagnosed to have a paroxysmal supraventricular tachycardia by ultrasonography and direct fetal electrocardiogram (ECG). Transplacental fetal therapy by maternal oral administration of propranolol resolved the fetal tachyarrhythmia. Although the wave forms of the fetal MCG at 32 weeks' gestation were normal, the fetal MCG at 35 weeks' gestation showed a short PR interval and a long QRS complex duration with a delta wave, indicating WPW syndrome. The findings of the fetal MCG were confirmed by the postnatal ECG. MCG made the prenatal diagnosis of WPW syndrome possible.

Surgical treatment of the Wolff-Parkinson-White syndrome in children.

From 1986 to 1989, seven children ranging in age from 5 months to 16 years underwent surgical treatment for the Wolff-Parkinson-White syndrome at the Shiga University of Medical Science. None of the patients had any other associated congenital heart disease. There was a right free wall accessory pathway in four patients and a left free wall accessory pathway in three. Surgical ablation of these accessory pathways was performed on eight occasions, using the endocardial approach three times and the epicardial approach five. All the children are alive and none has since had episodes of tachycardia. Only one patient had a recurrent delta wave, which was noted 18 months after the operation. Surgical ablation of the accessory pathway for the Wolff-Parkinson-White syndrome can be performed safely, even in infants and children; it is concluded that this useful procedure is capable of improving a patient's quality of life.

[Intrauterine therapy of fetal supraventricular tachycardia with digoxin and verapamil]. / Intrauterine Therapie fetaler supraventrikulärer Tachykardien mit Digoxin und Verapamil.

Fetal supraventricular tachycardia may cause intrauterine heart failure and thus require transplacental treatment. During a period of nine years, we treated nine of eleven fetuses (gestational age ranging from the 26th to the 36th week) suffering from paroxysmal supraventricular tachycardia (10) or atrial flutter (1). The remaining two fetuses did not receive antiarrhythmic therapy because of only short lasting supraventricular tachycardia. Two fetuses were hydropic at the onset of therapy. Diagnosis of the rhythm disorder was established by m-mode echocardiography. All nine fetuses treated received digoxin after diagnosis of supraventricular tachycardia. Three of these reverted to sinus rhythm, one remained in supraventricular tachycardia which, however, was well tolerated. Five fetuses (three because of failure of digoxin alone and two because of a severely symptomatic supraventricular tachycardia) were treated with a combination of digoxin and verapamil. All five fetuses responded to the combined treatment, two of them, however, were delivered prematurely because of recurrence of supraventricular tachycardia in one and amnion-infection syndrome in the other. All patients survived and no severe fetal or maternal side effects were observed. Our experience confirms that digoxin and verapamil are usually effective in treating fetal supraventricular tachycardia. Some fetuses with short lasting and self limiting supraventricular tachycardia may not need any treatment, and a few not responding to digoxin and verapamil may require other antiarrhythmic drugs.

[Supraventricular paroxysmal tachycardia without congenital heart disease: clinical, therapeutic aspects and course in 65 children]. / Taquicardia supraventricular paroxística sin cardiopatía congénita: aspectos clínicos, terapéuticos y evolutivos de 65 niños.

We have reviewed the records of 65 children with paroxysmal supraventricular tachycardia (PST) without congenital heart disease followed a mean of 4 years, with a total of 121 episodes. PST appeared before 6 months of age in 42 (64.6%) children. Thirteen patients (20%) had a present factor which might predispose to PST in 66.2% of the patients who were younger than 6 months of age, and in only 4.3% of those over 6 months. Wolff-Parkinson-White syndrome was present on surface ECG during sinus rhythm in 26.1% of children younger than 6 months, and in 39.1% of those over 6 months. Digoxin was the initial treatment in 84.3% of the episodes with a success rate of 75% when were employed alone and of 84.2% when were employed in combination of quinidine. PST recurred at least once in 35 children (53.8%), the 90% within three months of the first episode. All patients were alive and 63 (96.9%) doing well. One patient developed cerebral anoxia and now has hemiparesia and another patient has incessant PST. We conclude that children with PST without congenital heart disease and without delay in diagnosis had a good outcome.

Perinatal paroxysmal supraventricular tachycardia.

Three cases of perinatal paroxysmal supraventricular tachycardia are described. In two patients the tachycardia was present prior to delivery; in the third baby, who also had the Wolf-Parkinson-White Syndrome, the time of onset of tachycardia is not known. The risks of this condition to the foetus are largely unknown but severe intra-uterine cardiac failure can occur. Possible lines of management are discussed.

[Paroxysmal Hisian tachycardia and familial sarcoidosis]. / Tachycardie hisienne en salve et sarcoïdose familiale.

We report a His-bundle tachycardia in a 6 year-old patient without evidence of heart disease. Diagnosis and follow-up were assessed by exercise stress testing and repeated long-term electrocardiographic recording. As sarcoidosis was present in the mother and the aunt, this condition was discussed in this child also. However, since there is no absolute proof for this disease, the hypothesis of an antenatal injury of the bundle of His was discussed: this could be secondary to the transplacental transfer of abnormal maternal antibodies, as frequently observed in women with clinical or biologic evidence of connective tissue disease.

Wolff-Parkinson-White syndrome in the neonate.

Of 16 infants who presented with paroxysmal supraventricular tachycardia in the neonatal period, 50 percent had an electrocardiographic pattern consistent with Wolff-Parkinson-White conduction, type A. It is suggested that infants have bypass pathways similar to or identical with a Kent pathway as part of normal maturation. Infants with paroxysmal supraventricular tachycardia have electrically active bypass tracts but these are documented in only about one half of the patients because of the short duration of recordings or because of concealment (the bypass tract conducts only in retrograde fashion). The activity of these pathways is enhanced by the predominant cholinergic innervation of the neonatal heart. Resolution of the arrhythmias and the Wolff-Parkinson-White pattern in most patients occurs because of anatomic maturation of the conduction tissue, development of adrenergic innervation and a decrease in cholinergic dominance. In some children, maturation is incomplete and the bypass fibers remain quiescent or become active under certain circumstances such as those associated with increased autonomic discharge. Extended surveillance is recommended for all infants who present with paroxysmal supraventricular tachycardia and the Wolff-Parkinson-White pattern.

Congenital paroxysmal atrial tachycardia.

Ten infants who had paroxysmal atrial tachycardia in utero or at birth are reported. Because of apparent fetal distress, caesarean section was performed in 4 cases and labour was induced in 1. Birthweight was generally large for gestational age. Severe ascites and hydrops at birth were manifestations of cardiac failure. Atrial flutter was recorded in 4 infants and supraventricular tachycardia in 5. The WoLff-Parkinson-White syndrome became evident later in 2. Digoxin was given to all 10 infants, and cardioversion was required and was effective in 4. Known recurrences in childhood have occurred in only 1 patient. Congenital atrial tachyarrhythmias may be commoner than generally believed, and fetal electrocardiography may help to avoid unnecessary termination of pregnancy. Blood sugar determinations are important, since neonatal hypoglycaemia was found. Cardioversion should be performed promptly in severely ill infants or if there is no response to digoxin. Care is required to avoid digoxin toxicity.