Successful outcome in a pregnant woman with isolated noncompaction of the left ventricular myocardium.

A 24-year-old pregnant woman was referred to our hospital for the evaluation of her cardiac function. An electrocardiogram showed Wolff-Parkinson-White syndrome. Echocardiography revealed prominent trabeculation and deep intertrabecular recesses at the left ventricular apex and mid-portion of the inferior and lateral wall, with an impaired ejection fraction. She was diagnosed as having an isolated noncompaction of the ventricular myocardium (INVM). As the pregnancy progressed, severe restrictive hemodynamics became apparent. In consideration of the fetal growth, we decided to deliver the fetus by cesarean section at 32 weeks gestation; the patient successfully delivered a female infant. Interestingly, echocardiography demonstrated INVM in both the child and mother. This report is the first description of a successful pregnancy in a patient with familial INVM.

Evidence-based case report for analgesic and anesthetic management of a parturient with Ebstein's Anomaly and Wolff-Parkinson-White syndrome.

The rare congenital heart defect of Ebstein's anomaly is characterized by downward displacement and elongation of the septal cusp of tricuspid valve. As a result, it is often associated with a thin-walled and poorly contractile right ventricle, an enlarged atrium, tricuspid regurgitation, atrial septal defect with intracardiac shunt, pulmonary hypertension and tachyarrhythmia. Published reports or studies on analgesic and/or anesthetic management of laboring parturients with Ebstein's anomaly are limited. We present an evidence-based case report of the successful management of an obese laboring parturient with Ebstein's anomaly and Wolff-Parkinson-White syndrome under epidural analgesia. We reviewed the literature and present the best evidence available or its lack of, and the reasoning in supporting the choice of the analgesic and anesthetic management.

[Congenital atrioventricular block and Wolff-Parkinson-White Syndrome]. / Bloqueo auriculoventricular congénito y síndrome de Wolff-Parkinson-White.

We report the case of a patient followed since childhood for congenital complete atrioventricular block. At 28 years of age, atrioventricular conduction through an accessory pathway with long conduction times was detected. Periods of atrioventricular conduction alternated with periods of atrioventricular block. Sinus tachycardia and 1:1 exclusive conduction through the accessory pathway developed with increased sympathic activity (exercise, isoproterenol infusion). We discuss the special features of this case.

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.

[Catheter ablation of accessory pathways in patients with congenital cardiopathies]. / Ablación con catéter de vías accesorias en pacientes con cardiopatías congénitas.

The catheter ablation procedure in patients with accessory pathways and congenital heart defects can potentially become complicated because of abnormal anatomy and atypical conduction system. Eight patients ranging in age from 4.5 months to 18 years with accessory pathways and congenital heart defects underwent radiofrequency ablation. The cardiac diseases were diagnosed as Ebstein anomaly (n = 3), cardiac rhabdomyomas (n = 1), double outlet right ventricle (n = 1), endocardial cushion defect, partial form (n = 1), repaired Fallot's tetralogy (n = 1) and interventricular septal defect (n = 1). Six patients had manifest Wolff-Parkinson-White syndrome and 2 had concealed pathways. One patient with Ebstein anomaly had multiple accessory pathways. Radiofrequency ablation was initially successful in eight of the nine accessory pathways (89%). Two procedures were performed in one patient for pathway recurrence. Procedure was unsuccessful in 1 patient with Ebstein anomaly who later had surgical interruption of the accessory pathway. After a mean follow-up of 30.9 +/- 16.4 months, six of the 7 patients in which pathways were successfully ablated are tachycardia-free. One patient had late recurrence of tachycardia and is well controlled by propafenone.

Coexisting preexcitation syndrome and intermittent heart block presenting as neonatal seizures.

An infant presented with neonatal syncope and seizures. An ECG showed a preexcitation pattern, most compatible with Wolff-Parkinson-White (WPW) syndrome. Rhythm monitoring during an event demonstrated prolonged periods of complete AV block with no ventricular escape mechanism. We postulated that ventricular asystole was initiated by mechanical or autonomic influences on the accessory pathway and sustained by electrophysiologic interactions between the accessory pathway and the junctional escape focus. This is the first case report of a newborn having coexisting congenital AV block and WPW syndrome.

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.

[An intrapartal silent oscillation type in fetal Wolff-Parkinson-White syndrome]. / Ein intrapartaler silenter Oszillationstyp bei fetalem Wolff-Parkinson-White-Syndrom.

A discrepancy between auscultatory findings and the cardiotachogram was caused by technical limits of the cardiotocograph. The neonatal ECG showed a Wolff-Parkinson-White pattern with severe tachycardia. Some aspects of perinatal care in serious fetal and neonatal tachycardia are discussed.

Intrauterine and postnatal atrial fibrillation in the Wolff-Parkinson-White syndrome.

A fetal tachyarrhythmia was discovered at the thirty-second week of gestation of a 22-year old woman. Fetal echocardiography revealed atrial fibrillation with rapid ventricular rate, without any other demonstrable cardiac abnormality. In spite of therapeutic maternal blood levels of digoxin, the fetal ventricular rate and cardiac size increased, which prompted us to perform cesarean section at the thirty-fourth week of gestation. A baby with a Wolff-Parkinson-White syndrome but no other cardiac anomaly was delivered. Recurrent episodes of nonsustained atrial fibrillation with conduction over the accessory pathway occurred in the first hours of life. The Wolff-Parkinson-White pattern was not present on subsequent ECG recordings. The use of echocardiography in the diagnosis and management of this rare fetal tachyarrhythmia is emphasized.

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.