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Fetal onset of congenital long QT syndrome (LQTS) is a rare manifestation, and prenatal diagnosis is difficult. This report describes a boy who presented with both atrioventricular (AV) block and ventricular tachycardia during the antenatal period. The early postnatal electrocardiogram showed prolongation of the QT interval and AV block, subsequently leading to a polymorphic ventricular tachycardia torsade de pointes. This unique feature of congenital LQTS has a poor outcome, but the boy was successfully treated with beta-blockers and implantation of an automated cardioverter-defibrillator. The intrauterine manifestation of fetal AV block and ventricular tachycardia should raise a high suspicion of congenital LQTS, and the strong association with a malignant clinical course should warrant special evaluation. The literature on the prenatal diagnosis, fetal therapy, and neonatal outcome of this condition also are reviewed.

We describe polymorphic ventricular tachycardia (VT) diagnosed using fetal magnetocardiography (FMCG). The fetus of a 33-year-old Japanese female at 24 weeks of pregnancy was diagnosed as bradycardia (60 beats/min) by fetal cardiotocography (CTG). Ultrasound findings indicated a diagnosis of an atrioventricular (AV) block involving extrasystole, but FMCG revealed a polymorphic VT followed by ventricular asystole. Standard ECG immediately after cesarean section at 37 weeks of pregnancy confirmed long QT syndrome followed by nonsustained polymorphic VT and an advanced AV block with wide QRS. Echocardiography demonstrated moderate left ventricular dysfunction in the neonate requiring implantation with a permanent pacemaker.

Reentry is the main mechanism of life-threatening ventricular arrhythmias, including ventricular fibrillation and tachycardia. Its occurrence depends on the simultaneous presence of an arrhythmogenic substrate (a preexisting condition) and a "trigger," and is favored by electrophysiological heterogeneities. In the adult heart, electrophysiological heterogeneities of the ventricle exist along the apicobasal, left-right, and transmural axes. Also, conduction is preferentially slowed in the right ventricular outflow tract, especially during pharmacological sodium channel blockade. We propose that the origin of electrophysiological heterogeneities of the adult heart lies in early heart development. The heart is formed from several progenitor regions: the first heart field predominantly forms the left ventricle, whereas the second heart field forms the right ventricle and outflow tract. Furthermore, the embryonic outflow tract consists of slowly conducting tissue until it is incorporated into the ventricles and develops rapidly conducting properties. The subepicardial myocytes and subendocardial myocytes run distinctive gene programs from their formation onwards. This review discusses the hypothesis that electrophysiological heterogeneities in the adult heart result from persisting patterns in gene expression and function along the craniocaudal and epicardial-endocardial axes of the developing heart. Understanding the developmental origins of electrophysiological heterogeneity contributing to ventricular arrhythmias may give rise to new therapies.

Fetal tachyarrhythmias are a life-threatening condition complicating a small proportion of normal pregnancies. Despite major advances in the (intrauterine) pharmacologic treatment of these arrhythmias over the last years major uncertainties remain. Among these are controversies in the choice of agents in relation to arrhythmia type, and timing and duration of treatment. Currently, no evidence-based approach to the management of fetal tachyarrhythmias is available. An international registry is proposed as an important step toward obtaining the necessary data to develop evidence-based management strategies.

Background: Cardiogram signal amplitude is a key index of hypertrophy but has not been investigated extensively in utero. In this study, magnetocardiography was used to assess P and QRS amplitude in normal subjects and subjects with fetal arrhythmia., Methods and Results: The study cohort consisted of 68 normal fetuses and 25 with various arrhythmias: 9 reentrant supraventricular tachycardia (SVT), 2 ventricular tachycardia (VT), 2 sinus tachycardia, 2 blocked atrial bigeminy, 2 congenital second-degree atrioventricular (AV) block, and 8 congenital complete AV block. Subjects with congenital AV block, all presenting with bradycardia, showed large QRS amplitude, exceedingly large P-wave amplitude, and long P-wave duration. The 2 subjects with VT, both with poor ventricular function, also exhibited large P waves. SVT was associated with only moderate signal amplitude elevation., Conclusions: The data imply that AV block in utero is accompanied by hypertrophy, which is more pronounced for the atria than the ventricles. We hypothesize that the hypertrophy results from a compensatory response associated with regulation of cardiac output and is likely to be observable in other arrhythmias and disease states. Magnetocardiography may be more sensitive than fetal echocardiography for detection of atrial hypertrophy in utero.

Ventricular tachycardia is rare and poorly understood in the neonate. The authors undertook a retrospective study in 2 foetus and 8 neonates aged 1 to 20 days at the time of diagnosis. The tachycardia was permanent in 2 cases, observed in runs of variable variation in the other 8, incessant in 7 of these cases. Only two cases were symptomatic: cardiac failure with shock 16 hours after birth and hydramnios at 16 weeks gestation. The electrocardiographic criteria of ventricular tachycardia (wide QRS complexes of different morphology to the sinus QRS complexes, atrioventricular dissociation) were fulfilled in all patients. The arrhythmia was monomorphic 9 times out of 10 with a fixed (3 cases) or variable (7 cases) rate which was always > 150/min. Intravenous magnesium sulphate in the severe and permanent forms, oral betablockers in forms triggered by acceleration of the sinus rhythm, oral amiodarone alone or associated in one case with propranolol were prescribed but three neonates were not treated, either from the outset or after inefficacy of amiodarone: nine of the patients were cured and are treatment-free 12 to 24 months later: the other patient has a slow, well tolerated ventricular tachycardia. No aetiology was detected in 9 cases; the other had a metabolic disease of B-oxidation of long chain fatty acids. The authors conclude that isolated, idiopathic ventricular tachycardia of the neonate usually carry a good prognosis which is not dependent on the tachycardia of the permanence of the arrhythmia. Simple treatment (betablocker or amiodarone) is usually associate with restoration of sinus rhythm and definitive cure during the first year of life.