Follow-up of children or teenagers with paroxysmal supraventricular tachycardia, but without pre-excitation syndrome.

BACKGROUND: Paroxysmal supraventricular tachycardia (SVT) is considered benign in children if the electrocardiogram in sinus rhythm is normal, but causes anxiety in parents, children and doctors. AIMS: To report on the clinical and electrophysiological data from children with SVT, their follow-up and management. METHODS: Overall, 188 children/teenagers (mean age 15±2.8 years) with a normal electrocardiogram in sinus rhythm were studied for SVT, and followed for 2.3±4 years. RESULTS: SVT was poorly tolerated in 30/188 children (16.0%). SVT was related to atrioventricular nodal reentrant tachycardia (AVNRT) (n=133) or atrioventricular reentrant tachycardia (AVRT) over a concealed accessory pathway (n=55; 29.3%). Ablation of the slow pathway (n=66) or the accessory pathway (n=43) was performed without general anaesthesia, 2±3 years after initial evaluation. Failure or refusal to continue occurred in 18/109 (16.5%) children: 7/66 with AVNRT (10.6%), 11/43 with AVRT (25.6%) (P<0.001). Symptoms of SVT recurred in 20/91 children (22.0%) with apparently successful ablation: 6/91 (6.6%) had real SVT recurrence; 14/91 (15.4%) had only a sinus tachycardia, more frequent in AVNRT (11/59; 18.6%) than AVRT (3/32; 9.4%) (P<0.05). In 13 children treated with an antiarrhythmic drug (AAD), SVT recurred in four; two presented AAD-related syncope. In 66 untreated children, one death was noted after excessive AAD infusion to stop SVT; the others remained asymptomatic or had well-tolerated SVT. CONCLUSIONS: At the time of ablation, SVT management remains difficult in children. Indications for ablation are more common in AVRT than in AVNRT, but failures are frequent; 22.0% remained symptomatic after successful ablation, but false recurrences were frequent (15.4%). Without ablation, one third had a spontaneous favourable evolution.

Evolution of Clinical and Electrophysiological Data in Children with a Preexcitation Syndrome.

BACKGROUND: With ablation, the follow-up of preexcitation syndrome now is difficult to assess. The purpose was to collect data of children with a preexcitation syndrome studied on two separate occasions within a minimal interval of 1 year. METHODS: This is a retrospective chart review of 47 children initially aged 12 ± 4 years, who underwent two or more invasive electrophysiological studies (EPS) within 1-25 years of one another (6.3 ± 4.8) for occurrence of symptoms or new evaluation. RESULTS: Among initially symptomatic children (n = 25), four (19%) became asymptomatic and one presented life-threatening arrhythmia. Among asymptomatic children (n = 22), five became symptomatic (22.7%). Anterograde conduction disappeared in seven of 23 children with initially long accessory pathway-effective refractory period, but four of six had still induced atrioventricular reentrant tachycardia (AVRT). AVRT was induced at second EPS in three of 13 asymptomatic preexcitation syndrome with negative initial EPS. There were no spontaneous adverse events in the five children with criteria of malignancy at initial EPS; signs of malignancy disappeared in two. At multivariate analysis, AVRT at initial EPS was the only independent factor of symptomatic AVRT during follow-up. Absence of induced AVRT at initial EPS was the only factor of absence of symptoms and a negative study at the second EPS. CONCLUSIONS: There were no significant changes of data in children after 6.3 ± 4.8 years of follow-up. Most children with spontaneous/inducible AVRTs at initial EPS had still inducible AVRT at second EPS. Induced AF conducted with high rate has a relatively low prognostic value for the prediction of adverse events.

Factors of negativity of electrophysiological study in children and teenagers complaining of tachycardia and prognostic significance.

When non-invasive studies remain negative, the diagnosis of unexplained tachycardia in the young is a dilemma. The purpose of the study was to determine the factors of negativity of transesophageal electrophysiological study (EPS) in children/teenagers complaining of tachycardia and the prognostic value. Two hundred and seventy-three children with a normal ECG in sinus rhythm, aged from 6 to 19 years (15 ± 3), complained of tachycardia. Transesophageal EPS consisted of atrial stimulation in control state and after isoproterenol. Supraventricular tachycardia (SVT) was induced in 149 patients (group I) and EPS remained negative in 124 (group II). Age did not differ (15 ± 3 vs 15 ± 3). Female gender and familial history of SVT were as frequent in group I (47, 11%) than in group II (55%, p = 0.15; 7%;p = 0.2). Feeling of dizziness/syncope with tachycardia was less frequent in group I (12%) than in group II (48%) (p < 0.0001). Feeling of chest pain with tachycardia was less frequent in group I (2%) than in group II (28%) (p < 0.0001). The presence of non-cardiac disease was less frequent in group I (1.3%) than in group II (6.4%) (p < 0.025). Patients with negative study remained free of SVT after a follow-up of 3.5 ± 3 years, but one had a complete AV block. In children with apparently normal ECG in sinus rhythm, who complained of tachycardia clinical history (association with syncope, chest pain, or the presence of another disease) can predict negativity of EPS with a relatively high accuracy; EPS may not be necessary. In very symptomatic patients, transesophageal EPS, which is inexpensive and non-invasive, might be performed to stop investigations.

Arterial switch operation with a single coronary artery: the autograft concept.

A single coronary artery, especially if associated with anterior looping, remains a risk factor when performing an arterial switch operation for transposition of the great arteries. In such a situation, to avoid the risk of overstretching, we used a modification of the aortic autograft concept to transfer the single coronary artery, resulting in a tension-free relocation.

Rapid and low-cost method to prove the nature of no documented tachycardia in children and teenagers without pre-excitation syndrome.

Aims Symptoms in children are often difficult to interpret. The purpose of this study was to report the results of transoesophageal electrophysiological study (EPS) performed in children complaining of sudden onset tachycardia with normal non-invasive studies. Methods and results Eighty-two children and teenagers (mean age 15 +/- 3 years) presented with suspected but no documented paroxysmal supraventricular tachycardia (SVT). ECG was normal. Non-invasive studies were negative; 23 children had syncope with tachycardias. They underwent transoesophageal EPS in our out-patient clinic. The mean duration of transoesophageal EPS was 11 +/- 5 min. Electrophysiological study was negative in 25 children. AV nodal re-entrant tachycardia could be induced in 37 children, 11 of them associated with syncope. Wolff-Parkinson-White syndrome (WPW) was diagnosed in five children in which atrioventricular re-entrant tachycardia was inducible. Atrioventricular re-entrant tachycardia due to a concealed AP was induced in 14 children. Verapamil-sensitive ventricular tachycardia was induced in one patient. Factors associated with tachycardia inducibility were an older age (15.5 +/- 2 vs. 14 +/- 4 years) (P < 0.05) and the absence of syncope (81 vs. 52%) (P < 0.05). During a mean follow-up of 3 +/- 1 year, no patient with negative EPS developed documented tachycardia. In 17 children with inducible SVT, radiofrequency ablation of the re-entrant circuit was subsequently performed. Conclusion Transoesophageal EPS is a fast method for proving the nature of paroxysmal tachycardia in children and teenagers presenting with normal ECG and for demonstrating WPW syndrome not visible on standard ECG. The negative predictive value of transoesophageal EPS for the diagnosis of SVT was 100%.

Evidence of early impairments in both right and left ventricular inotropic reserves in children with Duchenne's muscular dystrophy.

In Duchenne's muscular dystrophy (DMD), cardiac function deteriorates with time and heart failure is one of the major causes of death. The aim of the study was to determine if a decrease in the ventricular inotropic reserves could be an early sign of cardiac dysfunction in these children. Nineteen children with DMD (aged 9 to 18 years, mean age 13.6 +/- 2.4) underwent equilibrium radionuclide angiography at rest and during an inotropic stimulation with low-dose dobutamine perfusion (7.5 to 15 microg. kg(-1). min(-1)). In all patients, this investigation was short (<30 minutes), successful, and uncomplicated. At rest, left ventricular (LV) ejection fraction (EF) was normal (>0.50) in 79% of patients, and right ventricular (RV) EF was normal (>0.45) in 95%. There was a trend toward a decrease with age for rest LVEF (p = 0.051) but not for rest RVEF (p = 0.8). By contrast, marked declines with age could be documented for the increases (Delta) in LVEF and RVEF during dobutamine perfusion (p = 0.002 for DeltaLVEF and p = 0.015 for DeltaRVEF). Thus, by multivariate analysis, the sole best indicator of decline in cardiac function with age was LVEF determined with dobutamine. In children with DMD, low-dose dobutamine radionuclide angiography gives evidence of an early decline with age of the inotropic reserves of both ventricles.