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.

close: Closure of patent foramen ovale, oral anticoagulants or antiplatelet therapy to prevent stroke recurrence: Study design.

RATIONALE: Currently available data do not provide definitive evidence on the comparative benefits of closure of patent foramen ovale, oral anticoagulants and antiplatelet therapy in patients with patent foramen ovale-associated cryptogenic stroke AIM: To assess whether transcatheter patent foramen ovale closure plus antiplatelet therapy is superior to antiplatelet therapy alone and whether oral anticoagulant therapy is superior to antiplatelet therapy, for secondary stroke prevention in patients aged 16 to 60 years with a large patent foramen ovale or a patent foramen ovale associated with an atrial septal aneurysm, and an otherwise unexplained ischaemic stroke or retinal ischaemia. SAMPLE SIZE: Six hundred and sixty-four patients were included in the study. METHODS AND DESIGN: CLOSE is an academic-driven, multicentre, randomized, open-label, three-group, superiority trial with blinded adjudication of outcome events. The trial has been registered with Clinical Trials Register (Clinicaltrials.gov, NCT00562289). Patient recruitment started in December 2007. Patient follow-up will continue until December 2016. Expected mean follow-up = 5.6 years. STUDY OUTCOMES: The primary efficacy outcome is the occurrence of fatal or nonfatal stroke. Safety outcomes include fatal, life-threatening or major procedure- or device-related complications and fatal, life-threatening or major haemorrhagic complications. DISCUSSION: CLOSE is the first specifically designed trial to assess the superiority of patent foramen ovale closure over antiplatelet therapy alone and the superiority of oral anticoagulants over antiplatelet therapy to prevent stroke recurrence in patients with patent foramen ovale-associated cryptogenic stroke.

Electrophysiological study generally is negative in patients <40 years suspected of supraventricular tachycardia but also complaining of chest pain and/or syncope.

BACKGROUND: The diagnosis of paroxysmal supraventricular tachycardia (SVT) frequently is a dilemma. Electrophysiological study (EPS) is the only means to evaluate the nature of symptoms when noninvasive studies remain negative. Our objectives were to determine the clinical factors of negativity or positivity of (EPS) in patients suspected of SVT. METHODS: EPS was performed in 2650 patients complaining of tachycardia and suspected of SVT. Transesophageal EPS consisted of programmed atrial stimulation in control state and after isoproterenol. Patients were followed from 1 month to 18 years (2.93 ± 4 years). RESULTS: SVT was induced in 1944 patients, age 48 ± 19.5. EPS remained negative in 706 patients, age 34 ± 17 (p<0.0001). Age <40 years, feeling of dizziness/syncope or chest pain associated with tachycardia, the absence of heart disease or short PR interval was more frequent in patients with negative EPS (respectively 64, 42, 26, 96, 88.5%) than in patients with induced SVT (34, 14, 4, 88, 59%) (p<0.0001).The positive predictive value for the prediction of a negative EPS of age <40, chest pain, syncope or their association was 63.5, 42, 26.5, 11% and negative predictive value was 66, 86, 94.5, 99.5%. At multivariate analysis, age <40 (0.000, OR 2.79), the presence of syncope associated with tachycardia (0.000, OR 5.075) or chest pain (0.000, OR 17.923) was an independent factor of negative EPS. CONCLUSIONS: Among patients complaining of nondocumented tachycardia, suspected of SVT, the association of tachycardia with chest pain and/or syncope and age <40 years generally was correlated with a negative EPS and did not indicate initially invasive studies. In the remaining patients transesophageal EPS is indicated.

A systematic variant screening in familial cases of congenital heart defects demonstrates the usefulness of molecular genetics in this field.

The etiology of congenital heart defect (CHD) combines environmental and genetic factors. So far, there were studies reporting on the screening of a single gene on unselected CHD or on familial cases selected for specific CHD types. Our goal was to systematically screen a proband of familial cases of CHD on a set of genetic tests to evaluate the prevalence of disease-causing variant identification. A systematic screening of GATA4, NKX2-5, ZIC3 and Multiplex ligation-dependent probe amplification (MLPA) P311 Kit was setup on the proband of 154 families with at least two cases of non-syndromic CHD. Additionally, ELN screening was performed on families with supravalvular arterial stenosis. Twenty-two variants were found, but segregation analysis confirmed unambiguously the causality of 16 variants: GATA4 (1 ×), NKX2-5 (6 ×), ZIC3 (3 ×), MLPA (2 ×) and ELN (4 ×). Therefore, this approach was able to identify the causal variant in 10.4% of familial CHD cases. This study demonstrated the existence of a de novo variant even in familial CHD cases and the impact of CHD variants on adult cardiac condition even in the absence of CHD. This study showed that the systematic screening of genetic factors is useful in familial CHD cases with up to 10.4% elucidated cases. When successful, it drastically improved genetic counseling by discovering unaffected variant carriers who are at risk of transmitting their variant and are also exposed to develop cardiac complications during adulthood thus prompting long-term cardiac follow-up. This study provides an important baseline at dawning of the next-generation sequencing era.

Age-related location of manifest accessory pathway and clinical consequences.

BACKGROUND: Accessory pathway (AP) ablation is not always easy. Our purpose was to assess the age-related prevalence of AP location, electrophysiological and prognostic data according to this location. METHODS: Electrophysiologic study (EPS) was performed in 994 patients for a pre-excitation syndrome. AP location was determined on a 12 lead ECG during atrial pacing at maximal preexcitation and confirmed at intracardiac EPS in 494 patients. RESULTS: AP location was classified as anteroseptal (AS)(96), right lateral (RL)(54), posteroseptal (PS)(459), left lateral (LL)(363), nodoventricular (NV)(22). Patients with ASAP or RLAP were younger than patients with another AP location. Poorly-tolerated arrhythmias were more frequent in patients with LLAP than in other patients (0.009 for ASAP, 0.0037 for RLAP, <0.0001 for PSAP). Maximal rate conducted over AP was significantly slower in patients with ASAP and RLAP than in other patients. Malignant forms at EPS were more frequent in patients with LLAP than in patients with ASAP (0.002) or PSAP (0.001). Similar data were noted when AP location was confirmed at intracardiac EPS. Among untreated patients, poorly-tolerated arrhythmia occurred in patients with LLAP (3) or PSAP (6). Failures of ablation were more frequent for AS or RL AP than for LL or PS AP. CONCLUSIONS: AS and RLAP location in pre-excitation syndrome was more frequent in young patients. Maximal rate conducted over AP was lower than in other locations. Absence of poorly-tolerated arrhythmias during follow-up and higher risk of ablation failure should be taken into account for indications of AP ablation in children with few symptoms.

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.

Longitudinal myocardial peak velocities using high temporal resolution phase-contrast and simple averaging are comparable to tissue Doppler echocardiography.

OBJECT: Phase contrast imaging is widely used to measure blood velocity. However tissue Doppler imaging (TDI) echocardiography is the reference for myocardial velocity assessment. This study aims at validating the ability of phase contrast (PC) sequences to correctly assess myocardial velocities and to compare these velocities to TDI. The phase contrast sequence was performed with breath-hold parameters and with parameters tuned to increase temporal resolution in free breathing. MATERIALS AND METHODS: Left and Right auriculo-ventricular annuluses longitudinal velocities were recorded on six healthy volunteers with different temporal resolutions (TDI: 5 ms, breath-hold PC: 94 ms and free-breathing PC: 19 ms). Free-breathing PC was obtained by averaging of three excitations. Amplitudes of four standard echocardiographic and clinically relevant myocardial longitudinal velocity waves were compared: Early filling and auricular, systolic and isovolumic contractions. RESULTS: Isovolumic contraction waves were only visible with free-breathing PC and TDI. The differences with the reference TDI wave velocities were lower (p = 0.02) for free-breathing PC (19.2 ± 2.6%) than for breath-hold PC (28.1 ± 2.9%). These differences for free-breathing PC were close to (p = 0.21) the coefficient of variation of the measurements provided by TDI (14.8 ± 1.2%). CONCLUSION: Myocardial longitudinal peak velocities can be assessed with a PC sequence tuned to optimize temporal resolution.

Age-related prognosis of syncope associated with a preexcitation syndrome.

BACKGROUND: Syncope in Wolff-Parkinson-White syndrome (WPW) is without relationship with WPW or reveals a poorly tolerated arrhythmia. Electrophysiologic study (EPS) is recommended. The purpose of the study was to evaluate the influence of the patient's age on the causes and prognosis of syncope. METHODS: A total of 98 patients, mean age 35 ± 18 years, with WPW were admitted for syncope. Note that 29 were aged between 9 and 19 years (mean 15 ± 3) (children and teenagers/group I), 45 between 20 and 49 years (mean 34 ± 8) (adults/group II), and 24 between 50 and 70 years (mean 60 ± 8) (elderly/group III). EPS consisted of atrial pacing and programmed atrial stimulation in control state and after isoproterenol. RESULTS: Potentially malignant form (rapid conduction in accessory pathway >240 beats/min in control state or >300 beats/min after isoproterenol and atrial fibrillation [AF] induction) was more frequent in group I (34%) than in groups II (7%) (P < 0.002) and III (0%) (P < 0.001). Orthodromic atrioventricular reentrant tachycardia (AVRT) and AF were induced as frequently in groups I (59, 34%), II (47, 15.5%), and III (54, 17%). AVRT was induced in all but one patient with malignant form. EPS was as frequently negative in groups I (27.5%), II (44%), and III (37.5%). Natural follow-up (mean 8 ± 6 years) indicated a favorable prognosis, only related to AVRT induction. Induced AF was without significance. CONCLUSIONS: Data in syncope and WPW syndrome depended on age: electrophysiological malignant form was frequent in children/teenagers, rare in adults, and absent in elderly. AVRT, the main cause of syncope, was as frequent in all ranges of age. AF's induction alone had no significance. Final prognosis was favorable.

Parental electrocardiographic screening identifies a high degree of inheritance for congenital and childhood nonimmune isolated atrioventricular block.

BACKGROUND: The origin of congenital or childhood nonimmune isolated atrioventricular (AV) block remains unknown. We hypothesized that this conduction abnormality in the young may be a heritable disease. METHODS AND RESULTS: A multicenter retrospective study (13 French referral centers, from 1980-2009) included 141 children with AV block diagnosed in utero, at birth, or before 15 years of age without structural heart abnormalities and without maternal antibodies. Parents and matched control subjects were investigated for family history and for ECG screening. In parents, a family history of sudden death or progressive cardiac conduction defect was found in 1.4% and 11.1%, respectively. Screening ECGs from 130 parents (mean age 42.0 ± 6.8 years, 57 couples) were compared with those of 130 matched healthy control subjects. All parents were asymptomatic and in sinus rhythm, except for 1 with undetected complete AV block. Conduction abnormalities were more frequent in parents than in control subjects, found in 50.8% versus 4.6%, respectively (P<0.001). A long PR interval was found in 18.5% of the parents but never in control subjects (P<0.0001). Complete or incomplete right bundle-branch block was observed in 39.2% of the parents and 1.5% of the control subjects (P<0.0001). Complete or incomplete left bundle-branch block was found in 15.4% of the parents and 3.1% of the control subjects (P<0.0006). Estimated heritability for isolated conduction disturbances was 91% (95% confidence interval, 80%-100%). SCN5A mutation screening identified 2 mutations in 2 patients among 97 children. CONCLUSIONS: ECG screening in parents of children affected by idiopathic AV block revealed a high prevalence of conduction abnormalities. These results support the hypothesis of an inheritable trait in congenital and childhood nonimmune isolated AV block.

Characteristics and long-term outcome of non-immune isolated atrioventricular block diagnosed in utero or early childhood: a multicentre study.

AIMS: The natural history of congenital or childhood non-immune, isolated atrioventricular (AV) block is poorly defined. METHODS AND RESULTS: We retrospectively studied 141 children with isolated, non-immune AV block diagnosed in utero, or up to 15 years of age, at 13 French medical centres, between 1980 and 2009. Patients with structural heart disease or maternal antibodies were excluded. Atrioventricular block was asymptomatic in 119 (84.4%) and complete in 100 (70.9%) patients. There was progression to complete AV block in 29/41 (70.7%) patients with incomplete AV block over 2.8 ± 3.4 years (1-155 months), but all patients with incomplete AV block may not have been included in the study. Narrow QRS complex was present in 18 of 26 patients (69.2%) with congenital, and 106 of 115 (92.2%) with childhood AV block. Pacemakers were implanted in 112 children (79.4%), during the first year of life in 18 (16.1%) and before 10 years of age in 90 (80.4%). The mean interval between diagnosis of AV block and pacemaker implants was 2.6 ± 3.9 years (0-300 months). The pacing indication was prophylactic in 70 children (62.5%). During a mean follow-up of 11.6 ± 6.7 years (1-32 years), no patient died or developed dilated cardiomyopathy (DCM). The long-term follow-up was uncomplicated in 127 children (90.1%). CONCLUSION: In this large multicentre study, the long-term outcome of congenital or childhood non-immune, isolated AV block was favourable, regardless of the patient's age at the time of diagnosis. No patient died or developed DCM, and pacemaker-related complications were few.

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%.

Are the results of electrophysiological study different in patients with a pre-excitation syndrome, with and without syncope?

AIMS: Syncope in Wolff-Parkinson-White (WPW) syndrome may reveal an arrhythmic event or is not WPW syndrome related. The aim of the study is to evaluate the results of electrophysiological study in WPW syndrome according to the presence or not of syncope and the possible causes of syncope. METHODS AND RESULTS: Among 518 consecutive patients with diagnosis of WPW syndrome, 71 patients, mean age 34.5 +/- 17, presented syncope. Transoesophageal electrophysiological study in control state and after isoproterenol infusion was performed in the out-patient clinic. Atrioventricular re-entrant tachycardia (AVRT) was more frequently induced than in asymptomatic patients (n = 38, 53.5%, P < 0.01), less frequently than in those with tachycardia; atrial fibrillation (AF) and/or antidromic tachycardia (ATD) was induced in 28 patients (39%) more frequently (P < 0.05) than in asymptomatic patients or those with tachycardia. The incidence of high-risk form [rapid conduction over accessory pathway (AP) and AF or ATD induction] was higher in syncope group (n = 18, 25%, P < 0.001) than in asymptomatic subjects (8%) or those with tachycardias (7.5%). Maximal rate conducted over AP was similar in patients with and without syncope, and higher in patients with spontaneous AF, but without syncope. Results were not age-related. CONCLUSION: Tachycardia inducibility was higher in patients with syncope than in the asymptomatic group. The incidence of malignant WPW syndrome was higher in patients with syncope than in asymptomatic or symptomatic population, but the maximal rate conducted over AP was not higher and another mechanism could be also implicated in the mechanism of syncope.

Experimental evaluation of a common susceptibility to motion sickness and vasovagal syncope in children.

In children, vasovagal syncope (VVS) is the most common cause of syncope and motion sickness (MS) is also very frequent, with many symptoms of an autonomic nature. To study a possible relationship between VVS susceptibility and MS susceptibility in young patients, 21 children (10 boys, 11.3+/-2.6 years) with recurrent syncope or presyncope were explored with a questionnaire concerning their vasovagal symptoms, susceptibility to MS and familial history. A tilt-table test and a dynamic posturography with Equitest (Sensory Organisation Test (SOT), in six conditions) were performed. Children were divided into two groups: A with a positive tilt-table test and particular susceptibility to VVS (n=13/21, six boys) and B with negative tilt-table test. A control group of 30 healthy children (15 boys, 11.4+/-2.4 years) was studied for MS susceptibility and familial history. VVS susceptibility was related to MS susceptibility (MS susceptibility was 69.3% in Group A versus 12.5% in Group B (p=0.0237) and 16.7% in control group (p=0.0028)) and also to SOT scores which are related both to the role of vestibule in equilibrium and to MS susceptibility, with lower values in Group A than Group B (condition 5: 47.9+/-12.3% versus 66.0+/-13.8%, p=0.0189 and vestibular (ratio of conditions 5/1): 51.8+/-12.7% versus 71.3+/-13.5%, p=0.0147). Our study demonstrates, for the first time, a relationship between VVS susceptibility and MS susceptibility in a population of children with a particular susceptibility to VVS. This paradigm may prove useful in better understanding the mechanisms underlying the susceptibility to VVS and MS.

Relation between motion sickness susceptibility and vasovagal syncope susceptibility.

Motion sickness is common in the population, especially in children, but its physiopathology is only partially understood and the true nature of the particular susceptibility of certain subjects remains completely unknown. Some symptoms of motion sickness, like pallor and cold sweating, are of an autonomic nature and the role of the autonomic nervous system in vasovagal syncope is well known. Our aim was therefore to study the relationship between motion sickness susceptibility and vasovagal syncope susceptibility. Questionnaires about susceptibility to motion sickness and to vasovagal syncope or presyncope in adulthood and childhood, filled in by 899 students (20.4 +/- 2.1 years, 405 men), were analysed. Motion sickness susceptibility in childhood was 31.1% and in adulthood 7.9% (p < 0.001). Vasovagal syncope susceptibility in childhood was 36.4% and in adulthood 33.9% (NS). A relationship between motion sickness susceptibility in adulthood and vasovagal syncope susceptibility in childhood and adulthood (p = 0.004 and 0.005, respectively) was found. Despite the limitations of a retrospective study this relationship between motion sickness susceptibility and vasovagal syncope susceptibility may indicate that a common mechanism exists, explaining the particular susceptibility of some subjects to both disorders. This paradigm may prove useful in better understanding the true nature of motion sickness and vasovagal syncope.

Value of programmed ventricular stimulation after tetralogy of fallot repair: a multicenter study.

BACKGROUND: Studies have consistently shown that ventricular tachycardia (VT) and sudden cardiac death (SCD) complicate the long-term outcome after tetralogy of Fallot repair, yet the diagnostic and predictive value of electrophysiological testing in this population is uncertain. METHODS AND RESULTS: A multicenter cohort of 252 patients with repaired tetralogy of Fallot undergoing programmed ventricular stimulation was followed up for 18.5+/-9.6 and 6.5+/-4.5 years after corrective surgery and electrophysiological testing, respectively. Clinical VT and/or SCD occurred in 24.6%. Sustained monomorphic VT and polymorphic VT were induced in 30.2% and 4.4%. Including polymorphic VT in the definition of inducibility improved sensitivity (66.1+/-6.0% versus 77.4+/-5.3%, P=0.0082) with a marginal reduction in specificity (81.6+/-2.8% versus 79.5+/-2.9%, P=0.0455). Positive and negative predictive values were 55.2+/-5.3% and 91.5+/-2.2%. Independent risk factors for inducibility were age at study > or =18 years (OR, 3.3), palpitations (OR, 2.8), prior palliative surgery (OR, 3.1), modified Lown criteria > or =2 (OR, 5.6), and cardiothoracic ratio > or =0.6 (OR, 3.3). Event-free survival rates in noninducible and inducible patients at 1, 5, 10, and 15 years were 97.9%, 92.8%, 89.3%, and 89.3% versus 79.4%, 62.6%, 58.7%, and 50.3%, respectively (P<0.0001). Both inducible monomorphic VT [relative risk (RR), 5.0; P=0.0002] and polymorphic VT (RR, 12.9; P<0.0001) predicted future clinical VT and SCD. In a multivariate analysis, inducible sustained VT was an independent risk factor for subsequent events (RR, 4.7; 95% CI, 1.2 to 18.5; P=0.0268). CONCLUSIONS: Programmed ventricular stimulation is of diagnostic and prognostic value in risk stratifying patients with repaired tetralogy of Fallot. In this patient population, inducible sustained polymorphic VT should not be disregarded as nonspecific.

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.