Multimodality imaging in bland-White-Garland syndrome in an adult with a left dominant coronary artery system.

Bland-White-Garland syndrome, also known as anomalous origin of the left coronary artery from the pulmonary artery, is a rare congenital disorder affecting around one in 300,000 live births. The majority of these present within the first year of life with 90% mortality rate if untreated and consequently is an extremely rare presentation in the adult. We present the first published case with a left dominant system in an adult presenting in their late 20s, illustrated by multimodality imaging.

A biventricular ICD system with biventricular defibrillation.

We describe the case of a 59-year-old gentleman with severe dilated cardiomyopathy requiring implantation of a dual-chamber biventricular implantable cardioverter-defibrillator (ICD). High defibrillation thresholds (DFT) were encountered at implant with an inadequate defibrillation safety margin. Testing of all possible shock vectors/polarities with and without the SVC coil and optimization of the distal RV coil position all proved inadequate. A satisfactory defibrillation safety margin was achieved following placement of a second lead in the coronary sinus to enable biventricular defibrillation. This case highlights an additional strategy for combating high DFTs and is an option even in dual-chamber biventricular ICD systems.

Improving the acceptability of the atrial defibrillator for the treatment of persistent atrial fibrillation: the atrial defibrillator sedation assessment study (ADSAS).

BACKGROUND: To compare the acceptability and effectiveness of three pre-medication regimens for manually activated cardioversion of recurrent persistent atrial fibrillation. METHODS: Eighteen patients implanted with the Jewel AF atrial defibrillator for drug-resistant persistent atrial fibrillation only were studied in an open-labelled randomised crossover study. Patients were assigned to sedation (S) with midazolam elixir, analgesia (A) with morphine sulphate or combination therapy (C) with dextromoramide and lorazepam. Pre-medication was taken up to 1 h before cardioversion. Patients rotated through each type of medication after undertaking at least one cardioversion. Visual analogue scales were completed immediately post-cardioversion and 24 h later for pain, anxiety and 'unpleasantness'. Higher scores represented a worse outcome. RESULTS: After 2 years' follow-up, 238 cardioversions were performed with S, 17 with A and 35 with C. The mean immediate combined score for S (10.9, 95% confidence interval (CI) 8.2-13.6) was significantly lower than for A (17.3, 95% CI 15.1-19.5, P = 0.01) and for C (15.9, 95% CI 12.3-19.6, P = 0.02). All patients who used S chose it as the most favourable pre-medicant. All patients who used A found it the least acceptable. CONCLUSION: Sedation rather than analgesia enhanced the acceptability of manually activated atrial defibrillation.

Temporal changes in atrial refractoriness following DC cardioversion of persistent atrial fibrillation in man.

AIMS: Studies have demonstrated shortening of the atrial effective refractory period (ERP) after episodes of atrial fibrillation (AF). This is termed atrial remodelling. It is unclear whether restoration of SR after persistent AF in patients with a clinical substrate results in reversal of this shortening and whether this is maintained long term. METHODS AND RESULTS: The ERP was determined at mid-lateral right atrial wall (MLRA) and right atrial appendage (RAA) at 600 ms and 400 ms drive cycle lengths and at basic sinus cycle length in 81 patients with persistent AF immediately, 24 h and 2 weeks following external DC cardioversion. All atrially active drugs were stopped for at least 5 half lives. (1) Prolongation of the ERP was observed at both atrial sites and all cycle lengths up to 24 h post cardioversion (p < 0.0001). (2) However, between 24 h and 2 weeks a subsequent shortening occurred in the ERP returning it to near post cardioversion levels. (3) The ERP was significantly longer at 24 h post cardioversion in patients who remained in SR for 2 weeks or longer compared with those who reverted to AF. CONCLUSION: Prolongation of the atrial ERP occurred following restoration of SR in persistent AF patients but was not maintained and displayed a biphasic pattern such that by 2 weeks the ERP had returned to baseline values. Despite this finding, a longer ERP at 24 h post cardioversion was associated with maintenance of SR in the medium-term.

Does sinus rhythm beget sinus rhythm? Long-term follow-up of the patient activated atrial defibrillator.

The aim of this study was to determine the effect of early patient activated cardioversion of atrial fibrillation (AF) using the atrial defibrillator on recurrence of AF. Fifteen patients, mean age 63 +/- 14 years, 80% men, with drug-resistant persistent AF were implanted with the Jewel AF atrial defibrillator. All patients performed self-administered cardioversion for AF recurrences. Over a 2 year follow-up, 238 patient-activated cardioversions were performed in 14 patients. Sinus rhythm was restored on every occasion with 96% of episodes terminating with a single shock. The median time from AF onset to patient awareness of symptoms was 2.5 hours. The median time from onset of symptoms to cardioversion was 3.5 hours. Comparison of the first and second six month period following implant showed a nonsignificant increase in mean total AF duration (75.7 +/- 107.8 hours vs 146.6 +/- 194.1 hours, P = 0.28). Two patients (13%) had a decreasing frequency of AF recurrences. The majority continued to have regular recurrences of AF. The atrial defibrillator is an extremely effective method of restoring sinus rhythm in patients with persistent AF. Regular early use of the atrial defibrillator, increased the duration of sinus rhythm in a minority of patients during long-term follow-up. Most patients had regular recurrences of AF requiring patient-activated cardioversion.

Long-term care of the patient with the atrial defibrillator.

With an aging population, atrial fibrillation is becoming an increasingly common cause of hospital admission. Patients with recurrent, symptomatic persistent atrial fibrillation often require repeated admissions to the hospital for cardioversion. The development of the atrial defibrillator has empowered such patients to take charge of their condition and perform cardioversion on themselves at home. This liberates them from the worry of hospitalization and can increase patient confidence. The implantation of an atrial defibrillator, however, has some disadvantages, and long-term use of the device exposes patients to some of the psychological adaptations that occur in recipients of implantable devices. This article discusses in depth the patient selection process, the implantation procedure, the use of the atrial defibrillator, and problems that can arise during long-term follow-up.

Circadian variation of arrhythmia onset patterns in patients with persistent atrial fibrillation.

BACKGROUND: The circadian onset patterns and cycle lengths of atrial tachyarrhythmias (AT) were determined in a group of patients with persistent atrial fibrillation. METHODS: Fifteen patients, mean age 63 +/- 14 years and 80% male, were implanted with the Jewel AF atrial defibrillator (Medtronic, Minneapolis, Minn) for persistent atrial fibrillation only. Onset times of AT and median onset atrial cycle lengths were determined from device memory. RESULTS: Over a follow-up period of 23.3 +/- 7 months, 227 episodes of persistent AT were treated by patient-activated atrial defibrillation. The peak onset of persistent AT was nocturnal, with 74% of episodes initiating between 8 pm and 8 am. Eighty-seven percent of the patients experienced an additional 403 paroxysmal AT episodes. These episodes showed a "double-peaked" pattern with the least number of episodes occurring between midnight and 8 am. The mean onset atrial cycle length of persistent AT was significantly shorter than the paroxysmal AT episodes (200 +/- 37 ms vs 240 +/- 39 ms, P <.005). The atrial cycle lengths at arrhythmia onset of both paroxysmal and persistent AT episodes also demonstrated circadian variation. CONCLUSION: There is a circadian distribution of onsets for persistent AT with predominance at night. Patients with persistent AF have >1 type of atrial arrhythmia with differences in the onset patterns and atrial cycle lengths, suggesting different triggers and onset mechanisms.

Psychosocial aspects of patient-activated atrial defibrillation.

UNLABELLED: Atrial Defibrillator. INTRODUCTION: The atrial defibrillator empowers patients to cardiovert themselves from atrial arrhythmias at a time that is socially and physically acceptable, thereby preventing hospitalization. The long-term psychosocial effects of repeated use of the patient-activated atrial defibrillator at home are unknown. METHODS AND RESULTS: Eighteen patients underwent placement of the Jewel AF atrial defibrillator for persistent atrial fibrillation only. All patients performed manually activated cardioversions at home under self-administered sedation. Automatic shock therapies were disabled. Hospital Anxiety and Depression Scale and Multidimensional Health Locus of Control questionnaires were obtained before implant. All patients completed questionnaires 1 year after device implant and at long-term follow-up. The spouse or partner of each patient was interviewed to identify positive and negative aspects of manual cardioversion at home. The baseline patient scores for both anxiety (5.7 +/- 2.7) and depression (3.4 +/- 2.3) fell within the predefined range of normality. At 1 year, there was no significant change in anxiety (4.9 +/- 3.7, P = 0.39) or depression (2.4 +/- 1.8, P = 0.06). At long-term follow-up (mean 28 months), a total of 377 patient-activated cardioversions were performed out of hospital (median 15 per patient). Scores for anxiety (6.0 +/- 4.0, P = 0.70) and depression (3.2 +/- 2.5, P = 0.68) remained unchanged. CONCLUSION: During long-term follow-up, patient-activated cardioversion using the atrial defibrillator was not associated with increased anxiety or depression. The procedure was well tolerated by patients and their partners, offering an acceptable treatment option for patients with recurrent persistent atrial fibrillation.

Echocardiographic changes and predictors of arrhythmia recurrence after long-term use of the atrial defibrillator.

BACKGROUND: The patient-activated atrial defibrillator allows patients to cardiovert themselves from atrial fibrillation soon after the onset of symptoms. The long-term effects of early cardioversion from persistent atrial fibrillation on left ventricular performance and left atrial size are unknown. METHODS: Eighteen patients, mean age 63.4, 83% male, had the Jewel((R)) AF atrial defibrillator implanted for persistent atrial fibrillation only. Transthoracic echocardiography was performed 3-monthly following implant. Parasternal long axis measurements were taken using conventional M-mode techniques. RESULTS: Over follow-up of 28.0+/-9 months, 377 episodes of persistent atrial fibrillation were terminated by patient-activated cardioversion (median 15 per patient). Echocardiographic measurements at implant were; left atrium 44+/-6 mm, left ventricular end-diastolic diameter 49+/-7 mm, left ventricular end-systolic diameter 34+/-7 mm, fractional shortening 33+/-10% and ejection fraction 65+/-17%. After 1 year there had been a significant decrease in mean left atrial size to 41+/-6 mm (P=0.02) and an increase in mean ejection fraction to 73+/-8% (P=0.04). At long-term follow-up however, all parameters reverted to pre-implant levels. Baseline echocardiographic variables did not predict which patients would demonstrate serial increases in sinus rhythm duration between shocks during long-term follow-up. Patients on antiarrhythmic drug therapy however were more likely to demonstrate "sinus rhythm begetting sinus rhythm". CONCLUSIONS: Use of the atrial defibrillator for spontaneous persistent atrial fibrillation is associated with a medium-term (1 year) reduction in left atrial size and an increase in ejection fraction. These changes were not maintained in the long-term. Synergistic therapy with antiarrhythmic drugs may prolong periods of sinus rhythm between arrhythmia recurrences.