Familial atrial fibrillation with fetal onset

A woman presented during two pregnancies (at 25 and 23 weeks' gestation, respectively) because the fetuses had rapid, irregular tachycardia and hydrops. After maternal drug treatment and achievement of slower fetal heart rates, the hydrops gradually resolved. Both babies were born full term with continuing atrial fibrillation. In the first, an ectopic atrial rhythm was temporarily achieved during high dose flecainide treatment but, in the younger sibling, all medications and repeated cardioversions failed even temporarily to convert the atrial fibrillation with an almost isoelectric baseline in ECG to sinus rhythm. Good rate control has been achieved with digoxin in both patients. No infective, immunological, or structural cause was found in either case, and thus an inherited aetiology is probable.

Keywords: atrial fibrillation; arrhythmias; fetal atrial fibrillation; familial arrhythmias     

Predictors of atrial rhythm after atrioventricular node ablation for the treatment of paroxysmal atrial arrhythmias

OBJECTIVE: To assess the natural history of the atrial rhythm of patients with paroxysmal atrial arrhythmias undergoing atrioventricular node ablation and permanent pacemaker implantation. DESIGN AND SETTING: A retrospective cohort study of consecutive patients identified from the pacemaker database and electrophysiology records of a tertiary referral hospital. PATIENTS: 62 consecutive patients with paroxysmal atrial arrhythmias undergoing atrioventricular node ablation and permanent pacemaker implantation between 1988 and July 1996. MAIN OUTCOME MEASURES: (1) Atrial rhythm on final follow up ECG, classified as either ordered (sinus rhythm or atrial pacing) or disordered (atrial fibrillation, atrial flutter or atrial tachycardia). (2) Chronic atrial fibrillation, defined as a disordered rhythm on two consecutive ECGs (or throughout a 24 hour Holter recording) with no ordered rhythm subsequently documented. RESULTS: Survival analysis showed that 75% of patients progressed to chronic atrial fibrillation by 2584 days (86 months). On multiple logistic regression analysis a history of electrical cardioversion, increasing patient age, and VVI pacing were associated with the development of chronic atrial fibrillation. A history of electrical cardioversion and increasing patient age were associated with a disordered atrial rhythm on the final follow up ECG. CONCLUSIONS: Patients with paroxysmal atrial arrhythmias are at high risk of developing chronic atrial fibrillation. A history of direct current cardioversion.     

Superiority of ibutilide (a new class III agent) over DL-sotalol in converting atrial flutter and atrial fibrillation. The Ibutilide/Sotalol Comparator Study Group

OBJECTIVE: To compare the efficacy and safety of a single dose of ibutilide, a new class III antiarrhythmic drug, with that of DL-sotalol in terminating chronic atrial fibrillation or flutter in haemodynamically stable patients. DESIGN: Double blind, randomised study. SETTING: 43 European hospitals. PATIENTS: 308 patients (mean age 60 years, 70% men, 48% with heart disease) with sustained atrial fibrillation (n = 251) or atrial flutter (n = 57) (duration three hours to 45 days) were randomised to three groups to receive a 10 minute infusion of 1 mg ibutilide (n = 99), 2 mg ibutilide (n = 106), or 1.5 mg/kg DL-sotalol (n = 103). Infusion was discontinued at termination of the arrhythmia. MAIN OUTCOME MEASURE: Successful conversion of atrial fibrillation or flutter, defined as termination of arrhythmia within one hour of treatment. RESULTS: Both drugs were more effective against atrial flutter than against atrial fibrillation. Ibutilide was superior to DL-sotalol for treating atrial flutter (70% and 56% v 19%), while the high dose of ibutilide was more effective for treating atrial fibrillation than DL-sotalol (44% v 11%) and the lower dose of ibutilide (44% v 20%, p < 0.01). The mean (SD) time to arrhythmia termination was 13 (7) minutes with 2 mg ibutilide, 19 (15) minutes with 1 mg ibutilide, and 25 (17) minutes with DL-sotalol. In all patients, the duration of arrhythmia before treatment was a predictor of arrhythmia termination, although this was less obvious in the group that received 2 mg ibutilide. This dose converted almost 48% of atrial fibrillation that was present for more than 30 days. Concomitant use of digitalis or nifedipine and prolongation of the QTc interval were not predictive of arrhythmia termination. Bradycardia (6.5%) and hypotension (3.7%) were more common side effects with DL-sotalol. Of 211 patients given ibutilide, two (0.9%) who received the higher dose developed polymorphic ventricular tachycardia, one of whom required direct current cardioversion. CONCLUSION: Ibutilide (given in 1 or 2 mg doses over 10 minutes) is highly effective for rapidly terminating persistent atrial fibrillation or atrial flutter. This new class III drug, under monitored conditions, is a potential alternative to currently available cardioversion options.     

Autonomic modulation of the atrial cycle length by the head up tilt test: non-invasive evaluation in patients with chronic atrial fibrillation

Objective—To determine the effects of upright posture compared with supine position on the dominant atrial cycle length (DACL) in patients with chronic atrial fibrillation.
Design—The power/frequency spectrum of QRST suppressed lead V1 ECG was studied in 14 patients in the supine position and during the head up tilt table test. The DACL changes were compared with changes in heart rate and blood pressure.
Results—Compared with the supine position, the upright position reduced the DACL from 160 to 150 ms (p < 0.01). The DACL was increased after returning to the supine position from the upright position, from 147 to 154 ms (p < 0.01). Heart rate increased from 91 beats/min in the supine position to 106 in the upright position (p < 0.01). There was a decrease in heart rate from 109 beats/min in the upright position to 93 after returning to the supine position (p < 0.01). No significant changes were seen in systolic or diastolic blood pressure. There were indications of an inverse relation between DACL and heart rate when comparing the supine position before and after tilt with the upright position (p < 0.001).
Conclusions—The sympathetic stimulation and vagal withdrawal induced by rising to upright body position are associated with a decrease in DACL during chronic atrial fibrillation. Thus a reflex increase in sympathetic discharge after induction of atrial fibrillation could favour the persistence of the arrhythmia.

Keywords: atrial fibrillation;  autonomic nervous system;  atrial cycle length;  heart rate     

Prediction of the onset of atrial fibrillation after cardiac surgery using the monophasic action potential

Objective—To show that the monophasic action potential (MAP) recorded continuously from human epicardium may be used to predict the imminent onset of atrial fibrillation or flutter (AF) following surgery, thus allowing prophylactic treatment to be started.
Patients—22 patients (14 male, 8 female; mean (SD) age 64 (12) years) undergoing aortic valve replacement.
Setting—Tertiary referral centre.
Methods—Over a mean observation period of 8 (2.7) days (range 4 to 14), nine episodes of AF were seen in six patients. Before AF, specific and significant alterations of the MAP morphology were observed. In seven of nine episodes the MAP shortened (25 (4)% 60 minutes before AF), developed a triangular shape, and the plateau amplitude decreased from 5.3 (1.2) to 2 (0.2) mV. In the two remaining episodes the beat to beat variability of cycle length and MAP duration at 90% repolarisation (MAPd₉₀) increased significantly from 24 (7) ms and 12 (8) ms (24 hours before AF) to 137 (27) ms and 56 (11) ms (30 minutes before AF) respectively. AF was successfully treated by the administration of sotalol in three cases and by a combination of verapamil and digoxin in a further four. Previously observed changes of MAPd₉₀ and MAP morphology regressed after conversion to sinus rhythm.
Conclusions—The continuous and intermediate term recording of the MAP from atrial epicardium appears to be a valid tool for detecting imminent AF after cardiac surgery with a high sensitivity (99%) and specificity (88%). Optimised antiarrhythmic treatment may thus be given selectively for prophylaxis.

Keywords: postoperative atrial fibrillation;  monophasic action potential;  postoperative management;  prophylactic antiarrhythmic treatment;  arrhythmias     

Radiofrequency catheter ablation for atrial flutter following orthotopic heart transplantation

A 26 year old woman with a diagnosis of limb girdle muscular dystrophy and peripartum cardiomyopathy underwent orthotopic heart transplantation using standard atrial anastomoses. Recurrent atrial flutter was demonstrated in the absence of histological evidence of cellular rejection. Radiofrequency ablation of the flutter circuit was successfully performed allowing the avoidance of long term antiarrhythmic therapy. Ten weeks following ablation and cessation of amiodarone, presyncopal symptoms were found, associated with daytime and nocturnal sinus pauses of 2.9 seconds and 4.2 seconds, respectively. There was no evidence of AV node conduction impairment. In view of the continued absence of cellular rejection and the evident denervated state of the heart, the pauses reflected significant donor sinus node dysfunction; therefore, an AAIR permanent pacing system was implanted. At the time of pacemaker implantation atrial pacing at 150 beats/min did not produce significant PR interval prolongation or AV block. This case serves to advise rigorous follow up in such patients to continue to seek evidence of sinus node dysfunction potentially requiring permanent pacemaker implantation.

Keywords: heart transplantation;  atrial flutter;  radiofrequency catheter ablation     

Pulmonary function in children with atrial septal defect before and after heart surgery

Objective—To test the effect of heart disease and heart surgery on lung function.
Design—A pulmonary function study of children undergoing surgery for atrial septal defect (ASD).
Settings—University hospital.
Patients—26 children tested before surgery (at mean (SD) age 11.8 (3.8) years) and 24 patients tested 1.8 (0.2) years after surgical correction.
Methods—Lung volumes, lung elasticity, and airway patency indices were measured using standard techniques.
Results—Before surgery: pulmonary function test abnormalities were found in 18 of the 26 patients. Stiff lung was found in 12, lung hyperinflation in five, and indices of decreased airway patency in four. Total lung capacity decreased in only two patients. After surgery: pulmonary function test abnormalities were found in 12 of the 24 patients (informed consent not given for two patients). Stiff lung was detected in nine and indices of peripheral airway obstruction in four. Mean values of specific airway conductance and peak expiratory flow were all normal. Lung hyperinflation was found only in one of 24 patients. No correlation between perioperative events and pulmonary function test data was found.
Conclusions—Pulmonary function test abnormalities persist in half the patients almost two years after surgery for ASD. A decrease in the total frequency of pulmonary function test abnormalities (in 19% of the patients), with a decrease in stiff lung in 8% and lung hyperinflation in 15%, was not significant. Impairment of lung function related to ASD is associated with the disease itself rather than the surgical procedure.

Keywords: atrial septal defect; heart surgery; pulmonary function; cardiopulmonary development     

Atrial fibrillation is associated with a lower exercise capacity in male chronic heart failure patients

Objective—To study the influence of atrial fibrillation on peak oxygen uptake (peak O₂) in chronic heart failure. An unfavourable effect of atrial fibrillation has been shown in several patient populations, but the results have not been consistent in chronic heart failure.
Methods—Data were analysed from male heart transplant candidates who were able to perform graded bicycle ergometry until exhaustion with respiratory gas analysis and measurement of heart rate. Patients in atrial fibrillation (n = 18) were compared with patients in sinus rhythm (n = 93).
Results—Age, weight, height, and aetiology of chronic heart failure did not differ significantly between the two groups. Cardiac catheterisation at supine rest showed that heart rate was comparable, but that stroke volume and cardiac output were lower (p < 0.05) in atrial fibrillation. Systolic and diastolic left ventricular function, assessed by radionuclide angiography at rest, were not significantly different. Peak O₂ (mean (SD): 13.8 (3.6) v 17.1 (5.6) ml/kg/min; p < 0.01) and peak work load (78 (27) v 98 (36) W; p < 0.05) were lower in the patients with atrial fibrillation, though respiratory gas exchange ratio and Borg score were similar in the two groups. Patients with atrial fibrillation had a higher heart rate sitting at rest before exercise (93 (16) v 84 (16) beats/min) and at peak effort (156 (23) v 140 (25) beats/min) (p < 0.05).
Conclusions—Atrial fibrillation is associated with a 20% lower peak O₂ in patients with chronic heart failure, suggesting that preserved atrial contraction or a regular rhythm, or both, are critical to maintain cardiac output and exercise performance.

Keywords: peak oxygen uptake;  exercise capacity;  chronic heart failure;  atrial fibrillation     

T wave alternans after sotalol: evidence for increased sensitivity to sotalol after conversion from atrial fibrillation to sinus rhythm

A 64 year old woman with an 11 year history of paroxysmal atrial fibrillation presented to the emergency room because of palpitations that had started two weeks previously. She had used sotalol 80 mg once daily for three years without any episodes of proarrhythmia or other adverse effects. However, she developed pronounced T wave alternans with giant inverted T waves and excessive QT prolongation following sotalol administration one day after conversion from atrial fibrillation to sinus rhythm. This case demonstrates bizarre T wave changes, T wave alternans, and extreme QT prolongation following sotalol administration shortly after conversion from atrial fibrillation to sinus rhythm. In this situation, sotalol administration may be proarrhythmic, because it enhances repolarisation inhomogeneities based on a spatially inhomogeneous distribution of repolarisation controlling ion channels to induce repolarisation abnormalities that may lead to torsade de pointes.

Keywords: T wave alternans; long QT syndrome; torsade de pointes; sotalol; atrial fibrillation     

Successful radiofrequency catheter ablation of atrial trigeminy in a young patient

A case is reported of a 35 year old man with atrial parasystolic trigeminy. The patient presented with a 10 year history of sustained supraventricular extrasystole causing symptoms leading to several hospitalisations and continuous unemployment. He had been treated ineffectively with several drug combinations. Radiofrequency catheter ablation of a right atrial focus completely suppressed the ectopic activity. This is the first report to demonstrate the efficacy and safety of radiofrequency catheter ablation in atrial ectopic trigeminy.

Keywords: atrial trigeminy;  ectopic atrial tachycardia;  radiofrequency catheter ablation     

Left atrial thrombus as an early consequence of blunt chest trauma

Thromboembolism is rarely considered in discussions of the complications of blunt chest trauma. The few cases of thromboembolism that have been reported in this setting have occurred in association with significant myocardial damage. A previously fit 23 year old woman was admitted to the intensive care unit following a road traffic accident. A day later, left atrial thrombus was demonstrated by transoesophageal echocardiography in the absence of any other evidence of important myocardial injury. Anticoagulation with heparin was cautiously introduced in spite of her extensive injuries, and there were no consequent bleeding complications. At hospital discharge on day 18 she was entirely well. Full anticoagulation with warfarin was continued for a further eight weeks at which time follow up transoesophageal echocardiography showed complete resolution of the thrombus.

Keywords: blunt chest trauma;  thromboembolism;  left atrial thrombus;  transoesophageal echocardiography     

Animal experimental implantation of an atrial septal defect occluder system

OBJECTIVE: To establish the implantation technique for the atrial septal defect occluder system (ASDOS) device in an experimental animal model and to determine long term mechanical stability of the device and its in vivo properties in terms of biocompatibility and tissue reaction. MATERIALS AND METHODS: An atrial septal defect was created and the device implanted in 17 pigs (mean weight 30 kg). The implantation technique was refined and modified because of initial technical and anatomical complications during nine acute pilot studies. The technique proved to be feasible in eight subsequent survival studies. Four pigs were electively killed three months after implantation (group 1). The remaining four pigs were killed six months after implantation (group 2). RESULTS: Necropsy showed all devices were embedded in soft tissue three months after implantation. Microscopic examination of atrial septal tissue showed an acute granulomatous inflammatory reaction in group 1 and fibrosis in group 2. The intensity of the inflammatory reaction around the device was clearly milder in group 2, indicating a decline in the inflammatory response with time. Clinical and biochemical investigations indicated acceptable biocompatibility of the device. CONCLUSION: The implantation technique for the ASDOS device in a chronic pig model has been established. Biocompatibility of the device was acceptable.     

自主神经在阵发性心房颤动发生中的作用

目的 探讨自主神经在阵发性心房颤动发生中的作用。方法 分析31例阵发性房颤患者47次房颤（〉30s）发作前动态心电图心率变异性时域和频域指标。患者分为器质性心脏病组（19例）和非器质性心脏病组（12例）。根据房颤发作前频域指标变化，将阵发性房颤分为3组：房颤发作前心率变异性频域指标低频（LF）成分增加，LFIHF比例增加为交感神经介导房颤，房颤发作前心率变异性频域指标高频（HF）成分增加，LF／HF比例减小为迷走神经介导房颤，其余的为未分类房颤。结果 器质性心脏病组房颤发作28次，其中交感神经介导房颤17次（60．7％），非器质性心脏病组房颤发作19次，迷走神经介导房颤12次（63．2％）。结论 自主神经在房颤的发作中起着重要的作用，器质性心脏病中的阵发性房颤多为交感神经介导房颤。非器质性心脏病中的阵发性房颤多为迷走神经介导房颤。     

Five minute recordings of heart rate variability for population studies: repeatability and age-sex characteristics

Objective—To evaluate the stability of short recordings of heart rate variability (HRV) with time, and the association of HRV with age and sex.
Design—Five minute Holter recordings were made twice over a two month interval (tracking study). In addition, HRV was measured in a cross sectional study.
Setting—Residents of 11 Israeli kibbutzim were examined in their settlements.
Subjects—32 men and 38 women (aged 31-67) participated in the tracking study and 294 (aged 35-65) were involved in the cross sectional study.
Main outcome measures—Time and frequency domain analyses on Holter recordings were undertaken in two breathing conditions: spontaneous and controlled breathing (15 respirations per minute). Regression was used to assess the relations of sex, age, heart rate, and logarithmically transformed HRV indices.
Results—HRV measures were highly consistent with time with correlations of 0.76-0.80 for high frequency and total power. Geometric mean total power declined with age by 45% in men and 32% in women, and was lower by 24% among women than among men (all p 0.005). Men had a 34% higher very low and low frequency power and a higher ratio of low to high frequency power (p < 0.001). Conversely, high frequency power in women represents a greater proportion of total power than in men.
Conclusion—Short recordings of HRV in a non-laboratory setting are stable over months and therefore characteristic of an individual. Strong age and sex effects were evident. HRV derived from short recordings can be informative in population based studies.

Keywords: heart rate variability; parasympathetic activity; reliability; sex differences     

Electrophysiological determinant for induction of isthmus dependent counterclockwise and clockwise atrial flutter in humans

OBJECTIVE: To investigate the electrophysiological determinant underlying the electrical induction of counterclockwise and clockwise isthmus dependent atrial flutter. PATIENTS AND METHODS: The isthmus bordered by the inferior vena caval orifice-tricuspid annulus-coronary sinus ostium (IVCO-TA-CSO) has been assumed to be the site of both slow conduction and unidirectional block critical to the initiation of atrial flutter. Trans-isthmus and the global atrial conduction were studied in 25 patients with isthmus dependent atrial flutter (group A) and in 21 patients without atrial flutter (group B), by pacing at the coronary sinus ostium and the low lateral right atrium (LLRA) and mapping with a 20 pole Halo catheter in the right atrium. RESULTS: Mean (SD) fluoroscopic isthmus length between the coronary sinus ostium and LLRA sites was 28.1 (4.0) mm in group A and 28.0 (3.9) mm in group B (p = 0.95), but the trans-isthmus conduction velocity of both directions at various pacing cycle lengths was nearly halved in group A compared with group B (mean 0.39-0.46 m/s v 0.83-0.89 m/s, p < 0.0001). Pacing at coronary sinus ostium directly induced counterclockwise atrial flutter in 14 patients and pacing at LLRA induced clockwise atrial flutter in 11 patients, following abrupt unidirectional trans-isthmus block. Transient atrial tachyarrhythmias preceded the onset of atrial flutter in 10 counterclockwise and six clockwise cases of atrial flutter. None of the group B patients had inducible atrial flutter even in the presence of trans-isthmus block. The intra- and interatrial conduction times, as well as the conduction velocities at the right atrial free wall and the septum, were similar and largely within the normal range in both groups. CONCLUSIONS: Critical slowing of the trans-IVCO-TA-CSO isthmus conduction, but not the unidirectional block or the global atrial performance, is the electrophysiological determinant of the induction of counterclockwise and clockwise isthmus dependent atrial flutter in man.     

Profibrillatory effects of verapamil but not of digoxin in the goat model of atrial fibrillation

INTRODUCTION: Verapamil and digoxin have been shown to modulate tachycardia-induced atrial electrical remodeling. The goal of the present study was to determine the direct effects of verapamil and digoxin on atrial fibrillation (AF), before and after electrical remodeling. METHODS AND RESULTS: In six goats we measured the AF cycle length (AFCL) and duration of AF (DurAF) of 50 consecutive induced paroxysms, before (t = 0) and after 24 hours (t = 24) of electrical remodeling. During AF, conduction velocity (CV(AF)), refractory period (RP(AF)), and type of AF (I, II, III) were determined. Verapamil was administered at a loading dose of 0.1 mg/kg, followed by a continuous (2-hour) infusion of 5 microg/kg/min. Digoxin was given intravenously as a single 0.02 mg/kg bolus. At t = 0 and t = 24, digoxin and verapamil caused a significant slowing of the ventricular rate of >40%. Digoxin had no effect on DurAF, AFCL, CV(AF), or RP(AF). Infusion of verapamil had a direct proarrhythmic effect. Both at t = 0 and t = 24, AFCL and RP(AF) were shortened by about 15%. During acute AF, verapamil prolonged the average duration of AF paroxysms from 7 to 16 seconds. After 24 hours of AF, the proarrhythmic effect was much stronger. Shortly after starting infusion (6 +/- 2 min), verapamil converted paroxysmal AF into sustained AF. As long as verapamil infusion was maintained, AF no longer terminated in any of the goats. This effect was associated with an increase in AF fragmentation from type I to type II-III. CONCLUSION: Verapamil shortens AFCL and RP(AF) in the presence and absence of electrical remodeling. After 24 hours, it exerted a marked proarrhythmic effect and converted paroxysmal (type I) into sustained (type III) AF. In contrast, digoxin had no effect on the rate or stability of AF.     

New electrocardiographic criteria for the differentiation between counterclockwise and clockwise atrial flutter: correlation with electrophysiological study and radiofrequency catheter ablation

Objective—To develop new electrocardiographic (ECG) criteria for the differentiation between counterclockwise and clockwise atrial flutters.
Background—Traditionally, the ECG differentiation between counterclockwise and clockwise atrial flutters is based on the flutter wave polarity in the inferior leads. However, determination of flutter wave polarity is subjective and sometimes difficult, especially in flutter waves of undulating pattern.
Patients—The study comprised 37 consecutive patients with drug resistant atrial flutter; 30 had counterclockwise and 17 had clockwise atrial flutter (10 had both forms of atrial flutter). The isthmus dependence was confirmed by entrainment study and catheter ablation. The ECG patterns of both types of atrial flutter were compared and the flutter wave polarity in the inferior leads was determined by four independent cardiologists.
Results—The flutter wave polarity in the inferior leads appeared negative in 24, positive in one, and equivocal in five of the counterclockwise atrial flutters; polarity appeared negative in one, positive in 10, and equivocal in six of the clockwise atrial flutters. However, the aVF/lead I flutter wave amplitude ratio was > 2.5 in all counterclockwise but < 2.5 in all clockwise atrial flutters. The flutter wave nadirs in the inferior leads corresponded to the upstrokes in V1 in all counterclockwise atrial flutters, but corresponded to the downstrokes in V1 in all clockwise atrial flutters.
Conclusions—The flutter wave polarity in the inferior leads does not correlate well with the flutter wave rotating direction. However, counterclockwise and clockwise atrial flutters can be differentiated by new ECG criteria with high accuracy.

Keywords: atrial flutter;  radiofrequency catheter ablation;  electrocardiography     

Role of orthotopic heart transplantation in the management of patients with recurrent ventricular tachyarrhythmias following myocardial infarction

Objective—To report the outcome of an intention to treat by heart transplantation strategy in two groups of patients after infarction, one with both left ventricular failure (LVF) and ventricular tachyarrhythmias (VTA) (group A) and the other with progressive LVF following antiarrhythmic surgery for VTA (group B).
Patients and methods—Group A comprised 17 consecutive patients for whom transplantation was considered the best primary non-pharmacological treatment; group B comprised five consecutive patients assessed and planned for transplantation after antiarrhythmic surgery.
Results—In group A, eight patients underwent transplantation and all survived the first 30 day period. At median follow up of 55 months (range 11 to 109) seven of this subgroup were still alive. Five patients died of recurrent VTA before transplantation, despite circulatory support. In the face of uncontrollable VTA, four of these underwent "high risk" antiarrhythmic surgery while awaiting transplantation: three died of LVF within 30 days and one was saved by heart transplantation two days after arrhythmia surgery. Mortality for the transplantation strategy in group A patients was 47% by intention to treat analysis. Quality of life in the eight actually transplanted, however, was good and only one died during median follow up of 56 months. The five patients in group B were accepted for transplantation for progressive LVF at a median of 21 months (range 12 to 28) after antiarrhythmic surgery. One died of LVF before transplantation, 22 months after initial surgery; another died of high output LVF three days after transplantation. Thus mortality of the intended strategy was 40%. The three transplanted patients are alive and well at 8-86 months.
Conclusions—Although the short and medium term outcome in category A or B patients who undergo transplantation is good, the overall success of the transplantation strategy in category A patients is limited by lack of donors in the short time frame in which they are required.

Keywords: heart transplantation;  ventricular arrhythmias;  myocardial infarction     

Sudden death in a young man with catecholaminergic polymorphic ventricular tachycardia and paroxysmal atrial fibrillation

Catecholaminergic polymorphic ventricular tachycardia (CPVT) is a familial condition that presents with exercise-induced syncope or sudden death in children or young adults. In most cases the disease is caused by a mutation in the cardiac ryanodine receptor (RyR2) gene. Current evidence suggests that primary therapy for CPVT is beta blockade and implantable cardioverter defibrillator (ICD) placement. There is a recent report of a patient with CPVT who died despite appropriate ICD therapies, and we report a similar case. Our patient died after probably initially receiving inappropriate ICD shocks for atrial fibrillation. We recommend that utmost efforts should be made to prevent shocks including repeated exercise testing to confirm suppression of PVT.