Bahram Kakavand

Pediatric nonpost-operative junctional ectopic tachycardia medical management and interventional therapies.

OBJECTIVES To determine the outcomes of medical management, pacing, and catheter ablation for the treatment of nonpost-operative junctional ectopic tachycardia (JET) in a pediatric population. BACKGROUND Nonpost-operative JET is a rare tachyarrhythmia that is associated with a high rate of morbidity and mortality. Most reports of clinical outcomes were published before the routine use of amiodarone or ablation therapies. METHODS This is an international, multicenter retrospective outcome study of pediatric patients treated for nonpost-operative JET. RESULTS A total of 94 patients with JET and 5 patients with accelerated junctional rhythm (age 0.8 year, range fetus to 16 years) from 22 institutions were identified. JET patients presenting at age < or =6 months were more likely to have incessant JET and to have faster JET rates. Antiarrhythmic medications were utilized in a majority of JET patients (89%), and of those, amiodarone was the most commonly reported effective agent (60%). Radiofrequency ablation was conducted in 17 patients and cryoablation in 27, with comparable success rates (82% radiofrequency vs. 85% cryoablation, p = 1.0). Atrioventricular junction ablation was required in 3% and pacemaker implantation in 14%. There were 4 (4%) deaths, all in patients presenting at age < or =6 months. CONCLUSIONS Patients with nonpost-operative JET have a wide range of clinical presentations, with younger patients demonstrating higher morbidity and mortality. With current medical, ablative, and device therapies, the majority of patients have a good clinical outcome.

Inside-out access: A new method of lead placement for patients with central venous occlusions

BACKGROUND Physicians will increasingly encounter patients who require rhythm management devices but have venous obstructions that prevent conventional access. Alternate access options, such as thoracotomy or transiliac approaches, exist but are associated with greater cost and morbidity. OBJECTIVE The purpose of this study is to describe a novel method of vascular access that allows prepectoral placement of conventional pacing and defibrillation leads in patients with complex central venous occlusions. METHODS Eight patients with central venous occlusions were referred for device implantation. Inside-out central venous access (IOCVA) was obtained via a percutaneous femoral approach. A catheter-dilator system was advanced via the right atrium to the most central point of venous occlusion. The occluded vein segment was punctured with a directionally guided needle, which was advanced along intravascular or extravascular tissue planes to the subclavian region. A solid wire needle was oriented toward the skin surface and advanced through the soft tissues until it exists from the body. The wire was used to pull rigid dilators through the occluded segment. Standard transvenous leads were implanted though the newly created channel. RESULTS All patients with total central venous occlusions (4 superior vena cava, 4 brachiocephalic and bilateral subclavian) had successful, prepectoral device implants (4 left-sided, 1 single-chamber, 4 dual-chamber, 3 biventricular). No procedure-related complications occurred. All patients had normal device function at follow-up of 485 ± 542 days. CONCLUSION IOCVA is an effective method of pacemaker and defibrillator implantation for patients with central venous occlusions. Further clinical evaluation of this novel method is needed.

Coincidence of Long QT Syndrome and Propionic Acidemia

Propionic acidemia and long QT syndrome (LQTS) are rare disorders. In addition, both conditions are potentially lethal. The patient presented in this article was initially diagnosed with propionic acidemia. Incidentally, she was found to have LQTS on electrocardiogram and verified by stress test and epinephrine challenge. Although the patient was asymptomatic and arrhythmia free, we started her on atenolol. This is the first report of the association between LQTS and propionic acidemia.

Is there an association between external cardioversions and long-term mortality and morbidity? Insights from the Atrial Fibrillation Follow-up Investigation of Rhythm Management study.

Background— Cardiac electric therapies effectively terminate tachyarrhythmias. Recent data suggest a possible increase in long-term mortality associated with implantable cardioverter-defibrillator shocks. Little is known about the association between external cardioversion episodes (ECVe) and long-term mortality. We sought to assess the safety of repeated ECVe with regard to cardiovascular mortality and morbidity. Methods and Results— We analyzed the data of the 4060 patients from the AFFIRM (Atrial Fibrillation Follow-up Investigation of Rhythm Management) trial. In particular, associations of ECVe with all-cause mortality, cardiovascular mortality, and hospitalizations after ECVe were studied. Over an average follow-up of 3.5 years, 660 (16.3%) patients died, 331 (8.2%) from cardiovascular causes. A total of 207 (5.1%) and 1697 (41.8%) patients had low ejection fraction and nonparoxysmal atrial fibrillation, respectively; 2460 patients received no ECVe, whereas 1600 experienced ≥1 ECVe. Death occurred in 412 (16.7%), 196 (16.5%), 39 (13.5%), and 13 (10.4%) of patients with 0, 1, 2, and ≥3 ECVe, respectively. There was no significant association between ECVe and mortality within any of the 4 subgroups defined by ejection fraction and atrial fibrillation type, although myocardial infarction, coronary artery bypass graft, and digoxin were significantly associated with death (estimated hazard ratios, 1.65, 1.59, and 1.62, respectively; P<0.0001). ECVe were associated with increased cardiac hospitalization reported at the next follow-up visit (39.3% versus 5.8%; estimated odds ratio, 1.39; P<0.0001). Conclusions— In the AFFIRM study, there was no significant association between ECVe and long-term mortality, even though ECVe were associated with increased hospitalizations from cardiac causes. Digoxin, myocardial infarction, and coronary artery bypass graft were significantly associated with mortality.Background —Cardiac electrical therapies effectively terminate tachyarrhythmias. Recent data suggest a possible increase in long-term mortality associated with implantable cardiac defibrillator shocks. Little is known about the association between external cardioversion episodes (ECVe) and long-term mortality. We sought to assess the safety of repeated ECVe with regards to cardiovascular mortality and morbidity. Methods and Results —We analyzed the data of the 4,060 patients from the AFFIRM trial. In particular, associations of ECVe with all-cause mortality, cardiovascular mortality and hospitalizations post ECVe were studied. Over an average follow-up of 3.5 years, 660 patients (16.3%) died, 331(8.2%) from cardiovascular causes. A total of 207(5.1%) and 1697(41.8%) patients had low ejection fraction (EF) and non-paroxysmal atrial fibrillation (AF) respectively. 2460 patients received no ECVe; while 1600 experienced ≥1 ECVe. Death occurred in 412(16.7%), 196(16.5%), 39(13.5%), and 13(10.4%) of patients with 0, 1, 2, and ≥3 ECVe respectively. There was no significant association between ECVe and mortality within any of the four subgroups defined by EF and AF type, although myocardial infarction (MI), coronary artery bypass graft (CABG), and digoxin were significantly associated with death (Estimated hazard ratios: 1.65; 1.59 and 1.62 respectively, p<0.0001). ECVe were associated with increased cardiac hospitalization reported at the next follow-up visit (39.3% vs. 5.8%; Estimated odds ratio: 1.39, p<0.0001). Conclusions —In the AFFIRM study, there was no significant association between ECVe and long-term mortality, even though ECVe were associated with increased hospitalizations from cardiac causes. Digoxin, MI, and CABG were significantly associated with mortality.

Successful Management of Acute Failure of the Systemic Right Ventricle with Cardiac Resynchronization Therapy

Management of systemic right ventricular (RV) failure can be challenging. Anatomical abnormality due to congenital heart disease adds to the complexity when interventions are performed. We report a patient with acute systemic RV failure who was successfully managed with cardiac resynchronization therapy.

Development of Quality Metrics in Ambulatory Pediatric Cardiology

The American College of Cardiology Adult Congenital and Pediatric Cardiology (ACPC) Section had attempted to create quality metrics (QM) for ambulatory pediatric practice, but limited evidence made the process difficult. The ACPC sought to develop QMs for ambulatory pediatric cardiology practice. Five areas of interest were identified, and QMs were developed in a 2-step review process. In the first step, an expert panel, using the modified RAND-UCLA methodology, rated each QM for feasibility and validity. The second step sought input from ACPC Section members; final approval was by a vote of the ACPC Council. Work groups proposed a total of 44 QMs. Thirty-one metrics passed the RAND process and, after the open comment period, the ACPC council approved 18 metrics. The project resulted in successful development of QMs in ambulatory pediatric cardiology for a range of ambulatory domains.

Is there an Association Between External Cardioversions and Long Term Mortality and Morbidity - Insights from the AFFIRM Study

Background— Cardiac electric therapies effectively terminate tachyarrhythmias. Recent data suggest a possible increase in long-term mortality associated with implantable cardioverter-defibrillator shocks. Little is known about the association between external cardioversion episodes (ECVe) and long-term mortality. We sought to assess the safety of repeated ECVe with regard to cardiovascular mortality and morbidity. Methods and Results— We analyzed the data of the 4060 patients from the AFFIRM (Atrial Fibrillation Follow-up Investigation of Rhythm Management) trial. In particular, associations of ECVe with all-cause mortality, cardiovascular mortality, and hospitalizations after ECVe were studied. Over an average follow-up of 3.5 years, 660 (16.3%) patients died, 331 (8.2%) from cardiovascular causes. A total of 207 (5.1%) and 1697 (41.8%) patients had low ejection fraction and nonparoxysmal atrial fibrillation, respectively; 2460 patients received no ECVe, whereas 1600 experienced ≥1 ECVe. Death occurred in 412 (16.7%), 196 (16.5%), 39 (13.5%), and 13 (10.4%) of patients with 0, 1, 2, and ≥3 ECVe, respectively. There was no significant association between ECVe and mortality within any of the 4 subgroups defined by ejection fraction and atrial fibrillation type, although myocardial infarction, coronary artery bypass graft, and digoxin were significantly associated with death (estimated hazard ratios, 1.65, 1.59, and 1.62, respectively; P<0.0001). ECVe were associated with increased cardiac hospitalization reported at the next follow-up visit (39.3% versus 5.8%; estimated odds ratio, 1.39; P<0.0001). Conclusions— In the AFFIRM study, there was no significant association between ECVe and long-term mortality, even though ECVe were associated with increased hospitalizations from cardiac causes. Digoxin, myocardial infarction, and coronary artery bypass graft were significantly associated with mortality.Background —Cardiac electrical therapies effectively terminate tachyarrhythmias. Recent data suggest a possible increase in long-term mortality associated with implantable cardiac defibrillator shocks. Little is known about the association between external cardioversion episodes (ECVe) and long-term mortality. We sought to assess the safety of repeated ECVe with regards to cardiovascular mortality and morbidity. Methods and Results —We analyzed the data of the 4,060 patients from the AFFIRM trial. In particular, associations of ECVe with all-cause mortality, cardiovascular mortality and hospitalizations post ECVe were studied. Over an average follow-up of 3.5 years, 660 patients (16.3%) died, 331(8.2%) from cardiovascular causes. A total of 207(5.1%) and 1697(41.8%) patients had low ejection fraction (EF) and non-paroxysmal atrial fibrillation (AF) respectively. 2460 patients received no ECVe; while 1600 experienced ≥1 ECVe. Death occurred in 412(16.7%), 196(16.5%), 39(13.5%), and 13(10.4%) of patients with 0, 1, 2, and ≥3 ECVe respectively. There was no significant association between ECVe and mortality within any of the four subgroups defined by EF and AF type, although myocardial infarction (MI), coronary artery bypass graft (CABG), and digoxin were significantly associated with death (Estimated hazard ratios: 1.65; 1.59 and 1.62 respectively, p<0.0001). ECVe were associated with increased cardiac hospitalization reported at the next follow-up visit (39.3% vs. 5.8%; Estimated odds ratio: 1.39, p<0.0001). Conclusions —In the AFFIRM study, there was no significant association between ECVe and long-term mortality, even though ECVe were associated with increased hospitalizations from cardiac causes. Digoxin, MI, and CABG were significantly associated with mortality.

Unusual Amiodarone Toxicity in a Child

metabolite were 0.3 µg/mL (0.47 µmol/L) and 0.4 ug/mL, respectively. The reference range for amiodarone in the serum is 0.5-2.5 µg/mL (0.8-3.9 0.47 µmol/L). Thyroid function test, ammonia level, plasma amino acids and urine organic acids were all normal or negative. The electrocardiogram showed normal sinus rhythm at 102 beats per minute. QRS was 89 msec, and QTc was 460 msec. A review of the literature revealed that central and peripheral neurotoxicity was indeed associated with amiodarone.

Prolonged Electrical Quiescence After Direct Current Cardioversion for Atrial Flutter in Congenital Heart Disease

Elective direct current cardioversion is considered first-line treatment in many cases of atrial flutter and fibrillation. This also is true in the pediatric population. This report describes a case of successful cardioversion that resulted in a very prolonged electrical quiescence.

Is There an Association Between External Cardioversions and Long-Term Mortality and Morbidity?Clinical Perspective: Insights From the Atrial Fibrillation Follow-Up Investigation of Rhythm Management Study

Background— Cardiac electric therapies effectively terminate tachyarrhythmias. Recent data suggest a possible increase in long-term mortality associated with implantable cardioverter-defibrillator shocks. Little is known about the association between external cardioversion episodes (ECVe) and long-term mortality. We sought to assess the safety of repeated ECVe with regard to cardiovascular mortality and morbidity. Methods and Results— We analyzed the data of the 4060 patients from the AFFIRM (Atrial Fibrillation Follow-up Investigation of Rhythm Management) trial. In particular, associations of ECVe with all-cause mortality, cardiovascular mortality, and hospitalizations after ECVe were studied. Over an average follow-up of 3.5 years, 660 (16.3%) patients died, 331 (8.2%) from cardiovascular causes. A total of 207 (5.1%) and 1697 (41.8%) patients had low ejection fraction and nonparoxysmal atrial fibrillation, respectively; 2460 patients received no ECVe, whereas 1600 experienced ≥1 ECVe. Death occurred in 412 (16.7%), 196 (16.5%), 39 (13.5%), and 13 (10.4%) of patients with 0, 1, 2, and ≥3 ECVe, respectively. There was no significant association between ECVe and mortality within any of the 4 subgroups defined by ejection fraction and atrial fibrillation type, although myocardial infarction, coronary artery bypass graft, and digoxin were significantly associated with death (estimated hazard ratios, 1.65, 1.59, and 1.62, respectively; P<0.0001). ECVe were associated with increased cardiac hospitalization reported at the next follow-up visit (39.3% versus 5.8%; estimated odds ratio, 1.39; P<0.0001). Conclusions— In the AFFIRM study, there was no significant association between ECVe and long-term mortality, even though ECVe were associated with increased hospitalizations from cardiac causes. Digoxin, myocardial infarction, and coronary artery bypass graft were significantly associated with mortality.Background —Cardiac electrical therapies effectively terminate tachyarrhythmias. Recent data suggest a possible increase in long-term mortality associated with implantable cardiac defibrillator shocks. Little is known about the association between external cardioversion episodes (ECVe) and long-term mortality. We sought to assess the safety of repeated ECVe with regards to cardiovascular mortality and morbidity. Methods and Results —We analyzed the data of the 4,060 patients from the AFFIRM trial. In particular, associations of ECVe with all-cause mortality, cardiovascular mortality and hospitalizations post ECVe were studied. Over an average follow-up of 3.5 years, 660 patients (16.3%) died, 331(8.2%) from cardiovascular causes. A total of 207(5.1%) and 1697(41.8%) patients had low ejection fraction (EF) and non-paroxysmal atrial fibrillation (AF) respectively. 2460 patients received no ECVe; while 1600 experienced ≥1 ECVe. Death occurred in 412(16.7%), 196(16.5%), 39(13.5%), and 13(10.4%) of patients with 0, 1, 2, and ≥3 ECVe respectively. There was no significant association between ECVe and mortality within any of the four subgroups defined by EF and AF type, although myocardial infarction (MI), coronary artery bypass graft (CABG), and digoxin were significantly associated with death (Estimated hazard ratios: 1.65; 1.59 and 1.62 respectively, p<0.0001). ECVe were associated with increased cardiac hospitalization reported at the next follow-up visit (39.3% vs. 5.8%; Estimated odds ratio: 1.39, p<0.0001). Conclusions —In the AFFIRM study, there was no significant association between ECVe and long-term mortality, even though ECVe were associated with increased hospitalizations from cardiac causes. Digoxin, MI, and CABG were significantly associated with mortality.