Two-to-One AV Block Associated with the Congenital Long QT Syndrome

2:1 AV Block in LQTS. Introduction Conduction abnormalities associated with long QT syndrome (LQTS) have been reported as "pseudo 2:1 AV block" due to sinus intervals shorter than ventricular refractoriness.
Method and Results: We report the electrophysiologic characteristics of a patient suffering from congenital LQTS with episodes of true 2:1 AV block. Induction of 2:1 infra-Hisian blocks and return to 1:1 conduction were observed using single atrial and ventricular extrastimuli. The block was located in the Purkinje network but not in the myocardium.
Conclusion: The His-Purkinje system of our LQTS patient displayed dynamic properties with a strong increase in refractoriness for short-long sequences and a decrease for long-short sequences that triggered intermittent 2:1 AV blocks.     

Catheter Ablation for Recurrent Tachyarrhythmias. Clinical Experience with Two Different Techniques of Ablation in 21 Patients

Between 1984 and 1988, 21 patients underwent catheter ablation for drug refractory arrhythmias. Nine patients presented atrial flutter, atrial fibrillation or atrial tachycardia, nine had supraventricular tachycardia (one AV nodal reentrant tachycardia, one reciprocating tachycardia due to concealed accessory pathway and seven XMPW syndrome). Three had ventricular tachycardia. Fourteen patients were treated with direct current shock ablation (DC) and seven patients with radiofrequency ablation (RF). Eight patients underwent ablation of the His bundle. In six patients permanent AV block could be induced and in two first-degree AV block. All became asymptomatic (two with additional antiarrhythmic drug therapy). In four patients with WPW syndrome DC ablation of the accessory pathway was attempted. In one patient a permanent block in the accessory pathway and in another an intermittent block were obtained. In the two remaining patients with accessory pathways the ablation failed to interrupt the retrograde conduction in one the retrograde conduction was modified: however, in the other no change could be demonstrated. Two patients underwent ventricular foci ablation, with one partial success (arrhythmia controlled with associated drug therapy) and one failure. Three patients had RF His bundle ablation (two for atrial flutter and one for atrial fibrillation). One complete atrioventricular block, one first degree AV block and one first degree AV block associated with right bundle branch block were induced. Recurrence of tachyarrhythmias was prevented only in the patient with complete atrioventricular block. RF ablation of accessory pathway was performed in three patients. It resulted in anterograde block in the accessory pathway in the first patient; a slight modification of the retrograde refractory period in the second and no change was noted in the last one. The first of these three patients could then be controlled with drug therapy. The other two patients underwent surgical dissection of the pathway. One patient underwent an unsuccessful attempt of ventricular focus ablation with RF energy. Complications were more common with DC than with RF ablation but serious ventricular arrhythmias were also observed during RF ablation. Thus, DC ablation was completely successful in eight of 14 patients (57%), partially successful with the addition of drug therapy in three patients (21%) and failed in 22%. HF ablation was successful in only one patient (14.5%) and partially successful in another one (14.5%). This relatively low success rate is due in part to the design of the device and the electrodes used in this study. With technical improvements of RF ablation it seems reasonable to expect that this method will play a significant role in the management of drug refractory arrhythmias, since RF ablation, when compared to DC ablation, has the major advantage not to require general anesthesia during the procedure.     

Trisomy 3 in marginal zone B-cell lymphoma: a study based on cytogenetic analysis and fluorescence in situ hybridization

Trisomy 3 represents the most frequent and consistent chromosomal abnormality characterizing the recently defined entity marginal zone B-cell lymphoma (MZBCL). By cytogenetic analysis and/or fluorescence in situ hybridization (FISH) on interphase nuclei we found an increased copy number of chromosome 3 in 22/36 (61%) successfully analysed cases, including 8/12 cases with extranodal MZBCL, 8/13 cases with nodal MZBCL, and 6/11 patients with splenic MZBCL. Sensitivity of interphase cytogenetics was somewhat higher than that of conventional cytogenetic investigation. Structural chromosomal changes involving at least one chromosome 3 were seen in 11/20 cases with an increased copy number of chromosome 3: +del(3)(p13) was demonstrated in three cases, and was the sole chromosomal abnormality in one of them; +i(3)(q10) was seen in two other patients; and rearrangements involving various breakpoints on the long arm of chromosome 3 were found in the remaining cases. FISH on metaphase spreads confirmed these structural abnormalities and additionally showed two unexpected translocations involving chromosome 3. We conclude that: (1) trisomy 3 occurs in a high proportion of extranodal, nodal and splenic MZBCL; (2) FISH on interphase nuclei is an additional and sensitive tool in detecting an increased copy number of chromosome 3 in MZBCL; (3) additional structural abnormalities involving the long arm of chromosome 3 are frequent but non-recurrent and are perhaps secondary changes; and (4) abnormalities such as +del(3)(p13) and +i(3)(q10) suggest that genes located on the long arm of chromosome 3 are of particular importance in the pathogenesis of MZBCL.     

Safety of High Energy Internal Cardioversion for Atrial Fibrillation

High energy internal cardioversion has been proposed as an alternative method to cardiovert drug refractory or external cardioversion refractory atrial fibrillation. However, the safety of this technique has not been clearly evaluated. We reviewed findings in 53 patients who underwent 55 sessions of high energy internal cardioversion (2 patients underwent 2 sessions] for termination of longstanding atrial fibrillation. Shocks energy varied from 70–270 J. Three patients had 3 shocks during the same session, 5 had 2, and 47 only 1. Success rate was 89% (success was defined as immediate conversion to normal sinus rhythm).
Low cardiac output occurred in two patients, and resulted in the death of one of these individuals, a patient with significant hypertrophic cardiomyopathy and heart failure. The other patient recovered completely. In 11% of the cases, shock induced transient atrioventricular block, necessitating ventricular pacing until sinus rhythm was restored. In three patients, a moderate but asymptomatic and uncomplicated pericardial effusion was diagnosed on echocardiogram. Finally, four patients had side effects related to venous puncture, which resolved spontaneously. These results suggest that high energy internal cardioversion is effective for conversion of atrial fibrillation. However, the technique may not be optimal in patients with advanced hypertrophic cardiomyopathy and in such cases the technique should be used carefully and only in the case of failure of external cardioversion; no more than two shocks should be delivered during the same procedure. Temporary ventricular pacing should be provided in all patients and an echocardiogram should be performed before patients are being discharged.     

Laparoscopic management of a transitional cell carcinoma arising in a right ectopic ureter

Ectopic ureter is a rare abnormality, so presenting a transitional cell carcinoma (TCC) arising from an ectopic ureter is extremely rare. We report here a case of a man with an invasive transitional cell carcinoma arising from a right ectopic ureter and managed by laparoscopy. To our knowledge, this is the fourth case described in the literature, and the second case of a TCC arising in a right ectopic ureter.     

Ten-Years Follow-Up of 20 Patients with Idiopathic Ventricular Tachycardia

The follow-up and characteristics of 20 patients with ventricular tachycardia (VT) and no detectable heart disease is reported. These were 16 men and four women with a mean age of 44 years. Symptoms were present in 18 patients (eight had syncope and ten palpitations or dizziness), VT was sustained in 11 patients and a left bundle branch block morphology with inferior axis was found in 17 patients. In three patients, VT had a right bundle branch block morphology and left-axis deviation. The VT was inducible in 13 patients during the electrophysiological testing (EP) and was sustained in five patients. Medical treatment was introduced in 19 patients. During a mean follow-up of 10 years from the onset of the symptoms and 6 years from the EP testing, one patient died suddenly. He had stopped taking amiodarone 5 months before. In seven patients symptoms recurred and were due to discontinuation of therapy in two cases and inefficacy of previous effective treatment in five patients. After modification of the treatment (three cases), implantation of a pacemaker (one case) and catheter ablation (one case), all patients became asymptomatic. Eleven patients became asymptomatic with the first administered antiarrhythmic therapy. One patient continues to be asymptomatic in spite of discontinuation of his medical therapy. We conclude that patients with VT and no detectable heart disease have a good long-term prognosis and that appropriate therapy can be found in almost all patients.     

Autotumour reactive T-cell clones among tumour-infiltrating T lymphocytes in B-cell non-Hodgkin's lymphomas

Summary. Seventy-three T-cell clones (TCC) were established from tumour-infiltrating lymphocytes-T (TIL-T) derived from lymph nodes involved by B-cell non-Hodgkin's lymphomas (B-NHL) in nine patients with different histological subtypes and clinical stages. 40 TCC (55%) expressed the CD25 Ag and were also able to proliferate in the presence of irradiated autologous B-NHL cells. Among them, 23 autotumour (AuTu) proliferative TCC were found not to proliferate to autologous EBV-transformed B-cell lines, indicating that the proliferative reactivity of these TCC was preferentially directed at autologous B-NHL cells. Tested against autologous B-NHL cells, only three AuTu prolifera- tive TCC (CD8 ⁺) showed a significant level of cytotoxicity (specific lysis > 15%). In blocking experiments, the AuTu proliferative reactivity of three TCC from one patient was strongly inhibited by anti-DR and anti-DQ mAbs, whereas that of three TCC from another patient was not affected by either anti-MHC class I or class II (DR., DP, DQ) mAbs. These findings suggest that the recognition of autologous B-NHL cells by AuTu proliferative TCC may occur through MHC-restricted as well as MHC-unrestricted mechanisms.     

Molecular diagnosis of t(11;14) in mantle cell lymphoma using two-colour interphase fluorescence in situ hybridization

t(11;14) is observed in up to 70% of mantle cell lymphoma (MCL) cases and is therefore an important diagnostic element. In routine practice, detection of t(11;14) by conventional cytogenetic techniques is hindered by the low yield and quality of tumour metaphases. Molecular techniques (Southern blot, PCR) are unable to detect a large number of 11q13 breakpoints due to scattering over distances up to 1 Mb. Using 23 MCL patients with karyotypically determined t(11;14) and eight negative controls, we have devised a two-colour interphase FISH assay for detection of the 14q+ chromosome. We chose an 11q13 probe telomeric to the major 11q13 translocation cluster sites and an IGH probe centromeric of the 14q32 breakpoints. This method detected the translocation in all 23 t(11;14) positive patients, with an overall average of 60% nuclei showing colocalized signals. Widespread application of this technique will constitute an important diagnostic aid in clinical management of MCL patients. Since FISH is a convenient method for retrospective analysis of large numbers of patient specimens, this method should contribute to an accurate estimation of t(11;14) frequency in MCL and other chronic B-cell malignancies and consequently to their better nosological characterization.