Mutations of SGO2 and CLDN14 collectively cause coincidental Perrault syndrome.

Perrault syndrome (PS) is a genetically heterogeneous disorder characterized by primary ovarian insufficiency (POI) in females and sensorineural hearing loss in males and females. In many PS subjects, causative variants have not been found in the five reported PS genes. The objective of this study was to identify the genetic cause of PS in an extended consanguineous family with six deaf individuals. Whole exome sequencing (WES) was completed on four affected members of a large family, and variants and co-segregation was confirmed by Sanger sequencing. All hearing impaired individuals, including the proband, are homozygous for a pathogenic variant of CLDN14, but this only explains the deafness. The PS proband is also homozygous for a frameshift variant (c.1453_1454delGA, p.(Glu485Lysfs*5)) in exon 7 of SGO2 encoding shugoshin 2, which is the likely cause of her concurrent ovarian insufficiency. In mouse, Sgol2a encoding shugoshin-like 2a is necessary during meiosis in both sexes to maintain the integrity of the cohesin complex that tethers sister chromatids. Human SGO2 has not previously been implicated in any disorder, but in this case of POI and perhaps others, it is a candidate for unexplained infertility.

USH1H, a novel locus for type I Usher syndrome, maps to chromosome 15q22-23.

Usher syndrome (USH) is a hereditary disorder associated with sensorineural hearing impairment, progressive loss of vision attributable to retinitis pigmentosa (RP) and variable vestibular function. Three clinical types have been described with type I (USH1) being the most severe. To date, six USH1 loci have been reported. We ascertained two large Pakistani consanguineous families segregating profound hearing loss, vestibular dysfunction, and RP, the defining features of USH1. In these families, we excluded linkage of USH to the 11 known USH loci and subsequently performed a genome-wide linkage screen. We found a novel USH1 locus designated USH1H that mapped to chromosome 15q22-23 in a 4.92-cM interval. This locus overlaps the non-syndromic deafness locus DFNB48 raising the possibility that the two disorders may be caused by allelic mutations.

Haplogroup analysis supports a pathogenic role for the 7510T>C mutation of mitochondrial tRNA(Ser(UCN)) in sensorineural hearing loss.

We ascertained a large North American family, LMG309, with matrilineal transmission of non-syndromic, progressive sensorineural hearing loss (SNHL). There was no history of aminoglycoside exposure, and penetrance was complete. We sequenced the entire mitochondrial genome and identified the previously reported 7510T>C transition in the tRNA(Ser(UCN)) gene. The 7510T>C was homoplasmic in all affected members. The LMG309 mitochondrial sequence belongs to an unnamed subgroup of mitochondrial haplogroup H. We demonstrate that the previously reported Spanish family S258 carries 7510T>C on a different mitochondrial sub-haplogroup, H1. We did not detect 7510T>C among 79 Caucasian haplogroup H control samples, including 11 from sub-haplogroup H1 and one from the same sub-haplogroup as LMG309. Our results provide strong genetic evidence that 7510T>C is a pathogenic mutation that causes non-syndromic SNHL.

Saline-cooled versus standard radiofrequency catheter ablation for infarct-related ventricular tachycardias.

BACKGROUND: Saline cooling of the electrode during radiofrequency (RF) ablation increases lesion size in animal models. If cooled RF also increases lesion size in human infarcts, it should facilitate the termination of ventricular tachycardia (VT). METHODS AND RESULTS: In 66 patients with VT due to prior infarction, 366 ablation sites, which were classified by entrainment and isolated potentials followed by ablation during VT with either standard RF energy (247 sites) or cooled RF (119 sites), were retrospectively reviewed to compare the efficacy for terminating VT. RF energy was applied at 259 isthmus sites, 62 bystander sites, 28 inner loop sites, and 17 outer loop sites. Compared with standard RF, cooled RF terminated VT more frequently at isthmus sites where an isolated potential was present (89% versus 54%, P=0.003), isthmus sites without an isolated potential (36% versus 21%, P=0.04), and at inner loop sites (60% versus 22%, P=0.04). Termination rates were similarly low for cooled and standard RF at bystander sites (14% versus 9%, P=0.56) and outer loop sites (13% versus 11%, P=0.93). CONCLUSIONS: Greater efficacy of cooled RF for terminating VT is consistent with the production of a larger lesion in human infarctions, which should facilitate successful ablation.

Efficacy of propafenone in Wolff-Parkinson-White syndrome: electrophysiologic findings and long-term follow-up.

The efficacy and safety of intravenous propafenone was studied in 10 patients with Wolff-Parkinson-White syndrome and in 2 patients with a concealed accessory pathway. During electrophysiologic study, the effect of propafenone on the effective refractory period of the accessory pathway was determined, as well as its effect during orthodromic atrioventricular (AV) reentrant tachycardia and atrial fibrillation. Propafenone caused significant increases in the accessory pathway refractory period, both in the anterograde direction (290 +/- 19 versus 474 +/- 50 ms, p less than 0.05) and in the retrograde direction (238 +/- 15 versus 408 +/- 44 ms, p less than 0.05). Complete anterograde accessory pathway conduction block occurred in four patients. Sustained AV reentrant tachycardia was inducible in 11 patients before administration of propafenone. Drug infusion during AV reentrant tachycardia promptly terminated arrhythmia in 10 of these 11 patients and caused slowing of AV reentrant tachycardia in the remaining patient. Before propafenone, sustained atrial fibrillation was inducible in six patients and nonsustained atrial fibrillation in four patients. After propafenone, no patient had inducible sustained atrial fibrillation. Furthermore, propafenone caused a marked decrease in peak ventricular rate during atrial fibrillation. Eight patients have been treated with oral propafenone and followed up for 12 +/- 2 months. All have remained virtually free of recurrent arrhythmia and none has developed significant side effects. Propafenone is a very promising agent for emergency intravenous therapy as well as long-term oral therapy in patients with Wolff-Parkinson-White syndrome.

Catheter ablation of ventricular tachycardia after myocardial infarction: relation of endocardial sinus rhythm late potentials to the reentry circuit.

OBJECTIVES: We sought to determine whether endocardial late potentials during sinus rhythm are associated with reentry circuit sites during ventricular tachycardia (VT). BACKGROUND: During sinus rhythm, slow conduction through an old infarct region may depolarize tissue after the end of the QRS complex. Such slow conduction regions can cause reentry. METHODS: Endocardial catheter mapping and radiofrequency ablation were performed in 24 patients with VT late after myocardial infarction. We selected for analysis a total of 103 sites where the electrogram was recorded during sinus rhythm and, without moving the catheter, VT was initiated and radiofrequency current applied in an attempt to terminate VT. RESULTS: Late potentials were present at 34 sites (33%). During pace mapping, the stimulus-QRS complex was longer at late potential sites, consistent with slow conduction, than at sites without late potentials (p < 0.0001). Late potentials were present at 15 (71%) of 21 sites classified as central or proximal in the reentry circuit based on entrainment, but also occurred frequently at bystander sites (13 [33%] of 39) and were often absent at the reentry circuit exit (3 [23%] of 13). Late potentials were present at 20 (54%) of 37 sites where ablation terminated VT, compared with 14 (21%) of 66 sites where ablation did not terminate VT (p = 0.004). Ablation decreased the amplitude of the late potentials present at sites where ablation terminated VT. CONCLUSIONS: Although sites with sinus rhythm late potentials often participate in VT reentry circuits, many reentry circuit sites do not have late potentials. Late potentials can also arise from bystander regions. Late potentials may help identify abnormal regions in sinus rhythm but cannot replace mapping during induced VT to guide ablation.

Characteristics of electrograms recorded at reentry circuit sites and bystanders during ventricular tachycardia after myocardial infarction.

OBJECTIVES: The purpose of this study was to determine the relation of isolated potentials (IPs) recorded during ventricular tachycardia (VT) to reentry circuit sites identified by entrainment. BACKGROUND: Reentry circuits causing VT late after myocardial infarction are complex. Both IPs and entrainment have been useful for identifying successful ablation sites, but the relation of IPs to the location in the reentry circuit as determined by entrainment has not been completely defined. METHODS: Data from catheter mapping of 70 monomorphic VTs in 36 patients with prior myocardial infarction were retrospectively analyzed. Entrainment followed by radiofrequency current (RF) ablation was performed at 384 sites. On the basis of entrainment, sites were classified as reentry circuit exit, central-proximal, inner or outer loop sites. Sites outside the circuit were divided into remote and adjacent bystanders. RESULTS: Isolated potentials were recorded at 50% (51 of 101) of reentry circuit exit, central and proximal sites as compared with only 8% (11 of 146, p < 0.001) of inner loop and outer loop sites and only 1.8% (2 of 106) of remote bystander sites (p < 0.001). Isolated potentials were also present at 45% of adjacent bystander sites. At central and proximal sites the presence of an IP increased the incidence of tachycardia termination by RF to 47.5% from 24% (p = 0.05). At exit sites tachycardia termination occurred frequently regardless of the presence or absence of IPs (45% vs. 48%, p = NS). Isolated potentials at exit, central and proximal sites had a shorter duration at sites where ablation terminated VT than at sites without termination (20.9 +/- 9.6 ms vs. 35.7 +/- 15.3 ms, p < 0.001). CONCLUSIONS: Isolated potentials are a useful guide to sites in the central-proximal region of the reentry circuit, but often fail to identify exit sites where ablation is successful. Entrainment and analysis of electrograms provide complementary information during mapping of VT.

Entrainment mapping and radiofrequency catheter ablation of ventricular tachycardia in right ventricular dysplasia.

OBJECTIVES: The purpose of this study was to determine if entrainment mapping techniques and predictors of successful ablation sites previously tested in coronary artery disease can be applied to ventricular tachycardia (VT) in arrhythmogenic right ventricular dysplasia (ARVD). BACKGROUND: VT in ARVD has not been well characterized. Reentry circuits in areas of abnormal myocardium are the likely cause, but these circuits have not been well defined. METHODS: Mapping of 19 VTs in 5 patients with ARVD was performed. At 58 sites pacing entrained VT and radiofrequency current (RF) was applied to assess acute termination of VT. RESULTS: Sites classified as exits, central/proximal, inner loop, outer loop, remote bystander and adjacent bystander were identified by entrainment criteria. The reentrant circuit sites were clustered predominantly around the tricuspid annulus and in the right ventricular outflow tract (RVOT). RF ablation acutely terminated VT at 13 sites or 22% of the applications. Of the 19 VTs, eight were rendered noninducible and three were modified to a longer cycle length. In 2 patients ablation at a single site abolished two VTs. CONCLUSION: VT in ARVD shows many of the characteristics of VT due to myocardial infarction. Entrainment mapping techniques can be used to characterize reentry circuits in ARVD. The use of entrainment mapping to guide ablation is feasible.

Detection of pacing-induced myocardial ischemia by endocardial electrograms recorded during cardiac catheterization.

Rapid atrial pacing confirms myocardial ischemia in patients with coronary artery disease when angina is provoked, and is accompanied by an increase in left ventricular end-diastolic pressure. In such cases, abnormalities in the surface electrocardiogram (ECG) are often not apparent. To enhance detection of subendocardial ischemia during rapid atrial pacing, local unipolar electrograms were recorded from the tip of a 0.025 in. (0.064 cm) diameter guidewire positioned against the endocardial surface of potentially ischemic regions. Endocardial electrograms, left ventricular end-diastolic pressure and multiple surface ECG leads were recorded during rapid atrial pacing in 21 patients with coronary artery disease. Before pacing, endocardial electrograms in all 21 patients were free of ST elevation. Marked ST elevation was apparent in 17 of the 21 patients after rapid atrial pacing and could be abolished by nitroglycerin. Moreover, in several patients, endocardial ST elevation after rapid atrial pacing was abolished after successful percutaneous transluminal coronary angioplasty of the critically stenosed artery supplying the ischemic region of myocardium. It is concluded that ST elevation in the endocardial electrogram after rapid atrial pacing is a reflection of myocardial ischemia and may be a sensitive marker of pacing-induced ischemia appearing earlier than angina, postpacing increase in left ventricular end-diastolic pressure or ST depression in the surface ECG.

Long-term oral propafenone therapy for suppression of refractory symptomatic atrial fibrillation and atrial flutter.

Sixty patients who had recurrent episodes of symptomatic atrial fibrillation or flutter, or both, and who had failed one to five prior drug trials were treated with open label oral propafenone hydrochloride. On a mean maximal tolerated dose of 795 +/- 180 mg/day, actuarial estimates of the percent of individuals free of recurrences of symptomatic atrial fibrillation/flutter during propafenone treatment were: 1 month, 54%; 3 months, 44% and 6 months, 40%. No individual baseline characteristic achieved statistical significance as a correlate of poor response to propafenone. Drug-related adverse reactions were reported in 22% of patients but were severe enough to require termination of propafenone in only 5%. Thus, oral propafenone is a useful and well tolerated drug for long-term suppression of symptomatic recurrences of atrial fibrillation/flutter despite a history of unresponsiveness to prior antiarrhythmic drug treatment.

Therapy of refractory symptomatic atrial fibrillation and atrial flutter: a staged care approach with new antiarrhythmic drugs.

One hundred nine patients with recurrent episodes of symptomatic atrial fibrillation or flutter, or both, who had failed one to five previous antiarrhythmic drug trials were treated with propafenone and, subsequently, sotalol if atrial fibrillation recurred. The clinical profile of the study group was as follows: age 63 +/- 13 years, left atrial anteroposterior dimension 4.4 +/- 0.9 cm and left ventricular ejection fraction 57 +/- 14%. Paroxysmal atrial fibrillation occurred in 56 patients (51%) and chronic atrial fibrillation occurred in 53 patients (49%). After loading and dose titration phases were completed, the maintenance doses of drugs were 450 to 900 mg/day for propafenone and 160 to 960 mg/day for sotalol. Life table estimates of the duration of freedom from atrial fibrillation were constructed for each drug trial. The percent of patients free of recurrent symptomatic arrhythmia at 6 months was 39% for propafenone and 50% for sotalol. The cumulative proportion of patients successfully treated with propafenone or sotalol, or both, by 6 months was 55% and remained relatively constant beyond that point. The incidence of intolerable side effects necessitating discontinuation of therapy ranged from 7% to 8%. Thus, despite previous unsuccessful drug trials, a substantial proportion of patients with recurrent symptomatic atrial fibrillation refractory to conventional therapy can be treated successfully and safely with newer antiarrhythmic drugs. Treatment failures tend to occur early in the course of follow-up, permitting easy identification of candidates for alternative therapeutic approaches.

Multi atrial maco-re-entry circuits in adults with repaired congenital heart disease: entrainment mapping combined with three-dimensional electroanatomic mapping.

OBJECTIVES: We sought to characterize re-entry circuits causing intra-atrial re-entrant tachycardias (IARTs) late after the repair of congenital heart disease (CHD) and to define an approach for mapping and ablation, combining anatomy, activation sequence data and entrainment mapping. BACKGROUND: The development of IARTs after repair of CHD is difficult to manage and ablate due to complex anatomy, variable re-entry circuit locations and the frequent co-existence of multiple circuits. METHODS: Forty-seven re-entry circuits were mapped in 20 patients with recurrent IARTs refractory to medical therapy. In the first group (n = 7), ablation was guided by entrainment mapping. In the second group (n = 13), entrainment mapping was combined with a three-dimensional electroanatomic mapping system to precisely localize the scar-related boundaries of re-entry circuits and to reconstruct the activation pattern. RESULTS: Three types of right atrial macro-re-entrant circuits were identified: those related to a lateral right atriotomy scar (19 IARTs), the Eustachian isthmus (18 IARTs) or an atrial septal patch (8 IARTs). Two IARTs originated in the left atrium. Radiofrequency (RF) lesions were applied to transect critical isthmuses in the right atrium. In three patients, the combined mapping approach identified a narrow isthmuses in the lateral atrium, where the first RF lesion interrupted the circuit; the remaining circuits were interrupted by a series of RF lesions across a broader path. Overall, 38 (81%) of 47 IARTs were successfully ablated. During follow-up ranging from 3 to 46 months, 16 (80%) of 20 patients remained free of recurrence. Success was similar in the first 7 (group 1) and last 13 patients (group 2), but fluoroscopy time decreased from 60 +/- 30 to 24 +/- 9 min/procedure, probably related to the increasing experience and ability to monitor catheter position non-fluoroscopically. CONCLUSIONS: Entrainment mapping combined with three-dimensional electroanatomic mapping allows delineation of complex re-entry circuits and critical isthmuses as targets for ablation. Radiofrequency catheter ablation is a reasonable option for treatment of IARTs related to repair of CHD.

Exploring postinfarction reentrant ventricular tachycardia with entrainment mapping.

Ventricular tachycardia late after myocardial infarction is usually due to reentry in the infarct region. These reentry circuits can be large, complex and difficult to define, impeding study in the electrophysiology laboratory and making catheter ablation difficult. Pacing through the electrodes of the mapping catheter provides a new approach to mapping. When pacing stimuli capture the effects on the tachycardia depend on the location of the pacing site relative to the reentry circuit. The effects observed allow identification of various portions of the reentry circuit, without the need for locating the entire circuit. Isthmuses where relatively small lesions produced by radiofrequency catheter ablation can interrupt reentry can often be identified. A classification that divides reentry circuits into one or more functional components helps to conceptualize the reentry circuit and predicts the likelihood that heating with radiofrequency current will terminate tachycardia. These methods are helping to define human reentry circuits.

The N + 1 difference: a new measure for entrainment mapping.

OBJECTIVES: The purpose of this study was to develop and test a new entrainment mapping measurement, the N + 1 difference. BACKGROUND: Entrainment mapping is useful for identifying re-entry circuit sites but is often limited by difficulty in assessing: 1) changes in QRS complexes or P-waves that indicate fusion, and 2) the postpacing interval (PPI) recorded directly from the stimulation site. METHODS: In computer simulations of re-entry circuits, the interval from a stimulus that reset tachycardia to a timing reference during the second beat after the stimulus was compared with the timing of local activation at the site during tachycardia to define an interval designated the N + 1 difference. The N + 1 difference was compared with the PPI-tachycardia cycle length (TCL) difference in simulations and at 65 sites in 10 consecutive patients with ventricular tachycardia (VT) after myocardial infarction and at 45 sites in 10 consecutive patients with atrial flutter. RESULTS: In simulations, the N + 1 difference was equal to the PPI-TCL difference. During mapping of VT and atrial flutter, the N + 1 difference correlated well with the PPI-TCL difference (r > or = 0.91, p < 0.0001), identifying re-entry circuit sites with sensitivity of > or = 86% and specificity of > or = 90%. Accuracy was similar using either the surface electrocardiogram or an intracardiac electrogram (Eg) as the timing reference. CONCLUSIONS: The N + 1 difference allows entrainment mapping to be used to identify re-entry circuit sites when it is difficult to evaluate Egs at the mapping site or fusion in the surface electrocardiogram.

Life-threatening ventricular arrhythmias due to transient or correctable causes: high risk for death in follow-up.

OBJECTIVES: This study evaluated the prognosis of patients resuscitated from ventricular tachycardia (VT) or ventricular fibrillation (VF) with a transient or correctable cause suspected as the cause of the VT/VF. BACKGROUND: Patients resuscitated from VT/VF in whom a transient or correctable cause has been identified are thought to be at low risk for recurrence and often receive no primary treatment for their arrhythmias. METHODS: In the Antiarrhythmics Versus Implantable Defibrillators (AVID) trial, patients with a potentially transient or correctable cause of VT/VF were not eligible for randomization. The mortality of these patients was compared with the mortality of patients with a known high risk of recurrence of VT/VF in the AVID registry. RESULTS: Compared with patients having high risk VT/VF, those with a transient or correctable cause for their presenting VT/VF were younger and had a higher left ventricular ejection fraction. These patients were more often treated with revascularization as the primary therapy, more commonly received a beta-blocker, less often required therapy for congestive heart failure and less commonly received either an antiarrhythmic drug or an implantable cardioverter defibrillator. Nevertheless, subsequent mortality of patients with a transient or correctable cause of VT/VF was no different or perhaps even worse than that of the primary VT/VF population. CONCLUSIONS: Patients identified with a transient or correctable cause for their VT/VF remain at high risk for death. Further research is needed to define truly reversible causes of VT/VF. Meanwhile, these patients may require more aggressive evaluation, treatment and follow-up than is currently practiced.

Amiodarone therapy and autoimmune thyroid disease. Increase in a new monoclonal antibody-defined T cell subset.

T cell subsets in 10 patients receiving amiodarone were evaluated, and their thyroid function and antithyroid antibodies were assessed. A generalized increase in a recently discovered subset of T cells expressing a complex ganglioside antigen reacting with monoclonal antibody 3G5 was found. Two patients, one with hyperthyroidism and the other with euthyroid Graves' ophthalmopathy, had an additional T cell abnormality--marked increase in Ia-positive T cells (an abnormality typical of patients with spontaneous Graves' disease). In the hyperthyroid patient, the Ia-positive T cells disappeared within three weeks after amiodarone was discontinued. The other patients receiving amiodarone had normal numbers of Ia-positive T cells. These studies indicate that amiodarone alters a major resting T cell subset for almost all patients and is associated with T cells expressing the Ia antigen in selected patients. These T cell abnormalities suggest that amiodarone precipitates organ-specific autoimmunity in susceptible persons.

Proarrhythmia.

Proarrhythmia is defined as the provocation of a new arrhythmia or the aggravation of a pre-existing one during therapy with a drug at doses or plasma concentrations below those considered to be toxic. Suggested criteria for proarrhythmia include (1) the new appearance of a sustained ventricular tachyarrhythmia; (2) change from a nonsustained to a sustained tachyarrhythmia; (3) acceleration of tachycardia rate; or (4) the new appearance of a clinically significant bradyarrhythmia or conduction defect. Proarrhythmia can be the direct result of a drug's electrophysiologic effects on conduction velocity, refractoriness, and automaticity. However, it may also be the result of metabolic abnormalities, changes in autonomic state, or drug/drug interactions that amplify or alter the drug's electrophysiologic effects. Some forms of ventricular proarrhythmia, such as torsade de pointes, are difficult to forecast and occur in patients with structurally normal hearts as well as in those with serious heart disease. Other forms of ventricular proarrhythmia, such as monomorphic ventricular tachycardia, occur predominantly in patients with structural heart disease or pre-existing ventricular arrhythmia. Atrial flutter with 1 : 1 conduction and bradyarrhythmias can be manifestations of proarrhythmia, particularly during drug therapy for atrial fibrillation. In patients with pacemakers or implantable cardiac defibrillators, antiarrhythmic drugs can change pacing thresholds and can alter the ability of a device to recognize or terminate a sustained ventricular tachyarrhythmia.

Effect of selective intracoronary antiarrhythmic drug administration in sustained ventricular tachycardia.

The effect of selective intracoronary antiarrhythmic drug infusion on inducibility of cardiac arrhythmias was studied in 3 patients with recurrent sustained monomorphic ventricular tachycardia referred for comprehensive electrophysiologic studies. Each patient had evidence of prior myocardial infarction, 1 or more occluded coronary arteries and a readily identifiable collateral vessel that provided collateral flow to the infarct-related artery. In each patient, the clinical arrhythmia was reproducibly inducible by programmed stimulation in the control state. After positioning a small infusion catheter in the collateral vessel, selective intracoronary lidocaine 0.3 to 0.6 mg/min (patients 1 and 2) or procainamide 0.1 to 1.4 mg/min (patient 3) was infused for a 10-minute period. In each patient the clinical arrhythmia was rendered noninducible during selective intracoronary drug infusion. The arrhythmia was again inducible after a 10-minute drug-washout period and also after standard intravenous doses of antiarrhythmic drug. Selective intracoronary antiarrhythmic drug infusion may help to localize the site of origin of some cardiac arrhythmias, may provide a means of testing the effects of several drugs during a single study and may be a new method for studying mechanisms of action of antiarrhythmic drugs.

Propafenone versus sotalol for suppression of recurrent symptomatic atrial fibrillation.

Because conventional antiarrhythmic therapy is often ineffective in maintaining sinus rhythm or is associated with adverse side effects in patients with atrial fibrillation (AF), there is a clinical need to test newer agents. One hundred patients with AF who had unsuccessful therapy with 1.9 +/- 1.0 type IA antiarrhythmic agents were randomized to receive either propafenone (n = 50) or sotalol (n = 50). Patients were stratified into 4 groups based on AF pattern (chronic vs paroxysmal) and left atrial size (large [> or = 4.5 cm] vs small [< 4.5]). The proportion of patients remaining in sinus rhythm on each agent was calculated for each group by the Kaplan-Meier method. For patients randomized to propafenone, 46 +/- 8%, 41 +/- 8% and 30 +/- 8% remained in sinus rhythm at 3, 6 and 12 months, respectively, after cardioversion. A similar proportion of patients treated with sotalol remained in sinus rhythm at follow-up (49 +/- 7%, 46 +/- 8% and 37 +/- 8% at 3, 6 and 12 months, respectively; p = NS). The proportion of patients remaining in sinus rhythm on propafenone and sotalol was not dependent on arrhythmia pattern or left atrial dimension. Except for constipation that occurred more frequently in patients treated with propafenone, adverse side effects were equally distributed between the 2 therapies. Two patients receiving sotalol died during follow-up. Propafenone and sotalol, 2 new antiarrhythmic agents, were found to be equally effective in maintaining sinus rhythm in 100 patients with recurrent AF. Response rates were not affected by arrhythmia pattern, left atrial size or unsuccessful prior drug therapy.(ABSTRACT TRUNCATED AT 250 WORDS)

Transtelephonic electrocardiographic transmission for management of cardiac arrhythmias.

Brief periods of transtelephonic electrocardiographic transmission conducted at periodic intervals or during sporadic symptoms may provide an inexpensive and reliable alternative to extended ambulatory electrocardiographic tape recordings. Sixty-one patients were enrolled in a transtelephonic electrocardiographic transmissions program. In 51 patients with documented arrhythmias (group I), telephone electrocardiographic transmissions were used to monitor antiarrhythmic drug therapy. In 10 patients, telephone electrocardiographic transmission was used in an attempt to diagnose infrequent symptoms suggestive of arrhythmia (group II). Of the 650 telephone electrocardiographic transmissions received, 73 (11%) revealed a clinically significant event, whereas 577 (89%) did not show any significant disturbances of cardiac rhythm. Of the 61 patients entered into the program, 29 (48%) had a clinically significant event identified during 1 or more transmissions. In group I, transtelephonic electrocardiographic transmission prompted a change in therapy in 37% of the patients. Of the 10 patients in group II, clinically significant events were noted during telephone electrocardiographic transmissions in each patient. Assuming a yield of 1 clinically significant event detected per 10 telephone electrocardiographic transmissions and a similar yield on long-term ambulatory electrocardiographic recordings, use of telephone electrocardiographic transmissions offers a cost-effective means of following patients with significant cardiac arrhythmias who are receiving potent antiarrhythmic drugs. In addition, telephone electrocardiographic transmission is a suitable diagnostic technique for patients with infrequent symptoms suggestive of cardiac arrhythmias.