Joyce C. Pressley

Familial Occurrence of Accessory Atrioventricular Pathways (Preexcitation Syndrome)

Accessory atrioventricular pathways, the anatomical structures responsible for the preexcitation syndromes, may result from a developmental failure to eradicate the remnants of the atrioventricular connections present during cardiogenesis. To study whether preexcitation syndromes could also be transmitted genetically, we determined the prevalence of preexcitation in the first-degree relatives of 383 of 456 consecutive patients (84 percent) with electrophysiologically proved accessory pathways. We compared the observed prevalence of preexcitation among the 2343 first-degree relatives with the frequency reported in the general population (0.15 percent). For 13 of the 383 index patients (3.4 percent), accessory pathways were documented in one or more first-degree relatives. At least 13 of the 2343 relatives identified (0.55 percent) had preexcitation; this prevalence was significantly higher than that in the general population (P less than 0.0001). Identification of affected relatives may have been incomplete because clinical information was obtained only about symptomatic relatives. Patients with familial preexcitation have a higher incidence of multiple accessory pathways and possibly an increased risk of sudden cardiac death. Our data suggest a hereditary contribution to the development of accessory pathways in humans. The pattern of inheritance appears to be autosomal dominant.

Frequency, diagnosis and clinical characteristics of patients with multiple accessory atrioventricular pathways

Multiple accessory atrioventricular (AV) pathways were documented in 52 of 388 patients (13%) who underwent detailed electrophysiologic evaluation. Multiple AV pathways were identified during intraoperative mapping or electrophysiologic study by different patterns of ventricular preexcitation during atrial fibrillation, flutter or atrial pacing with different delta-wave morphologic and ventricular activation patterns; different sites of atrial activation during right ventricular pacing or orthodromic reciprocating tachycardia; or preexcited reciprocating tachycardia using a second pathway as the retrograde limb of the tachycardia. A logistic model was used to determine which clinical, electrocardiographic and electrophysiologic variables were associated with multiple AV pathways. Right free-wall and posteroseptal accessory AV pathways were more common in patients with multiple AV pathways and were frequently associated. Multivariate logistic regression identified Ebsteins anomaly, and a history of preexcited reciprocating tachycardia as significant variables (p less than 0.0001). Pathway location was not subjected to statistical analysis because of confounding variables.

Effect of Ebstein's anomaly on short- and long-term outcome of surgically treated patients with Wolff-Parkinson-White syndrome.

BackgroundEbsteins anomaly is the most commonly occurring congenital abnormality associated with the Wolif-Parkinson-White (WPW) syndrome. However, the effects of Ebsteins anomaly on the risks and benefits of surgical ablation of accessory pathways in patients with WPW syndrome are unknown Methods and ResultsThis study compared the long-term outcome of 38 WPW patients with Ebsteins anomaly undergoing accessory pathway ablation to a reference population of 384 similarly treated patients without the anomaly. Ebsteins anomaly was mild in 21 patients (55%) and moderate-to-severe in 17 patients (45%). Sixteen patients (42%) required tricuspid valve surgery, and 23 (61%) had an atrial septal defect or patent foramen ovale repaired. Baseline clinical characteristics and preoperative clinical arrhythmias were similar in both groups. Ten-year survival was 92.4% and 91.2% for patients with and without Ebsteins anomaly, respectively (p = NS). During a mean follow-up of 6.2±3.8 and 5.3±3.6 years, 82% of patients with and 90% without Ebsteins anomaly had either clinically insignificant or no arrhythmias, and 18% versus 10% reported symptoms suggesting arrhythmias lasting longer than 1 minute, respectively. Atrial fibrillation was reduced postoperatively to 9% (p < 0.00l) in patients with and to 4% (p < 0.00l) in those without the anomaly. Fewer hospitalizations were reported postoperatively by 90% versus 96% of patients with and without Ebsteins anomaly; 9.4% versus 6.0% of patients were disabled at follow-up, respectively (p = NS) ConclusionsPatients with Ebsteins anomaly are improved significantly after accessory pathway ablation. The presence of this anomaly should not preclude accessory pathway ablation in these patients.

New observations on atrial fibrillation before and after surgical treatment in patients with the Wolff-Parkinson-White syndrome

The records of 342 patients who received surgical treatment for the Wolff-Parkinson-White syndrome between 1968 and 1986 were reviewed to evaluate the characteristics of atrial fibrillation. The patients were classified into two groups according to the presence (n = 166) or absence (n = 176) of documented episodes of atrial fibrillation preoperatively. The mean follow-up duration was 6 years (range 2 to 20). As compared with reports based on smaller patient groups and shorter follow-up, the study revealed several new findings. 1) During follow-up, nine patients in the atrial fibrillation group developed recurrent atrial fibrillation after a successful operation; five of these nine patients did not have associated heart disease. 2) All three patients with a history of atrial fibrillation and an accessory pathway conducting in the anterograde direction only had a successful surgical procedure and no postoperative atrial fibrillation. 3) The cycle length of atrioventricular (AV) reciprocating tachycardia was significantly shorter in the atrial fibrillation group (304 +/- 42 ms, mean +/- SD) than in the no-atrial fibrillation group (321 +/- 54 ms, p less than 0.005), and the cycle length of AV reciprocating tachycardia that degenerated into atrial fibrillation (289 +/- 26 ms) was shorter than that for the AV reciprocating tachycardia without subsequent atrial fibrillation (316 +/- 51 ms, p less than 0.005). 4) Sustained atrial fibrillation was induced in 30% of patients without a history of atrial fibrillation. 5) Atrial fibrillation occurred in four patients with an accessory pathway that conducted only in the retrograde direction.(ABSTRACT TRUNCATED AT 250 WORDS)

Out-of-hospital management of cardiac arrest by basic emergency medical technicians

The outcome in 126 consecutive patients with nontraumatic out-of-hospital cardiac arrest was analyzed to determine the effectiveness of a standard ambulance system over 22 months. Therapy was limited to basic life support (that is, administration of oxygen by mask, i.v. fluids, closed-chest massage and artificial respiration) by emergency medical technicians in a community in which less than 1% of the population had been trained in cardiopulmonary resuscitation (CPR). Analyses of patient data were performed to determine the relations between survival to hospital admission or discharge and 6 variables; response time, prior CPR, initial rhythm, acute myocardial infarction, initial blood pressure and initial pulse. Of 126 patients, 28 (22%) survived to hospital admission and 11 (9%) to hospital discharge. Two patient subgroups had a higher discharge rate: those with an initial rhythm of ventricular tachycardia or fibrillation (7 of 50, 14%), and those with an initial blood pressure greater than or equal to 90 mm Hg and a pulse rate of greater than 50 beats/min (3 of 6, 50%). For patients in arrest before ambulance arrival, there was no difference in outcome between those who did or those who did not receive prior CPR. Results of this study can be used as a basis for evaluating and comparing interventions directed toward stabilization of patients during the prehospital phase of cardiac arrest.

Predicting the outcomes of electrophysiologic studies of patients with unexplained syncope - Preliminary validation of a derived model

Purpose:To develop and validate a predictive model that would allow clinicians to determine whether an electrophysiologic (EP) study is likely to result in useful diagnostic information for a patient who has unexplained syncope.Patients:One hundred seventy-nine consecutive patients with unexplained syncope who underwent EP studies at two university medical centers comprised the training sample. A test sample to validate the model was made up of 138 patients from the clinical literature who had undergone EP studies for syncope.Design:Retrospective analysis of patients undergoing EP studies for syncope. The data collector was blinded to the study hypothesis; the electrophysiologist assessing outcomes was blinded to clinical and historical data. Clinical predictor variables available from the history, the physical examination, electrocardiography (ECG), and Holter monitoring were analyzed via two multivariable predictive modeling strategies (ordinal logistic regression and recursive partitioning) for their abilities to predict the results of EP studies, namely tachyarrhythmic and bradyarrhythmic outcomes. These categories were further divided into full arrhythmia and borderline arrhythmia groups.Results:Important outcomes were 1) sustained monomorphic ventricular tachycardia (VT) and 2) bradyarrhythmias, including sinus node and atrioventricular (AV) conducting disease. The results of the logistic regression (in this study, the superior strategy) showed that the presence of organic heart disease [odds ratio (OR)=3.0, p<0.001] and frequent premature ventricular contractions on ECG (OR=6.7, p<0.004) were associated with VT, while the following abnormal ECG findings were associated with bradyarrhythmias: first-degree heart block (OR=7.9, p<0.001), bundle-branch block (OR=3.0, p<0.02), and sinus bradycardia (OR=3.5, p<0.03). Eighty-seven percent of the 31 patients with important outcomes at EP study had at least one of these clinical risk factors, while 95% of the patients with none of these risk factors had normal or nondiagnostic EP studies. In the validation sample, the presence of one or more risk factors would have correctly identified 88% of the test VT patients and 65% of the test bradyarrhythmia patients as needing EP study.Conclusion:These five identified predictive factors, available from the history, the physical examination, and the initial ECG, could be useful to clinicians in selecting those patients with unexplained syncope who will have a serious arrhythmia identified by EP studies.

A comparison of paramedic versus basic emergency medical care of patients at high and low risk during acute myocardial infarction

This prospective study compares the outcome of patients with acute myocardial infarction managed by mobile intensive care (paramedic phase) with that of similar patients managed by basic emergency medical care (control phase) in the same community before the introduction of paramedics. All paramedic-transported patients were managed according to a standard chest pain protocol with use of prophylactic lidocaine and, as needed, treatment for sinus bradycardia, hypotension and life-threatening ventricular arrhythmia. There were no specific interventions for supraventricular tachyarrhythmia or hypertension. All patients were treated under similar in-hospital protocols. Percent mortality in patients with hypotension, the highest risk subgroup in the control phase, was significantly lowered with paramedic-level care (69 versus 10%, p = 0.01). Patients with hypertension, a relatively low risk subgroup during the control phase (16% mortality), were also at lower risk during the paramedic phase (10% mortality). In fact, there was no mortality in either study phase for patients with an initial systolic blood pressure greater than 180 mm Hg. During the combined study phases, patients with normotension and tachycardia demonstrated a tendency toward higher percent mortality (33%) than either patients with normotension without tachycardia (10%) or those with hypertension and tachycardia (6%). Although the overall percent mortality was reduced by 24% (from 21 to 16%), this decrease was largely due to the improvement of patients with hypotension. Investigation into the feasibility of prehospital interventions for the high risk patient with acute myocardial infarction normotension and tachycardia appears warranted.

Comparison of survival of amiodarone-treated patients with coronary artery disease and malignant ventricular arrhythmias with that of a control group with coronary artery disease.

Although amiodarone is effective in the treatment of ventricular arrhythmias, it is associated with serious toxic effects. In addition, the prognosis of patients with malignant ventricular arrhythmias and coronary artery disease treated with amiodarone remains poor. The survival of 54 consecutive patients with angiographically documented coronary artery disease and symptomatic ventricular tachycardia or ventricular fibrillation treated with amiodarone was compared with that of 5,125 medically treated patients with coronary artery disease. The amiodarone group was older, with worse left ventricular function and more peripheral and cerebrovascular disease. The 1 year survival probability was 0.73 for the amiodarone group and 0.94 for the control coronary artery disease group. At 2 years of follow-up, the survival probabilities were 0.60 and 0.90 for the amiodarone and the control group, respectively. When the survival curves were adjusted for group differences in baseline prognostic characteristics (integrated as a previously published hazard score), there was no difference in the prognosis of the two groups. These findings suggest that treatment with amiodarone of malignant ventricular arrhythmias associated with coronary artery disease maintains patients on an underlying survival curve determined by the degree of myocardial dysfunction, clinical characteristics and coronary anatomy, and that amiodarone does not have a deleterious effect on survival.

Basic emergency medical care of patients with acute myocardial infarction: initial prehospital characteristics and in-hospital complications

This prospective study documents the natural history of the prehospital phase of 110 patients with acute myocardial infarction transported by a basic emergency medical system during a 22 month period. Ambulances in a mixed urban-rural county were staffed by basic emergency medical technicians certified in basic life support and the administration of intravenous fluids. Systolic blood pressure, pulse rate and cardiac rhythm were noted for all patients at the time of ambulance arrival and intermittently during transport. Analyses of patient data were performed to determine the relation between the occurrence of subsequent in-hospital urgent complications and death and 1) patient delay time, 2) initial pulse rate, 3) initial systolic blood pressure, and 4) initial cardiac rhythm. Twenty-three (21%) of the 110 patients died and 66 (60%) experienced at least one in-hospital urgent complication. When initial rhythm, pulse rate and blood pressure were considered, patients with hypotension had a higher mortality rate than did those who were either normotensive or hypertensive. The 10 patients with initial sinus bradycardia but no hypotension constituted a subgroup with zero mortality. These results identify high and low risk patient subgroups that may benefit from either providing or withholding interventions directed toward hemodynamic stabilization during the prehospital phase of acute myocardial infarction.

Cryosurgical versus catheter ablation of the atrioventricular function

Results of catheter ablation of the atrioventricular (AV) junction in 41 patients were compared with results of cryosurgical ablation in 42 patients. Mean follow-up was 29 months among patients who underwent catheter ablation and 53 months among those who underwent cryosurgical ablation. In both groups complete heart block was produced in most patients (88% in the catheter ablation group, 86% in the cryosurgery group), and similar proportions of patients continued to receive antiarrhythmic drugs (27% in the catheter ablation group, 36% in the cryosurgery group). However, the short-term morbidity rate was significantly lower among patients who underwent catheter ablation (12% vs 42%) (p = 0.004). Long-term mortality and morbidity rates were not significantly different; most deaths were related to underlying cardiopulmonary disease and morbidity to problems with permanent pacemakers. Both catheter ablation and cryosurgical ablation of the AV junction are effective in creating complete AV block and controlling supraventricular tachycardia in medically refractory patients. Because catheter ablation is associated with lower short-term morbidity and avoids the need for a major surgical procedure, it is preferable to cryosurgical ablation of the AV junction when permanent abolition of AV conduction is necessary.