Telangiectasias of the breasts showing diffuse dermal angiomatosis in a patient with diffuse livedo reticularis.

The breasts are a common location for diffuse dermal angiomatosis (DDA) in a context of obesity and macromastia. The typical clinical presentation includes erythematous or purplish plaques, reticulated telangiectasias, and sometimes livedo reticularis, often complicated by painful ulcerations of the breasts. Biopsy usually confirms a dermal proliferation of endothelial cells staining positively for CD31, CD34 and SMAa and negatively for HHV8. We report herein a woman with DDA of the breasts presenting as diffuse livedo reticularis and acrocyanosis, both long-standing and considered idiopathic following extensive investigations. Since a biopsy of the livedo did not document DDA features in our case, we suggest that our patient's livedo reticularis and telangiectasias could constitute a vascular predisposition for DDA, as its pathogenesis frequently involves an underlying disease involving ischemia, hypoxia, or hypercoagulability.

Initial clinical experience with ambulatory use of an implantable atrial defibrillator for conversion of atrial fibrillation. Metrix Investigators.

BACKGROUND: A recent study has shown that the implantable atrial defibrillator can restore sinus rhythm in patients with recurrent atrial fibrillation when therapy was delivered under physician observation. The objective of this study was to evaluate the safety and efficacy of ambulatory use of the implantable atrial defibrillator. METHODS AND RESULTS: An atrial defibrillator was implanted in 105 patients (75 men; mean age, 59+/-12 years) with recurrent, symptomatic, drug-refractory atrial fibrillation. After successful 3-month testing, patients could transition to ambulatory delivery of shock therapy. Patients completed questionnaires regarding shock therapy discomfort and therapy satisfaction using a 10-point visual-analog scale (1 represented "not at all," 10 represented "extremely") after each treated episode of atrial fibrillation. During a mean follow-up of 11.7 months, 48 of 105 patients satisfied criteria for transition and received therapy for 275 episodes of atrial fibrillation. Overall shock therapy efficacy was 90% with 1.6+/-1.2 shocks delivered per episode (median, 1). Patients rated shock discomfort as 5.2+/-2.4 for successful therapy and 4.2+/-2.2 for unsuccessful therapy (P:>0.05). The satisfaction score was higher for successful versus unsuccessful therapy (3.4+/-3. 3 versus 8.7+/-1.3, P:<0.05). There was no ventricular proarrhythmia observed throughout the course of this study. CONCLUSIONS: Ambulatory use of an implantable atrial defibrillator can safely and successfully convert most episodes of atrial fibrillation, often requiring only a single shock. Successful therapy is associated with high satisfaction and only moderate discomfort.

Ventricular fibrillation in six adults without overt heart disease.

Findings are described in six patients with no clinical evidence of heart disease who had documented ventricular fibrillation (five patients) or ventricular flutter (one patient). The mean age of the six patients, all men, was 34 years (range 26 to 43). Cardiovascular collapse occurred in all and was followed by successful cardioversion. No patient had electrolyte or QT abnormalities. One patient had slight right ventricular enlargement on M-mode echocardiography, and another had a left ventricular pressure gradient at rest of 30 mm Hg with a normal two-dimensional echocardiogram. Holter electrocardiographic monitoring revealed incessant ventricular tachycardia in one patient and nonsustained ventricular tachycardia in three others. Exercise testing revealed nonsustained ventricular tachycardia in one patient. Ventricular fibrillation was induced at the time of programmed electrical stimulation in four of the six patients. Documented recurrence of ventricular fibrillation or ventricular flutter occurred in three patients, but in only one patient receiving antiarrhythmic drugs. Four patients were treated with amiodarone and one received an automatic implantable cardioverter-defibrillator. All patients are alive after a mean follow-up period of 78 months after the first documentation of their arrhythmia and 37 months after programmed electrical stimulation. Ventricular fibrillation can occur in the apparently structurally normal human heart. Antiarrhythmic treatment can provide effective control of this malignant arrhythmia.

Q waves in hypertrophic cardiomyopathy in relation to the distribution and severity of right and left ventricular hypertrophy.

The cause of abnormal Q waves in hypertrophic cardiomyopathy remains unclear. Myocardial wall thickness was assessed by two-dimensional echocardiography at 8 left ventricular and 10 right ventricular sites in 67 patients (mean age 40 years) with hypertrophic cardiomyopathy and the findings were analyzed in relation to the presence of abnormal Q waves on the 12 lead rest electrocardiogram (ECG). Nineteen (28%) of the 67 patients had abnormal Q waves. Right ventricular hypertrophy was significantly more common in patients without abnormal Q waves (25 [52%] of 48 versus 2 [11%] of 19, p less than 0.001). With univariate analysis, there were six measurements that were significantly associated with abnormal Q waves: an increase in upper anterior septal thickness (p less than 0.005) and maximal left ventricular wall thickness (p less than 0.02), a decrease in mean and maximal right ventricular wall thickness (both p less than 0.005) and an increase in the ratio of both upper anterior septal to mean right ventricular wall thickness (p less than 0.005) and upper anterior septal to upper posterior wall thickness (p less than 0.005). With multivariate analysis, only the ratios of upper anterior septal to mean right ventricular wall thickness (p less than 0.005) and to upper posterior wall thickness (p less than 0.05) were significantly related to the presence of abnormal Q waves and predicted Q wave location with a sensitivity, specificity and predictive accuracy of 90%, 88% and 89%, respectively. In hypertrophic cardiomyopathy, the presence of abnormal Q waves on the 12 lead ECG is primarily a function of the relation of right ventricular wall thickness and upper anterior septal thickness.

Clinical significance of giant negative T waves in hypertrophic cardiomyopathy.

To assess the clinical significance of "giant" negative T waves in patients with hypertrophic cardiomyopathy from Western nations, clinical, echocardiographic, radionuclide and 48 h electrocardiographic (ECG) monitoring findings were compared in 27 patients with and 56 patients without giant negative T waves. Patients with giant negative T waves were older at diagnosis (43 +/- 15 versus 32 +/- 14 years, p less than 0.005), had greater ECG voltage (SV1 + RV5 = 57 +/- 20 versus 37 +/- 18 mm, p less than 0.001) and had a more vertical frontal plane axis (38.4 +/- 34 versus 13.4 +/- 45 degrees, p less than 0.05). Left ventricular wall thickness on two-dimensional echocardiography was similar at the mitral valve level (mean 16.5 +/- 4 versus 16.6 +/- 3 cm), but was greater at the papillary muscle level (mean 20.7 +/- 5 versus 17.6 +/- 3 mm, p less than 0.01) and apex (mean 23.3 +/- 5 versus 17.3 +/- 3 mm, p less than 0.001) in patients with giant negative T waves. Fewer patients with giant negative T waves had asymmetric septal hypertrophy (12 [44%] of 27 versus 36 [64%] of 56, p = 0.08) or systolic anterior motion of the mitral valve (4 [14%] of 27 versus 25 [45%] of 56, p less than 0.01), whereas left ventricular end-diastolic (44.1 +/- 6 versus 39.6 +/- 5 mm, p = 0.01) and end-systolic dimensions (27.8 +/- 4 versus 24 +/- 6 mm, p less than 0.05) were greater in this group. Nonsustained ventricular tachycardia was seen on ECG monitoring in 21% of patients in both groups.(ABSTRACT TRUNCATED AT 250 WORDS)

Nonischemic sustained ventricular tachycardia: clinical outcome in 12 patients with arrhythmogenic right ventricular dysplasia.

The clinical course and long-term follow-up of 12 patients with arrhythmogenic right ventricular dysplasia causing ventricular tachycardia are presented. No patient had a history of congestive heart failure and the cardiothoracic ratio measured less than or equal to 0.5 in all patients. All 12 patients were symptomatic during ventricular tachycardia; syncope occurred in 4. Exercise-related symptoms were present in 8 (73%) of 11 patients. The mean right ventricular ejection fraction was 31% (range 20% to 54%), and the mean left ventricular ejection fraction was 68% (range 44% to 88%). Signal averaging of the rest electrocardiogram (ECG) revealed late potentials in five of eight patients. During programmed electrical stimulation, sustained or nonsustained ventricular tachycardia showing a left bundle branch block configuration was induced in all patients. One patient underwent right ventricular disconnection and died 1 week after operation of low cardiac output failure. The remaining 11 patients were all treated medically and are alive at a mean follow-up time of 7.9 years after the onset of symptoms. Recurrence of symptomatic and documented sustained monomorphic ventricular tachycardia occurred in eight patients and could not be predicted by results of long-term ECG monitoring, treadmill exercise testing or programmed stimulation. In conclusion, despite recurrence of ventricular tachycardia, patients with arrhythmogenic right ventricular dysplasia have a favorable outcome when treated medically. Noninvasive studies (imaging techniques, ambulatory ECG monitoring and exercise testing) provide data that may be sufficient in diagnosing arrhythmogenic right ventricular dysplasia.

Atrial fibrillation in patients with an accessory pathway: importance of the conduction properties of the accessory pathway.

To investigate how the electrophysiologic properties of the accessory pathway affect the occurrence of atrial fibrillation in the Wolff-Parkinson-White syndrome, programmed stimulation data of 57 patients with overt pre-excitation and 33 patients with a concealed accessory pathway with documented circus movement tachycardia were reviewed. Atrial fibrillation had occurred spontaneously in 31 (54%) of the 57 patients with the Wolff-Parkinson-White syndrome and in 1 (3%) of the 33 with a concealed accessory pathway (p less than 0.001). Sustained atrial fibrillation was induced in 23 of 31 patients with the Wolff-Parkinson-White syndrome and spontaneous atrial fibrillation (Group A), in 7 of 26 patients with the Wolff-Parkinson-White syndrome without spontaneous atrial fibrillation (Group B) and in 5 of 33 patients with a concealed accessory pathway (Group C). The anterograde effective refractory period of the accessory pathway was shorter in Group A than in Group B (252 versus 297 ms, p less than 0.001). There were no differences among groups in PA interval, right to left atrium conduction time, cycle length of tachycardia and atrial and retrograde accessory pathway effective refractory period. Atrial fibrillation is more frequent in patients with the Wolff-Parkinson-White syndrome than in those with a concealed accessory pathway. Patients with overt pre-excitation and atrial fibrillation have a shorter anterograde accessory pathway refractory period. It seems therefore that the anterograde rather than the retrograde conduction properties of the accessory pathway are the critical determinants of atrial fibrillation in the Wolff-Parkinson-White syndrome.

Antiarrhythmic drug therapy in the Multicenter UnSustained Tachycardia Trial (MUSTT): drug testing and as-treated analysis.

OBJECTIVES: Using data from the Multicenter UnSustained Tachycardia Trial (MUSTT), we examined the factors used to select antiarrhythmic drug therapy and their impact on outcomes. BACKGROUND: The MUSTT examined the use of programmed ventricular stimulation (PVS) to guide antiarrhythmic therapy in patients with coronary arteriosclerosis, left ventricular dysfunction and asymptomatic, unsustained ventricular tachycardia (VT). Trial outcomes may reflect factors used to select antiarrhythmic drug therapy. METHODS: We compared subgroups of patients with inducible sustained VT randomized to PVS-guided antiarrhythmic therapy (n = 351), in particular those receiving PVS-guided antiarrhythmic drug therapy (n = 142) versus no antiarrhythmic therapy (controls, n = 353). RESULTS: "Effective" antiarrhythmic drug therapy (i.e., the term "effective" was used to denote therapy that resulted in noninducible VT or hemodynamically stable induced VT) was found for 142 of the 351 patients (43%), most often at the first or second PVS session (125/142, 88%). Mortality among the 142 patients did not differ from that among control patients. Of these 142 patients, the PVS end point was noninducibility in 91 patients and stable VT in 51 patients. Mortality did not differ between these two groups either, but arrhythmia was numerically more frequent in the PVS-induced stable VT group. Mortality was greatest in the few patients receiving propafenone (unadjusted p = 0.07, adjusted p = 0.14 vs. controls), but mortality with all agents did not differ from that of controls, even after adjustment. CONCLUSIONS: Even when presenting the results as favorably as possible, we found no benefit with PVS-guided drug therapy in patients with clinical unsustained VT who had inducible sustained VT. These findings are unaltered by using different end points for PVS or considering the response to individual drugs.

Prognosis in rupture of the ventricular septum after acute myocardial infarction and role of early surgical intervention.

Since 1944, 91 patients (50 men and 41 women, mean age 68 years [range 39 to 86]) with ventricular septal rupture after acute myocardial infarction were seen at the Mayo Clinic. Patients were divided into 4 groups according to therapy and timing of surgical intervention. Fourteen patients seen before 1965, when surgery was not performed for such a complication or not readily available, were excluded from the analysis. Group 1 (n = 22) had surgery within 48 hours of septal rupture, group 2 (n = 6) underwent operation between 2 and 14 days, group 3 (n = 24) had surgery after 14 days, and group 4 (n = 25) only received medical treatment. Short-term (30 days) survivors (45%, 35 of 77 patients) were compared with nonsurvivors. Using logistic regression, by univariate analysis, 3 variables were significantly associated with outcome: age (p less than 0.01), cardiogenic shock (p less than 0.00001), and long delay between ventricular septal rupture and surgical intervention (p less than 0.004). By multivariate analysis, however, only cardiogenic shock (p less than 0.00001) and age (p less than 0.007) correlated with an adverse outcome. In patients with cardiogenic shock after septal rupture, the prognosis was uniformly fatal unless patients undergo early surgery. None of the 23 patients in groups 2, 3 or 4 survived, whereas 5 of 13 patients (38%) who had surgery within 48 hours of septal rupture survived.(ABSTRACT TRUNCATED AT 250 WORDS)

Cost savings with nonthoracotomy implantable cardioverter-defibrillators.

We analyzed hospital and physician charges for 99 consecutive patients who underwent implantable cardioverter-defibrillator (ICD) implantation at our institution. Eighteen patients received an epicardial lead system and 81 were scheduled to receive a nonthoracotomy lead system, the generator being implanted either abdominally (n = 62) or pectorally (n = 19). The epicardial group had a significantly longer convalescent stay (11.6 +/- 2.5 days; mean +/- SEM) than the abdominal nonthoracotomy group, analyzed by intention to treat (4.6 +/- 0.5 days) or by treatment received (3.8 +/- 0.2 days; p <0.0001). Postoperative stay for the pectoral group was shorter still (2.9 +/- 0.4 days; p <0.033). Total charges for the epicardial group were $99,081 +/- $25,094, significantly higher than those for any of the nonthoracotomy groups (p <0.017). Total charges for the pectoral group were $44,128 +/- $2,465, significantly less than those for the abdominal nonthoracotomy group, analyzed by intention to treat ($59,961 +/- $1,369; p <0.05) or by treatment received ($56,679 +/- $635; p <0.05). Cost reductions in the nonthoracotomy groups were primarily due to decreased in-hospital convalescence period, lower surgeon and anesthesiologist fees, and lower procedure-day hospital charges in the pectoral group. The use of ICDs with nonthoracotomy leads can result in significantly shorter in-hospital convalescence and a reduction in total implant-related charges of 40% to 55%. The use of pectorally implanted ICDs results in further reduction in hospital stay and further cost reduction of 22% to 26%. The trend toward shorter convalescent stay without postimplant testing is likely to reduce further the overall costs of ICD implantation.

A transcardiac lead system for identification and termination of supraventricular and ventricular tachycardia.

The value of a transcardiac lead system (coronary sinus to right ventricular apex) to record atrial and ventricular electrical activity and its pacing capabilities was assessed in 20 patients with a variety of tachycardias (atrial tachycardia in 3 patients, atrial flutter in 4, intranodal tachycardia in 6, circus movement tachycardia using an accessory pathway in 1 patient, and ventricular tachycardia in 9). The transcardiac lead invariably showed both atrial and ventricular electrical activity during sinus rhythm and tachycardias, allowing application of the same criteria as used when analyzing cardiac rhythm on the surface electrocardiogram. Atrial complexes had a mean amplitude of 4.2 mV during sinus rhythm and varied from 3.0 to 4.1 mV during the different types of tachycardia. Ventricular complexes had a mean amplitude of 9.8 mV during sinus rhythm, 13.8 mV during supraventricular tachycardia and 16.1 mV during ventricular tachycardia. The duration of the QRS complex on the transcardiac lead was equal to the duration of the QRS complex on the surface electrocardiogram during tachycardias with a small or wide QRS complex. By varying the intensity of current delivered through the transcardiac lead, only right ventricular pacing (mean current intensity 1.2 +/- 0.4 mA) or simultaneous atrioventricular pacing (mean current intensity 4.7 +/- 3.3 mA) could be achieved. Termination of all episodes of tachycardia was achieved with either ventricular pacing or simultaneous atrioventricular pacing. This transcardiac lead system allows clear identification of atrial and ventricular events, is suitable for tachycardia analysis using simple surface electrocardiographic algorithms and allows pacing termination of a variety of tachycardias.

Evaluation of outpatient initiation of antiarrhythmic drug therapy in patients reverting to sinus rhythm after an episode of atrial fibrillation.

The safety of a method of outpatient antiarrhythmic drug loading utilizing a continuous loop event recorder was evaluated. The findings suggest that the standard 2-day hospital admission for drug loading is not necessary in all patients and a method of outpatient loading may be equally safe.

Catheter ablation using radiofrequency or low-energy direct current in pediatric patients with the Wolff-Parkinson-White syndrome.

Percutaneous ablation of accessory pathways was performed in 22 consecutive children and adolescents (9 boys and 13 girls, age range 8 to 18 years). Low-energy direct current (DC) was used exclusively in the first 6 patients, whereas ablation was performed with radiofrequency energy in the following 16. Accessory pathways were located in the left free wall in 15 patients, were posteroseptal in 3, were in the right free wall in 3 and were anteroseptal in 1. A concealed accessory pathway was present in 7 patients (32%). There was no significant difference in clinical or electrophysiologic variables between both groups. Catheter ablation was successful in the initial 6 patients using low-energy DC, as compared with 13 of 16 patients using radiofrequency ablation. Low-energy DC was successful as a backup power source in all 3 patients who had unsuccessful radiofrequency ablation. There was no complication. The median procedural and fluoroscopic times for successful ablation were 2.5 hours and 49 minutes, respectively (p = NS between both power sources). Accessory pathway conduction recurred in 2 patients (33%) who had low-energy DC as compared with 1 (6%) who had radiofrequency ablation (p = NS). These 3 patients had successful reablation of their accessory pathways. In children and adolescents with accessory pathways, both new power sources compare favorably, with an overall success rate of ablation of 100% (22 of 22 patients). Radiofrequency ablation should be used initially because it does not require general anesthesia and is associated with a lower rate of recurrence of accessory pathway conduction.(ABSTRACT TRUNCATED AT 250 WORDS)

Cardiac sarcoidosis: a potentially treatable form of myocarditis.

A 39-year-old woman had a 2-year history of heart block, which had necessitated pacemaker implantation, and a 6-month history of heart failure. Endomyocardial biopsy specimens initially revealed lymphocytic myocarditis but subsequently showed giant cell myocarditis. She died suddenly, and autopsy disclosed extensive cardiac sarcoidosis with minimal extracardiac involvement. Cardiac sarcoidosis may be difficult to diagnose clinically because the extent of cardiac and the extent of extracardiac involvement tend to be inversely related. Endomyocardial biopsy may be helpful in diagnosing such cases.

Reverse atrial electrical remodelling following atrial defibrillation as determined by signal-averaged ECG.

BACKGROUND: Following successful cardioversion for atrial fibrillation (AF), the rate of early recurrence remains high. Analysis of the signal-averaged electrocardiogram of the P wave has been proposed as a noninvasive method of predicting those at risk of recurrence. PURPOSE: To determine the change in signal-averaged P wave duration (SAPWD) following cardioversion from AF, and to determine whether SAPWD is associated with the risk of recurrence. METHODS: SAPWD was determined in 76 patients immediately following successful electrical cardioversion and three days later. Patients were then followed clinically for one year. RESULTS: Recurrent AF was observed in 32 of 76 patients at 90 days following cardioversion. There was no difference in SAPWD immediately following cardioversion (158+/-28 ms versus 164+/-31 ms, P=NS) or three days following cardioversion (152+/-24 ms versus 158+/-36 ms, P=0.4) in patients with and without recurrent AF. There was, however, a significant decrease in the SAPWD during the first three days following cardioversion in the patients who remained in sinus rhythm (158+/-28 ms initially versus 152+/-24 ms on day three, P=0.009). Among the patients with recurrent AF, the decrease was smaller and not statistically significant (161+/-30 ms versus 158+/-36 ms, P=0.3). CONCLUSION: Shortening of the SAPWD occurs following atrial defibrillation in patients who maintain sinus rhythm at 90 days. This provides evidence for reverse atrial electrical remodelling and its association with the maintenance of sinus rhythm.

Programmed ventricular stimulation during variant angina: report of a case.

Programmed ventricular stimulation was performed in a 74-year-old patient who had a history of syncope following chest pain. In the baseline state, ventricular tachycardia was not inducible. Immediately following the study protocol, the patient complained of her usual chest pain and ST elevation was documented in lead II with reciprocal ST depression in leads AVF and V1. Programmed ventricular stimulation was repeated (presumably during the occlusive phase of coronary spasm) and a polymorphic ventricular tachycardia with a cycle length of 200 msec was repeatedly induced. Following intravenous nitroglycerin and resolution of chest pain, ventricular tachycardia was not inducible. Coronary angiography with ergonovine testing confirmed coronary spasm of the right coronary artery. We speculate that syncope was caused by ventricular tachycardia following coronary artery spasm. During a 12-month follow-up with calcium blockers and nitrates, there has been no recurrence of chest pain or syncope.