A Case of Multiple Ectopic Pheochromocytomas Occurring in the Urinary Bladder and the Para-Aortic Region

A patient with urinary bladder pheochromocytoma and anotherpheo-chromocytoma in the para-aortic region is presented. Also,nine cases which have already been reported in Japan are reviewed. The following conclusions are made: bladder pheochromocytomacan appear at any age in either sex. The clinical triad consistingof hypertension, gross intermittent or transient painless hematuriaand micturitional attacks typical of pheochromocytoma are seenin most cases. Attention is called to such urinary bladder     

Pseudomyxoma Peritonei with High Carcinoembryonic Antigen (CEA) Level: Report of Two Cases

Two cases of pseudomyxoma peritonei with high serum carcinoembryonicantigen (CEA) are reported. CEA levels in serum increased incorrelation with the accumulation of ascites. Systemic chemotherapywith anticancer agents such as 5-fluorouraciI (5-FU), cyclophosphamide,mitomycin C (MMC) and chromomycin A_a showed no effect on reducingeither the production of mucinous materials or the CEA level,but repeated intraperi-toneal instillation of large amountsof MMC reduced them in one case. In the other case, the CEAlevel returned to normal after removal of the tumor. These resultssuggest that CEA may be a useful indicator of the effect oftreatment and of the prognosis of pseudomyxoma peritonei.     

Radiofrequency Catheter Ablation of Concealed Atrioventricular Accessory Pathways Using a "Simultaneous Pacing Method"

The retrograde atrial potential at a successful ablation site is usually obscured by the wide and large ventricular potential during atrioventricular reentrant tachycardia or ventricular pacing, which makes it difficult to determine the appropriate ablation site for concealed accessory pathway. A pacing maneuver named the “simultaneous pacing method” is proposed herein to differentiate the retrograde atrial potential from the ventricular potential for a successful ablation of the concealed accessory pathway. Catheter ablation was performed in 12 patients with a single left free-wall concealed accessory pathway. The atrial insertion site was determined by the simultaneous pacing method in six patients (group I) and by ventricular pacing in six patients (group II), In the simultaneous pacing method, electrograms recorded during ventricular pacing in the earliest retrograde atrial activation site are a fusion of the ventricular potential and the following retrograde atrial potential. When atrial and ventricular pacings are performed simultaneously (simultaneous pacing), the end portion of the electrograms recorded at the same site is solely the ventricular component, because atrial is activated earlier. The atrial potential can be confirmed during ventricular pacing in comparison with the electrograms during the “simultaneous pacing.” Radiofrequency catheter ablation was successful in eliminating conduction through the accessory pathway in all 12 patients. The radiofrequency applications in group I were significantly fewer than those in group II (1.7 ± 1.0 in group I, 5.3 ± 3.2 in group II, P < 0.05). The total procedure time in group I was significantly shorter than in group II (57.8± 15.7 vs 106.7 ± 41.6 mins in group II. respectively, P < 0.05). The fluoroscopy time in group I was significantly shorter than in group II (54.0 ± 7.9 vs 81.3± 26.3 mins, respectively, P < O.05). We were able to determine the atrial insertion site of accessory pathways by the simultaneous pacing method. The simultaneous pacing method was useful in eliminating concealed left free-wall accessory pathways.     

Significance of Ventricular Pacing Site in Manifest Entrainment During Orthodromic Atrioventricular Reentrant Tachycardia with Left-Sided Accessory Pathway

We examined entrainment by ventricular pacing in six patients during orthodromic atrioventricular reentrant tachycardia (AVRT) utilizing a left-sided lateral accessory pathway. Constant fusion and progressive fusion were demonstrated in all patients by left ventricular pacing during tachycardia, but in none of the patients by right ventricular pacing. When left ventricular pacing was performed during AVRT, the antidromic wave front from the pacing impulse (n) collided with the orthodromic wave front of the previous pacing beat (n - 1) within the ventricle, therefore, constant fusion and progressive fusion were demonstrated in the surface electrocardiographic QRS complexes. On the other hand, when right ventricular pacing was performed during orthodromic AVRT, the antidromic wave front from the pacing impulse (n) collided with the orthodromic wave front of the previous paced beat (n - 1) within the normal atrioventricular pathway, and constant fusion and progressive fusion were therefore not demonstrated. These phenomena were explained by the relationship of the ventricular pacing site and the reentrant circuit. This study demonstrates the importance of the pacing site in manifest entrainment of orthodromic AVRT during ventricular pacing.     

Acute Effects of Different Atrial Pacing Sites in Patients with Atrial Fibrillation: Comparison of Single Site and Biatrial Pacing

It has been reported that a trial single site or biatrial pacing can suppress the occurrence of AF. However, its mechanism remains unclear. The study population included 32 patients with AF (n = 20: AF group), or without paroxysmal AF (n = 12: control group). The mechanism and efficacy of atrial pacing were investigated by electrophysiological studies to determine which was more effective for suppressing AF induction; single site pacing of the right atrial appendage (RAA) or distal coronary sinus (CS-d), or biatrial (simultaneous BAA and CS-d) pacing. In the AF group, AF inducibility was significantly higher with BAA extrastimulus during RAA (12/20; P < 0.0001) or biatrial paced drive (7/20; P < 0.01) than during CS-d paced drive (0/20). In the control group, AF was not induced at any site paced. In the AF group, the conduction delay and other parameters of atrial vulnerability significantly improved during CS-d paced drive. The atrial recovery time (ART) at RAA and CS-d was measured during each basic pacing mode. ART was defined as the sum of the activation time and refractory period, and the difference between ARTs at RAA and CS-d was calculated as the ART difference (ARTD). The ARTD was significantly longer during BAA pacing in the AF group than in control group (155.0 +/- 32.8 vs 128.8 +/- 32.9 ms, P < 0.05). In the AFgroup, ARTDs during biatrial (52.0 +/- 24.2 ms) and CS-d pacing (51.7 +/- 26.0 ms) were significantly shorter than ARTD during RAA pacing. The CS-d paced drive was more effective for suppressing AF induction than biatrial or RAA paced drive by alleviating conduction delay. CS-d and biatrial pacing significantly reduced ARTD compared with RAA pacing.     

Differential Response of QTU Interval to Exercise, Isoproterenol, and Atrial Pacing in Patients with Congenital Long QT Syndrome

Sympathetic stimulation is well known to contribute to the genesis of QTU prolongation and ventricular lachyarrhythmias in patients with congenital long QT syndrome. In this study, we performed exercise treadmill testing, isoproterenol infusion (1–2 μg/min), and right atrial pacing (cycle length 500 msec) in 11 patients with congenital long QT (LQT) syndrome (LQT group) and in 12 age- and sex-matched controls (control group). The responses of the corrected QT (QT_c; Bazett's method) interval and the TU wave complex tvere evaluated. The QT_c interval was prolonged from 482 ± 63 msec^1/2 to 548 ± 28 msec^1/2 by exercise in the LQT group (n = 11; P < 0.005), and this was associated with fusion of the T waves with enlarged U waves, whereas the QT_c interval did not increase with exercise in the control group (n = 12; 402 ± 19 msec^1/2 vs 409 ± 22 msec^1/2). The QT_c interval was also prolonged from 466 ± 50 msec^1/2 to 556 ± 33 msec^1/2 by isoproterenol in the LQT group (n = 7; P < 0.005) in association with morphological changes of the TU wave complex like those seen with exercise, whereas it was only slightly increased from 399 ± 10 msec^1/2 to 436 ± 13 msec^1/2 by isoproterenol in the control group (n = 77; P < 0.001). However, the QT_c interval did not increase with atrial pacing in the LQT group (n = 8; 476 ± 57 msec^1/2 vs 486 ± 59 msec^1/2), whereas it was slightly increased from 400 ± 21 msec^1/2 to 426 ± 18 msec^1/2 by atrial paring in (he control group (n = 8; P < 0.005). These results suggest that sympathetic stimulation plays an important role in the QTU prolongation and marked TU wave complex abnormalities in patients with congenital long QT syndrome.     

Peritoneoscopic Findings of Autoimmune Hepatitis

Abstract: To clarify the morphologic differences between hepatitis C virus (HCVI-negative autoimmune hepatitis (AIH) and HCV-positive AIH, peritoneoscopic findings were studied. Among twenty three patients with AIH according to the Japanese criteria (1992), 15 were HCV-negative and 8 were HCV-positive. The terms grooved depression, coarse depression, coarse elevation, coarse undulation, and round-shaped reddish marking (RM) were used in this study to evaluate the peritoneoscopic findings. Grooved depressions, coarse depressions, coarse elevations, coarse undulations and round-shaped RMs were all common findings (53%, 87%, 73%, 80%, and 80%, respectively) in HCV-negative AIH patients, but they were less common (13%, 25%, 13%, 13%, and 0%, respectively) in HCV-positive AIH patients. This study revealed that HCV-negative AIH patients had different peritoneoscopic findings from HCV-positive AIH patients. Thus HCV-negative AIH may be typical AIH, and HCV-positive AIH may essentially be a subset of type C chronic hepatitis.     

Mechanism of Double Ventricular Response to a Single Atrial Extrastimulus in Patients with Wolff-Parkinson-White Syndrome

The purpose of this study is to elucidate electrophysiological determinants of double ventricular response (DVR) to a single atrial extrastimulus in Wolff-Parkinson-White (WPW) syndrome. DVR was observed in 5 (3.4%) out of 146 consecutive patients with WPW syndrome. The site of accessory pathway was located in left lateral free wall in four patients and posterior septum in one. DVR was induced by extrastimulus from coronary sinus in four patients with left-sided accessory pathway, and from both coronary sinus and high right atrium in a patient with septal accessory pathway. However, it was not possible to induce DVR from high right atrium in patients with left-sided accessory pathway, because 50 to 80 ms are needed for intra-atrial conduction from high right atrium to coronary sinus. Critical prolongation of normal AV conduction allowing DVR was seen in the slow pathway of AV node in four patients. In the remaining patients requisite conduction delay occurred in both AV node and His-Purkinje system. Single right ventricular extrastimulus could easily elicit orthodromic AV reciprocating tachycardia or echo beat in four out of five patients and incremental ventricular stimulation induced it in the remaining patient, indicating the presence of retrograde block in the normal AV pathway. As requisites of DVR to a single atrial extrastimulus in WPW syndrome: (1) slow antegrade conduction and retrograde block in the normal AV pathway; and (2) stimulation site in the vicinity of accessory pathway, are needed.     

Epinephrine-Induced Ventricular Premature Complexes Due to Early Afterdepolarizations and Effects of Verapamil and Propranolol in a Patient with Congenital Long QT Syndrome

Epinephrine-Induced VPCs and EADs in Congenital LQTS. We report a patient with congenital long QT syndrome in whom early afterdcpolarizations (RAl)s) were demonstrated on monophasic action potential (MAP) recordings in the left ventricular mid-base inferior wall. Epinephrine infusion at 5 fig/mm increased the amplitude of the EADs and the late component of the T(U) wave. Epinephrine also induced ventricular premature complexes (VPCs) with right hundle branch block morphology and left-axis deviation that occurred from the peak of the EADs. Verapamil injection (5 nig) during continuous epinephrine infusion abolished all VPCs with a slight reduction in the amplitude of the EADs. Propranolol injection (5 mg) in addition to verapamil further reduced the amplitude of the EADs and the late component of the T(U) wave. These findings suggest that the epinephrinc-induced VPCs were closely related to triggered rhythm arising from the EADs, and that both verapumil and propranoloi were effective for the suppression of VPCs and EADs.     

Increased Dispersion of Repolarization Time Determined by Monophasic Action Potentials in Two Patients with Familial Idiopathic Ventricular Fibrillation

Increased Dispersion of RT in Familial Idiopathic VF. Introduction: The role of increased dispersion of repolarization in the genesis of torsades de pointes in patients with long QT syndrome has been clarified, but its role in the genesis of idiopathic ventricular fibrillation (VF) is not yet known. To investigate the pathogenesis of VF, we recorded monophasic action potentials (MAPs) from two siblings (48- and 36-year-old males) with familial idiopathic VF. Methods and Results: The elder brother (patient I) showed a late r’ wave in lead V¹ and ST segment elevation in leads V¹ through V³. The younger brother (patient 2) had late r’ waves and ST segment elevation in leads II, III, and aVF, and the configurations were very similar to those of patient I. MAPs were recorded from several sites in the right ventricular (RV) and left ventricular (LV) endocardium during constant right atrial pacing. The repolarization time (RT) was defined as the sum of the activation time (AT) and action potential duration (APD) at 90% repolarization. In patient 1, marked prolongation of the AT (140 msec) and the RT (380 msec) was recorded in the RV septum of the outflow tract, and the RT dispersion was markedly increased (125 msec). In contrast, patient 2 showed prolongation of the AT (80 msec) and RT (310 msec), and fractionated electrograms in the RV floor of the inflow tract. The RT dispersion was also increased (80 msec). VF and nonsustained polymorphic ventricular tachycardia were induced by double premature stimulation in patients 1 and 2, respectively. Chronic amiodarone therapy decreased the RT dispersion and suppressed the induction of ventricular tachyarrhythmias in patient 2, although late r’ waves and slight ST segment elevation were unmasked in leads V₁, and V₂. Conclusion: Our data suggest that the increased dispersion of the RT, which was due mainly to a localized conduction delay in the RV, created an arrhythmogenic substrate in the two patients with familial idiopathic VF.