Kunitake Hashiba

Takayasu's Arteritis Clinical Report of Eighty-four Cases and Immunological Studies of Seven Cases

The manifestations of Takayasus arteritis of the aorta were studied in 84 patients. The extent of the involvement of the aorta was classified on aortographic examination in 54 patients and from the clinical manifestations in 30. Involvement of the aorta was classified as: (1) arch type in 47 cases; (2) extensive type (whole aorta and its branches involved) in 27 cases; and (3) descending thoracic and abdominal type (only descending thoracic and abdominal aortas involved) in 10 cases. The three types resembled one another in clinical manifestations and laboratory findings except for ischemic signs which varied with the type of lesion and a slight difference in the ratio of male to female patients. Generalized, cardiac and pulmonary symptoms were noted by about two thirds of the patients in the early stage. About one third complained of local pain. The erythrocyte sedimentation rate and C-reactive protein were high values during the active stage of this disease. The hemagglutination test using tannic acid-treated erythrocytes was positive in five of seven cases. It is not clear yet that circulating anti-arterial antibodies are the direct cause of Takayasus arteritis.

Prolonged and fractionated right atrial electrograms during sinus rhythm in patients with paroxysmal atrial fibrillation and sick sinus node syndrome.

Intraatrial catheter mapping of the right atrium was performed during sinus rhythm in 92 patients: Group I = 43 control patients without paroxysmal atrial fibrillation or sick sinus node syndrome; Group II = 31 patients with paroxysmal atrial fibrillation but without sick sinus node syndrome; and Group III = 18 patients with both paroxysmal atrial fibrillation and sick sinus node syndrome. Atrial electrograms were recorded at 12 sites in the right atrium. The duration and number of fragmented deflections of the atrial electrograms were quantitatively measured. The mean duration and number of fragmented deflections of the 516 atrial electrograms in Group I were 74 +/- 11 ms and 3.9 +/- 1.3, respectively. The criteria for an abnormal atrial electrogram were defined as a duration of greater than or equal to 100 ms or eight or more fragmented deflections, or both. Abnormal atrial electrograms were observed in 10 patients (23.3%) in Group I, 21 patients (67.7%) in Group II and 15 patients (83.3%) in Group III (Group II versus Group I, p less than 0.001; Group III versus Group I, p less than 0.001). The mean number of abnormal electrograms per patient with an abnormal electrogram was 1.3 +/- 0.7 in Group I, 2.5 +/- 1.9 in Group II and 3.5 +/- 2.5 in Group III (Group I versus Group II, p less than 0.01; Group II versus Group III, p less than 0.05). A prolonged and fractionated atrial electrogram characteristic of paroxysmal atrial fibrillation can be closely related to the vulnerability of the atrial muscle.(ABSTRACT TRUNCATED AT 250 WORDS)

Patients with idiopathic cardiomyopathy belong to the same mitochondrial DNA gene family of Parkinson's disease and mitochondrial encephalomyopathy

Comparison of total mitochondrial DNA sequences of patients with idiopathic (deleted or hypertrophic) cardiomyopathy with those of patients with Parkinsons disease and mitochondrial encephalomyopathies revealed distinct clustering of point mutations among patients. Furthermore, an inverse relation was found between the total number of base-substitution and life span of the patients. Among point mutations found in each patient, sequentially diverged six clusters consisting of 14, 10, 7, 1, 2, and 3 mutations, respectively, were detected. Five sub-clusters consisting of 2, 2, 11, 1, and 1 mutations, respectively, were detected. From each cluster, the patients unique mutations were diverged with three types of the mutations specific for the disease. The divergence allowed construction of a phylogenetic tree which clearly indicated that patients with idiopathic cardiomyopathy belong to the same mitochondrial DNA gene family of Parkinsons disease and mitochondrial encephalomyopathies.

Impaired left ventricular filling in borderline hypertensive patients without cardiac structural changes

To determine the effect of borderline hypertension on left ventricular diastolic performance, 16 patients with borderline hypertension who did not have left ventricular hypertrophy and 16 age-sex-matched patients with normotension of similar age and body mass index were investigated. Pulsed Doppler echocardiography was used to record left ventricular filling signals at rest and immediately after supine ergometer exercise. All subjects had normal left ventricular structure and systolic function. At rest the borderline hypertension group in comparison with the normotension group had a depressed peak velocity of early filling (E) (44 +/- 7 vs 54 +/- 10 cm/sec; p < 0.01), no enhanced peak velocity of late filling (A) (52 +/- 8 vs 50 +/- 11 cm/sec; not significant), and a reduced E/A ratio (0.9 +/- 0.2 vs 1.1 +/- 0.3; p < 0.05). Atrial filling time and preejection period were similar in the two groups. The effect of exercise on left ventricular filling velocity in patients with borderline hypertensive resembled that in those with normotension. Percentage changes in E (+14% +/- 12% vs +14% +/- 13%) and A (+13% +/- 8% vs 11% +/- 12%) were equivalent, suggesting a preserved diastolic reserve for exercise in the borderline hypertension group. In conclusion, borderline hypertension appears to be predictive of early filling impairment, and a late filling compensative mechanism is not yet apparent. These findings in borderline hypertension without apparent cardiac damage call for clinical attention to prevent further deterioration in cardiac function.

Electrophysiologic properties of atrial muscle in paroxysmal atrial fibrillation

The electrophysiologic properties of atrial muscle were studied by programmed atrial stimulation in 42 patients with paroxysmal atrial fibrillation (PAF) and in 53 control patients without PAF. Single premature atrial stimulation was given at the right atrial appendage following 8 basic stimuli with a basic cycle length of 500 ms. Repetitive atrial firing (RAF) was defined as the occurrence of 2 or more successive premature atrial activations following single premature atrial stimulation. Fragmented atrial activity (FAA) was defined as an increase by more than 75% of the duration of the atrial electrogram in response to a single premature stimulation. Interatrial conduction delay was defined as an increase of the conduction time by more than 50 ms in response to a single premature stimulation. RAF was induced in 26 of 42 patients (61.9%) with PAF and in 14 of 53 control patients (26.4%). FAA and interatrial conduction delay were elicited in 69.0 and 80.9% of patients with PAF and in 34.0 and 56.6% of control patients, respectively. In 16 patients with PAF in whom RAF was not induced, FAA developed in 11 patients (68.8%). In 88.1% of 42 patients with PAF and in 41.5% of 53 controls, RAF or FAA, or both, were elicited by atrial premature stimulation. It is concluded that the incidence of RAF and FAA were significantly higher in patients with PAF than in the control group, and the induction of RAF or FAA, or both, was closely related to the vulnerability of the atrial muscle to atrial fibrillation.

Electrophysiological Abnormalities of the Atrial Muscle in Patients with Manifest Wolff-Parkinson-White Syndrome Associated with Paroxysmal Atrial Fibrillation

We investigated the electrophysiological properties of the atrial muscle in 33 patients with manifest Wolff‐Parkinson‐White syndrome. Group I consisted of 13 patients with paroxysmal atrial fibrillation and group II consisted of 20 patients without paroxysmal atrial fibrillation. The anterograde and retrograde effective refractory periods of the accessory pathway and the inducibility of atrioventricular reciprocating tachycardia were not significantly different between the two groups, Endocardial electrograms, obtained by right atrial catheter mapping, were recorded during sinus rhythm from 12 sites of the right atrium in 12 of the 13 group I patients and in all group II patients. An abnormal atrial electrogram was defined as 100 msec or longer in duration, and/or the occurrence of eight or more deflections. Ten (83%) of the 12 group I patients had abnormal atrial electrograms, while only two (10%) of the 20 group II patients had abnormal atrial electrograms, and the difference was significant (P < 0.01). Thirty‐six (26%) of the total 139 electrograms obtained from 12 group I patients and two (1%) of the total 199 electrograms obtained from 20 group II patients fulfilled the criteria for an abnormal atrial electrogram, and the difference was significant (P < 0.01). The fragmented atrial activity zone, interatrial conduction delay zone, and repetitive atrial firing zone obtained by right atrial extrastimulation were significantly wider in group I than in group II, respectively. It was concluded that electrical abnormalities of the atrial muscle may play an important role in the occurrence of paroxysmal atrial fibrillation in patients with Wolff‐Parkinson‐White syndrome.

Sex Differences in Phenotypic Manifestation and Gene Transmission in the Romano-Ward Syndrome

Hereditary QT prolongation syndrome is characterized by idiopathic prolongation, syncopal attack and sudden death in some cases due to severe ventricular tachycardias, and is divided into two major different types, Jervell and Lange-Nielsen syndrome and Romano-Ward syndrome, on a genetic basis and by the association with congenital deafness. The Romano-Ward syndrome is inherited in a simple autosomal dominant pattern and is not associated with congenital deafness. It is of interest to note that a kind of female predominance in clinical manifestations has been observed in the Romano-Ward syndrome,2 as I myself already first reported in 1978 on the basis of some of the clinical aspect^.^ If sex differences really exist, this should be considered a contradictory, or at least, a very peculiar characteristic for a simple non-sex-linked autosomal dominant trait. The purpose of the study presented here was to investigate the sex difference in the Romano-Ward syndrome using phenotypic manifestations and gene transmission in 16 families with whom we had experience for over 20 years.

Electrophysiological abnormalities of the atrial muscle in patients with sinus node dysfunction without tachyarrhythmias

The duration and the number of fragmented deflections of the right atrial electrograms were assessed and quantitatively measured in 74 patients who underwent endocardial catheter mapping during sinus rhythm. The bipolar electrograms were recorded at 12 sites in the right atrium. An abnormal atrial electrogram was defined as a duration of 100 ms or longer, and/or 8 or more fragmented deflections, according to our previous criteria. The patients were divided into two groups. The control group consisted of 41 age-matched patients with normal sinus node function and without paroxysmal atrial fibrillation. The study group comprised 33 patients with sinus node dysfunction but without tachyarrhythmias. Abnormal atrial electrograms were observed in 8 (19.5%) control patients, and in 16 (48.5%) sinus node dysfunction patients; p < 0.02. The total number of abnormal electrograms was 14 (2.89%) of 483 atrial electrograms in controls, and 36 (9.38%) of 384 in the study group; p < 0.0002. The mean duration (75.6 +/- 17) and the mean number of fragmented deflections (4.1 +/- 2) of the total atrial electrograms in the sinus node dysfunction group was significantly greater than that in controls (70.9 +/- 11 and 3.6 +/- 1, respectively); p < 0.01. The mean number of abnormal electrograms per patient in the study group (1.06 +/- 1.8) was significantly higher than that in the control group (0.3 +/- 0.8); p < 0.05. These data suggest that: (1) there is a significantly greater electrophysiological abnormality of the atrial muscle in patients with sinus node dysfunction but without paroxysmal atrial fibrillation than in age-matched controls, and (2) not only the sinus node but also the atrial muscle is electrophysiologically altered in patients with sinus node dysfunction but without tachyarrhythmias.

Asystole with syncope following strenuous exercise in a man without organic heart disease

A 29-year-old man without organic heart disease who had a history of six episodes of syncope following sudden cessation of strenuous exercise over the last 18 years was reported. Resting electrocardiogram and 24-hour electrocardiographic monitoring did not show any rhythm disturbance or ischemic changes. Sinus node recovery time, sinoatrial conduction time and A-V conduction were normal on electrophysiologic study. Asystole with syncope occurred immediately following sudden cessation of strenuous exercise and following release of intrathoracic pressure during Valsalvas maneuver performed after subcutaneous administration of 0.5 mg of epinephrine. However, Masters single two-step test and Valsalvas maneuver performed without epinephrine could not evoke asystole. It was assumed from these results that transient overactivity of the autonomic nervous system and sympathetic-parasympathetic interactions were the underlying factors responsible for the occurrence of asystole following sudden cessation of strenuous exercise in the present case.

Mechanism of the suppression of repetitive atrial firing by isoproterenol — comparison with disopyramide

To investigate whether isoproterenol (Iso) could suppress the initiation of repetitive atrial firing (RAF), we investigated its effect on RAF in comparison with that of disopyramide (Diso). Extrastimuli at a basic cycle length of 500 ms were delivered from the high right atrium in 49 patients who received an intravenous infusion of Iso (0.01 microgram/kg per min) and in 39 patients given intravenous Diso (2 mg/kg per 10 min). Induction of RAF, the atrial effective refractory period (A-ERP), and the maximum conduction delay (MCD) were measured. Iso abolished the induction of RAF in 13/19 (68%) patients, while Diso did so in 13/22 (59%) patients. Thirty-four of the 41 patients with RAF in the baseline study had an A-ERP < 250 ms and an MCD > 40 ms. Iso significantly decreased the A-ERP from 205 +/- 26 to 194 +/- 23 ms (P < 0.01) and significantly decreased the MCD from 67 +/- 24 to 39 +/- 16 ms (P < 0.0001) in 19 patients with RAF. On the other hand, Diso significantly increased the A-ERP from 203 +/- 31 to 235 +/- 36 ms (P < 0.0001), and significantly diminished the MCD from 68 +/- 31 to 55 +/- 30 ms (P < 0.01) in 22 patients with RAF. In patients with new RAF (n = 7) or re-induced RAF (n = 14) during Iso or after Diso, the MCD was more than 40 ms. Our results suggest that there are two different modes of RAF suppression, i.e. shortening or lengthening of the A-ERP.(ABSTRACT TRUNCATED AT 250 WORDS)