Primary cardiomyopathy in identical twins.

Seventeen year old identical twin brothers with no family history of cardiopathy began experiencing palpitations almost simultaneously. In both, examination revealed marked cardiomegaly and hypokinesia of the ventricular walls, and clinical and radiologic signs of progressive cardiac failure developed a few days later. Both boys died suddenly, 49 days and 5 months, respectively, after the initial examination. Electrocardiographic and vectorcardiographic studies revealed a severe intraventricular conduction disturbance that coincided with histologic changes in the myocardial tissue, including profuse interstitial fibrosis, hypertrophy and degeneration of the myocardial fibers, aberrant arrangement of the muscular fibers and considerable alteration of the structure of cardiac tissue. In the absence of hereditary and chromosomal factors, and excluding possible viral intervention during fetal life, it is believed that a teratogenic factor can produce the structural alterations of the tissue and derangement of the fibers observed in these hearts. The irregular contractions of the heart at the level of the net-like meshwork, disarrangement of myocardial fibers, and adaptative mechanisms of the heart inherent in the destruction of the contractile tissue contributed to the functional cardiac disorders that resulted in congestive heart failure and sudden death in these twins.

Paradoxical behavior of the QT interval during exercise and recovery and its relationship with cardiac memory.

BACKGROUND: Few studies have dealt with the behavior of the corrected (QTc) and uncorrected QT intervals during exercise and recovery. HYPOTHESIS: Based on previously published dynamics of the QT interval during treadmill testing, this study attempted to reevaluate the computer-proposed underlying mechanisms of these dynamics and to determine whether the so-called memory phenomenon could be operative in some subjects without evidence of structural heart disease. METHODS: This study included 42 unmedicated healthcare volunteers, 23 men and 19 women aged between 20 and 42 (mean 31.7) years. All had normal physical examinations, x-rays, and transthoracic echocardiograms. The electrocardiograms were also normal with 12-lead QT interval dispersions of < 90 ms. RESULTS: During exercise and recovery, the behavior of the QT intervals permitted the categorization into two groups. In Group 1 (31/42; 73.8% of subjects) the uncorrected QT interval showed a biphasic pattern consisting of a gradual decrease during incremental exercise followed by a gradual increase during recovery. In contrast, the QTc interval had a triphasic pattern resulting from a slight increase during the early phase of exercise, a gradual decrease at the highest rates, and a final increase during recovery as the rate slowed to control values. In Group 2 (11/42; 36.2% of subjects) the behavior was considered as paradoxical since the uncorrected QT interval displayed in a triphasic pattern whereas the QTc interval yielded a tetraphasic pattern due to the fact that both showed a second decrease during recovery while the rate was decreasing. CONCLUSIONS: Analysis of dynamics behavior of the QTc and the uncorrected QT intervals during exercise showed that some normal subjects had a paradoxical behavior which, because of its temporal relation to the phases of exercise, could be an expression of the so-called memory phenomenon.

[The usefulness of the application of nonlinear dynamics in the analysis of electrocardiograms in Chagas' disease patients]. / Utilidad de la aplicación de técnicas de modelado no lineal en el análisis de electrocardiogramas de pacientes con infección chagásica.

INTRODUCTION AND OBJECTIVES: The application of nonlinear techniques allows the definition of early risk markers in patients with Chagas infection and without any evidence of cardiac involvement evaluated by standard diagnostic test. Nonlinear modeling techniques have proved to be effective in cardiac rhythm analysis, thereby justifying its use in Chagas' disease. PATIENTS AND METHOD: The routine noninvasive test and heart rate variability analysis were performed in Chagas' disease patients and in a group of healthy subjects. In a second phase we used nonlinear analysis in the evaluation of patients with Chagas infection and no evidence of heart disease, Chagasic patients with minimal electrocardiographic abnormalities and healthy controls. RESULTS: Twenty-four-hour electrocardiographic ambulatory monitoring and heart rate variability allowed us to establish differences between the healthy subjects and patients with Chagas infection without evidence of cardiac disease (p c 0.05 and p <0.005). In sharp contrast nonlinear analysis characterized 4 subgroups in Chagasic patients without cardiac involvement (sensitivity and specificity of 1 00%). CONCLUSIONS: Our findings suggest that nonlinear modeling techniques have a high sensitivity and specificity in the early detection of cardiac involvement and very early autonomic disturbance. We recommend that these techniques be applied to patients with high risk of cardiac disease other than Chagasic myocarditis. Our findings should be corroborated with studies in larger populations. We are currently developing a prospective study to this end.

Microelectrode study of high grade block in canine Purkinje fibers.

Previous reports from this laboratory described an electrotonic mechanism for simple impulse transmission through blocked segments of canine Purkinje tissue with slow diastolic depolarization assuming a vital role in second degree block. Utilizing an electrical blocking current, a blocked segment of canine Purkinje tissue was produced. Transmembrane events were recorded from blocked segments during higher grades of block (3:1 to complete) to delineate further mechanisms responsible for a periodic distal boundary response. Our results confirm that slow diastolic depolarization is an important determinant in sustaining periodic impulse conduction. Its importance is related to (1) progressive decrease of the resting membrane potential toward threshold at the distal block boundary, and (2) augmentation of the transmitted electrotonic potential in accordance with voltage dependent changes in membrane resistance. These data further lend definition to the distinction between electrotonic, partially active, and active transmembrane potentials. Impulse transmission through a segment of inactivated tissue is electrotonic and slow diastolic depolarization plays an important role in the maintenance of periodic impulse transmission.