Familial amyloidosis with polyneuropathy (FAP) was first reported from Portugal in 1952, but since then this syndrome has been recognized in many countries including Sweden. In this investigation cardiac and pulmonary functions in the Swedish variety of FAP were studied. A retrospective survey of the ECG findings in 71 patients showed a high prevalence of atrioventricular (38%) and intraventricular (41%) conduction defects, and also a high prevalence of atrial fibrillation (14%). In several patients a progression in the conduction defects to advanced disturbances could be observed and 10 out of 71 patients (14%) in the present series required pacemaker treatment. A histopathological study of the atrioventricular part of the conduction system showed marked amyloid infiltration in each case, which may explain the high prevalence of conduction defects. In an échocardiographie study which emcompassed 22 consecutive patients, all but those two patients with the shortest duration of symptomatic disease showed abnormal features. The most frequent and characteristic findings were hypertrophy of the interventricular septum (86%) and a hyperrefractile appearance of the iryocardium (68%). This unusual association of échocardiographie features is considered almost diagnostic of cardiac amyloidosis. A hemodynamic study showed an essentially normal systolic heart function, but in several patients there were signs of impaired diastolic function with increased myocardial rigidity. Several patients showed signs of obstruction of the ventricular outflow tracts. This finding, as well as the échocardiographie features, is in accordance with altered anatomical and functional properties of the interventricular septum. The major pulmonary function abnormalities were decreased maximum respiratory pressure which indicate that the neuropathy in FAP involves the respiratory musculature, and impaired diffusing capacity consistent with an alveo-capillary block caused by amyloid deposits. / digitalisering@umu

Intracardiac pathways mediating the parasympathetic control of various cardiac functions are incompletely understood. Several intracardiac ganglia have been demonstrated to potently influence cardiac rate [the sinoatrial (SA) ganglion], atrioventricular (AV) conduction (the AV ganglion), or left ventricular contractility (the cranioventricular ganglion). However, there are numerous ganglia found throughout the heart whose functions are poorly characterized. One such ganglion, the posterior atrial (PA) ganglion, is found in a fat pad on the rostral dorsal surface of the right atrium. We have investigated the potential impact of this ganglion on cardiac rate and AV conduction. We report that microinjections of a ganglionic blocker into the PA ganglion significantly attenuates the negative chronotropic effects of vagal stimulation without significantly influencing negative dromotropic effects. Because prior evidence indicates that the PA ganglion does not project to the SA node, we neuroanatomically tested the hypothesis that the PA ganglion mediates its effect on cardiac rate through an interganglionic projection to the SA ganglion. Subsequent to micro-injections of the retrograde tracer fast blue into the SA ganglion, >70% of the retrogradely labeled neurons found within five intracardiac ganglia throughout the heart were observed in the PA ganglion. The neuroanatomic data further indicate that intraganglionic neuronal circuits are found within the SA ganglion. The present data support the hypothesis that two interacting cardiac centers, i.e., the SA and PA ganglia, mediate the peripheral parasympathetic control of cardiac rate. These data further support the emerging concept of an intrinsic cardiac nervous system.

La fibrilación auricular (FA) es una de las arritmias cardiacas más comunes, la cual afecta alrededor del 10% de la población de más de 70 años. En FA, los impulsos eléctricos auriculares generados por el nodo sinusal son sustituidos por impulsos eléctricos desorganizados. Esto esta asociado con un bombardeo irregular de activaciones auriculares hacia el nodo AV. Dado que el nodo AV no puede conducir todas estas activaciones, algunas de ellas son bloqueadas en el nodo. Esta propiedad de filtrado que tiene el nodo es fundamental para mantener el ritmo cardiaco en un rango compatible con la vida. Sin embargo, la respuesta ventricular durante FA presenta intervalos RR (tiempo entre dos activaciones) más cortos e irregulares que durante ritmo sinusal. Al ser el nodo AV la única estructura responsable para la conducción de los latidos auriculares hacia los ventrículos, las estrategias terapéuticas para controlar el ritmo cardiaco durante FA tratan de utilizar y ajustar las propiedades de conducción del nodo. Sin embargo, sigue sin estar suficientemente entendido el papel que dichas propiedades de conducción juegan para controlar y modular la respuesta ventricular durante FA. Durante el desarrollo de la presente tesis se han investigado en diferentes especies y con diversas técnicas algunas de las principales características de la conducción del nodo AV con la intención de aportar mayor conocimiento sobre esta intrigante estructura del corazón. Específicamente, se ha analizado uno de los fenómenos más enigmáticos de la respuesta ventricular durante FA: la aparición de patrones de respuesta ventricular multimodales al construir histogramas de RR obtenidos a partir de registros de larga duración. En la literatura se han sugerido diversas teorías que pudiesen explicar la aparición de estos múltiples intervalos RR predominantes. En el desarrollo de la presente disertación se mostrarán algunos resultados incompatibles con dichas teorías, razón por la cual se presenta y defiende una nueva hipótesis que sugiere que los intervalos RR predominantes están relacionados con el proceso fibrilatorio auricular. / Martínez Climent, BA. (2011). Characterization and Modeling of Atrioventricular Conduction during Atrial Fibrillation [Tesis doctoral]. Universitat Politècnica de València. https://doi.org/10.4995/Thesis/10251/10985