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Eight patients (different families) with syncopal attacks and a long QT interval in the ECG died suddenly. Five heard normally and three were born deaf. At postmortem examination of all eight hearts the single consistent abnormality was focal neuritis and neural degeneration within the sinus node, A-V node, His bundle and ventricular myocardium. Although the etiology of this intracardiac neural disease is uncertain, a chronic viral infection or some noninfectious degenerative process are among the plausible causes discussed. If intracardiac neuritis and neural degeneration prove to be a prevalent finding among other victims dying from the long QT syndromes, further consideration should be given to whether there is any genuine hereditary component in the pathogenesis. Because of the asymmetrical and focal distribution of the cardioneural lesions, the response to present forms of medical or surgical treatment of the lung QT syndromes may vary from benefit to harm. Until more is known of the true etiology of the neural disease, treatment will probably remain empirical in nature and should be conducted with cautious clinical observation.

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A method is described of producing pulmonary embolism in the dog by the use of various-sized autologous clots made radiopaque. This method of experimental pulmonary embolism results in dilatation of the large- to small-sized pulmonary arteries and a consistent hemodynamic response in the pulmonary circulation, characterized by a marked rise in pulmonary arteriolar resistance with increased pulmonary arterial pressure but persistently normal pulmonary venous and "capillary" pressures. Data are presented to support the concept that the pulmonary hemodynamic changes that result from an initial pulmonary thromboembolism are primarily the result of vasoconstriction of the small pulmonary arteries or arterioles, presumably by a reflex mechanism. Mechanical blockage of the pulmonary vascular bed is considered to be of importance in causing hemodynamic alterations only after massive or repeated pulmonary embolism. The increase in pulmonary arteriolar resistance that results from the vasomotor response to pulmonary embolism is transient, whereas that due to mechanical blockage results in sustained pulmonary hypertension. Systemic arterial pressure does not show a consistent response to pulmonary embolism and could not be related to the hemodynamic alterations that occurred in the pulmonary circulation. Severe systemic hypotension resulted unpredictably but was more frequent after repeated or extensive pulmonary embolism. The systemic hypotensive reaction to pulmonary embolism is only in part due to a decreased cardiac output. The more severe responses are best explained on the basis of a vasomotor reflex producing a decrease in systemic arterial resistance. The two mechanisms of death in this experimental series were severe systemic hypotension with development of fatal ventricular arrhythmias and, almost as frequent, pulmonary edema that was characterized by severe pulmonary hypertension in the presence of a normal pulmonary venous pressure. There are many similarities between the experimental findings of this study and the clinical event of human pulmonary thromboembolism. Attention is drawn to the need for further investigation of the mechanism of the systemic arterial pressure response that often causes death and appears to be independent of the magnitude of the pulmonary hemodynamic changes.

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