Search

Pure acute Pandysautonomia (PAP), first described in 1969 by Young, is a rare but well defined clinical entity, in which the isolate involvement of Autonomic Nervous System (ANS) gives rise to a characteristic clinical admixture of symptoms and signs all due to the alteration of parasympathetic and sympathetic control of neurovegetative functions. The most prominent clinical features are: 1) postural hypotension without changes in heart rate leading to orthostatic syncopal or near-syncopal episodes, 2) abnormalities of intestinal tract and urinary bladder motility, leading to constipation alternating with diarrhea and urinary retention, 3) loss of sweating, 4) ocular abnormalities. We report a case of a 17 year old man with PAP, in whom almost complete resolution of clinical symptoms and signs of ANS involvement was observed within 9 months from the onset of illness. In our case, besides an accurate clinical and laboratory follow-up, particular attention was paid to investigate the cardiac responses to autonomic reflex tests and pharmacological interventions (atropine and beta-blocking drugs administration) during the entire course of illness. During the acute phase, complete vagal and almost complete beta-sympathetic "denervation" of the heart leading to disabling postural hypotension, fixed resting tachycardia and total unresponsiveness to Valsalva manoeuvre, were observed. PAP is a rare condition which must be known from cardiologists for a more complete knowledge of the etiology and pathophysiological mechanisms of postural syncopal attacks and must be distinguished from the less favourable progressive autonomic failure of the Shy-Drager syndrome.

Eight cases of top-ranking athletes with "repolarization disorders" are reported. All subjects were asymptomatic and were otherwise suited for excellent cardiovascular performances. Seven athletes did not show any evidence of heart disease. Seven had MVP (mitral valve prolapse). Umprompted variability of ECG tracings was observed in three cases. Both isoproterenol infusions (IS) and maximal physical effort (EX) normalized T wave (abnormalities in 100% of cases, while atropine (AT) was ineffective despite an increase in heart rate greater than that caused by IS. The authors emphasize the usefulness of combined use of the EX and IS tests in ascertaining the clinical significance of T wave changes in healthy athletes. A "neurogenic" mechanism is proposed by the authors for the pathogenesis of these T wave abnormalities. This hypothesis may explain the umprompted variability of ECG tracings and T wave normalization after maximal physical effort and isoproterenol infusion.

The occurrence of Wenckebach second-degree (Mobitz I) A-V block in apparently normal persons still provides a puzzle for the cardiologist, as the benign nature of this event has been recently questioned. This problem becomes more intriguing when Wenckebach A-V block is encountered in asymptomatic top-ranking athletes, because of medico-legal implications. We report 10 cases of highly-trained athletes, including three with mitral valve prolapse (MVP) features, with a spontaneous or induced Wenckebach second-degree A-V block. Previous ECGs of six subjects, dating from a maximum of 6 years to a minimum of 18 months, were available. Deterioration of A-V conduction has never been documented and all six cases have remained asymptomatic for the whole follow-up period. Athletes have been submitted to a protocol study consisting of ECG recording at rest, during, and after vagal and sympathetic reflex maneuvers, drug administration (isoproterenol and atropine), submaximal and maximal exercise. Nine subjects have been considered to have "normal" responses of the A-V node to provocative tests, since conduction disturbances were improved or normalized by reflex sympathetic stimulations and were completely normalized by autonomic drug administration and exercise. One athlete showed "abnormal" responses to tests. In order to give a conclusive prognostic and medico-legal assessment, we advised him to submit to an invasive electrophysiological investigation. Wenckebach second-degree A-V block in athletes may be a more common finding than so far described, especially when a systematic search is made. In our opinion, this event can still be considered a vagally-induced benign feature of athlete's heart, provided that an immediate improvement of A-V conduction is obtained in response to reflex sympathetic maneuvers, and that a complete normalization after sympathomimetic and vagolytic drug administration and physical exercise is observed. The clinical histories of our athletes and the observed complete disappearance of conduction disturbances after detraining, strongly support this opinion. Wenckebach second-degree A-V block in asymptomatic athletes with MVP features probably does not affect the prognosis if similar favorable responses to the aforesaid tests are observed.