[Neurocardiogenic convulsive syncope--differential diagnosis, pathophysiology and therapy based on a case report]. / Die neurokardiogene konvulsive Synkope--Differentialdiagnose, Pathophysiologie und Therapie anhand eines Fallberichts.

Neurocardiogenic convulsive syncope as a disease at the border between cardiovascular and neurologic dysfunction can pose considerable diagnostic challenges. We report on the case of a 19 year-old female patient with recurrent neurocardiogenic convulsive syncope where the time from the onset of symptoms to the correct diagnosis and initiation of an effective therapy spanned more than three years. Based on this case report, we discuss differential diagnosis, pathophysiology and therapy of this disorder of autonomic cardiovascular regulation. Neurocardiogenic convulsive syncope should be considered whenever a patient has both syncope that exhibits a typical cardiovascular pattern (e.g., fainting of short duration with rapid reorientation phase) and prolonged loss of consciousness with characteristic neurological features (e.g., cerebral seizures with postictal state of confusion). Head-up tilt testing, introduced into clinical practice in 1986, is an efficient tool to diagnose neurocardiogenic syncope with comparatively high sensitivity in patients with recurrent syncope of unknown origin. Besides orthostatic training and pharmacotherapy, permanent dual-chamber cardiac pacing has gained increasing importance as treatment for cardioinhibitory forms of neurocardiogenic syncope.

Dynamic restitution of action potential duration during electrical alternans and ventricular fibrillation.

The restitution kinetics of action potential duration (APD) were investigated in paced canine Purkinje fibers (P; n = 9) and endocardial muscle (M; n = 9), in isolated, perfused canine left ventricles during ventricular fibrillation (VF; n = 4), and in endocardial muscle paced at VF cycle lengths (simulated VF; n = 4). Restitution was assessed with the use of two protocols: delivery of a single extrastimulus after a train of stimuli at cycle length = 300 ms (standard protocol), and fixed pacing at short cycle lengths (100-300 ms) that induced APD alternans (dynamic protocol). The dynamic protocol yielded a monotone increasing restitution function with a maximal slope of 1.13 +/- 0.13 in M and 1.14 +/- 0.17 in P. Iteration of this function reproduced the APD dynamics found experimentally, including persistent APD alternans. In contrast, the standard protocol yielded a restitution relation with a maximal slope of 0.57 +/- 0.18 in M and 0.84 +/- 0.20 in P, and iteration of this function did not reproduce the APD dynamics. During VF, the restitution kinetics at short diastolic interval were similar to those determined with the dynamic protocol (maximal slope: 1.72 +/- 0.47 in VF and 1.44 +/- 0.49 in simulated VF). Thus APD dynamics at short coupling intervals during fixed pacing and during VF were accounted for by the dynamic, but not the standard, restitution relation. These results provide further evidence for a strong relationship among the kinetics of electrical restitution, the occurrence of APD alternans, and complex APD dynamics during VF.

Electrical restitution and spatiotemporal organization during ventricular fibrillation.

Despite recent advances in our understanding of the mechanism for ventricular fibrillation (VF), important electrophysiological aspects of the development of VF still are poorly defined. It has been suggested that the onset of VF involves the disintegration of a single spiral wave into many self-perpetuating waves. It has been further suggested that such a process requires that the slope of the electrical restitution relation be >/=1. The same theory anticipates that a single spiral wave will be stable (not disintegrate) if the maximum slope of the restitution relation is <1. We have shown previously that the slope of the restitution relation during rapid pacing and during VF is >/=1 in canine ventricle. We now show that drugs that reduce the slope of the restitution relation (diacetyl monoxime and verapamil) prevent the induction of VF and convert existing VF into a periodic rhythm. In contrast, a drug that does not reduce the slope of the restitution relation (procainamide) does not prevent the induction of VF, nor does it regularize VF. These results indicate that the kinetics of electrical restitution is a key determinant of VF. Moreover, they suggest novel approaches to preventing the induction or maintenance of VF.

[Recurrent hypertensive crises associated with severe orthostatic hypotension due to baroreflex failure syndrome]. / Rezidivierende hypertensive Krisen verbunden mit schwerer orthostatischer Hypotonie bei Baroreflex-Insuffizienz-Syndrom.

The baroreflex mechanism is a central part of the regulation of the cardiovascular system, particularly in the control of vagal and sympathetic outflow to the heart and the peripheral circulation. Failure of the baroreflex is a rare cause of secondary hypertension. It is characterized by drastic changes in sympathetic activation and blood pressure following complete denervation of the baroreflex. Here, we report a case of baroreflex failure following bilateral carotid artery surgery and radiation. Moreover, postoperative orthostatic hypotension with recurrent syncope suggests a rare subform, the selective baroreflex failure, where efferent parasympathetic activity is preserved. Both high blood pressure and orthostatically induced syncope improved substantially after treatment with clonidine.

Effects of [K(+)](o) on electrical restitution and activation dynamics during ventricular fibrillation.

To test whether hyperkalemia suppresses ventricular fibrillation (VF) by reducing the slope of the action potential duration (APD) restitution relation, we determined the effects of the extracellular K(+) concentration ([K(+)](o)) ([KCl] = 2.7-12 mM) on the restitution of APD and maximum upstroke velocity (V(max)) the magnitude of APD alternans and spatiotemporal organization during VF in isolated canine ventricle. As [KCl] was increased incrementally from 2.7 to 12 mM, V(max) was reduced progressively. Increasing [KCl] from 2.7 to 10 mM decreased the slope of the APD restitution relation at long, but not short, diastolic intervals (DI), decreased the range of DI over which the slope was >/=1, and reduced the maximum amplitude of APD alternans. At [KCl] = 12 mM, the range of DI over which the APD restitution slope was >/=1 increased, and the maximum amplitude of APD alternans increased. For [KCl] = 4-8 mM, the persistence of APD alternans at short DI was associated with maintenance of VF. For [KCl] = 10-12 mM, the spontaneous frequency during VF was reduced, and activation occurred predominantly at longer DI. The lack of APD alternans at longer DI was associated with conversion of VF to a periodic rhythm. These results provide additional evidence for the importance of APD restitution kinetics in the development of VF.

Postcardiac injury syndrome following radiofrequeny ablation of atrial flutter.

We report the case of a 64-year-old woman who was admitted to our hospital for radiofrequency ablation of isthmus-dependent counterclockwise atrial flutter. Following an initially uncomplicated right atrial linear isthmus ablation that was associated with conversion of atrial flutter to sinus rhythm and evidence of complete isthmus block, the patient developed a small pericardial effusion, a marked and recurrent left-sided pleural effusion, and had significantly elevated inflammatory markers. After an extensive diagnostic work-up which excluded infectious, malignant and thromboembolic causes of the effusions, a diagnosis of postcardiac injury syndrome was made and the patient was treated with oral corticosteroids and nonsteroidal anti-inflammatory drugs. Over a treatment period of 2 months there was complete resolution of the pericardial and left-sided pleural effusions and normalization of inflammatory markers. Postcardiac injury syndrome is a rare complication of radiofrequency ablation that is characterized by signs of pericardial, pleural and pulmonary parenchymal inflammation.