Persistent Atrial Standstill Research Articles

Persistent Atrial Standstill—Clinical, Electrophysiological, and Morphological Study

Persistent atrial standstill (PAS) is a rare disorder characterized by absence of atrial activity on the surface and intracavity electrograms, absence of atrial mechanical activity, and inability to electrically stimulate the atria. Four patients (ages 18-60 years) with PAS were evaluated. One of these (no. 3) only had right atrial (RA) standstill, whereas left atrium (LA) showed spontaneous activity and could be stimulated electrically. As RA biopsy is not possible, right ventricular (RV) endomyocardial biopsy (EMB) was obtained to identify possible atrial pathology that revealed inflammatory myocarditis, 2; amyloidosis, 1; and myocardial hypertrophy with fibrosis, 1. Three patients were given permanent pacemakers. One of these with amyloidosis died suddenly. One is lost to follow-up. The others cases are persisting with PAS.

Chronobiological study of heart rate and ventricular extrasystole in the course of acute myocardial infarction (AMI)

Persistent atrial standstill is a very rare pathophysiologic condition whose diagnosis is established when both electrical and mechanical silence of the atria are confirmed. To test the hypothesis that secretion of atrial natriuretic peptide is disturbed in patients with persistent atrial standstill, the response of atrial natriuretic peptide secretion and other neurohormonal factors during exercise was investigated in three patients with a rate-responsive ventricular demand (VVI) pacemaker implanted for confirmed persistent atrial standstill. The results were compared with those observed in eight normal subjects and patients with a rate-responsive VVI (Group A) or atrial demand (AAI) (Group B) pacemaker implanted for confirmed sick sinus syndrome.Patients in Group A displayed significant elevation of alpha-human atrial natriuretic peptide secretion both before and during exercise (122.5 ± 14.8 and 207.5 ± 8.3 pg/ml, respectively) compared with those in Group B (55 ± 14.1 and 116.4 ± 51.5 pg/ml, respectively) and the normal subjects (18.9 ± 9.8 and 30.8 ± 19.2 pg/ml, respectively). This indicated development of a nonphysiologic increase in atrial volume or pressure overload, or both, in rate-responsive VVI pacing because of lack of atrioventricular synchrony. However, patients with persistent atrial standstill had undetectable (<10 pg/ml) or almost undetectable secretion of atrial natriuretic peptide as well as lower levels of cyclic guanosine monophosphate in the circulation both before and during exercise. Changes in plasma catecholamines during exercise were similar in patients with persistent atrial standstill compared with the other groups.This study indicates that “endocrinologic silence” accompanies electrical and mechanical silence of the atria, which may constitute a third diagnostic clue to persistent atrial standstill.

Myocarditis manifesting as persistent atrial standstill

Persistent atrial standstill complicating inflammatory myocarditis in a young boy is presented. The disease was marked by a stuttering course characterized by intermittent return of atrial rhythm in the form of atrial flutter and fibrillation until persistent standstill lasting till the last follow-up occurred. The relevant literature is reviewed.

A case of persistent atrial standstill with a history of six years

A case of persistent atrial standstill with a history of six years

A case of persistent atrial standstill, associated with marked bradycardia since childhood

A case of persistent atrial standstill, associated with marked bradycardia since childhood

Familial endemic persistent atrial standstill in a small mountain community: review of eight cases.

Familial persistent atrial standstill (PAS) has been rarely documented. Five patients, three male and two female (mean age 46 years at first observation), with familial 'complete' PAS, all from a small mountain community (900 residents) have been studied. Diagnostic criteria were: absence of P wave in any lead of the standard electrocardiogram; no electrical activity of the atria with extremely slow junctional escape rhythm on the endocavitary recordings; lack of atrial excitability; absence of atrial wall movement at fluoroscopy; no mitral A wave on the echocardiogram. All patients had marked cardiac enlargement primarily due to atrial enlargement and all had impaired functional class. Some had bradycardia for many years but none had syncope. Permanent cardiac pacing was carried out in all. Two had cerebral embolism. In the same community there were three patients, one male and two female (mean age 34 years at first observation), with 'partial' atrial standstill characterized by absence of P waves in any lead of the standard electrocardiogram and by endocavitary recording of electrical activity limited to a localized region of the atrium. These three patients also had cardiac enlargement primarily due to atrial enlargement but to a lesser degree than the patients with complete PAS. One patient had cerebral embolism. The familial and endemic character of the disease is stressed. Cardiac enlargement due primarily to atrial enlargement seems to be a common feature of both the complete and partial form of the PAS syndrome.

Dissimilar atrial rhythms. A patient with triple right atrial rhythm

A patient with complete A-V block and atrial fibrillation was analyzed by multiple intra-atrial electrograms. Three areas were recorded in the right atrium, each of them with different electrophysiological properties: (1) persistent atrial standstill, (2) irregular atrial activity with a rate of 50/minute, and (3) fibrilloflutter waves with a rate of 450/minute. The latter were also registered in the left atrium. This case illustrates another form of dissimilar atrial rhythms, and provides additional evidence of the importance of recording multiple electrograms from each atrium in the evaluation of the atrial events in atypical atrial arrhythmias.

Flutter and fibrillation-like phenomenon of his bundle observed in a patient with persistent atrial standstill

A diagnosis of persistent atrial standstill was made in a patient with syncopal attacks, based on: (1) the lack of P waves both in the routine 12-lead electrocardiogram (ECG) and in the right atrial cavity lead; (2) the absence of "a waves in the right atrial pressure curve; and (3) the failure of the atria to respond to electrical stimulation. Compared with previously reported cases of persistent atrial standstill with a slow, regular escape rhythm of supraventricular origin, the present case was characterized by alternate periods of bradycardia and tachycardia, the latter being suggested as AV junctional tachycardia with exit block. Furthermore, the His bundle electrogram (HBE) showed either regular, high frequency deflictions or irregular, deformed potentials, suggesting flutter and fibrillation-like phenomena in the His bundle.

Persistent atrial standstill, report of three cases

Three cases, of which two are brothers, of persistent atrial standstill are reported. The diagnosis was made by the lack of P wave in routine 12 leads and right atrial cavity lead, no response of atrium to electrical stimulation and absence of "a" wave in right atrial pressure curve.

Persistent atrial standstill

A 24 year old woman presented with evidence of possible cerebral embolism following a transient episode of temporoparietal headache and sudden inability to move her left arm and leg. The electromyographic studies were within normal limits, but the electrocardiogram revealed junctional rhythm with no evidence of atrial activity. Her condition was diagnosed as persistent atrial standstill, a rare arrhythmia; only 19 other case reports could be retrieved from the literature.

Familial Atrial Cardiomyopathy with Heart Block

A cardiomyopathy is described affecting three of five siblings, characterized by first-degree heart block and ectopic supraventricular rhythms progressing over some years to persistent atrial standstill with complete loss of response to direct atrial stimulation. At least two children of one of the patients appear to be similarly affected. We have found only 11 documented cases of this persistent arrhythmia. A familial incidence has been reported twice, but in both was due to amyloidosis. This appears to be the amyloid description of familial persistent atrial standstill not due to amyloid disease.

Transient and persistent atrial standstill with His bundle lesions. Electrophysiologic and pathologic correlations.

Electrophysiologic studies, including His bundle recording and atrial pacing (AP), were performed in one patient with transient (TAS) and another with persistent atrial standstill (PAS). Both subsequently expired, and postmortem examinations with serial sections of the conduction system were obtained. The patient with TAS had left bundle-branch (LBB) block, a P-H interval of 140 msec (normal, 80-140 msec), an H-Q of 40 msec (normal, 35-55 msec), and an AP threshold of 1.2 ma. Postmortem examination revealed total left circumflex occlusion proximal to the sinoatrial (SA) nodal artery takeoff, SA nodal arteriolosclerosis without infarction, and left-sided cardiac skeletal sclerosis (LSCSS) disrupting the penetrating portion of the His bundle and the LBB. Intracardiac recordings in the patient with PAS showed absent atrial activity and an H-Q of 60 msec. The atria were inexcitable with AP up to 15 ma at multiple sites. Postmortem examination revealed a previously undescribed atrial disease characterized by arteriolosclerosis, fibroelastosis, fatty infiltration, and vacuolar degeneration of muscle cells, with only moderate SA and A-V nodal involvement. LSCSS was present, disrupting the branching portion of the His bundle. Thus, TAS reflected SA nodal ischemia without infarction; PAS reflected a new atrial degenerative disease; H-Q was normal with a lesion in the penetrating portion of the His bundle and was prolonged with a similar lesion in the branching portion.

Persistent atrial standstill in a family with myocardial disease

Three siblings with slow heart rates and mild congestive heart failure are described. Their electrocardiograms showed normal QRS complexes but no P waves. Intra-atrial electrocardiograms confirmed the absence of atrial activity. In one patient Adams-Stokes' attacks developed for which he required an artificial pacemaker. A biopsy specimen of his right atrium showed interstitial and perivascular deposits of amyloid. Atrial standstill may be suspected when P or fibrillary waves are absent from the electrocardiogram. It may be confirmed by intracardiac electrocardiograms. Inability to stimulate the atria, using the internal pacemaker, indicates that the myocardium, rather than the sinus node, is responsible for the atrial standstill.

Persistent atrial standstill in a patient affected with facioscapulohumeral dystrophy.

Persistent atrial standstill in a patient affected with facioscapulohumeral dystrophy.

Persistent atrial standstill

A patient with persistent atrial standstill complicated by facioscapulohumeral muscular dystrophy is described. The rarity of both persistent atrial arrest and the association of facioscapulohumeral dystrophy with heart disease is evident from the scarcity of such reports in the literature. A classification of atrial standstill is proposed, with categories of temporary, terminal, and persistent duration. The criteria for diagnosing atrial standstill are expanded by the inclusion of intracardiac pressures, intracardiac electrocardiography and cineangiocardiography. The successful treatment of the patient with atropine sulphate, with an increase in heart rate and cardiac output, is described.