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Persistent atrial standstill
CASE REPORTS
Persistent Atrial Standstill
OARREL T. COMBS,
M.D.,
Major
MC, USA
HENRY
M.D.,
Major
MC, USA
F. BELLACI.
HAROLD
H.
SHIVELY.
M.D.,
Major
MC.
USA GABRIEL
GREGORATOS,
M.D.,
Colonel
MC, USA San Francisco,
California
A 24 year old woman presented with evidence of possible cerebra1 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. standstill is an unusual arrhythmia characterP waves on the electrocardiogram and lack of “a” waves in the jugular venous pulse. lntraatrial recordings reveal no atrial electrogram, and intraatrial pressure recordings reveal no atrial pressure phenomenon. We recently studied a patient with persistent atrial standstill in whom right and left heart catheterization and electrophysiologic studies, including His bundle recordings and atrial and ventricular stimulation, were performed. The patient’s younger sister has also demonstrated supraventricular conduction abnormalities. Persistent ized
by
atrial
absent
CASE REPORT
From the Cardiology Service, Department of Medicine, Letterman Army Medical Center, Presidio of San Francisco. Requests for reprints should be addressed to Technical Publications Editor, Letterman Army Medical Center, Presidio of San Francisco, California 94129. Manuscript accepted September 17, 1973.
This 24 year old woman was in apparently good health until the morning of admission when she noted the onset of a mild right temporoparietal headache. The headache increased in intensity, and she became aware suddenly of inability to move her left arm and leg when she attempted to arise. The episode lasted for approximately 5 minutes, was observed by her husband and was followed by a gradual return of function to the extremities. There was no associated loss of consciousness, seizure activity, confusion, aphasia or palpitations. The patient was seen a half hour later by a physician. She had mild diffuse hyperreflexia and a slow irregular pulse with bigeminal rhythm. Past medical history disclosed possible rheumatic fever at age 7, at which time a heart murmur was noted with an associated slow, irregular pulse. At age 18, she had a similar episode which prompted medical evaluation, particularly since her younger sister had required a permanent pacemaker for symptomatic sinoatrial block 1 year earlier. The electrocardiogram of our patient at age 18 had revealed junctional rhythm with no evidence of P waves, and a Master’s exercise test demonstrated short runs of ventricular tachycardia. At that time a diagnosis of congenital heart block with junctional rhythm was made, and the patient was told that a pacemaker might be necessary at a later date. For the subsequent 6 years she was asymptomatic. She had taken oral contraceptives previously for a year without difficulty, and had resumed taking them approximately 2 weeks before the onset of the transient left hemiparesis. Family history disclosed that her 16 year old sister has evi-
February
1974
The American
Journal
of Medicine
Volume
56
231
PERSISTENT ATRIAL STANDSTILL-COMBS
ET AL
(left} and Figure 1. Posteroanterior lateral (right) chest roentgenograms. There is slight prominence of the left atrial appendage and main pulmonary artery in the posteroanterior view. Straightening of the thoracic spine is noted on the lateral view. dence of sinoatrial block and atrioventricular conduction abnormalities associated with recurrent syncopal attacks which have been controlled with ventricular pacing and antiarrhythmic drugs. Two brothers, ages 24 and 26, have no symptoms of cardiovascular disease; however, the electrocardiogiram of one, who also has diabetes mellitus, indicates a left axis deviation. The patient’s mother has diffuse nonspecific ST-T wave changes but is asymptomatic. Physical examination on admission revealed an alert and cooperative young woman. She weighed 85 pounds. Blood pressure was lOO/SO mm Hg, pulse rate 46 beats/min in a bigeminal rhythm, with occasional runs of slow, regular rhythm. Neck examination revealed visible “v” waves but
no distinct “a” waves. On cardiac examination the point of maximal impulse was in the 5th left intercostal space at the mid-clavicular line. The first heart sound was loud; the second heart sound split paradoxically. A grade 2/6 :systolic ejection murmur was heard most distinctly along the upper left sternal border and was also audible over the left carotid artery. The murmur was not audible at the apex nor in the aortic area. In addition, a grade 2/6 late systolic high-pitched murmur was audible at the apex which radiated to the axilla. Laboratory studies, including the complete blood count, sedimentation rate, serum electrolytes, sequential multiple analyzer (SMA-12, Technicon@) series, cardiac enzymes and aldolase determinations, were all within normal limits. A
Figure
2.
Electrocardiogram
ob-
tained on admission. P waves are absent. Anterior forces are greatly diminished. There is T wave inversion in the precordial leads. The frontal plane QRS axis is - 20 degrees.
232
February 1974
The American Journal of Medicine
Volume 56
PERSISTENT ATRIAL STANDSTILL-COMBS
Figure 3. Electrocardiogram reveals aberrant beats in bigeminy. These were considered to represent premaalventricular contractions, ture thouuh junctional beats with aberrant con&&on cannot be entirely exeluded.
ET AL.
v,
chest roentgenogram showed the heart to be at the upper limits of normal for size and displaced slightly leftward. The pulmonary outflow tract was prominent (Figure 1). The lateral chest roentgenogram revealed straightening of the thoracic spine which may account for some of the roentgenographic findings. Electrocardiogram showed no evidence of atrial activity with a junctional pacemaker at a rate of 50/min, narrow QRS complex, generalized T wave inversion, absent initial septal forces and left axis deviation (Figure 2). At times, frequent unifocal premature ventricular contractions in bigeminy were noted (Figure 3). A vectorcardiogram was consistent with left anterior hemiblock and revealed leftward and anterior initial forces. A mitral valve echocardiogram was within normal limits. An electromyographic study was also within normal limits. During hospitalization numerous electrocardiograms revealed junctional rhythm with no atrial activity. Cardiac catheterization was performed. Right heart pressures were mildly elevated at rest with “a” waves absent in both the right atrial and pulmonary capillary pressure recordings. There was no gradient across the aortic or pulmonic valves. Cardiac output and indicator dilution studies for shunt detection were normal. Calculated angiographic ejection fraction, stroke volume and stroke index were at the upper limits of normal. A left ventriculogram revealed normal left ventricular contractions. The posterior leaflet of the mitral valve prolapsed into the left atrium resulting in minimal mitral insufficiency. A forward angiocar-
February
diogram from the right atrium demonstrated passive movement of both atria. There was no evidence of active atrial contraction, and no atrial filling defects were noted. An intracardiac electrocardiogram revealed no evidence of atrial electrical activity during exploration of the right atrium from the sinoatrial node to the tricuspid valve (Figure 4). A His bundle electrogram demonstrated a His potential preceding each QRS wave with a normal H-V interval. Right atrial stimulation was attempted at multiple sites from the sinoatrial node to the free wall of the right atrium at energy levels up to 25 ma, but no atrial response could
Atrial
ECG
100 mEjec
His bundle and atrial electrocardiogram. 4. There is no evidence of atrial activity. The H-V interval is 50 msec. Figure
1974
The American
Journal
of Medicine
Volume
56
233
PERSISTENT ATRIAL STANDSTILL-COMBS
pacer
at
25
ET AL
Figure 5. His bund’le and atria/ elecwith atria/ pacing. trocardioaram Again no‘ie absence of atria/ .activity despite atria/ pacing at 25 ma.
m.a.
be elicited (Figure 5). Conversely, stimulation of the right ventricle with 0.5 ma produced prompt ventricular capture with no evidence of retrograde atrial capture. Tlhe patient remained asymptomatic during her hospitalization. Because her transient left hemiparesis could have been the result of cerebral embolization from thrombus in the noncontracting left atrium, anticoagulation with warfarin sodium was carried out, and birth control pills were discontinued. Our patient therefore demonstrates atrial standstill which has probably been present since age 7. In addition, prolapse of the mitral valve with trivial mitral insufficiency is present. COMMENTS Perhaps the first case of persistent atrial standstill appearing in the literature was that in a patient with myocarditis reported by Lewis in 1913 [l]. However, his data are insufficient to rule out the possibility of fine atrial fibrillation. The first well documented case of persistent atrial standstill was reported by Chavez et al. [2] in 1944. A 25 year old man, in whom phlebograms and esophageal electrocardiograms were obtained, had no signs of atrial activity. The patient suffered from intractable heart failure, and at postmortem examination several old infarcts jnvolving the atria, septum and the ventricles were ‘found. The electrocardiogram revealed right bundle branch block and left anterior hemiblock. Three patients with facioscapulohumeral dystrophy and persistent atria.1 standstill have been described [3-51. All three were young men (ages 26, 35 and 32). The electrocardiogram in these cases showed, in addi-
234
February 1974
The American Journal of Medicine
tion to absence of atrial activity, left axis deviation and poor precordial “R” wave progression. The patient described by Baldwin et al. [5] demonstrated transient neurologic symptoms similar to those experienced by our patient and was given anticoagulant therapy. This patient also had evidence of a prolapsing mitral leaflet, again similar to the findings in our patient. Persistent atrial standstill in relation to abdominal surgery was reported by W.ada et al. [6]. In their patient, a 46 year old man, atrial standstill developed which these workers proposed was related to a previous surgical procedure. Juve et al. [7], in a review of atrial standstill, mentioned one case which persisted for several years, the etiology of which was unknown. Persistent atrial standstill was also reported by Raynaud et al. [8]. Their patient subsequently came to autopsy and was found to have a paper thin atrium which these investigators suggested as the cause of the electrical findings. Four families in which persistent atrial standstill occurred have been described previously [9-l 21. Harrison and Derrick [9] described a 34 year old man who underwent cardiac catheterization and atrial biopsy at the time an electrical pacemaker was placed. Based on the biopsy findings, a diagnosis of primary amyloidosis was made. The electrocardiogram of this patient’s 33 year old sister showed absent “P” waves, but no other studies were made. Allensworth et al. [IO] described a family in which “P” waves were absent on the electrocardiograms of four siblings. In three of these four patients further studies established the diagnosis of persistent atrial standstill. One of the patients underwent biopsy of the atrial appendage when the pacemaker was implanted. The biopsy
Volume 56
PERSISTENT ATRIAL STANDSTILL-COMBS
specimen revealed primary amyloidosis. Schuilenburg [ll] described two sisters who had apparent atrial standstill. The rhythm disturbance had been present in one for 11 years and in the other for 20 years. The etiology of the atrial standstill was unknown, but a hereditary cardiomyopathy was suggested. In a family studied by Nagle et al. [12] three of five siblings had a cardiomyopathy characterized by first degree heart block and ectopic supraventricular arrhythmias which progressed over some years to persistent atrial standstill. These investigators also reported that two children of one of the patients appeared to be similarly affected. A 47 year old man was seen by Messinger and Mirkinson [13] with recurrent chest pain, hypertension and persistent atrial standstill. A definite diagnosis of coronary artery disease could not be made, but these investigators speculated that arteriosclerotic heart disease might be the cause of the rhythm disturbance. To our knowledge two previous cases of persistent atrial standstill with His bundle electrocardiography have been reported. The first was by Rosen et al. [14]. Their patient, a 73 year old woman, initially presented with atrial fibrillation and subsequently atrial standstill developed. His bundle study revealed a slightly prolonged H-V interval and complete absence of atrial activity. The patient was examined at autopsy, and these investigators proposed that she represented a new atrial disease with marked fibrosis, fibroelastosis and fatty infiltration of the atrium. Atrial muscle cells showed marked vacuolization; in some areas they were engulfed by hyalinized connective tissue. A second case was reported by Patton et al. [15]. Their patient, a 54 year old man, had a prolonged H-V interval and attempts to pace the atria were unsuccessful. He subsequently died from a malignancy, and autopsy revealed diffuse fibrosis of the atria. Rosenbaum and Levine [16], in their review of atrial standstill, referred to a patient who was considered to have atrial standstill in relation to rheumatic pancarditis. Our patient’s history of rheumatic fever is not well documented. Although it is conceivable that a myocarditis involving the atria could have resulted in atrial standstill, it seems unlikely. Including our case, we were able to find 20 reported cases of atrial standstill. There were six additional cases in which “P” waves were absent in the electrocardiogram, but no further studies had been carried out. Four of these patients were related to patients with definite atrial standstill. In
February
ET AL.
15 of the 19 well proved cases, the sex of the patient was identified; nine were male and six were female. The age of the patients in the cases in which the age was reported (10 cases) ranged from 24 to 73 years, with an average of 39.6 years. Theoretically, it would appear that three possible mechanisms might result in atrial standstill: (1) sinus arrest, (2) sinoatrial exit block, or (3) diffuse disease of the atria. In the first two situations atrial pacing should be possible. In our patient and in nine of the previously described patients, atrial stimulation was attempted without success, implying that the abnormality was generalized throughout the atria. This diffuse involvement of the atria is supported by the available pathologic data. In four cases autopsy data were reported [2,8,14,15], and in two other cases [9,10] atrial biopsy was performed. The cases seen at postmortem examination revealed diffuse disease throughout the atria, and the two atrial biopsy specimens demonstrated amyloidosis which was probably generalized throughout the atrial musculature. It would appear then that persistent atrial standstill may occur when diffuse involvement of the atria, from whatever underlying cause, results in replacement of normal atrial cells with subsequent loss of responsiveness to electrical stimulation. This mechanism apparently differs from that of transient atrial standstill which has been well documented clinically [16-181, and which has been reported in the setting of drug toxicity [16], hypoxia [19] and recently in a patient after surgery [20]. It has been postulated that the standstill in this situation results from depolarization of atria and subsequent lack of responsiveness which can be corrected by reversing the underlying mechanism or, as recently reported, by the administration of small amounts of isoproterenol
[201. Therapeutic attempts to treat persistent atrial standstill have been varied. In several cases, therapy was either thought unnecessary or was not reported. In four patients parasympathetic blocking agents were used with some success [3,6,8,9], and in three patients cardiac pacing was used after attempts at other therapy were unsuccessful [9,10]. Our patient was asymptomatic except for the episode which resulted in her admission, and she refused cardiac pacing. Anticoagulation W;IS begun because of the possibility of embolism from the noncontracting left atrium. The sister of our patient has had several syncopal episodes, and electrocardiograms have re-
1974
The American
Journal
of Medicine
Volume
56
235
PERSISTENT ATRIAL STANDSTILL-COMBS
vea.led
supraventricular
ET At
arrhythmias
with evidence
ACKNOWLEDGMENT
of junctional rhythm resulting in retrograde atrial activity and probable episodes of sinoatrial block. She has required ventricular pacing to control her symptoms. One may speculate that she may have disease of her atrial conduction system which ma’y also eventually result in atrial standstill.
We wish
to express
pert technical white, Medical
our
appreciation
for
the
ex-
assistance of Mrs. Lottie AppleEditor, Letterman Army Medical
Center, and for the secretarial Gloria G. Swanson.
assistance
of Mrs.
REFERENCES
2.
3. 4.
9.
10.
236
Lewis T: Exceptional types of slow heart action. Q J Med 6: 221, 1913. Chavez I, Brumlik J. Sodi-Pollares D: Sobre un case extraordinario de paralisis auricular permanente con deckneracion del nodulo de Keith Y Flack. Arch lnst Cardiol Mex 16: 159, 1946. Bloomfield DA, Sinclair-Smith BC: Persistent atrial paralysis. Am J Med 39: 335, 1965. Caponnetto S, Pastorini C, Tirelli G: Persistent atrial standstill in a patient affected with facioscapulohumeral muscular dystrophy. Cardiology 53: 341, 1968. Baldwin B, Talley R, Johnson C, Nutter D: Permanent paralysis of the atrium in a patient with facioscapulohumeral muscular dystrophy. Am J Cardiol 31: 649, 1973. Wada M, Takeda C. Mise J: A case report of atrial standstill. Jap Circ J 30: 543, 1966. Juve A, Delaage M, Torresani J, Nicolai P, Heuillet G, Pinas E: La paralysie auriculaire. Arch Mal Coeur 60: 865, 1967. Raynaud R, Jobard P, Brochier M, Fauchier J, Benatre A, Raynaud P: Paralysie auriculaire chronique et oreillette papyracee. Ann Cardiol Angeiol (Paris) 17: 117, 1968. Harrison WH, Derrick JR: Atrial standstill. Angiology 20: 610, 1969. Allensworth DC, Rise DJ, Lowe GW: Persistent atrial standstill in a family with myocardial disease. Am J Med 47: 775, 1969.
February
1974
The American
Journal
of Medicine
Volume
11.
12.
13. 14.
15.
16.
17. 18.
19.
20.
56
Schuilenburg RM: Prolonged atrial standstill (abstract), World Congress of Cardiology, London, British Medical Assn, 1970, p 280. Naole RE. Smith B. Williams DD: Familial atrial cardiomyopathy with heart block (abstract). Brit Heart J 34: 205, 1972. Messinger WJ, Mirkinson AM: Permanent atrial standstill. Arch Intern Med 124: 211, 1969. Rosen KM, Shahbuden RH, Gunnar RM. Lev MA: Transient and permanent atrial standstill with His bundle recordings. Circulation 44: 220, 1971. Patton RD, Damata AN, Berkowitz WD, Lau SU. Stein EM: Electrically silent right atrium. J Electrocardiol 3: 239, 1970. Rosenbaum F, Levine SA: Auricular standstill, its occurrence and significance. Am J Med Sci 198: 774, 1939. James TN: Myocardial infarction and atrial arrhythmias. Circulation 24: 761, 1961. James TN, Rupe CF. Monte RW: Pathology of the cardiac conduction system in systemic lupus erythematosus. Ann Intern Med 62: 402, 1965. Greene CW, Gilbert NC: Studies on the responses of the circulation to low oxygen tension. I I I. Changes in the pacemaker and in the conduction during extreme oxygen want, as shown in the human electrocardiogram. Arch Intern Med 27: 517, 1921. Waldo AL, Vitikainen KJ: Atrial standstill secondary to atrial inexcitability (atrial quiescence). Circulation 46: 690. 1972.
Persistent Atrial Standstill
OARREL T. COMBS,
M.D.,
Major
MC, USA
HENRY
M.D.,
Major
MC, USA
F. BELLACI.
HAROLD
H.
SHIVELY.
M.D.,
Major
MC.
USA GABRIEL
GREGORATOS,
M.D.,
Colonel
MC, USA San Francisco,
California
A 24 year old woman presented with evidence of possible cerebra1 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. standstill is an unusual arrhythmia characterP waves on the electrocardiogram and lack of “a” waves in the jugular venous pulse. lntraatrial recordings reveal no atrial electrogram, and intraatrial pressure recordings reveal no atrial pressure phenomenon. We recently studied a patient with persistent atrial standstill in whom right and left heart catheterization and electrophysiologic studies, including His bundle recordings and atrial and ventricular stimulation, were performed. The patient’s younger sister has also demonstrated supraventricular conduction abnormalities. Persistent ized
by
atrial
absent
CASE REPORT
From the Cardiology Service, Department of Medicine, Letterman Army Medical Center, Presidio of San Francisco. Requests for reprints should be addressed to Technical Publications Editor, Letterman Army Medical Center, Presidio of San Francisco, California 94129. Manuscript accepted September 17, 1973.
This 24 year old woman was in apparently good health until the morning of admission when she noted the onset of a mild right temporoparietal headache. The headache increased in intensity, and she became aware suddenly of inability to move her left arm and leg when she attempted to arise. The episode lasted for approximately 5 minutes, was observed by her husband and was followed by a gradual return of function to the extremities. There was no associated loss of consciousness, seizure activity, confusion, aphasia or palpitations. The patient was seen a half hour later by a physician. She had mild diffuse hyperreflexia and a slow irregular pulse with bigeminal rhythm. Past medical history disclosed possible rheumatic fever at age 7, at which time a heart murmur was noted with an associated slow, irregular pulse. At age 18, she had a similar episode which prompted medical evaluation, particularly since her younger sister had required a permanent pacemaker for symptomatic sinoatrial block 1 year earlier. The electrocardiogram of our patient at age 18 had revealed junctional rhythm with no evidence of P waves, and a Master’s exercise test demonstrated short runs of ventricular tachycardia. At that time a diagnosis of congenital heart block with junctional rhythm was made, and the patient was told that a pacemaker might be necessary at a later date. For the subsequent 6 years she was asymptomatic. She had taken oral contraceptives previously for a year without difficulty, and had resumed taking them approximately 2 weeks before the onset of the transient left hemiparesis. Family history disclosed that her 16 year old sister has evi-
February
1974
The American
Journal
of Medicine
Volume
56
231
PERSISTENT ATRIAL STANDSTILL-COMBS
ET AL
(left} and Figure 1. Posteroanterior lateral (right) chest roentgenograms. There is slight prominence of the left atrial appendage and main pulmonary artery in the posteroanterior view. Straightening of the thoracic spine is noted on the lateral view. dence of sinoatrial block and atrioventricular conduction abnormalities associated with recurrent syncopal attacks which have been controlled with ventricular pacing and antiarrhythmic drugs. Two brothers, ages 24 and 26, have no symptoms of cardiovascular disease; however, the electrocardiogiram of one, who also has diabetes mellitus, indicates a left axis deviation. The patient’s mother has diffuse nonspecific ST-T wave changes but is asymptomatic. Physical examination on admission revealed an alert and cooperative young woman. She weighed 85 pounds. Blood pressure was lOO/SO mm Hg, pulse rate 46 beats/min in a bigeminal rhythm, with occasional runs of slow, regular rhythm. Neck examination revealed visible “v” waves but
no distinct “a” waves. On cardiac examination the point of maximal impulse was in the 5th left intercostal space at the mid-clavicular line. The first heart sound was loud; the second heart sound split paradoxically. A grade 2/6 :systolic ejection murmur was heard most distinctly along the upper left sternal border and was also audible over the left carotid artery. The murmur was not audible at the apex nor in the aortic area. In addition, a grade 2/6 late systolic high-pitched murmur was audible at the apex which radiated to the axilla. Laboratory studies, including the complete blood count, sedimentation rate, serum electrolytes, sequential multiple analyzer (SMA-12, Technicon@) series, cardiac enzymes and aldolase determinations, were all within normal limits. A
Figure
2.
Electrocardiogram
ob-
tained on admission. P waves are absent. Anterior forces are greatly diminished. There is T wave inversion in the precordial leads. The frontal plane QRS axis is - 20 degrees.
232
February 1974
The American Journal of Medicine
Volume 56
PERSISTENT ATRIAL STANDSTILL-COMBS
Figure 3. Electrocardiogram reveals aberrant beats in bigeminy. These were considered to represent premaalventricular contractions, ture thouuh junctional beats with aberrant con&&on cannot be entirely exeluded.
ET AL.
v,
chest roentgenogram showed the heart to be at the upper limits of normal for size and displaced slightly leftward. The pulmonary outflow tract was prominent (Figure 1). The lateral chest roentgenogram revealed straightening of the thoracic spine which may account for some of the roentgenographic findings. Electrocardiogram showed no evidence of atrial activity with a junctional pacemaker at a rate of 50/min, narrow QRS complex, generalized T wave inversion, absent initial septal forces and left axis deviation (Figure 2). At times, frequent unifocal premature ventricular contractions in bigeminy were noted (Figure 3). A vectorcardiogram was consistent with left anterior hemiblock and revealed leftward and anterior initial forces. A mitral valve echocardiogram was within normal limits. An electromyographic study was also within normal limits. During hospitalization numerous electrocardiograms revealed junctional rhythm with no atrial activity. Cardiac catheterization was performed. Right heart pressures were mildly elevated at rest with “a” waves absent in both the right atrial and pulmonary capillary pressure recordings. There was no gradient across the aortic or pulmonic valves. Cardiac output and indicator dilution studies for shunt detection were normal. Calculated angiographic ejection fraction, stroke volume and stroke index were at the upper limits of normal. A left ventriculogram revealed normal left ventricular contractions. The posterior leaflet of the mitral valve prolapsed into the left atrium resulting in minimal mitral insufficiency. A forward angiocar-
February
diogram from the right atrium demonstrated passive movement of both atria. There was no evidence of active atrial contraction, and no atrial filling defects were noted. An intracardiac electrocardiogram revealed no evidence of atrial electrical activity during exploration of the right atrium from the sinoatrial node to the tricuspid valve (Figure 4). A His bundle electrogram demonstrated a His potential preceding each QRS wave with a normal H-V interval. Right atrial stimulation was attempted at multiple sites from the sinoatrial node to the free wall of the right atrium at energy levels up to 25 ma, but no atrial response could
Atrial
ECG
100 mEjec
His bundle and atrial electrocardiogram. 4. There is no evidence of atrial activity. The H-V interval is 50 msec. Figure
1974
The American
Journal
of Medicine
Volume
56
233
PERSISTENT ATRIAL STANDSTILL-COMBS
pacer
at
25
ET AL
Figure 5. His bund’le and atria/ elecwith atria/ pacing. trocardioaram Again no‘ie absence of atria/ .activity despite atria/ pacing at 25 ma.
m.a.
be elicited (Figure 5). Conversely, stimulation of the right ventricle with 0.5 ma produced prompt ventricular capture with no evidence of retrograde atrial capture. Tlhe patient remained asymptomatic during her hospitalization. Because her transient left hemiparesis could have been the result of cerebral embolization from thrombus in the noncontracting left atrium, anticoagulation with warfarin sodium was carried out, and birth control pills were discontinued. Our patient therefore demonstrates atrial standstill which has probably been present since age 7. In addition, prolapse of the mitral valve with trivial mitral insufficiency is present. COMMENTS Perhaps the first case of persistent atrial standstill appearing in the literature was that in a patient with myocarditis reported by Lewis in 1913 [l]. However, his data are insufficient to rule out the possibility of fine atrial fibrillation. The first well documented case of persistent atrial standstill was reported by Chavez et al. [2] in 1944. A 25 year old man, in whom phlebograms and esophageal electrocardiograms were obtained, had no signs of atrial activity. The patient suffered from intractable heart failure, and at postmortem examination several old infarcts jnvolving the atria, septum and the ventricles were ‘found. The electrocardiogram revealed right bundle branch block and left anterior hemiblock. Three patients with facioscapulohumeral dystrophy and persistent atria.1 standstill have been described [3-51. All three were young men (ages 26, 35 and 32). The electrocardiogram in these cases showed, in addi-
234
February 1974
The American Journal of Medicine
tion to absence of atrial activity, left axis deviation and poor precordial “R” wave progression. The patient described by Baldwin et al. [5] demonstrated transient neurologic symptoms similar to those experienced by our patient and was given anticoagulant therapy. This patient also had evidence of a prolapsing mitral leaflet, again similar to the findings in our patient. Persistent atrial standstill in relation to abdominal surgery was reported by W.ada et al. [6]. In their patient, a 46 year old man, atrial standstill developed which these workers proposed was related to a previous surgical procedure. Juve et al. [7], in a review of atrial standstill, mentioned one case which persisted for several years, the etiology of which was unknown. Persistent atrial standstill was also reported by Raynaud et al. [8]. Their patient subsequently came to autopsy and was found to have a paper thin atrium which these investigators suggested as the cause of the electrical findings. Four families in which persistent atrial standstill occurred have been described previously [9-l 21. Harrison and Derrick [9] described a 34 year old man who underwent cardiac catheterization and atrial biopsy at the time an electrical pacemaker was placed. Based on the biopsy findings, a diagnosis of primary amyloidosis was made. The electrocardiogram of this patient’s 33 year old sister showed absent “P” waves, but no other studies were made. Allensworth et al. [IO] described a family in which “P” waves were absent on the electrocardiograms of four siblings. In three of these four patients further studies established the diagnosis of persistent atrial standstill. One of the patients underwent biopsy of the atrial appendage when the pacemaker was implanted. The biopsy
Volume 56
PERSISTENT ATRIAL STANDSTILL-COMBS
specimen revealed primary amyloidosis. Schuilenburg [ll] described two sisters who had apparent atrial standstill. The rhythm disturbance had been present in one for 11 years and in the other for 20 years. The etiology of the atrial standstill was unknown, but a hereditary cardiomyopathy was suggested. In a family studied by Nagle et al. [12] three of five siblings had a cardiomyopathy characterized by first degree heart block and ectopic supraventricular arrhythmias which progressed over some years to persistent atrial standstill. These investigators also reported that two children of one of the patients appeared to be similarly affected. A 47 year old man was seen by Messinger and Mirkinson [13] with recurrent chest pain, hypertension and persistent atrial standstill. A definite diagnosis of coronary artery disease could not be made, but these investigators speculated that arteriosclerotic heart disease might be the cause of the rhythm disturbance. To our knowledge two previous cases of persistent atrial standstill with His bundle electrocardiography have been reported. The first was by Rosen et al. [14]. Their patient, a 73 year old woman, initially presented with atrial fibrillation and subsequently atrial standstill developed. His bundle study revealed a slightly prolonged H-V interval and complete absence of atrial activity. The patient was examined at autopsy, and these investigators proposed that she represented a new atrial disease with marked fibrosis, fibroelastosis and fatty infiltration of the atrium. Atrial muscle cells showed marked vacuolization; in some areas they were engulfed by hyalinized connective tissue. A second case was reported by Patton et al. [15]. Their patient, a 54 year old man, had a prolonged H-V interval and attempts to pace the atria were unsuccessful. He subsequently died from a malignancy, and autopsy revealed diffuse fibrosis of the atria. Rosenbaum and Levine [16], in their review of atrial standstill, referred to a patient who was considered to have atrial standstill in relation to rheumatic pancarditis. Our patient’s history of rheumatic fever is not well documented. Although it is conceivable that a myocarditis involving the atria could have resulted in atrial standstill, it seems unlikely. Including our case, we were able to find 20 reported cases of atrial standstill. There were six additional cases in which “P” waves were absent in the electrocardiogram, but no further studies had been carried out. Four of these patients were related to patients with definite atrial standstill. In
February
ET AL.
15 of the 19 well proved cases, the sex of the patient was identified; nine were male and six were female. The age of the patients in the cases in which the age was reported (10 cases) ranged from 24 to 73 years, with an average of 39.6 years. Theoretically, it would appear that three possible mechanisms might result in atrial standstill: (1) sinus arrest, (2) sinoatrial exit block, or (3) diffuse disease of the atria. In the first two situations atrial pacing should be possible. In our patient and in nine of the previously described patients, atrial stimulation was attempted without success, implying that the abnormality was generalized throughout the atria. This diffuse involvement of the atria is supported by the available pathologic data. In four cases autopsy data were reported [2,8,14,15], and in two other cases [9,10] atrial biopsy was performed. The cases seen at postmortem examination revealed diffuse disease throughout the atria, and the two atrial biopsy specimens demonstrated amyloidosis which was probably generalized throughout the atrial musculature. It would appear then that persistent atrial standstill may occur when diffuse involvement of the atria, from whatever underlying cause, results in replacement of normal atrial cells with subsequent loss of responsiveness to electrical stimulation. This mechanism apparently differs from that of transient atrial standstill which has been well documented clinically [16-181, and which has been reported in the setting of drug toxicity [16], hypoxia [19] and recently in a patient after surgery [20]. It has been postulated that the standstill in this situation results from depolarization of atria and subsequent lack of responsiveness which can be corrected by reversing the underlying mechanism or, as recently reported, by the administration of small amounts of isoproterenol
[201. Therapeutic attempts to treat persistent atrial standstill have been varied. In several cases, therapy was either thought unnecessary or was not reported. In four patients parasympathetic blocking agents were used with some success [3,6,8,9], and in three patients cardiac pacing was used after attempts at other therapy were unsuccessful [9,10]. Our patient was asymptomatic except for the episode which resulted in her admission, and she refused cardiac pacing. Anticoagulation W;IS begun because of the possibility of embolism from the noncontracting left atrium. The sister of our patient has had several syncopal episodes, and electrocardiograms have re-
1974
The American
Journal
of Medicine
Volume
56
235
PERSISTENT ATRIAL STANDSTILL-COMBS
vea.led
supraventricular
ET At
arrhythmias
with evidence
ACKNOWLEDGMENT
of junctional rhythm resulting in retrograde atrial activity and probable episodes of sinoatrial block. She has required ventricular pacing to control her symptoms. One may speculate that she may have disease of her atrial conduction system which ma’y also eventually result in atrial standstill.
We wish
to express
pert technical white, Medical
our
appreciation
for
the
ex-
assistance of Mrs. Lottie AppleEditor, Letterman Army Medical
Center, and for the secretarial Gloria G. Swanson.
assistance
of Mrs.
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3. 4.
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236
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