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Partial atrial standstill: a case report
Journal of Electrocardiology 38 (2005) 252 – 255 www.elsevier.com/locate/jelectrocard
Partial atrial standstill: a case report Ergun Demiralp, MD, Ata Kirilmaz, MDT, Bekir Sitki Cebeci, MD, Rifat Eralp Ulusoy, MD Electrophysiology Section, Department of Cardiology, Gulhane Military Medical Academy Haydarpasa Training Hospital, 34668 Istanbul, Turkey Received 12 July 2004; revised 16 December 2004; accepted 13 January 2005
Abstract
Twelve-lead electrocardiograms revealed no atrial activity and a wide QRS escape rhythm at 38 beats/min in a 20-year-old man who presented with syncope. Doppler echocardiography documented the absence of A wave both in the tricuspid and mitral valve flow. The only mechanical activity was documented at the left atrial appendage. An electrophysiologic study demonstrated electrical inactivity in the right atrium and an atrial tachycardia in the left atrium. Atrial pacing with maximum output did not capture the atria. Our case represents an advanced stage of partial atrial standstill, with a mechanical and electrical atrial activity confined only to the left trial appendage. The patient remained asymptomatic after receiving a VVIR pacemaker and anticoagulation therapy. D 2005 Elsevier Inc. All rights reserved.
Keywords:
Atrial fibrillation; Atrial standstill; Electrophysiology; Atrial tachycardia
1. Introduction Atrial standstill (AS) is characterized by failure of atrial activity either spontaneously or in response to electrical stimulation. It has been reported in digitalis or quinidine toxicity, hypoxia, muscular dystrophia, valvular dysfunction, polymorphism of connexin 40, proscillaridin and verapamil intoxication, amyloidosis, ischemic heart disease, and acute coronary syndromes [1-7]. It is a rare arrhythmia diagnosed on the basis of specific electrocardiographic, electrophysiologic, and hemodynamic findings. Partial AS has also been reported. In this report, we describe a case of AS with a preserved mechanical and electrical activity confined only to the left atrial appendage. 2. Case presentation A 20-year-old man presented after his first syncopal episode. He complained of weakness, dyspnea on exertion, and frequent presyncopal events. Physical examination was unremarkable except for a grade 2/6 ejection systolic murmur at the mid left sternal border and bradycardia. Twelve-lead electrocardiogram (ECG) showed an escape T Corresponding author. Gulhane Askeri Tip Akademisi Haydarpasa Egitim Hastanesi Kardiyoloji Servisi, 34668 Istanbul, Turkey. Tel.: +90 216 5422473, +90 532 3307496; fax: +90 216 3487880. E-mail address: [email protected] (A. Kirilmaz). 0022-0736/$ – see front matter D 2005 Elsevier Inc. All rights reserved. doi:10.1016/j.jelectrocard.2005.01.009
distal rhythm at 33 beats/min with a QRS duration of 120 milliseconds but no atrial activity (Fig. 1). Chest x-ray revealed cardiomegaly with a prominent right atrial border. Echocardiography revealed a massively dilated right atrium (RA) with moderate dilation of the left atrium (LA) along with moderate tricuspid (TR) and mitral regurgitation (Fig. 2A). Although a Doppler study did not demonstrate any atrial activity (A wave) in the mitral and tricuspid inflow regions, fluttering of the left atrial appendage was documented both by tissue Doppler and by Doppler flow with transesophageal echocardiography (Fig. 2B and C). Pulmonary arterial pressure measured from TR by Doppler echocardiography was 35 mm Hg. The patient underwent an electrophysiologic study. A steerable decapolar coronary sinus catheter and a quadripolar diagnostic catheter were placed under fluoroscopic guidance via the right femoral vein. There was no electrical activity recorded from the RA. Left atrial activity recorded from the distal coronary sinus revealed an atrial tachycardia at a cycle length of 300 milliseconds (Fig. 3A and B). Neither the RA nor the LA could be paced with the highest voltage output (25 V at 2 milliseconds).
3. Discussion We report a case of AS coexisting with a dilated RA. Although the surface ECG failed to disclose any atrial
E. Demiralp et al. / Journal of Electrocardiology 38 (2005) 252–255
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Fig. 1. Twelve-lead ECG at the presentation. No P wave was demonstrated in the ECG. Heart rate was 38 beats/min and QRS duration was measured 140 milliseconds.
electrical activity, the electrical and the mechanical activity of the left atrial appendage was detected by intracardiac electrography and Doppler recordings. Although a few autopsy reports have described the pathological changes in AS, fibroelastosis and fatty infiltration in the atria have been incriminated [8]. The high lateral RA is usually involved first with a progression toward the lower site of RA. Finally, atrial electrograms can be recorded only in the vicinity of the tricuspid valve or interatrial septum. This phenomenon was observed in most cases despite the different underlying diseases. On the basis of these observations, our case represents a relatively late stage of the disease process. In addition, in our case, the electrical activity was confined at most to the LA with only demonstrable mechanical activity confined to the left atrial appendage. In a study by Nakazato et al [9], the absence of P wave was observed in 6 of 11 patients with atrial standstill. They observed the loss of P waves in the remaining 5 patients in time with the progression of the underlying disease. The absence of His activity in our case also supports the advanced stage of disease. No systemic disease or causative agent was detected in our case, and the patient did not present any sign or symptom of a causative disease for years. Although we could not search for a possible genetic defect such as polymorphism of connexin 40, cardiac evaluation including echocardiography of the patient’s relatives did not reveal any cardiac abnormalities. In this case, partial AS assumed to be idiopathic has been confirmed by giant RA, progression of the disease process to the LA, and spared left atrial appendage documented by both echocardiography and intracardiac electrography.
Syncope, stroke, congestive heart failure, and sudden death are among the reported complications of AS. Therapeutic measures include long-term anticoagulation and implantation of a permanent pacemaker. Our patient
Fig. 2. A, Echocardiographic apical 4-chamber view. The RA diameter was 95 85 mm. B, Tissue Doppler flow taken from the left atrial appendage region. Arrows indicate the focal mechanical atrial activity with a frequency of 3/s. C, Transesophageal echocardiographic Doppler flow of the left atrial appendage, sampling volume marker (*) at the base of the appendage. Arrows indicate the blood flow of the left atrial appendage at a frequency of 3/s. LV indicates left ventricle, MV, mitral valve.
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E. Demiralp et al. / Journal of Electrocardiology 38 (2005) 252–255
Fig. 3. A, Surface and intracardiac electrograms demonstrating an atrial tachycardia with a cycle length of 301 milliseconds. The atrial activity recorded from the catheter located in the coronary sinus is very fractionated and long in duration (70 milliseconds). This pattern reflects the slowed and diseased propagation of the electrical activation in the LA. Electrogram recorded from the catheter located in the high RA reveals no right atrial activity but far-field ventricular signal. CS, electrogram recorded from the coronary sinus. A indicates atrial activity; V, ventricular activity. B, Fluoroscopic image in 408 left anterior oblique position showing the localization of the catheters during recording the electrograms in A. HRA indicates high RA; CS, coronary sinus; LAO, left anterior oblique.
received a VVIR pacemaker and anticoagulation and remained free of syncope. References [1] Bloomfield DA, Singlair-Smith BC. Persistent atrial standstill. Am J Med 1965;39:335.
[2] Wolff L, White P. Auricular standstill during quinidine therapy. Heart 1929;14:295. [3] Rosenbaum FF, Levine SA. Auricular standstill. Its occurrence and significance. Am J Med Sci 1939;198:774. [4] Magnusson P. Auricular standstill. Acta Med Scand 1946;123:519. [5] Koshimizu TA, Komori S, Ishihara T, et al. Restored atrial excitability after late recanalization in a patient with atrial standstill and acute myocardial infarction. Pacing Clin Electrophysiol 2002;25(2):217.
E. Demiralp et al. / Journal of Electrocardiology 38 (2005) 252–255 [6] Boriani G, Gallina M, Merlini L, et al. Clinical relevance of atrial fibrillation/flutter, stroke, pacemaker implant, and heart failure in Emery-Dreifuss muscular dystrophy: A Long-term Longitudinal Study. Stroke 2003;34:901. [7] Groenewegen WA, Firouzi M, Bezzina CR, et al. A cardiac sodium channel mutation cosegregates with a rare connexin40 genotype in familial atrial standstill. Circ Res 2003;92(1):14.
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[8] Rosen KM, Rahimtoola SH, Gunnar RM, et al. Transient and persistent atrial standstill with His bundle lesions. Electrophysiologic and pathologic correlations. Circulation 1971;44:220. [9] Nakazato Y, Nakata Y, Hisaoka T, et al. Clinical and electrophysiological characteristics of atrial standstill. Pacing Clin Electrophysiol 1995;18:1244.
Partial atrial standstill: a case report Ergun Demiralp, MD, Ata Kirilmaz, MDT, Bekir Sitki Cebeci, MD, Rifat Eralp Ulusoy, MD Electrophysiology Section, Department of Cardiology, Gulhane Military Medical Academy Haydarpasa Training Hospital, 34668 Istanbul, Turkey Received 12 July 2004; revised 16 December 2004; accepted 13 January 2005
Abstract
Twelve-lead electrocardiograms revealed no atrial activity and a wide QRS escape rhythm at 38 beats/min in a 20-year-old man who presented with syncope. Doppler echocardiography documented the absence of A wave both in the tricuspid and mitral valve flow. The only mechanical activity was documented at the left atrial appendage. An electrophysiologic study demonstrated electrical inactivity in the right atrium and an atrial tachycardia in the left atrium. Atrial pacing with maximum output did not capture the atria. Our case represents an advanced stage of partial atrial standstill, with a mechanical and electrical atrial activity confined only to the left trial appendage. The patient remained asymptomatic after receiving a VVIR pacemaker and anticoagulation therapy. D 2005 Elsevier Inc. All rights reserved.
Keywords:
Atrial fibrillation; Atrial standstill; Electrophysiology; Atrial tachycardia
1. Introduction Atrial standstill (AS) is characterized by failure of atrial activity either spontaneously or in response to electrical stimulation. It has been reported in digitalis or quinidine toxicity, hypoxia, muscular dystrophia, valvular dysfunction, polymorphism of connexin 40, proscillaridin and verapamil intoxication, amyloidosis, ischemic heart disease, and acute coronary syndromes [1-7]. It is a rare arrhythmia diagnosed on the basis of specific electrocardiographic, electrophysiologic, and hemodynamic findings. Partial AS has also been reported. In this report, we describe a case of AS with a preserved mechanical and electrical activity confined only to the left atrial appendage. 2. Case presentation A 20-year-old man presented after his first syncopal episode. He complained of weakness, dyspnea on exertion, and frequent presyncopal events. Physical examination was unremarkable except for a grade 2/6 ejection systolic murmur at the mid left sternal border and bradycardia. Twelve-lead electrocardiogram (ECG) showed an escape T Corresponding author. Gulhane Askeri Tip Akademisi Haydarpasa Egitim Hastanesi Kardiyoloji Servisi, 34668 Istanbul, Turkey. Tel.: +90 216 5422473, +90 532 3307496; fax: +90 216 3487880. E-mail address: [email protected] (A. Kirilmaz). 0022-0736/$ – see front matter D 2005 Elsevier Inc. All rights reserved. doi:10.1016/j.jelectrocard.2005.01.009
distal rhythm at 33 beats/min with a QRS duration of 120 milliseconds but no atrial activity (Fig. 1). Chest x-ray revealed cardiomegaly with a prominent right atrial border. Echocardiography revealed a massively dilated right atrium (RA) with moderate dilation of the left atrium (LA) along with moderate tricuspid (TR) and mitral regurgitation (Fig. 2A). Although a Doppler study did not demonstrate any atrial activity (A wave) in the mitral and tricuspid inflow regions, fluttering of the left atrial appendage was documented both by tissue Doppler and by Doppler flow with transesophageal echocardiography (Fig. 2B and C). Pulmonary arterial pressure measured from TR by Doppler echocardiography was 35 mm Hg. The patient underwent an electrophysiologic study. A steerable decapolar coronary sinus catheter and a quadripolar diagnostic catheter were placed under fluoroscopic guidance via the right femoral vein. There was no electrical activity recorded from the RA. Left atrial activity recorded from the distal coronary sinus revealed an atrial tachycardia at a cycle length of 300 milliseconds (Fig. 3A and B). Neither the RA nor the LA could be paced with the highest voltage output (25 V at 2 milliseconds).
3. Discussion We report a case of AS coexisting with a dilated RA. Although the surface ECG failed to disclose any atrial
E. Demiralp et al. / Journal of Electrocardiology 38 (2005) 252–255
253
Fig. 1. Twelve-lead ECG at the presentation. No P wave was demonstrated in the ECG. Heart rate was 38 beats/min and QRS duration was measured 140 milliseconds.
electrical activity, the electrical and the mechanical activity of the left atrial appendage was detected by intracardiac electrography and Doppler recordings. Although a few autopsy reports have described the pathological changes in AS, fibroelastosis and fatty infiltration in the atria have been incriminated [8]. The high lateral RA is usually involved first with a progression toward the lower site of RA. Finally, atrial electrograms can be recorded only in the vicinity of the tricuspid valve or interatrial septum. This phenomenon was observed in most cases despite the different underlying diseases. On the basis of these observations, our case represents a relatively late stage of the disease process. In addition, in our case, the electrical activity was confined at most to the LA with only demonstrable mechanical activity confined to the left atrial appendage. In a study by Nakazato et al [9], the absence of P wave was observed in 6 of 11 patients with atrial standstill. They observed the loss of P waves in the remaining 5 patients in time with the progression of the underlying disease. The absence of His activity in our case also supports the advanced stage of disease. No systemic disease or causative agent was detected in our case, and the patient did not present any sign or symptom of a causative disease for years. Although we could not search for a possible genetic defect such as polymorphism of connexin 40, cardiac evaluation including echocardiography of the patient’s relatives did not reveal any cardiac abnormalities. In this case, partial AS assumed to be idiopathic has been confirmed by giant RA, progression of the disease process to the LA, and spared left atrial appendage documented by both echocardiography and intracardiac electrography.
Syncope, stroke, congestive heart failure, and sudden death are among the reported complications of AS. Therapeutic measures include long-term anticoagulation and implantation of a permanent pacemaker. Our patient
Fig. 2. A, Echocardiographic apical 4-chamber view. The RA diameter was 95 85 mm. B, Tissue Doppler flow taken from the left atrial appendage region. Arrows indicate the focal mechanical atrial activity with a frequency of 3/s. C, Transesophageal echocardiographic Doppler flow of the left atrial appendage, sampling volume marker (*) at the base of the appendage. Arrows indicate the blood flow of the left atrial appendage at a frequency of 3/s. LV indicates left ventricle, MV, mitral valve.
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E. Demiralp et al. / Journal of Electrocardiology 38 (2005) 252–255
Fig. 3. A, Surface and intracardiac electrograms demonstrating an atrial tachycardia with a cycle length of 301 milliseconds. The atrial activity recorded from the catheter located in the coronary sinus is very fractionated and long in duration (70 milliseconds). This pattern reflects the slowed and diseased propagation of the electrical activation in the LA. Electrogram recorded from the catheter located in the high RA reveals no right atrial activity but far-field ventricular signal. CS, electrogram recorded from the coronary sinus. A indicates atrial activity; V, ventricular activity. B, Fluoroscopic image in 408 left anterior oblique position showing the localization of the catheters during recording the electrograms in A. HRA indicates high RA; CS, coronary sinus; LAO, left anterior oblique.
received a VVIR pacemaker and anticoagulation and remained free of syncope. References [1] Bloomfield DA, Singlair-Smith BC. Persistent atrial standstill. Am J Med 1965;39:335.
[2] Wolff L, White P. Auricular standstill during quinidine therapy. Heart 1929;14:295. [3] Rosenbaum FF, Levine SA. Auricular standstill. Its occurrence and significance. Am J Med Sci 1939;198:774. [4] Magnusson P. Auricular standstill. Acta Med Scand 1946;123:519. [5] Koshimizu TA, Komori S, Ishihara T, et al. Restored atrial excitability after late recanalization in a patient with atrial standstill and acute myocardial infarction. Pacing Clin Electrophysiol 2002;25(2):217.
E. Demiralp et al. / Journal of Electrocardiology 38 (2005) 252–255 [6] Boriani G, Gallina M, Merlini L, et al. Clinical relevance of atrial fibrillation/flutter, stroke, pacemaker implant, and heart failure in Emery-Dreifuss muscular dystrophy: A Long-term Longitudinal Study. Stroke 2003;34:901. [7] Groenewegen WA, Firouzi M, Bezzina CR, et al. A cardiac sodium channel mutation cosegregates with a rare connexin40 genotype in familial atrial standstill. Circ Res 2003;92(1):14.
255
[8] Rosen KM, Rahimtoola SH, Gunnar RM, et al. Transient and persistent atrial standstill with His bundle lesions. Electrophysiologic and pathologic correlations. Circulation 1971;44:220. [9] Nakazato Y, Nakata Y, Hisaoka T, et al. Clinical and electrophysiological characteristics of atrial standstill. Pacing Clin Electrophysiol 1995;18:1244.