Clin. Cardiol. 14, 336-340 (1991)

D@G@Ophp3\ This srction edited by A . J . Cumm, M . D . , F. R.C.P., F. A. C.C.

The Pacemaker Syndrome: Old and New Causes H . SCHULLER. M

D

,

J . BRANDT,M

D

Department of Cardiothoracic Surgery, University Hospital, Lund, Sweden

Summary: The pacemaker syndrome refers to symptoms and signs in the pacemaker patient caused by inadequate timing of atrial and ventricular contractions. The lack of normal atrioventricular synchrony may result in decreased cardiac output and venous “cannon A waves.” A sudden increase in atrial pressure at the onset of asynchrony may elicit a systemic hypotensive reflex response. A wide range of symptoms can be observed. The pacemaker syndrome is encountered in a significant number of patients with ventricular (VVI) pacemakers, mostly when 1: 1 retrograde ventriculoatrial conduction is present. The risk of occurrence of the pacemaker syndrome is minimized if pacemaker systems are used which restore or maintain the normal atrioventricular contraction sequence. Hence, in sinus node disease, atrial stimulation with o r without ventricular stimulation should be employed, while in highgrade atrioventricular block dual-chamber pacing is recommended. The pacemaker syndrome is not restricted to the VVI stimulation mode. It can be seen, though rarely, in atrial and dual-chamber pacing, and an awareness of these new causes is necessary. An established pacemaker syndrome can often be counteracted by ad.justing the pulse generator function. Key words: pacemaker syndrome, retrograde conduction, pacemaker complications

Introduction A decade after the advent of the implantable cardiac pacemaker, it was realized that artificial stimulation of the heart is sometimes followed by worsening of the patient’s condition, despite normal pulse generator function. Mitsui and colleagues,’ as early as 1969, described patients who experienced adverse effects of ventricular pacing. The authors attributed this phenomenon to mismatching of the pacing rate with the patient’s physiological demands. Several subsequent reports of patients with similar problems2-llbrought recognition that these were the result of an asynchrony between atrial systole and pacemakerinitiated ventricular contractions. Such patients may experience palpitations and neck pulsations, and the asynchrony can result in low cardiac output, hypotension leading to neurologic symptoms, and congestive heart failure. A comprehensive review in 1985 by Ausubel and Furman’* defined the pacemaker syndrome as occurring only during single-chamber ventricular pacing. Recent technical developments within the field of cardiac pacing have been very rapid, resulting in the introduction of several different pacing modes. It has been recognized that identical hemodynamic situations may occur with stimulation modes other than ventricular pacing. Based on this, the pacemaker syndrome should be redefined in a somewhat broader aspect.

Definition The pacemaker syndrome refers to symptoms and signs present in the pacemaker patient, which are caused by inadequate timing of atrial and ventricular contractions. Address for reprints: Hans Schiiller, M.D. Department of Cardiothoracic Surgery University Hospital S-221 85 Lund. Sweden Received: December 27, 1990 Accepted January 3 , 1991

Hernodynamic Aspects When the normal atrioventricular ( A V ) contraction sequence is lost, cardiac output is typically acutely decreased by about 20% under resting conditions;I3-l6however, the

H. Schuller and J . Brandt: The pacemaker syndrome

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relative contribution of atrial systole to cardiac output time relation between atrial and ventricular activity may varies considerably between individuals. I 4 - l 6 Studies be at random, or fixed in the presence of retrograde VA evaluating the relative importance of atrial systole in disconduction (“negative AV synchrony”). The adverse eased hearts have yielded conflicting results. I 5 - I 8 hemodynamic effects of lack of the normal synchrony are If an atrial contraction occurs during ventricular sysmore pronounced in the presence of 1: 1 VA conductole, that is, when the atrioventricular valves are closed, tion,8.9.i9,23 and the risk of a patient experiencing the regurgitunt cannon A waves may occur in the atria, resultpacemaker syndrome is much higher in this ~ i t u a t i o n . ~ . ~ ing in a pressure increase in the systemic and pulmonary The prevalence of retrograde VA conduction in patients veins, with concomitant drops in right ventricular and left with total AV block is reported to be about 15%, while ventricular as well as arterial p r e ~ s u r e . ~ The , ~ , in~ ~ . ~ ~it is 20-36% in second-degree AV b l o ~ k .Among ~ ~ - ~pa~ creased atrial pressure caused by the cannon A waves may tients with sinus node disease, approximately 70% have counteract the normal baroreceptor-mediated vasopressor 1: 1 VA conduction during ventricular s t i m u l a t i ~ n . ~ ~ ~ ~ ~ ~ ~ response, resulting in aggravation of systemic hypotenAmong the patients with VVI pacemakers and VA consion.2n0-22 The extent of the arterial pressure drop varies duction, the pacemaker syndrome has been reported to ocindividuully and is obviously determined by several faccur in up to 40%.8.28 tors, such as the mechanical status of the atria, the body As VVI pacing cames a substantial risk of the pacemakposition, and the balance of the autonomic nervous syser syndrome, pacing modes retaining the atrioventricular tem. An abrupt decrease in arterial blood pressure with activation sequence are preferable. In the patient with AV block a dual-chamber pacemaker should be the first the onset of atrioventricular asynchrony is recognized as choice.29For treatment of the patient with symptomatic an important feature of the pacemaker syndrome. This hemodynamic response is illustrated in Figure 1 . The sinus node disease a pacemaker system with atrial stimuretrograde conduction causes an increase in central venous lation is r e ~ o m m e n d e d Furthermore, .~~ atrial pacing has pressure, and the arterial pressure drops substantially. Such been shown to result in lower incidences of permanent atrial fibrillation, thromboembolism, and congestive heart changes between adequate and inadequate timing of the failure. 31-33 Long-term follow-up has found this to result atrial and ventricular contractions may result in considerable distress, especially if they occur frequently. in a lower mortality than seen with VVI p a ~ i n g . ~ ~ , ~ ~ In the patient with a VVI pacemaker and established pacemaker syndrome, reoperation with change of pacing mode must be considered. If episodes of bradycardia are The Pacemaker Syndrome in Ventricular Pacing infrequent, unnecessary ventricular stimulation can be avoided by selecting a low pulse generator escape rate Ventricular pacing in high-degree AV block results in and/or by employing rate hysteresis. An attempt to supinadequate timing of atrial and ventricular activity. Atripress VA conduction by means of antiarrhythmic drugs oventricular asynchrony without AV block occurs in the has been suggested.28 presence of sinus rhythm and competitive VVI pacing. VVI pacing remains the most commonly used stimulaIn both ventricular-paced AV block and sinus rhythm, the tion mode in the treatment of patients with sinus node disease.34This is remarkable in light of the numerous reports in the literature and presentations at pacemaker meetings regarding the negative influence of VVI pacing, especially when VA conduction prevails.35Thus, the main and earliest defined cause of pacemaker syndrome continues to preECG vail to an unnecessary extent.

The Pacemaker Syndrome in Atrial Pacing AP

CVP

FIG I Variation in arterial (AP) and central venous (CVP) pressure i n relation to changes between sinus and ventricular-paced rhythm.

In atrial pacing, a considerable delay between the atrial pacing stimulus and the ventricular contraction may result in the next atrial stimulus falling during the mechanical ventricular systole (Fig. 2). The atrial contraction against closed AV valves will then result in the hemodynamic effects described above. In atrial-inhibited (AAI) pacing at standard basic rates this is an unlikely scenario, as it would demand an extreme spike-QRS interval. the phenomenon has been described a few cases of rate-ahptive atrial ( M - R ) pacing, when the atrial stimulation rate is i n c r e a ~ e d .Normally, ~ ~ - ~ ~ a heart rate

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....

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FIG 2 Atrial pacing at 120 inipimin, resulting in a spike-Q interval of 320 ms.

increase during exercise is accompanied by enhanced AV conduction.39If the increase in atrial pacing rate is disproportionate to the degree of exercise, or if AV conduction is depressed due to conduction system disorders or drug effects, the pacemaker syndrome may result. It follows that AAI-R pacing demands normal or nearnormal AV conduction, and this factor should be evaluated prior to or during pacemaker implantation. If AV conduction is impaired, a dual-chamber rate-adaptive (DDD-R or DDI-R) system is indicated. In AAI-R pacing, careful matching of the pacing rate increase with the degree of exercise is mandatory, and drugs with negative effects on AV conduction should be used with caution. With these precautions, AAI-R pacing has been shown to be a successful treatment with a low risk of complications in selected patients with sinus node d i s e a ~ e . ~ ~ , ~ ’

Pacemaker Syndrome in Dual-Chamber Pacing Does the use of dual-chamber systems eliminate the risk of pacemaker syndrome completely? It is obvious that some malfunctions may result in this syndrome: for example, in atrial exit block when the subsequent ventricular stimulation is followed by retrograde VA conduction. Unfortunately, not even a dual-chamber system with normal function will maintain or restore AV synchrony in every instance. Hence, some new causes for the pacemaker syndrome must be recognized. Under certain circumstances, a DDD unit, functioning in accordance with the technical specifications and program setting and without lead-related problems, may perform functionally like a VVI unit.42The condition for this is the presence of VA conduction and critical timing in relation to the programmed duration of the postventricular atrial refractory period (PVARP) and basic rate interval. A retrograde or ectopic P wave occurring during an ongoing PVARP remains unsensed, thus obviating a subsequent ventricular triggering. However, the P wave is followed by a period of atrial myocardial refractoriness. If the next atrial pacing stimulus is delivered during this period, a new atrial depolarization cannot be accomplished, a situation which can be referred to as “pseudo atrial exit block” (Fig. 3). Should the following ventricular stimulation cause retrograde VA conduction, this se-

FIG.3 Dual-chamber pacing at 80 imp/min. Retrograde P waves and atrial stimulation during atrial refractoriness (“pseudo atrial exit block”).

FIG.4 DDI mode, 60 implmin. Myopotential sensing via the atrial lead inhibits the atrial output, as do the subsequent retrogradely conducted P waves as they occur after the end of the PVARP (275 n i h ) .

uence of events may become repetitive. While retrograde P waves sensed after the end of a PVARP cause the wellknown pacemaker-mediated endless loop tachycardia (PMT),43 P waves within a PVARP can result in functional ventricular stimulation at the baseline rate with continued VA conduction. A similar situation may arise in DDI systems when a retrograde P wave occurs after the end of the PVARP, inhibiting the atrial channel (Fig. 4). The combination of a long technical AV delay and a relatively high baseline rate promotes the occurrence of this phenomenon. The DDI system will, under these circumstances, act as a VVI unit due to the repetitive retrograde c o n d ~ c t i o n . ~ ~ The risk for these functional problems due to VA conduction in dual-chamber pacing demands an awareness of the problem. Counteraction is usually possible by adjusting the pulse generator program. The presence of VA conduction and VA conduction intervals should be evaluated both at primary implantation and during follow-up, as VA conduction characteristics may change with time.45 In modem microprocessor-based D D D systems, special VA conduction test programs should be implemented for convenient and comprehensive evaluation during follow-up. A further step would be automatic adaption of dual-chamber units to problems such as loss of atrial capture and the consequences of retrogradely conducted P waves. The theory that changes between different atrial rhythms induce pacemaker syndrome is elucidated by the clinical observation of symptom variations after His bundle ablation in patients with paroxysmal atrial tachycardias. These patients usually are equipped with VVI-R pacemaker systems. A successful ablation precludes conducted tachycardias, and a well-functioning VVI-R system will guarantee a satisfactory ventricular rate adaption to exercise.

H. Schiiller and J . Brandt: The pacemaker syndrome

However, some patients may experience symptoms when atrial tachyarrhythmias spontaneously convert to normal atrial activity. This probably is due to the variation of stroke volume and intermittent cannon A waves caused by thc random occurrence of atrial contractions. In this patient group, the pacemaker syndrome can be obviated by using dual-chamber units that automatically shift from atrial-triggered ventricular stimulation to VVI-R pacing in the event of atrial fibrillation or flutter; when the tachyanhythmia ceases, the unit reverts to the atrialtriggered mode.46

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The pacemaker syndrome: old and new causes.

The pacemaker syndrome refers to symptoms and signs in the pacemaker patient caused by inadequate timing of atrial and ventricular contractions. The l...
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