Electrical activation of the heart normally originates in the sinoatrial (SA) node, the predominant pacemaker. Other subsidiary pacemakers in the atrioventricular (AV) node, specialized conducting system, and muscle may initiate electrical activation if the SA node is dysfunctional or suppressed. Typically, subsidiary pacemakers discharge at a slower rate and, in the absence of an appropriate increase in stroke volume, may result in tissue hypoperfusion.
Spontaneous activation and contraction of the heart are a consequence of the specialized pacemaking tissue in these anatomic locales. As described in Chap. 231, action potentials in the heart are regionally heterogeneous. The action potentials in cells isolated from nodal tissue are distinct from those recorded from atrial and ventricular myocytes (Fig. 232-1). The complement of ionic currents present in nodal cells results in a less negative resting membrane potential compared with atrial or ventricular myocytes. Electrical diastole in nodal cells is characterized by slow diastolic depolarization (phase 4), which generates an action potential as the membrane voltage reaches threshold. The action potential upstrokes (phase 0) are slow compared with atrial or ventricular myocytes, being mediated by calcium rather than sodium current. Cells with properties of SA and AV nodal tissue are electrically connected to the remainder of the myocardium by cells with an electrophysiologic phenotype between that of nodal cells and that of atrial or ventricular myocytes. Cells in the SA node exhibit the most rapid phase 4 depolarization and thus are the dominant pacemakers in a normal heart.
Action potential profiles recorded in cells isolated from sinoatrial or atrioventricular nodal tissue compared with those of cells from atrial or ventricular myocardium. Nodal cell action potentials exhibit more depolarized resting membrane potentials, slower phase 0 upstrokes, and phase 4 diastolic depolarization.
Bradycardia results from a failure of either impulse initiation or impulse conduction. Failure of impulse initiation may be caused by depressed automaticity resulting from a slowing or failure of phase 4 diastolic depolarization (Fig. 232-2), which may result from disease or exposure to drugs. Prominently, the autonomic nervous system modulates the rate of phase 4 diastolic depolarization and thus the firing rate of both primary (SA node) and subsidiary pacemakers. Failure of conduction of an impulse from nodal tissue to atrial or ventricular myocardium may produce bradycardia as a result of exit block. Conditions that alter the activation and connectivity of cells (e.g., fibrosis) in the heart may result in failure of impulse conduction.
Schematics of nodal action potentials and the currents that contribute to phase 4 depolarization. Relative increases in depolarizing L- (ICa-L) and T- (ICa-T) type calcium and pacemaker currents (If) along with a reduction in repolarizing inward rectifier (IK1) and delayed rectifier (IK) potassium currents result in depolarization. Activation of ACh-gated (IKACh) potassium current and beta blockade slow the rate of phase 4 and decrease the pacing rate. (Modified from J Jalife et al: Basic Cardiac Electrophysiology for the Clinician, Blackwell Publishing, 1999.
SA node dysfunction and AV conduction block are the most common causes of pathologic bradycardia. SA node dysfunction may be difficult to distinguish from physiologic sinus bradycardia, particularly in the young. SA node dysfunction increases in frequency between the fifth and sixth decades of life and should be considered in patients with fatigue, exercise intolerance, or syncope and sinus bradycardia. Transient AV block is common in the young and probably is a result of the high vagal tone found in up to 10% of young adults. Acquired and persistent failure of AV conduction is decidedly rare in healthy adult populations, with an estimated incidence of ~200/million population per year.
Permanent pacemaking is the only reliable therapy for symptomatic bradycardia in the absence of extrinsic and reversible etiologies such as increased vagal tone, hypoxia, hypothermia, and drugs (Table 232-1). Approximately 50% of the 150,000 permanent pacemakers implanted in the United States and 20–30% of the 150,000 of those in Europe were implanted for SA node disease.
Table 232–1. Etiologies of SA Node Dysfunction |Favorite Table|Download (.pdf)
Table 232–1. Etiologies of SA Node Dysfunction
|Autonomic||Sick-sinus syndrome (SSS)|
|Carotid sinus hypersensitivity ||Coronary artery disease (chronic and acute MI) |
|Vasovagal (cardioinhibitory) stimulation||Inflammatory|
|Beta blockers||Myocarditis (including viral)|
|Calcium channel blockers||Rheumatic heart disease|
|Digoxin||Collagen vascular diseases|
|Antiarrhythmics (class I and III)||Lyme disease|
|Clonidine (other sympatholytics)||Congenital heart disease|
|Lithium carbonate||TGA/Mustard and Fontan repairs |
|Narcotics (methadone) ||Chest trauma|
|Hypothyroidism||AD SSS, OMIM #163800 (15q24-25)|
|Sleep apnea Hypoxia||AR SSS, OMIM #608567 (3p21)|
|Endotracheal suctioning (vagal maneuvers)||SA node disease with myopia, OMIM 182190|
|Hypothermia||Kearns-Sayre syndrome, OMIM #530000|
|Increased intracranial pressure||Myotonic dystrophy|
|Type 1, OMIM #160900 (19q13.2-13.3) |
|Type 2, OMIM #602668 (3q13.3-q24) |
|Friedreich's ataxia, OMIM #229300 9q13, 9p23-p11)|
Structure and Physiology of the Sa Node
The SA node is composed of a cluster of small fusiform cells in the sulcus terminalis on the epicardial surface of the heart at the right atrial–superior vena caval junction, where they envelop the SA nodal artery. The SA node is structurally heterogeneous, but the central prototypic nodal cells have fewer distinct myofibrils than does the surrounding atrial myocardium, no intercalated disks visible on light microscopy, a poorly developed sarcoplasmic reticulum, and no T-tubules. Cells in the peripheral regions of the SA node are transitional in both structure and function. The SA nodal artery arises from the right coronary artery in 55–60% and the left circumflex artery in 40–45% of persons. The SA node is richly innervated by sympathetic and parasympathetic nerves and ganglia.
Irregular and slow propagation of impulses from the SA node can be explained by the electrophysiology of nodal cells and the structure of the SA node itself. The action potentials of SA nodal cells are characterized by a relatively depolarized membrane potential (Fig. 232-1) of −40 to −60 mV, slow phase 0 upstroke, and relatively rapid phase 4 diastolic depolarization compared with the action potentials recorded in cardiac muscle cells. The relative absence of inward rectifier potassium current (IK1) accounts for the depolarized membrane potential; the slow upstroke of phase 0 results from the absence of available fast sodium current (INa) and is mediated by L-type calcium current (ICa-L); and phase 4 depolarization is a result of the aggregate activity of a number of ionic currents. Prominently, both L- and T-type (ICa-T) calcium currents, the pacemaker current (so-called funny current, or If) formed by the tetramerization of hyperpolarization-activated cyclic nucleotide-gated channels, and the electrogenic sodium-calcium exchanger provide depolarizing current that is antagonized by delayed rectifier (IKr) and acetylcholine-gated (IKACh) potassium currents. ICa-L, ICa-T, and If are modulated by β-adrenergic stimulation and IKACh by vagal stimulation, explaining the exquisite sensitivity of diastolic depolarization to autonomic nervous system activity. The slow conduction within the SA node is explained by the absence of INa and poor electrical coupling of cells in the node, resulting from sizable amounts of interstitial tissue and a low abundance of gap junctions. The poor coupling allows for graded electrophysiologic properties within the node, with the peripheral transitional cells being silenced by electrotonic coupling to atrial myocardium.
Etiology of Sa Nodal Disease
SA nodal dysfunction has been classified as intrinsic or extrinsic. The distinction is important because extrinsic dysfunction is often reversible and generally should be corrected before pacemaker therapy is considered (Table 232-1). The most common causes of extrinsic SA node dysfunction are drugs and autonomic nervous system influences that suppress automaticity and/or compromise conduction. Other extrinsic causes include hypothyroidism, sleep apnea, and conditions likely to occur in critically ill patients such as hypothermia, hypoxia, increased intracranial pressure (Cushing's response), and endotracheal suctioning via activation of the vagus nerve.
Intrinsic sinus node dysfunction is degenerative and often is characterized pathologically by fibrous replacement of the SA node or its connections to the atrium. Acute and chronic coronary artery disease (CAD) may be associated with SA node dysfunction, although in the setting of acute myocardial infarction (MI; typically inferior), the vabnormalities are transient. Inflammatory processes may alter SA node function, ultimately producing replacement fibrosis. Pericarditis, myocarditis, and rheumatic heart disease have been associated with SA nodal disease with sinus bradycardia, sinus arrest, and exit block. Carditis associated with systemic lupus erythematosus (SLE), rheumatoid arthritis (RA), and mixed connective tissue disorders (MCTDs) may also affect SA node structure and function. Senile amyloidosis is an infiltrative disorder in patients typically in the ninth decade of life; deposition of amyloid protein in the atrial myocardium can impair SA node function. Some SA node disease is iatrogenic and results from direct injury to the SA node during cardiothoracic surgery.
Rare heritable forms of sinus node disease have been described, and several have been characterized genetically. Autosomal dominant sinus node dysfunction in conjunction with supraventricular tachycardia [(i.e., tachycardia-bradycardia variant of sick-sinus syndrome (SSS)] has been linked to mutations in the pacemaker current (If) subunit gene HCN4 on chromosome 15. An autosomal recessive form of SSS with the prominent feature of atrial inexcitability and absence of P waves on the electrocardiogram (ECG) is caused by mutations in the cardiac sodium channel gene, SCN5A, on chromosome 3. SSS associated with myopia has been described but not genetically characterized. There are several neuromuscular diseases, including Kearns-Sayre syndrome (ophthalmoplegia, pigmentary degeneration of the retina, and cardiomyopathy) and myotonic dystrophy, that have a predilection for the conducting system and SA node.
SSS in both the young and the elderly is associated with an increase in fibrous tissue in the SA node. The onset of SSS may be hastened by coexisting disease, such as CAD, diabetes mellitus, hypertension, and valvular diseases and cardiomyopathies.
Clinical Features of Sa Node Disease
SA node dysfunction may be completely asymptomatic and manifest as an ECG anomaly such as sinus bradycardia; sinus arrest and exit block; or alternating supraventricular tachycardia, usually atrial fibrillation, and bradycardia. Symptoms associated with SA node dysfunction, in particular tachycardia-bradycardia syndrome, may be related to both slow and fast heart rates. For example, tachycardia may be associated with palpitations, angina pectoris, and heart failure, and bradycardia may be associated with hypotension, syncope, presyncope, fatigue, and weakness. In the setting of SSS, overdrive suppression of the SA node may result in prolonged pauses and syncope upon termination of the tachycardia. In many cases, symptoms associated with SA node dysfunction result from concomitant cardiovascular disease. A significant minority of patients with SSS develop signs and symptoms of heart failure that may be related to slow or fast heart rates.
One-third to one-half of patients with SA node dysfunction develop supraventricular tachycardia, usually atrial fibrillation or atrial flutter. The incidence of persistent atrial fibrillation in patients with SA node dysfunction increases with advanced age, hypertension, diabetes mellitus, left ventricular dilation, valvular heart disease, and ventricular pacing. Remarkably, some symptomatic patients may experience an improvement in symptoms with the development of atrial fibrillation, presumably from an increase in their average heart rate. Patients with the tachycardia-bradycardia variant of SSS, similar to patients with atrial fibrillation, are at risk for thromboembolism, and those at greatest risk, including patients ≥65 years and patients with a prior history of stroke, valvular heart disease, left ventricular dysfunction, or atrial enlargement, should be treated with anticoagulants. Up to one-quarter of patients with SA node disease will have concurrent AV conduction disease, although only a minority will require specific therapy for high-grade AV block.
The natural history of SA node dysfunction is one of varying intensity of symptoms even in patients who present with syncope. Symptoms related to SA node dysfunction may be significant, but overall mortality usually is not compromised in the absence of other significant comorbid conditions. These features of the natural history need to be taken into account in considering therapy for these patients.
Electrocardiography of Sa Node Disease
The electrocardiographic manifestations of SA node dysfunction include sinus bradycardia, sinus pauses, sinus arrest, sinus exit block, tachycardia (in SSS), and chronotropic incompetence. It is often difficult to distinguish pathologic from physiologic sinus bradycardia. By definition, sinus bradycardia is a rhythm driven by the SA node with a rate of <60 beats/min; sinus bradycardia is very common and typically benign. Resting heart rates <60 beats/min are very common in young healthy individuals and physically conditioned subjects. A sinus rate of <40 beats/min in the awake state in the absence of physical conditioning generally is considered abnormal. Sinus pauses and sinus arrest result from failure of the SA node to discharge, producing a pause without P waves visible on the ECG (Fig. 232-3). Sinus pauses of up to 3 s are common in awake athletes, and pauses of this duration or longer may be observed in asymptomatic elderly subjects. Intermittent failure of conduction from the SA node produces sinus exit block. The severity of sinus exit block may vary in a manner similar to that of AV block (see below). Prolongation of conduction from the sinus node will not be apparent on the ECG; second-degree SA block will produce intermittent conduction from the SA node and a regularly irregular atrial rhythm.
Sinus slowing and pauses on the ECG. The ECG is recorded during sleep in a young patient without heart disease. The heart rate before the pause is slow, and the PR interval is prolonged, consistent with an increase in vagal tone. The P waves have a morphology consistent with sinus rhythm. The recording is from a two-lead telemetry system in which the tracing labeled II mimics frontal lead II and V represents Modified Central Lead 1, which mimics lead V1 of the standard 12-lead ECG.
Type I second-degree SA block results from progressive prolongation of SA node conduction with intermittent failure of the impulses originating in the sinus node to conduct to the surrounding atrial tissue. Second-degree SA block appears on the ECG as an intermittent absence of P waves (Fig. 232-4). In type II second-degree SA block, there is no change in SA node conduction before the pause. Complete or third-degree SA block results in no P waves on the ECG. Tachycardia-bradycardia syndrome is manifest as alternating sinus bradycardia and atrial tachyarrhythmias. Although atrial tachycardia, atrial flutter, and atrial fibrillation may be observed, the latter is the most common tachycardia. Chronotropic incompetence is the inability to increase the heart rate in response to exercise or other stress appropriately and is defined in greater detail below.
Mobitz type I SA nodal exit block. A theoretical SA node electrogram (SAN EG) is shown. Note that there is grouped beating producing a regularly irregular heart rhythm. The SA node EG rate is constant with progressive delay in exit from the node and activation of the atria, inscribing the P wave. This produces subtly decreasing P-P intervals before the pause, and the pause is less than twice the cycle length of the last sinus interval.
SA node dysfunction is most commonly a clinical or electrocardiographic diagnosis. Sinus bradycardia or pauses on the resting ECG are rarely sufficient to diagnose SA node disease, and longer-term recording and symptom correlation generally are required. Symptoms in the absence of sinus bradyarrhythmias may be sufficient to exclude a diagnosis of SA node dysfunction.
Electrocardiographic recording plays a central role in the diagnosis and management of SA node dysfunction. Despite the limitations of the resting ECG, longer-term recording employing Holter or event monitors may permit correlation of symptoms with the cardiac rhythm. Many contemporary event monitors may be automatically triggered to record the ECG when certain programmed heart rate criteria are met. Implantable ECG monitors permit long-term recording (12–18 months) in particularly challenging patients.
Failure to increase the heart rate with exercise is referred to as chronotropic incompetence. This is alternatively defined as failure to reach 85% of predicted maximal heart rate at peak exercise or failure to achieve a heart rate >100 beats/min with exercise or a maximal heart rate with exercise less than two standard deviations below that of an age-matched control population. Exercise testing may be useful in discriminating chronotropic incompetence from resting bradycardia and may aid in the identification of the mechanism of exercise intolerance.
Autonomic nervous system testing is useful in diagnosing carotid sinus hypersensitivity; pauses >3 s are consistent with the diagnosis but may be present in asymptomatic elderly subjects. Determining the intrinsic heart rate (IHR) may distinguish SA node dysfunction from slow heart rates that result from high vagal tone. The normal IHR after administration of 0.2 mg/kg propranolol and 0.04 mg/kg atropine is 117.2 − (0.53 × age) in beats/min; a low IHR is indicative of SA disease.
Electrophysiologic testing may play a role in the assessment of patients with presumed SA node dysfunction and in the evaluation of syncope, particularly in the setting of structural heart disease. In this circumstance, electrophysiologic testing is used to rule out more malignant etiologies of syncope, such as ventricular tachyarrhythmias and AV conduction block. There are several ways to assess SA node function invasively. They include the sinus node recovery time (SNRT), defined as the longest pause after cessation of overdrive pacing of the right atrium near the SA node (normal: <1500 ms or, corrected for sinus cycle length, <550 ms), and the sinoatrial conduction time (SACT), defined as one-half the difference between the intrinsic sinus cycle length and a noncompensatory pause after a premature atrial stimulus (normal <125 ms). The combination of an abnormal SNRT, an abnormal SACT, and a low IHR is a sensitive and specific indicator of intrinsic SA node disease.
Treatment: Sinoatrial Node Dysfunction
Since SA node dysfunction is not associated with increased mortality rates, the aim of therapy is alleviation of symptoms. Exclusion of extrinsic causes of SA node dysfunction and correlation of the cardiac rhythm with symptoms is an essential part of patient management. Pacemaker implantation is the primary therapeutic intervention in patients with symptomatic SA node dysfunction. Pharmacologic considerations are important in the evaluation and management of patients with SA nodal disease. A number of drugs modulate SA node function and are extrinsic causes of dysfunction (Table 232-1). Beta blockers and calcium channel blockers increase SNRT in patients with SA node dysfunction, and antiarrhythmic drugs with class I and III action may promote SA node exit block. In general, such agents should be discontinued before decisions regarding the need for permanent pacing in patients with SA node disease are made. Chronic pharmacologic therapy for sinus bradyarrhythmias is limited. Some pharmacologic agents may improve SA node function; digitalis, for example, has been shown to shorten SNRT in patients with SA node dysfunction. Isoproterenol or atropine administered IV may increase the sinus rate acutely. Theophylline has been used both acutely and chronically to increase heart rate but has liabilities when used in patients with tachycardia-bradycardia syndrome, increasing the frequency of supraventricular tachyarrhythmias, and in patients with structural heart disease, increasing the risk of potentially serious ventricular arrhythmias. At the current time, there is only a single randomized study of therapy for SA node dysfunction. In patients with resting heart rates <50 and >30 beats/min on a Holter monitor, patients who received dual-chamber pacemakers experienced significantly fewer syncopal episodes and had symptomatic improvement compared with patients randomized to theophylline or no treatment.
In certain circumstances, sinus bradycardia requires no specific treatment or only temporary rate support. Sinus bradycardia is common in patients with acute inferior or posterior MI and can be exacerbated by vagal activation induced by pain or the use of drugs such as morphine. Ischemia of the SA nodal artery probably occurs in acute coronary syndromes more typically with involvement with the right coronary artery, and even with infarction, the effect on SA node function most often is transient.
Sinus bradycardia is a prominent feature of carotid sinus hypersensitivity and neurally mediated hypotension associated with vasovagal syncope that responds to pacemaker therapy. Carotid hypersensitivity with recurrent syncope or presyncope associated with a predominant cardioinhibitory component responds to pacemaker implantation. Several randomized trials have investigated the efficacy of permanent pacing in patients with drug-refractory vasovagal syncope, with mixed results. Although initial trials suggested that patients undergoing pacemaker implantation have fewer recurrences and a longer time to recurrence of symptoms, at least one follow-up study did not confirm these results.
The details of pacing modes and indications for pacing in SA node dysfunction are discussed below.
Atrioventricular Conduction Disease
Structure and Physiology of the AV Node
The AV conduction axis is structurally complex, involving the atria and ventricles as well as the AV node. Unlike the SA node, the AV node is a subendocardial structure originating in the transitional zone, which is composed of aggregates of cells in the posterior-inferior right atrium. Superior, medial, and posterior transitional atrionodal bundles converge on the compact AV node. The compact AV node (~1 × 3 × 5 mm) is situated at the apex of the triangle of Koch, which is defined by the coronary sinus ostium posteriorly, the septal tricuspid valve annulus anteriorly, and the tendon of Todaro superiorly. The compact AV node continues as the penetrating AV bundle where it immediately traverses the central fibrous body and is in close proximity to the aortic, mitral, and tricuspid valve annuli; thus, it is subject to injury in the setting of valvular heart disease or its surgical treatment. The penetrating AV bundle continues through the annulus fibrosis and emerges along the ventricular septum adjacent to the membranous septum as the bundle of His. The right bundle branch (RBB) emerges from the distal AV bundle in a band that traverses the right ventricle (moderator band). In contrast, the left bundle branch (LBB) is a broad subendocardial sheet of tissue on the septal left ventricle. The Purkinje fiber network emerges from the RBB and LBB and extensively ramifies on the endocardial surfaces of the right and left ventricles, respectively.
The blood supply to the penetrating AV bundle is from the AV nodal artery and first septal perforator of the left anterior descending coronary artery. The bundle branches also have a dual blood supply from the septal perforators of the left anterior descending coronary artery and branches of the posterior descending coronary artery. The AV node is highly innervated with postganglionic sympathetic and parasympathetic nerves. The bundle of His and distal conducting system are minimally influenced by autonomic tone.
The cells that constitute the AV node complex are heterogeneous with a range of action potential profiles. In the transitional zones, the cells have an electrical phenotype between those of atrial myocytes and cells of the compact node (Fig. 232-1). Atrionodal transitional connections may exhibit decremental conduction, defined as slowing of conduction with increasingly rapid rates of stimulation. Fast and slow AV nodal pathways have been described, but it is controversial whether these two types of pathway are anatomically distinct or represent functional heterogeneities in different regions of the AV nodal complex. Myocytes that constitute the compact node are depolarized (resting membrane potential ~−60 mV) and exhibit action potentials with low amplitudes, slow upstrokes of phase 0 (<10 V/s), and phase 4 diastolic depolarization; high-input resistance; and relative insensitivity to external [K+]. The action potential phenotype is explained by the complement of ionic currents expressed. AV nodal cells lack IK1 and INa; ICa-L is responsible for phase 0; and phase 4 depolarization reflects the composite activity of the depolarizing currents If, ICa-L, ICa-T, and INCX and the repolarizing currents IKr and IKACh. Electrical coupling between cells in the AV node is tenuous due to the relatively sparse expression of gap junction channels (predominantly connexin-40) and increased extracellular volume.
The His bundle and the bundle branches are insulated from ventricular myocardium. The most rapid conduction in the heart is observed in these tissues. The action potentials exhibit very rapid upstrokes (phase 0), prolonged plateaus (phase 2), and modest automaticity (phase 4 depolarization). Gap junctions, composed largely of connexin-40, are abundant, but bundles are poorly connected transversely to ventricular myocardium.
Etiology of AV Conduction Disease
Conduction block from the atrium to the ventricle can occur for a variety of reasons in a number of clinical situations, and AV conduction block may be classified in a number of ways. The etiologies may be functional or structural, in part analogous to extrinsic and intrinsic causes of SA nodal dysfunction. The block may be classified by its severity from first to third degree or complete AV block or by the location of block within the AV conduction system. Table 232-2 summarizes the etiologies of AV conduction block. Those which are functional (autonomic, metabolic/endocrine, and drug-related) tend to be reversible. Most other etiologies produce structural changes, typically fibrosis, in segments of the AV conduction axis that are generally permanent. Heightened vagal tone during sleep or in well-conditioned individuals can be associated with all grades of AV block. Carotid sinus hypersensitivity, vasovagal syncope, and cough and micturition syncope may be associated with SA node slowing and AV conduction block. Transient metabolic and endocrinologic disturbances as well as a number of pharmacologic agents also may produce reversible AV conduction block.
Table 232–2. Etiologies of Atrioventricular Block |Favorite Table|Download (.pdf)
Table 232–2. Etiologies of Atrioventricular Block
|Carotid sinus hypersensitivity||Vasovagal|
|Calcium channel blockers||Antiarrhythmics (class I and III)|
Congenital heart disease
|Facioscapulohumeral MD, OMIM #158900 (4q35)|
|Kearns-Sayre syndrome, OMIM #530000||Emery-Dreifuss MD, OMIM #310300 (Xq28)|
Type 1, OMIM #160900 (19q13.2-13.3)
Type 2, OMIM #602668 (3q13.3-q24)
|Progressive familial heart block, OMIM #113900 (19q13.2-q13.3, 3p21)|
|Lev disease||Lenègre disease|
|Coronary Artery Disease|
Several infectious diseases have a predilection for the conducting system. Lyme disease may involve the heart in up to 50% of cases; 10% of patients with Lyme carditis develop AV conduction block, which is generally reversible but may require temporary pacing support. Chagas' disease, which is common in Latin America, and syphilis may produce more persistent AV conduction disturbances. Some autoimmune and infiltrative diseases may produce AV conduction block, including SLE, RA, MCTD, scleroderma, amyloidosis (primary and secondary), sarcoidosis, and hemochromatosis; rare malignancies also may impair AV conduction.
Idiopathic progressive fibrosis of the conduction system is one of the more common and degenerative causes of AV conduction block. Aging is associated with degenerative changes in the summit of the ventricular septum, central fibrous body, and aortic and mitral annuli and has been described as "sclerosis of the left cardiac skeleton." The process typically begins in the fourth decade of life and may be accelerated by atherosclerosis, hypertension, and diabetes mellitus. Accelerated forms of progressive familial heart block have been identified in families with mutations in the cardiac sodium channel gene (SCN5A) and other loci that have been mapped to chromosomes 1 and 19.
AV conduction block has been associated with heritable neuromuscular diseases, including the nucleotide repeat disease myotonic dystrophy, the mitochondrial myopathy Kearns-Sayre syndrome (Chap. 387), and several of the monogenic muscular dystrophies. Congenital AV block may be observed in complex congenital cardiac anomalies (Chap. 236), such as transposition of the great arteries, ostium primum atrial septal defects (ASDs), ventricular septal defects (VSDs), endocardial cushion defects, and some single-ventricle defects. Congenital AV block in the setting of a structurally normal heart has been seen in children born to mothers with SLE. Iatrogenic AV block may occur during mitral or aortic valve surgery, rarely in the setting of thoracic radiation, and as a consequence of catheter ablation. AV block is a decidedly rare complication of the surgical repair of VSDs or ASDs but may complicate repairs of transposition of the great arteries.
CAD may produce transient or persistent AV block. In the setting of coronary spasm, ischemia, particularly in the right coronary artery distribution, may produce transient AV block. In acute MI, AV block transiently develops in 10–25% of patients; most commonly this is first- or second-degree AV block, but complete heart block (CHB) may also occur. Second-degree and higher-grade AV block tends to occur more often in inferior than in anterior acute MI; however, the level of block in inferior MI tends to be in the AV node with more stable, narrow escape rhythms. In contrast, acute anterior MI is associated with block in the distal AV nodal complex, His bundle, or bundle branches and results in wide complex, unstable escape rhythms and a worse prognosis with high mortality rates.
Electrocardiography and Electrophysiology of AV Conduction Block
Atrioventricular conduction block typically is diagnosed electrocardiographically, which characterizes the severity of the conduction disturbance and allows one to draw inferences about the location of the block. AV conduction block manifests as slow conduction in its mildest forms and failure to conduct, either intermittent or persistently, in more severe varieties. First-degree AV block (PR interval >200 ms) is a slowing of conduction through the AV junction (Fig. 232-5). The site of delay is typically in the AV node but may be in the atria, bundle of His, or His-Purkinje system. A wide QRS is suggestive of delay in the distal conduction system, whereas a narrow QRS suggests delay in the AV node proper or, less commonly, in the bundle of His. In second-degree AV block there is an intermittent failure of electrical impulse conduction from atrium to ventricle. Second-degree AV block is subclassified as Mobitz type I (Wenckebach) or Mobitz type II. The periodic failure of conduction in Mobitz type I block is characterized by a progressively lengthening PR interval, shortening of the RR interval, and a pause that is less than two times the immediately preceding RR interval on the ECG. The ECG complex after the pause exhibits a shorter PR interval than that immediately preceding the pause (Fig. 232-6). This ECG pattern most often arises because of decremental conduction of electrical impulses in the AV node.
First-degree AV block with slowing of conduction in the AV node as indicated by the prolonged atrial-to-His bundle electrogram (AH) interval, in this case 157 ms. The His bundle-to-earliest ventricular activation on the surface ECG (HV) interval is normal. The normal HV interval suggests normal conduction below the AV node to the ventricle. I and V1 are surface ECG leads, HIS is the recording of the endocavitary electrogram at the His bundle position. A, H, and V are labels for the atrial, His bundle, and right ventricular electrograms, respectively.
Mobitz type I second-degree AV block. The PR interval prolongs before the pause, as shown in the ladder diagram. The ECG pattern results from slowing of conduction in the AV node.
It is important to distinguish type I from type II second-degree AV nodal block because the latter has more serious prognostic implications. Type II second-degree AV block is characterized by intermittent failure of conduction of the P wave without changes in the preceding PR or RR intervals. When AV block is 2:1, it may be difficult to distinguish type I from type II block. Type II second-degree AV block typically occurs in the distal or infra-His conduction system, is often associated with intraventricular conduction delays (e.g., bundle branch block), and is more likely to proceed to higher grades of AV block than is type I second-degree AV block. Second-degree AV block (particularly type II) may be associated with a series of nonconducted P waves, referred to as paroxysmal AV block (Fig. 232-7), and implies significant conduction system disease and is an indication for permanent pacing. Complete failure of conduction from atrium to ventricle is referred to as complete or third-degree AV block. AV block that is intermediate between second degree and third degree is referred to as high-grade AV block and, as with CHB, implies advanced AV conduction system disease. In both cases, the block is most often distal to the AV node, and the duration of the QRS complex can be helpful in determining the level of the block. In the absence of a preexisting bundle branch block, a wide QRS escape rhythm (Fig. 232-8B) implies a block in the distal His or bundle branches; in contrast, a narrow QRS rhythm implies a block in the AV node or proximal His and an escape rhythm originating in the AV junction (Fig. 232-8A). Narrow QRS escape rhythms are typically faster and more stable than wide QRS escape rhythms and originate more proximally in the AV conduction system.
Paroxysmal AV block. Multiple nonconducted P waves after a period of sinus bradycardia with a normal PR interval. This implies significant conduction system disease, requiring permanent pacemaker implantation.
High-grade AV block. A. Multiple nonconducted P waves with a regular narrow complex QRS escape rhythm probably emanating from the AV junction. B. A wide complex QRS escape and a single premature ventricular contraction. In both cases there is no consistent temporal relationship between the P waves and QRS complexes.
Diagnostic testing in the evaluation of AV block is aimed at determining the level of conduction block, particularly in asymptomatic patients, since the prognosis and therapy depend on whether the block is in or below the AV node. Vagal maneuvers, carotid sinus massage, exercise, and administration of drugs such as atropine and isoproterenol may be diagnostically informative. Owing to the differences in innervation of the AV node and infranodal conduction system, vagal stimulation and carotid sinus massage slow conduction in the AV node but have less of an effect on infranodal tissue and may even improve conduction due to a reduced rate of activation of distal tissues. Conversely, atropine, isoproterenol, and exercise improve conduction through the AV node and impair infranodal conduction. In patients with congenital CHB and a narrow QRS complex, exercise typically increases heart rate; by contrast, those with acquired CHB, particularly with wide QRS, do not respond to exercise with an increase in heart rate.
Additional diagnostic evaluation, including electrophysiologic testing, may be indicated in patients with syncope and suspected high-grade AV block. This is particularly relevant if noninvasive testing does not reveal the cause of syncope or if the patient has structural heart disease with ventricular tachyarrhythmias as a cause of symptoms. Electrophysiologic testing provides more precise information regarding the location of AV conduction block and permits studies of AV conduction under conditions of pharmacologic stress and exercise. Recording of the His bundle electrogram by a catheter positioned at the superior margin of the tricuspid valve annulus provides information about conduction at all levels of the AV conduction axis. A properly recorded His bundle electrogram reveals local atrial activity, the His electrogram, and local ventricular activation; when it is monitored simultaneously with recorded body surface electrocardiographic traces, intraatrial, AV nodal, and infranodal conduction times can be assessed (Fig. 232-5). The time from the most rapid deflection of the atrial electrogram in the His bundle recording to the His electrogram (AH interval) represents conduction through the AV node and is normally <130 ms. The time from the His electrogram to the earliest onset of the QRS on the surface ECG (HV interval) represents the conduction time through the His-Purkinje system and is normally ≤55 ms.
Rate stress produced by pacing can unveil abnormal AV conduction. Mobitz I second-degree AV block at short atrial paced cycle lengths is a normal response. However, when it occurs at atrial cycle lengths >500 ms (<120 beats/min) in the absence of high vagal tone, it is abnormal. Typically, type I second-degree AV block is associated with prolongation of the AH interval, representing conduction slowing and block in the AV node. AH prolongation occasionally is due to the effect of drugs (beta blockers, calcium channel blockers, digitalis) or increased vagal tone. Atropine can be used to reverse high vagal tone; however, if AH prolongation and AV block at long pacing cycle lengths persist, intrinsic AV node disease is likely. Type II second-degree block is typically infranodal, often in the His-Purkinje system. Block below the node with prolongation of the HV interval or a His bundle electrogram with no ventricular activation (Fig. 232-9) is abnormal unless it is elicited at fast pacing rates or short coupling intervals with extra stimulation. It is often difficult to determine the type of second-degree AV block when 2:1 conduction is present; however, the finding of a His bundle electrogram after every atrial electrogram indicates that block is occurring in the distal conduction system.
High-grade AV block below the His. The AH interval is normal and is not changing before the block. Atrial and His bundle electrograms are recorded consistent with block below the distal AV junction. I, II, III, and V1 are surface ECG leads. HISp, HISd, and RVA are the proximal HIS, distal HIS, and right ventricular apical electrical recordings. A, H, and V represent the atrial, His, and ventricular electrograms on the His bundle recording. (Tracing courtesy of Dr. Joseph Marine; with permission.)
Intracardiac recording at electrophysiologic study that reveals prolongation of conduction through the His-Purkinje system (i.e., long HV interval) is associated with an increased risk of progression to higher grades of block and is generally an indication for pacing. In the setting of bundle branch block, the HV interval may reveal the condition of the unblocked bundle and the prognosis for developing more advanced AV conduction block. Prolongation of the HV interval in patients with asymptomatic bundle branch block is associated with an increased risk of developing higher-grade AV block. The risk increases with greater prolongation of the HV interval such that in patients with an HV interval >100 ms, the annual incidence of complete AV block approaches 10%, indicating a need for pacing. In patients with acquired CHB, even if intermittent, there is little role for electrophysiologic testing, and pacemaker implantation is almost always indicated.
Treatment: Management of AV Conduction Block
Temporary or permanent artificial pacing is the most reliable treatment for patients with symptomatic AV conduction system disease. However, exclusion of reversible causes of AV block and the need for temporary heart rate support based on the hemodynamic condition of the patient are essential considerations in each patient. Correction of electrolyte derangements and ischemia, inhibition of excessive vagal tone, and withholding of drugs with AV nodal blocking properties may increase the heart rate. Adjunctive pharmacologic treatment with atropine or isoproterenol may be useful if the block is in the AV node. Since most pharmacologic treatment may take some time to initiate and become effective, temporary pacing may be necessary. The most expeditious technique is the use of transcutaneous pacing, where pacing patches are placed anteriorly over the cardiac apex (cathode) and posteriorly between the spine and the scapula or above the right nipple (anode). Acutely, transcutaneous pacing is highly effective, but its duration is limited by patient discomfort and longer-term failure to capture the ventricle owing to changes in lead impedance. If a patient requires more than a few minutes of pacemaker support, transvenous temporary pacing should be instituted. Temporary pacing leads can be placed from the jugular or subclavian venous system and advanced to the right ventricle, permitting stable temporary pacing for many days, if necessary. In most circumstances, in the absence of prompt resolution, conduction block distal to the AV node requires permanent pacemaking.
Nomenclature and Complications
The main therapeutic intervention in SA node dysfunction and AV conduction block is permanent pacing. Since the first implementation of permanent pacing in the 1950s, many advances in technology have resulted in miniaturization, increased longevity of pulse generators, improvement in leads, and increased functionality. To better understand pacemaker therapy for bradycardias, it is important to be familiar with the fundamentals of pacemaking. Pacemaker modes and function are named using a five-letter code. The first letter indicates the chamber(s) that is paced (O, none; A, atrium; V, ventricle; D, dual; S, single), the second is the chamber(s) in which sensing occurs (O, none; A, atrium; V, ventricle; D, dual; S, single), the third is the response to a sensed event (O, none; I, inhibition; T, triggered; D, inhibition + triggered), the fourth refers to the programmability or rate response (R, rate responsive), and the fifth refers to the existence of antitachycardia functions if present (O, none; P, antitachycardia pacing; S, shock; D, pace + shock). Almost all modern pacemakers are multiprogrammable and have the capability for rate responsiveness using one of several rate sensors: activity or motion, minute ventilation, or QT interval. The most commonly programmed modes of implanted single- and dual-chamber pacemakers are VVIR and DDDR, respectively, although multiple modes can be programmed in modern pacemakers.
Although pacemakers are highly reliable, they are subject to a number of complications related to implantation and electronic function. In adults, permanent pacemakers are most commonly implanted with access to the heart by way of the subclavian–superior vena cava venous system. Rare, but possible, acute complications of transvenous pacemaker implantation include infection, hematoma, pneumothorax, cardiac perforation, diaphragmatic/phrenic nerve stimulation, and lead dislodgment. Limitations of chronic pacemaker therapy include infection, erosion, lead failure, and abnormalities resulting from inappropriate programming or interaction with the patient's native electrical cardiac function. Rotation of the pacemaker pulse generator in its subcutaneous pocket, either intentionally or inadvertently, often referred to as "twiddler's syndrome," can wrap the leads around the generator and produce dislodgment with failure to sense or pace the heart. The small size and light weight of contemporary pacemakers make this a rare complication.
Complications stemming from chronic cardiac pacing also result from disturbances in atrioventricular synchrony and/or left ventricular mechanical synchrony. Pacing modes that interrupt or fail to restore atrioventricular synchrony may lead to a constellation of signs and symptoms, collectively referred to as pacemaker syndrome, that include neck pulsation, fatigue, palpitations, cough, confusion, exertional dyspnea, dizziness, syncope, elevation in jugular venous pressure, canon A waves, and stigmata of congestive heart failure, including edema, rales, and a third heart sound. Right ventricular apical pacing can induce dyssynchronous activation of the left ventricle, leading to compromised left ventricular (LV) systolic function, mitral valve regurgitation, and the previously mentioned stigmata of congestive heart failure. Maintenance of AV synchrony can minimize the sequelae of pacemaker syndrome. Selection of pacing modes that minimize unnecessary ventricular pacing or implantation of a device capable of right and left ventricular pacing (biventricular pacing) can help minimize the deleterious consequences of pacing-induced mechanical dyssynchrony at the ventricular level.
Pacemaker Therapy in Sa Node Dysfunction
Pacing in SA nodal disease is indicated to alleviate symptoms of bradycardia. Consensus guidelines published by the American Heart Association (AHA)/American College of Cardiology/Heart Rhythm Society (ACC/HRS) outline the indications for the use of pacemakers and categorize them by class based on levels of evidence. Class I conditions are those for which there is evidence or consensus of opinion that therapy is useful and effective. In class II conditions there is conflicting evidence or a divergence of opinion about the efficacy of a procedure or treatment; in class IIa conditions the weight of evidence or opinion favors treatment, and in class IIb conditions efficacy is less well established by the evidence or opinion of experts. In class III conditions, the evidence or weight of opinion indicates that the therapy is not efficacious or useful and may be harmful.
Class I indications for pacing in SA node dysfunction include documented symptomatic bradycardia, sinus node dysfunction–associated long-term drug therapy for which there is no alternative, and symptomatic chronotropic incompetence. Class IIa indications include those outlined previously in which sinus node dysfunction is suspected but not documented and for syncope of unexplained origin in the presence of major abnormalities of SA node dysfunction. Mildly symptomatic individuals with heart rates consistently <40 beats/min constitute a class IIb indication for pacing. Pacing is not indicated in patients with SA node dysfunction who do not have symptoms and in those in whom bradycardia is associated with the use of nonessential drugs (Table 232-3).
Table 232–3. Summary of Guidelines for Pacemaker Implantation in SA Node Dysfunction |Favorite Table|Download (.pdf)
Table 232–3. Summary of Guidelines for Pacemaker Implantation in SA Node Dysfunction
|1. SA node dysfunction with symptomatic bradycardia or sinus pauses|
|2. Symptomatic SA node dysfunction as a result of essential long-term drug therapy with no acceptable alternatives|
|3. Symptomatic chronotropic incompetence|
|4. Atrial fibrillation with bradycardia and pauses >5s|
|1. SA node dysfunction with heart rates <40 beats/min without a clear and consistent relationship between bradycardia and symptoms|
|2. SA node dysfunction with heart rates <40 beats/min on an essential long-term drug therapy with no acceptable alternatives, without a clear and consistent relationship between bradycardia and symptoms|
|3. Syncope of unknown origin when major abnormalities of SA node dysfunction are discovered or provoked by electrophysiologic testing|
|1. Mildly symptomatic patients with waking chronic heart rates <40 beats/min|
|1. SA node dysfunction in asymptomatic patients, even those with heart rates <40 beats/min|
|2. SA node dysfunction in which symptoms suggestive of bradycardia are not associated with a slow heart rate|
|3. SA node dysfunction with symptomatic bradycardia due to nonessential drug therapy|
There is some controversy about the mode of pacing that should be employed in SA node disease. A number of randomized, single-blind trials of pacing mode have been performed. There are no trials that demonstrate an improvement in mortality rate with AV synchronous pacing compared with single-chamber pacing in SA node disease. In some of these studies, the incidence of atrial fibrillation and thromboembolic events was reduced with AV synchronous pacing. In trials of patients with dual-chamber pacemakers designed to compare single-chamber with dual-chamber pacing by crossover design, the need for AV synchronous pacing due to pacemaker syndrome was common. Pacing modes that preserve AV synchrony appear to be associated with a reduction in the incidence of atrial fibrillation and improved quality of life. Because of the low but finite incidence of AV conduction disease, patients with SA node dysfunction usually undergo dual-chamber pacemaker implantation.
Pacemaker Therapy in Carotid Sinus Hypersensitivity and Vasovagal Syncope
Carotid sinus hypersensitivity, if accompanied by a significant cardioinhibitory component, responds well to pacing. In this circumstance, pacing is required only intermittently and single-chamber ventricular pacing is often sufficient. The mechanism of vasovagal syncope is incompletely understood but appears to involve activation of cardiac mechanoreceptors with consequent activation of neural centers that mediate vagal activation and withdrawal of sympathetic nervous system tone. Several randomized clinical trials have been performed in patients with drug-refractory vasovagal syncope, with some studies suggesting reduction in the frequency and the time to recurrent syncope in patients who were paced compared with those who were not. A recent follow-up study to one of those initial trials, however, found less convincing results, casting some doubt on the utility of pacing for vagally mediated syncope.
Pacemakers in AV Conduction Disease
There are no randomized trials that evaluate the efficacy of pacing in patients with AV block, as there are no reliable therapeutic alternatives for AV block and untreated high-grade AV block is potentially lethal. The consensus guidelines for pacing in acquired AV conduction block in adults provide a general outline for situations in which pacing is indicated (Table 232-4). Pacemaker implantation should be performed in any patient with symptomatic bradycardia and irreversible second- or third-degree AV block, regardless of the cause or level of block in the conducting system. Symptoms may include those directly related to bradycardia and low cardiac output or to worsening heart failure, angina, or intolerance to an essential medication. Pacing in patients with asymptomatic AV block should be individualized; situations in which pacing should be considered are patients with acquired CHB, particularly in the setting of cardiac enlargement; left ventricular dysfunction; and waking heart rates ≤40 beats/min. Patients who have asymptomatic second-degree AV block of either type should be considered for pacing if the block is demonstrated to be intra- or infra-His or is associated with a wide QRS complex. Pacing may be indicated in asymptomatic patients in special circumstances, in patients with profound first-degree AV block and left ventricular dysfunction in whom a shorter AV interval produces hemodynamic improvement, and in the setting of milder forms of AV conduction delay (first-degree AV block, intraventricular conduction delay) in patients with neuromuscular diseases that have a predilection for the conduction system, such as myotonic dystrophy and other muscular dystrophies, and Kearns-Sayre syndrome.
Table 232–4. Guideline Summary for Pacemaker Implantation in Acquired AV Block |Favorite Table|Download (.pdf)
Table 232–4. Guideline Summary for Pacemaker Implantation in Acquired AV Block
|1. Third-degree or high-grade AV block at any anatomic level associated with:|
|a. Symptomatic bradycardia|
|b. Essential drug therapy that produces symptomatic bradycardia|
|c. Periods of asystole >3 s or any escape rate <40 beats/min while awake|
|d. Postoperative AV block not expected to resolve|
|e. Catheter ablation of the AV junction|
|f. Neuromuscular diseases such as myotonic dystrophy, Kearns-Sayre syndrome, Erb dystrophy, and peroneal muscular atrophy, regardless of the presence of symptoms|
|2. Second-degree AV block with symptomatic bradycardia|
|3. Type II second-degree AV block with a wide QRS complex with or without symptoms|
|4. Exercise-induced second- or third-degree AV block in the absence of ischemia|
|5. Atrial fibrillation with bradycardia and pauses >5s|
|1. Asymptomatic third-degree AV block regardless of level|
|2. Asymptomatic type II second-degree AV block with a narrow QRS complex|
|3. Asymptomatic type II second-degree AV block with block within or below the His at electrophysiologic study|
|4. First- or second-degree AV block with symptoms similar to pacemaker syndrome|
|1. Asymptomatic third-degree AV block regardless of level|
|2. Asymptomatic type II second-degree AV block with a narrow|
|3. Asymptomatic type II second-degree AV block with block within or below the His at electrophysiologic study|
|4. First- or second-degree AV block with symptoms similar to pacemaker syndrome|
|1. Marked first-degree AV block (PR interval >300 ms) in patients with LV dysfunction in whom shortening the AV delay would improve hemodynamics|
|2. Neuromuscular diseases such as myotonic dystrophy, Kearns-Sayre syndrome, Erb dystrophy, and peroneal muscular atrophy with any degree of AV block regardless of the presence of symptoms|
|1. Asymptomatic first-degree AV block|
|2. Asymptomatic type I second-degree AV block at the AV node level|
|3. AV block that is expected to resolve or is unlikely to recur (Lyme disease, drug toxicity)|
Pacemaker Therapy in Myocardial Infarction
Atrioventricular block in acute MI is often transient, particularly in inferior infarction. The circumstances in which pacing is indicated in acute MI are persistent second- or third-degree AV block, particularly if symptomatic, and transient second- or third-degree AV block associated with bundle branch block (Table 232-5). Pacing is generally not indicated in the setting of transient AV block in the absence of intraventricular conduction delays or in the presence of fascicular block or first-degree AV block that develops in the setting of preexisting bundle branch block. Fascicular blocks that develop in acute MI in the absence of other forms of AV block also do not require pacing (Table 232-5 and Table 232-6).
Table 232–5. Guideline Summary for Pacemaker Implantation in AV Conduction Block in Acute Myocardial Infarction (AMI) |Favorite Table|Download (.pdf)
Table 232–5. Guideline Summary for Pacemaker Implantation in AV Conduction Block in Acute Myocardial Infarction (AMI)
|1. Persistent second-degree AV block in the His-Purkinje system with bilateral bundle branch block or third-degree block within or below the His after AMI|
|2. Transient advanced (second- or third-degree) infranodal AV block and associated bundle branch block. If the site of block is uncertain, an electrophysiologic study may be necessary|
|3. Persistent and symptomatic second- or third-degree AV block|
|1. Persistent second- or third-degree AV block at the AV node level|
|1. Transient AV block in the absence of intraventricular conduction defects|
|2. Transient AV block in the presence of isolated left anterior fascicular block|
|3. Acquired left anterior fascicular block in the absence of AV block|
|4. Persistent first-degree AV block in the presence of bundle branch block that is old or age-indeterminate|
Table 232–6. Indications for Pacemaker Implantation in Chronic Bifascicular and Trifascicular Block |Favorite Table|Download (.pdf)
Table 232–6. Indications for Pacemaker Implantation in Chronic Bifascicular and Trifascicular Block
|1. Intermittent third-degree AV block|
|2. Type II second-degree AV block|
|3. Alternating bundle branch block|
|1. Syncope not demonstrated to be due to AV block when other likely causes (e.g., ventricular tachycardia) have been excluded|
|2. Incidental finding at electrophysiologic study of a markedly prolonged HV interval (>100 ms) in asymptomatic patients|
|3. Incidental finding at electrophysiologic study of pacing-induced infra-His block that is not physiologic|
|1. Neuromuscular diseases such as myotonic dystrophy, Kearns-Sayre syndrome, Erb dystrophy, and peroneal muscular atrophy with any degree of fascicular block regardless of the presence of symptoms, because there may be unpredictable progression of AV conduction disease|
|1. Fascicular block without AV block or symptoms|
|2. Fascicular block with first-degree AV block without symptoms|
Pacemaker Therapy in Bifascicular and Trifascicular Block
Distal forms of AV conduction block may require pacemaker implantation in certain clinical settings. Patients with bifascicular or trifascicular block and symptoms, particularly syncope that is not attributable to other causes, should undergo pacemaker implantation. Pacemaking is indicated in asymptomatic patients with bifascicular or trifascicular block who experience intermittent third-degree, type II second-degree AV block or alternating bundle branch block. In patients with fascicular block who are undergoing electrophysiologic study, a markedly prolonged HV interval or block below the His at long cycle lengths also may constitute an indication for permanent pacing. Patients with fascicular block and the neuromuscular diseases previously described should also undergo pacemaker implantation (Table 232-6).
In general, a pacing mode that maintains AV synchrony reduces complications of pacing such as pacemaker syndrome and pacemaker-mediated tachycardia. This is particularly true in younger patients; the importance of dual-chamber pacing in the elderly, however, is not well established. Several studies have failed to demonstrate a difference in mortality rate in older patients with AV block treated with a single- (VVI) compared with a dual- (DDD) chamber pacing mode. In some of the studies that randomized pacing mode, the risk of chronic atrial fibrillation and stroke risk decreased with physiologic pacing. In patients with sinus rhythm and AV block, the very modest increase in risk with dual-chamber pacemaker implantation appears to be justified to avoid the possible complications of single-chamber pacing.