Download 15 Complete Heart Block—Third

15
Complete Heart Block—Third-Degree
Heart Block
Mohamad Al-Ahdab
Complete atrioventricular (AV) block can be
defined as interruption in the transmission
of the cardiac impulse from the atria to the
ventricles due to an anatomical or functional
impairment in the AV conduction system.
The conduction disturbance can be transient
or permanent.
Congenital complete heart block (CHB),
the most common and important form in children, was first described in 1901 by Morquio,
who also noted a familial occurrence and an
association with Stokes-Adams attacks and
death. The presence of fetal bradycardia (40–
80 bpm) as a manifestation of CHB was first
noted in 1921. The incidence of congenital CHB in the general population varies between 1 in 15,000 to 1 in 22,000 live-born
infants.
almost all cases presenting in utero or during the neonatal period. Rarely it may explain
a few cases occurring later (5% in one report). Other causes include myocarditis and
various structural cardiac defects, particularly
congenitally corrected transposition of the
great arteries, AV discordance, or polysplenia
with AV canal defect. Several genetic disorders such as familial atrial septal defect and
Kearns-Sayre syndrome (Chapter 18) have
been identified (Table 1). In most cases, CHB
is characterized pathologically by fibrous tissue that either replaces the AV node and its
surrounding tissue or by an interruption between the atrial myocardium and the AV node;
other lesions that can occur include congenital absence of the AV node. The net effect
is that the block is usually at the level of the
AV node. The heart is otherwise structurally
normal in these children.
ETIOLOGY
In the absence of congenital heart disease, neonatal lupus is responsible for 60%
to 90% of cases of congenital CHB. Antibodies from a mother with an autoimmune connective tissue disorder, most frequently lupus
erythematosus, cross the placenta to the fetus during the first trimester and account for
Neonatal Lupus
Complete heart block, hepatobiliary disease, malar rash, thrombocytopenia and, less
frequently, myocarditis comprise the neonatal lupus primarily presenting in utero or in
the neonate. Frequently the only manifestation of neonatal lupus, and by extension an
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TABLE 1. Congenital Complete Heart Block (1 in 20,000 to 25,000 Live Births)
No associated structural heart disease
Accounts for 67% to 75% of affected infants
Immune-mediated complete heart block
Accounts for 80% of congenital complete heart block in the absence of structural defect
Associated with maternal autoantibodies (anti-SSA/Ro antibodies and anti-SSB/La antibodies) which are the
putative etiologic agents
Because the mentioned antibodies are of the IgG class, transplacental passage and complete heart block do not
appear until after 16 to 20 weeks of gestation
High fetal wastage
At least 80% of mothers with affected infants will have autoantibodies; most mothers will either have or
develop overt symptoms of a systemic connective tissue disorder
Discontinuity within the cardiac conduction tissue at level of the atrial axis, within the nodal-ventricular
conduction tissue, or within the intraventricular conduction tissue∗
Other associations: (applies to complete heart block at birth as well as letter)
Tumors and neoplasia
Myocarditis and infections
Familial, genetic, and metabolic
Long QT syndrome
Congestive heart failure occurs but less commonly
Fair to good postnatal prognosis
Pacemaker in the newborn period indicated by presence of congestive heart failure or a very slow heart rate
(<50 to 55 bpm), or at an older age by symptoms
Associated with complex structural heart disease
Accounts for 25% to 33% of affected infants
Most common forms of cardiac malformations:
Corrected (I-loop) transposition of the great arteries
Single ventricle
Defects in atrial and ventricular septation and looping
Guarded prognosis: high fetal and newborn mortality (even with pacemaker)
Usually with congestive heart failure
Early permanent cardiac pacemaker frequently necessary
∗
See Ho et al., Am J Cardiol 1986, 58:291–294.
autoimmune abnormality in the mother, is
CHB in the newborn.
Neonatal lupus is due to transplacental
passage of maternal anti-Ro/SSA and/or antiLa/SSB antibodies. Among women with such
antibodies, CHB occurs in approximately 2%
of pregnancies. Once such a woman has given
birth to an infant with CHB, the recurrence
rate of CHB in subsequent pregnancies is
about 15%; another 6% have an isolated rash
consistent with neonatal lupus.
Anti-Ro/SSA and/or anti-La/SSB antibodies bind to fetal cardiac tissue, leading
to immune-mediated injury to the AV node
and its surrounding tissue. Both Ro/SSA and
La/SSB antigens are abundant in fetal heart
tissue between 18 and 24 weeks. Apoptosis
induces translocation of Ro/SSA and La/SSB
to the surface of fetal cardiomyocytes; anti-
Ro and anti-La antibodies then bind to the
surface of the fetal cardiomyocytes and induce the release of tumor necrosis factor by
macrophages, resulting in fibrosis. In addition to inducing tissue damage, anti-Ro/SSA
and/or anti-La/SSB antibodies inhibit calcium
channel activation of the cardiac L- and T-type
calcium channels themselves; L-type channels are crucial to action potential propagation
and conduction in the AV node. The sinoatrial
(SA) node also may be involved; sinus bradycardia has been described in 3.8% percent of
fetuses but is usually not permanent.
CLINICAL MANIFESTATION
The manifestations of CHB vary with
the age at presentation. Patients with the
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neonatal lupus syndrome tend to present earlier than those with CHB not due to neonatal
lupus.
Presentation in Utero
Congenital heart block may present with
fetal bradycardia between 18 and 28 weeks of
gestation. Almost all of these cases (95% in
one series) are due to neonatal lupus, demonstrated by the presence of anti-Ro/SSA and/or
anti-La/SSB antibodies in the maternal serum.
In utero detection is made by echocardiography, which can estimate the fetal PR interval
(Chapter 19). Complications in utero include
hydrops fetalis, myocarditis, endocardial fibroelastosis, pericardial effusion, and spontaneous intrauterine fetal death. In one report,
among 29 cases diagnosed in utero, there was
one therapeutic abortion and six intrauterine
fetal deaths; a lower rate of intrauterine death
(6 of 87) was seen in another study. Seconddegree block detected in utero can progress to
CHB.
Infants who present with heart block in
utero, but who survive until birth, have a high
neonatal mortality rate. In one report, 6 of 22
such infants (27%) died within one week of
birth. In another series, 10 of 107 (9%) died
within the first three months. Infants born before 34 weeks have a higher mortality rate than
those born later (52% vs. 9%). Infants with
first- or second-degree heart block at birth can
progress to CHB.
Presentation in the Neonate
As in the fetus, the cardinal finding in
CHB in the neonate is a slow heart rate. In
addition to bradycardia, other clinical clues in
the neonate include intermittent cannon waves
in the neck, a first heart sound that varies in intensity, and intermittent gallops and murmurs.
As with cases presenting in utero, almost all
presenting in the neonatal period (90% in one
series) are due to neonatal lupus. The newborn
at greatest risk has a rapid atrial rate, often
150 bpm or faster, and a ventricular rate less
than 50 bpm. Similar to acquired CHB, the
ECG most commonly shows a narrow QRS
complex due to a junctional or AV nodal escape or ectopic rhythm (Figure 1). First- or
second-degree heart block found in infants at
birth can progress to CHB. The outcome for
II
P
I
P
P
P
P
III
V
H
H
V
V
V
HBE
H
V
AVF
FIGURE 1. Leads II, I, III, and aVF with a His bundle electrogram (HBE) and ventricular (V) electrogram demonstrating CHB with a His bundle (H) escape rhythm. The ventricular rate is 45 bpm. Reprinted with permission from
Ho SY et al. The anatomy of congenital heart block. J Am Cardiol 1986;58(3):292–294 and Elsevier.
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COMPLETE HEART BLOCK THIRD-DEGREE HEART BLOCK
FIGURE 2. Monitor electrocardiographic tracing in a 10-year-old boy presenting with syncope. The tracing shows
intermittent CHB with long pauses. A pacemaker was implanted although his AV conduction improved. Myocarditis
was presumed but unproven (negative cardiac biopsy). Reprinted with permission from Ho SY et al. The anatomy of
congenital heart block. J Am Cardiol 1986;58(3):292–294 and Elsevier.
patients diagnosed as neonates is better than
for those diagnosed in utero. In the above cited
review, 33 patients presented in the perinatal
period; five had signs of heart failure, but none
had hydrops fetalis. None died within the first
six months, but two died at 0.9 and 1.5 years
of age. Our experience has been not as grave.
Presentation in Childhood
As many as 40% of cases of congenital heart block do not present until childhood
(mean age five to six years) (Figure 2). Few of
these patients (5%) have neonatal lupus. The
diagnosis is usually made when by presentation such as syncope or by detecting a slow
pulse. Heart block is confirmed by ECG or by
ambulatory ECG monitoring.
Complete heart block may be intermittent when first detected, but usually becomes
persistent in later childhood. It has been suggested that most unexplained CHB diagnosed
for the first time beyond infancy is congenital in origin and has escaped notice because
of a higher ventricular rate and the absence
of symptoms. However, given that prenatal
COMPLETE HEART BLOCK THIRD-DEGREE HEART BLOCK
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TABLE 2. Disorders that Can Cause Third-Degree Heart Block
Fibrosis and Sclerosis: Fibrosis and sclerosis of the conduction system accounts for about one-half of cases of AV
block.
Lenegre’s disease has been traditionally used to describe a progressive, fibrotic, sclerodegenerative affliction of
the conduction system in younger individuals associated with slow progression to CHB and may be hereditary.
Lev’s disease has referred to “sclerosis of the left side of the cardiac skeleton” in older patients, such as that
associated with calcific involvement of the aortic and mitral rings.
Familial Disease: Familial AV conduction block (Chapter 18).
Valvular Disease: Calcification and fibrosis of the aortic or mitral valve rings can extend into the conducting system.
Cardiomyopathies: Includes hypertrophic obstructive cardiomyopathy and infiltrative processes such as
amyloidosis and sarcoidosis.
Hyperthyroidism, myxedema, and thyrotoxic periodic paralysis.
Neuromuscular heredodegenerative disease, dermatomyositis, rheumatoid disease, and Paget’s disease.
Infections: Myocarditis due to rheumatic fever, diphtheria, viruses, systemic lupus erythematosus, toxoplasmosis,
bacterial endocarditis, syphilis, and Lyme disease.
Malignancies: Such as Hodgkin’s disease and other lymphomas; multiple myeloma; and cardiac tumors.
Drugs: A variety of drugs can impair conduction and cause AV block (Chapter 21).
Ischemic heart disease.
ultrasound is now well-developed and in wide
use, it may be that cases currently missed in
fetal life have preserved AV conduction at
birth and acquire progressive AV nodal disease thereafter. Consistent with this notion are
two observations. First, in a report from one
center, the number of childhood case referrals
remained constant from 1980 to 1998 despite
the introduction of fetal echocardiography
and the wide availability of heart rate monitoring during pregnancy and labor. Second,
in a series of 102 patients who were asymptomatic through age 15 and were followed for
7 to 30 years thereafter, a slow decline in ventricular rate was noted with increasing age,
with a mean heart rate at age 15 of 46 bpm
and a mean heart rate after age 40 of 39 bpm.
Some patients present with bradycardiarelated symptoms, including reduced exercise
tolerance, presyncope, or syncope. Sudden
death has also been described. In the above review of 102 patients who were without symptoms through age 15, 27 (26%) had a subsequent syncopal episode, eight of which were
fatal. Six of these eight episodes represented
a first syncopal episode.
Many other disorders can disrupt the AV
conduction system (Table 2). These disorders
are rare in the young.
TREATMENT
Management of congenital heart block
in utero and in the perinatal period can
include steroid therapy if associated with
anti-Ro/SSA and anti-La/SSB antibodies, and
isoproterenol and/or pacemaker insertion immediately postpartum.
The principal therapeutic decision after the immediate perinatal period involves
the need for pacemaker placement. Most patients ultimately have a pacemaker inserted,
regardless of the time of onset of the syndrome. In one study, by 20 years of age,
only 11% of neonatal and 12% of childhood cases had not required pacemaker implantation. In another report that included 40
patients free of symptoms at age 15, pacemakers were required in 90% by age 60
(Chapter 17).
The Report of the American College
of Cardiology/American Heart Association/
North American Society for Pacing and
Electrophysiology Task Force on Practice
Guidelines (Committee on Pacemaker Implantation) outlines management of thirddegree AV block in children, adolescents, and patients with congenital heart
disease.
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TABLE 3. Class I Indications for
Pacemaker Implant in Children
Symptomatic bradycardia (syncope or presyncope)
Moderate to marked exercise intolerance
Heart failure thought related to the bradycardia
Left ventricular dysfunction or low cardiac output
A wide QRS escape rhythm or block below the His
bundle
Complex ventricular arrhythmias
In an infant, ventricular rates <50−55 bpm or <70 bpm
when associated with congenital heart disease
Sustained pause-dependent VT, with or without
prolonged QT, in which the efficacy of pacing is
thoroughly documented
Advanced second- or third-degree AV block persisting
at least seven days after cardiac surgery
Class I Conditions: Class I conditions are
those for which there is evidence and/or general
agreement that a permanent pacemaker should be
implanted. This class includes patients with advanced second- or third-degree heart block, which
is permanent or intermittent (Table 3).
These guidelines are reasonable but should
be tailored to the patients needs. Infants with
CHB but otherwise a normally structural heart
may be followed without a pacemaker even
if the heart rate is in the 40s when asleep.
Equally important is the variation in the heart
rate and the overall status of the child. In
contrast, an infant with CHB (congenital or
surgical) and structural heart disease should
receive a pacemaker. Other useful guidelines
are outlined in Table 4.
Class II Conditions: Class II conditions
are those for which permanent pacemakers are
TABLE 4. Other Customized Guidelines
Cardiac enlargement
QT interval prolongation, which may represent a
substrate for ventricular arrhythmias (Class II by the
ACC/AHA/NASPE guidelines)
Ventricular arrhythmias related to a slow rate or that can
be abolished by a more rapid heart rate
Ectopic rhythms or other medical conditions are present
that require drugs that suppress the automaticity of
escape pacemakers and result in symptomatic
bradycardia
frequently used, but there is divergence of opinion with respect to the necessity of their insertion
(Table 5). Some of these conditions reflect advanced but not complete heart block.
LONG-TERM PROGNOSIS
Complete heart block, as noted, presenting in utero or the neonatal period due
to neonatal lupus, is associated with a significant early mortality. Of 175 cases described in two reports, 29 (17%) died either
in utero or within the first three months of
life. Infants and young children with CHB
who are asymptomatic usually remain so
until later childhood, adolescence, or adulthood. Children with a mean heart rate below
50 bpm and evidence of an unstable junctional
escape rhythm may benefit from early pacemaker implant. One particular risk related to
the AV block and the ventricular bradycardia is the development of torsades de pointes
(Figure 3).
Those patients who do not experience
symptoms or syncopal attacks may nonetheless experience physiologic consequences of
bradycardia. The ventricular rate tends to fall
slowly with age. To compensate for the slow
heart rate, the heart enlarges to produce a
higher stroke volume; in some cases, this can
lead to voltage criteria for left ventricular
enlargement and nonspecific ST-T wave
changes as well as to heart failure.
In general, the prognosis for the majority of young patients following pacemaker
implantation for isolated congenital CHB is
excellent. However, one report evaluated 16
patients (10 with neonatal lupus) in whom
a pacemaker was implanted within the first
two weeks of life; 12 developed heart failure before age 24. The major findings on
myocardial biopsy were hypertrophy and interstitial fibrosis. During follow-up, four patients died from progressive heart failure
and seven required transplantation. In another
study of 149 patients followed for 10 years,
6% developed a dilated cardiomyopathy by
COMPLETE HEART BLOCK THIRD-DEGREE HEART BLOCK
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TABLE 5. Class II Indications
Second- or third-degree AV block within the bundle of His in an asymptomatic patient (consider as Class I)
Prolonged subsidiary pacemaker recovery time with a pause greater than three seconds
Transient surgical second- or third-degree AV block that reverts to bifascicular block
Asymptomatic second- or third-degree AV block and a ventricular rate below 50 bpm when awake beyond the first
year of life
Complete AV block with double or triple rest cycle length pauses or minimal heart rate variability
Asymptomatic neonate with congenital CHB and bradycardia in relation to age
Long QT syndrome (especially with ventricular arrhythmias)
Congenital heart disease and impaired hemodynamics due to sinus bradycardia or loss of AV synchrony
Neuromuscular disease with any degree of AV block (including first-degree AV block), with or without symptoms,
because there may be unpredictable progression of AV conduction disease
6.5 years of age; risk factors included antiRo/SSA or anti-La/SSB antibodies, increased
heart size at initial evaluation, and the absence
of improvement with a pacemaker.
While the development of heart failure in such patients may be a consequence
of myocardial fibrosis associated with CHB,
another factor may be the long-term consequences of right ventricular pacing with
consequent ventricular asynchrony. Recently,
a report compared 23 patients with con-
genital CHB, each of whom had a pacemaker, to 30 matched healthy control subjects.
Echocardiography was performed before
pacemaker implantation and after at least five
years of right ventricular pacing in the CHB
patients. The CHB patients with pacemakers,
not surprisingly when compared to perfectly
normal controls, developed asynchronous left
ventricular contraction, an increase in left ventricular end-diastolic diameter, a decrease in
cardiac output, and a decrease in exercise
FIGURE 3. Eight-year-old boy with congenital CHB developed ventricular extrasystoles and torsades de pointes. He
spontaneously converted and a dual-chamber pacemaker was implanted. Reprinted with permission from Ho SY et
al. The anatomy of congenital heart block. J Am Cardiol 1986;58(3):292–294 and Elsevier.
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COMPLETE HEART BLOCK THIRD-DEGREE HEART BLOCK
performance. When pacemaker therapy is initially placed in children, there are often limited choices. Biventricular pacing, when the
patient is of sufficient size, may, in part, address this observation (Chapter 17).
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