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Etiology of atrioventricular block

Author: William H Sauer, MD
Section Editor: Leonard I Ganz, MD, FHRS, FACC
Deputy Editor: Gordon M Saperia, MD

All topics are updated as new evidence becomes available and our peer review process is complete.

Literature review current through: Oct 2020. | This topic last updated: Oct 26, 2020.

INTRODUCTION

Atrioventricular (AV) block is defined as a delay or interruption in the transmission of

an impulse from the atria to the ventricles due to an anatomical or functional
impairment in the conduction system. The conduction disturbance can be transient or
permanent, with conduction that is delayed, intermittent, or absent. Commonly used
terminology includes:

● First degree AV block – Delayed conduction from the atrium to the ventricle
(defined as a prolonged PR interval of >200 milliseconds) without interruption in
atrial to ventricular conduction.

● Second-degree AV block – Intermittent atrial conduction to the ventricle, often in

a regular pattern (eg, 2:1, 3:2, or other pattern), which are further classified into
Mobitz type I (Wenckebach) and Mobitz type II second degree AV block.

● Third-degree (complete AV) block – No atrial impulses conduct to the ventricle.

● High-grade AV block – Intermittent atrial conduction to the ventricle with two or

more consecutive blocked P waves but without complete AV block.

AV block has a variety of causes ( table 1). The various etiologies of AV block will be
reviewed here. The management of the specific types of AV block is discussed
separately. (See "First degree atrioventricular block" and "Second degree
atrioventricular block: Mobitz type I (Wenckebach block)" and "Second degree
atrioventricular block: Mobitz type II" and "Third degree (complete) atrioventricular
block".)

PHYSIOLOGIC AV BLOCK

AV block can result from physiologic slowing of cardiac conduction in response to

increased parasympathetic nervous system output. Enhanced vagal tone due to
athletic training, sleep, pain, carotid sinus massage, or carotid sinus hypersensitivity
syndrome can result in slowing of the sinus rate and/or the development of AV
conduction disturbances. In general, enhanced vagal tone leads to lower degrees of
AV block (ie, first degree or Mobitz type I second degree); higher degree AV block that
occurs in the setting of enhanced vagal tone could suggest other pathologic
contributions to AV conduction disturbance. (See "Sinus node dysfunction:
Epidemiology, etiology, and natural history" and "Carotid sinus hypersensitivity and
carotid sinus syndrome".)

PATHOPHYSIOLOGIC AV BLOCK

Fibrosis and sclerosis of the conduction system, which appears idiopathic, accounts for
about one-half of cases of AV block. Conduction system fibrosis and sclerosis may be
induced by several different conditions that often cannot be distinguished clinically [1].
Additionally, some degree of fibrosis and sclerosis occurs as part of the normal aging
process, with the prevalence increasing progressively with age with approximately a
2:1 male:female predominance. Among a prospective cohort of more than half a
million United Kingdom residents, the prevalence of conduction system disease (which
included all levels of AV block, as well as bundle branch blocks) was approximately 11
per 10,000 persons under age 55 and increased to between 55 per 10,000 persons ≥65
years of age [2].

Idiopathic — Apparently idiopathic progressive cardiac conduction defects are the

most common cause of AV block, occurring in approximately 50 percent of cases.
Idiopathic AV conduction abnormalities are characterized by progressive impairment
of the conduction system which occurs gradually over decades:

● Lenegre's disease – The term Lenegre's disease has been traditionally used to
describe a progressive, fibrotic, sclerodegenerative affliction of the conduction
system in younger (age <60 years) individuals. Lenegre's disease is frequently
associated with slow progression to complete heart block and may be hereditary.
(See 'Familial disease' below.)

● Lev's disease – The term Lev's disease has been used to refer to "sclerosis of the
left side of the cardiac skeleton" in older patients (age >70 years old), such as that
associated with calcific involvement of the aortic and mitral rings [3-5]. Lev's
disease is caused by fibrosis or calcification extending from any of the fibrous
structures adjacent to the conduction system into the conduction system [4,5].

Depending upon the anatomic location of the areas of fibrosis and sclerosis, various
conduction abnormalities can result:

● Fibrosis of the superior and basal aspect of the muscular septum is a common
cause of right bundle branch block (RBBB) with left anterior fascicular block in the
elderly patient.

● Involvement of the mitral ring or the central fibrous body, for example, may be the
most common cause of complete heart block with a narrow QRS complex in the
elderly.

● Aortic valve calcification, on the other hand, can invade the bundle of His, the right
and/or left bundle branch as well as the left anterior fascicle. Thus, the QRS
complex may be prolonged.

Associated with other cardiac disease

● Ischemic heart disease – Ischemic heart disease accounts for about 40 percent of
cases of AV block [1]. Conduction can be disturbed with either chronic ischemic
heart disease or during an acute myocardial infarction (MI) [6-10]. Up to 20
percent of patients with an acute MI develop some degree of AV block, with the
likelihood and severity related to the area and extent of ischemia/infarction [8-10].
While restoration of perfusion in the setting of acute MI frequently leads to
 improved conduction, coronary revascularization in stable patients with AV block
rarely if ever improves AV conduction.

Intraventricular conduction disturbances (IVCDs), including bundle and fascicular

blocks, also occur in 10 to 20 percent of cases of acute MI [11-17]. Left bundle
branch block (LBBB) and RBBB with left anterior fascicle block are most common.
(See "Conduction abnormalities after myocardial infarction".)

● Cardiomyopathies and myocarditis – AV block can be seen in patients with

cardiomyopathies, including hypertrophic obstructive cardiomyopathy and
infiltrative processes such as amyloidosis and sarcoidosis, and in patients with
myocarditis due to a variety of causes including rheumatic fever, Lyme disease,
diphtheria, viruses, systemic lupus erythematosus, toxoplasmosis, bacterial
endocarditis, and syphilis [4,5,18-29]. Patients with COVID-19-associated
myocarditis have been observed to have varying degrees of AV block [30]. The
development of AV block in myocarditis is often a poor prognostic sign. (See
"Clinical manifestations and diagnosis of myocarditis in adults" and "Lyme
carditis", section on 'Atrioventricular conduction abnormalities'.)

● Congenital heart disease – AV block of varying degrees can be associated with a

variety of congenital heart defects that result in structural abnormalities (eg,
congenitally corrected transposition of the great arteries, large primum atrial
septal defects, and large AV septal defects [AV canal defects]). Additionally,
complete heart block may be an isolated lesion (ie, with no associated structural
heart disease), most commonly associated with neonatal lupus, which results from
transplacental passage of anti-Ro/SSA or anti-La/SSB antibodies from the mother.
(See "Atrial arrhythmias (including AV block) in congenital heart disease", section
on 'Congenital AV block' and "Congenital third degree (complete) atrioventricular
block" and "Neonatal lupus: Epidemiology, pathogenesis, clinical manifestations,
and diagnosis".)

Familial disease — Familial AV block, characterized by a progression in the degree of

AV block in association with a variable apparent site of block, may be transmitted as an
autosomal dominant trait. One form of AV conduction block has been mapped to a
genetic locus at chromosome 19q13 and the other to chromosome 3p21, where the
cardiac sodium channel, SCN5A, is encoded [31]. Several SCN5A mutations have been
associated with AV conduction block [32-38]. Some patients with hereditary AV block
are identified in childhood due to the presents of bundle branch disease (ie, RBBB,
LBBB, left anterior fascicular block [LAFB], or left posterior fascicular block [LPFB]),
while others present in middle-age and have been called hereditary Lenegre's disease
[32,33,35,39]. In the latter setting, it has been proposed that haploinsufficiency
combined with aging leads to a progressive decline in conduction [40]. (See 'Idiopathic'
above.)

Different SCN5A mutations are associated with other cardiac abnormalities including
congenital long QT syndrome, the Brugada syndrome, familial sinus node dysfunction,
and familial dilated cardiomyopathy with conduction defects and susceptibility to atrial
fibrillation. (See "Congenital long QT syndrome: Pathophysiology and genetics" and
"Sinus node dysfunction: Epidemiology, etiology, and natural history" and "Genetics of
dilated cardiomyopathy" and "Brugada syndrome: Epidemiology and pathogenesis",
section on 'SCN5A'.)

Other genetic forms of familial AV block have been described, including a form of
progressive cardiac conduction system disease mapped to a locus on chromosome
19q13 and a form associated with congenital heart disease for which a point mutation
has been identified in the cardiac transcription factor CSX/NKX2-5 [39,41-46]. (See
'Associated with other cardiac disease' above.)

Miscellaneous causes — AV block can also occur in a variety of other disorders:

● Hyperkalemia, usually when the plasma potassium concentration is above 6.3

meq/L [47-49]. (See "Clinical manifestations of hyperkalemia in adults", section on
'Conduction abnormalities and arrhythmias'.)

● Hyperthyroidism and hypothyroidism, myxedema, and thyrotoxic periodic

paralysis [4,5,50]. (See "Cardiovascular effects of hypothyroidism", section on
'Rhythm disturbances' and "Thyrotoxic periodic paralysis", section on 'Clinical
features'.)

● Hereditary neuromuscular heredodegenerative disease such as myotonic

dystrophy, Kearns-Sayre syndrome, and Erb's dystrophy [51-53]. (See "Inherited
syndromes associated with cardiac disease".)
 ● Cardiac tumors, cysts, myocardial bridging, and trauma [3-5,54-56]. (See "Cardiac
tumors" and "Myocardial bridging of the coronary arteries", section on 'Clinical
relevance'.)

● Rheumatologic disorders including dermatomyositis [3-5]. (See "Clinical

manifestations of dermatomyositis and polymyositis in adults", section on 'Cardiac
involvement'.)

IATROGENIC AV BLOCK

Iatrogenic AV block, which can result from either medications or invasive procedures,
is common. As with physiologic AV block, iatrogenic AV block can occur in isolation, but
can also exacerbate underlying pathophysiologic AV block.

Medications — A variety of drugs can impair AV conduction, resulting in AV block. The

common medications which can result in AV block include:

● Beta blockers
● Non-dihydropyridine calcium channel blockers (especially verapamil and to a
lesser extent diltiazem)
● Digoxin
● Adenosine
● Antiarrhythmic medications, commonly amiodarone but also drugs that modulate
the sodium channel (eg, quinidine, procainamide, disopyramide, etc)

Most patients with AV block who are taking drugs that can impair conduction probably
have some degree of underlying conduction system disease, although toxicity may
result from medication overdose (either intentional or as a result of decreased
clearance in the setting of renal or hepatic dysfunction). The association between
medications altering AV conduction and underlying conduction system disease was
suggested by a study of 169 patients with second or third degree AV block not related
to acute MI, digitalis toxicity, or vasovagal syncope [57]. Of these, 92 (54 percent) were
receiving beta blockers and/or verapamil or diltiazem. Drug discontinuation resulted in
resolution of AV block in 32 of 79 cases; however, AV block later recurred in the
absence of therapy in 18 of these patients.
A 2020 scientific statement from the American Heart Association details drugs
associated with AV block [58].

Cardiac procedures — AV conduction abnormalities may result from a variety of

invasive cardiac procedures.

● Open heart surgery – AV block may occur following replacement of either the
aortic or mitral valve, closure of a ventricular septal defect, or other surgical
procedures [5,59-64]. In many instances, this is a transient phenomenon related to
periprocedural edema which resolves in the hours to days following surgery and
can be managed with temporary pacing. However, surgery may result in a
permanent conduction abnormality requiring a permanent pacemaker.

● Transcatheter aortic valve implantation (TAVI) – Between 2 and 8 percent of

patients who undergo percutaneous TAVI develop AV block following the
procedure. Pre-existing disturbances of cardiac conduction (particularly right
bundle branch block), a narrow left ventricular outflow tract, and the severity of
mitral annular calcification appear to be predictors of this complication. There may
also be a higher rate of heart block observed with self-expanding implanted aortic
valves compared with balloon expandable versions [65]. (See "Transcatheter aortic
valve implantation: Complications", section on 'High degree heart block'.)

● Catheter ablation for arrhythmias – AV block is a potential complication of

catheter ablation of reentrant arrhythmias when the reentrant pathway lies within
or near the AV node. As an example, catheter ablation for AV nodal reentrant
tachycardia typically involves areas of the atrium very close to the AV node, with a
resulting 1.4 percent risk of heart block following this procedure [66]. (See
"Overview of catheter ablation of cardiac arrhythmias", section on
'Complications'.)

● Transcatheter closure of VSD – A variety of devices have been used to

percutaneously close muscular ventricular septal defects (VSDs), both congenital
and those that occur after myocardial infarction. The Amplatzer ventricular septal
defect occluder, for example, completely occluded 28 of 30 VSDs in one report
[67]. One patient with complete left bundle branch block after the procedure
progressed to complete heart block at one year. The presumed mechanism is that
the right ventricular retention disk overlaps the ventricular conduction system as
 it passes above or anterosuperiorly to the defect. (See "Management and
prognosis of ventricular septal defect in adults", section on 'Arrhythmias'.)

● Alcohol (ethanol) septal ablation – Percutaneous transluminal alcohol (ethanol)

septal ablation is an invasive septal reduction therapy for patients with
hypertrophic cardiomyopathy and significant left ventricular outflow tract
obstruction. This intervention consists of infarction and thinning of the proximal
interventricular septum via infusion of alcohol into the first septal perforating
branch of the left anterior descending coronary artery through an angioplasty
catheter. Complete heart block is seen in approximately 8 to 10 percent of patients
after this procedure. (See "Hypertrophic cardiomyopathy: Nonpharmacologic
treatment of left ventricular outflow tract obstruction", section on 'Alcohol
(ethanol) septal ablation'.)

INFORMATION FOR PATIENTS

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articles are written at the 10th to 12th grade reading level and are best for patients who
want in-depth information and are comfortable with some medical jargon.

Here are the patient education articles that are relevant to this topic. We encourage
you to print or e-mail these topics to your patients. (You can also locate patient
education articles on a variety of subjects by searching on "patient info" and the
keyword(s) of interest.)

● Basics topics (see "Patient education: Heart block in adults (The Basics)")

SUMMARY AND RECOMMENDATIONS

● Atrioventricular (AV) block is defined as a delay or interruption in the transmission
of an impulse from the atria to the ventricles due to an anatomical or functional
impairment in the conduction system. The conduction disturbance can be
transient or permanent, and it can have many causes ( table 1). (See
'Introduction' above.)

● Fibrosis and sclerosis of the conduction system accounts for about 50 percent of
cases of AV block and may be induced by several different conditions that often
cannot be distinguished clinically. When fibrosis and sclerosis of the conduction
system are present, they are frequently progressive and may ultimately progress
to complete heart block. (See 'Idiopathic' above.)

● Ischemic disease accounts for about 40 percent of cases of AV block. Conduction

can be disturbed with either chronic ischemic heart disease or during an acute
myocardial infarction. AV block can be seen in patients with cardiomyopathies and
in the setting of congenital heart disease. (See 'Associated with other cardiac
disease' above.)

● Familial AV block, characterized by a progression in the degree of AV block in

association with a variable apparent site of block, may be transmitted as an
autosomal dominant trait. (See 'Familial disease' above and "Congenital third
degree (complete) atrioventricular block", section on 'Etiology'.)

● A variety of drugs, including beta blockers, non-dihydropyridine calcium channel

blockers (especially verapamil and to a lesser extent diltiazem), digitalis,
adenosine, and antiarrhythmic medications, can impair AV conduction,
occasionally resulting in AV block. In most cases, the resulting AV block is at least
partially reversible following withdrawal of the offending medication(s). (See
'Medications' above.)

● A variety of procedures performed on the heart may result in AV block, including

most commonly open heart surgery, but also following transcatheter aortic valve
implantation, catheter ablation of arrhythmias, transcatheter closure of a
ventricular septal defect, and alcohol septal ablation. (See 'Cardiac procedures'
above.)

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REFERENCES
1. ZOOB M, SMITH KS. THE AETIOLOGY OF COMPLETE HEART-BLOCK. Br Med J 1963;
2:1149.

2. Khurshid S, Choi SH, Weng LC, et al. Frequency of Cardiac Rhythm Abnormalities
in a Half Million Adults. Circ Arrhythm Electrophysiol 2018; 11:e006273.

3. LENEGRE J. ETIOLOGY AND PATHOLOGY OF BILATERAL BUNDLE BRANCH BLOCK

IN RELATION TO COMPLETE HEART BLOCK. Prog Cardiovasc Dis 1964; 6:409.

4. LEV M. ANATOMIC BASIS FOR ATRIOVENTRICULAR BLOCK. Am J Med 1964;

37:742.

5. LEV M. THE PATHOLOGY OF COMPLETE ATRIOVENTRICULAR BLOCK. Prog

Cardiovasc Dis 1964; 6:317.

6. Begg FR, Magovern GJ, Cushing WJ, et al. Selective cine coronary arteriography in
patients with complete heart block. J Thorac Cardiovasc Surg 1969; 57:9.

7. Simon AB, Zloto AE. Atrioventricular block: natural history after permanent
ventricular pacing. Am J Cardiol 1978; 41:500.

8. LEVINE SA, MILLER H, PENTON GB. Some clinical features of complete heart
block. Circulation 1956; 13:801.

9. HEJTMANCIK MR, HERRMANN GR, SHIELDS AH, WRIGHT JC. A clinical study of
complete heart block. Am Heart J 1956; 52:369.

10. ROWE JC, WHITE PD. Complete heart block: a follow-up study. Ann Intern Med
1958; 49:260.

11. Killip T 3rd, Kimball JT. Treatment of myocardial infarction in a coronary care unit.
A two year experience with 250 patients. Am J Cardiol 1967; 20:457.

12. Godman MJ, Lassers BW, Julian DG. Complete bundle-branch block complicating
acute myocardial infarction. N Engl J Med 1970; 282:237.
13. Sugiura T, Iwasaka T, Hasegawa T, et al. Factors associated with persistent and
transient fascicular blocks in anterior wall acute myocardial infarction. Am J
Cardiol 1989; 63:784.

14. Mullins CB, Atkins JM. Prognoses and management of venticular conduction
blocks in acute myocardial infarction. Mod Concepts Cardiovasc Dis 1976; 45:129.

15. Hindman MC, Wagner GS, JaRo M, et al. The clinical significance of bundle branch
block complicating acute myocardial infarction. 1. Clinical characteristics, hospital
mortality, and one-year follow-up. Circulation 1978; 58:679.

16. Scheinman MM, Gonzalez RP. Fascicular block and acute myocardial infarction.
JAMA 1980; 244:2646.

17. Dubois C, Piérard LA, Smeets JP, et al. Short- and long-term prognostic
importance of complete bundle-branch block complicating acute myocardial
infarction. Clin Cardiol 1988; 11:292.

18. Harris A, Davies M, Redwood D, et al. Aetiology of chronic heart block. A clinico-
pathological correlation in 65 cases. Br Heart J 1969; 31:206.

19. Bernstein, M . Auriculoventricular dissociation following scarlet fever: Report of a

case. Am Heart J 1938; 16:582.

20. Rantz, LA, Spink, et al. Abnormalities in the electrocardiogram following

hemolytic streptococcus sore throat. Arch Intern Med 1946; 77:66.

21. Lev M, Bharati S, Hoffman FG, Leight L. The conduction system in rheumatoid
arthritis with complete atrioventricular block. Am Heart J 1975; 90:78.

22. CLARK NS. Complete heart block in children; report of three cases possibly
attributable to measles. Arch Dis Child 1948; 23:156.

23. Rosenberg, DH . Electrocardiographic changes in epidemic parotitis (mumps).

Proc Soc Exp Biol Med 1945; 58:9.

24. ENGLE MA. Recovery from complete heart block in diphtheria. Pediatrics 1949;
3:222.
25. Menon TB, Rao CK. Tuberculosis of the Myocardium Causing Complete Heart
Block. Am J Pathol 1945; 21:1193.

26. SHEE JC. STOKES-ADAMS ATTACKS DUE TO TOXOPLASMA MYOCARDITIS. Br Heart

J 1964; 26:151.

27. Lim CH, Toh CC, Chia BL, Low LP. Stokes-Adams attacks due to acute nonspecific
myocarditis. Am Heart J 1975; 90:172.

28. Wray R, Iveson M. Complete heart block and systemic lupus erythematosus. Br
Heart J 1975; 37:982.

29. Kleid JJ, Kim ES, Brand B, et al. Heart block complicating acute bacterial
endocarditis. Chest 1972; 61:301.

30. Kir D, Mohan C, Sancassani R. Heart Brake: An Unusual Cardiac Manifestation of

COVID-19. JACC Case Rep 2020; 2:1252.

31. Schott JJ, Alshinawi C, Kyndt F, et al. Cardiac conduction defects associate with
mutations in SCN5A. Nat Genet 1999; 23:20.

32. Tan HL, Bink-Boelkens MT, Bezzina CR, et al. A sodium-channel mutation causes
isolated cardiac conduction disease. Nature 2001; 409:1043.

33. Wang DW, Viswanathan PC, Balser JR, et al. Clinical, genetic, and biophysical
characterization of SCN5A mutations associated with atrioventricular conduction
block. Circulation 2002; 105:341.

34. Herfst LJ, Potet F, Bezzina CR, et al. Na+ channel mutation leading to loss of
function and non-progressive cardiac conduction defects. J Mol Cell Cardiol 2003;
35:549.

35. Probst V, Kyndt F, Potet F, et al. Haploinsufficiency in combination with aging

causes SCN5A-linked hereditary Lenègre disease. J Am Coll Cardiol 2003; 41:643.

36. McNair WP, Ku L, Taylor MR, et al. SCN5A mutation associated with dilated
cardiomyopathy, conduction disorder, and arrhythmia. Circulation 2004;
110:2163.
37. Olson TM, Michels VV, Ballew JD, et al. Sodium channel mutations and
susceptibility to heart failure and atrial fibrillation. JAMA 2005; 293:447.

38. Makiyama T, Akao M, Tsuji K, et al. High risk for bradyarrhythmic complications in
patients with Brugada syndrome caused by SCN5A gene mutations. J Am Coll
Cardiol 2005; 46:2100.

39. https://www.ncbi.nlm.nih.gov/gtr/conditions/C1879286/ (Accessed on January 04,

2017).

40. Royer A, van Veen TA, Le Bouter S, et al. Mouse model of SCN5A-linked hereditary
Lenègre's disease: age-related conduction slowing and myocardial fibrosis.
Circulation 2005; 111:1738.

41. Brink PA, Ferreira A, Moolman JC, et al. Gene for progressive familial heart block
type I maps to chromosome 19q13. Circulation 1995; 91:1633.

42. Schott JJ, Benson DW, Basson CT, et al. Congenital heart disease caused by
mutations in the transcription factor NKX2-5. Science 1998; 281:108.

43. Ikeda Y, Hiroi Y, Hosoda T, et al. Novel point mutation in the cardiac transcription
factor CSX/NKX2.5 associated with congenital heart disease. Circ J 2002; 66:561.

44. Jay PY, Harris BS, Maguire CT, et al. Nkx2-5 mutation causes anatomic hypoplasia
of the cardiac conduction system. J Clin Invest 2004; 113:1130.

45. Pashmforoush M, Lu JT, Chen H, et al. Nkx2-5 pathways and congenital heart
disease; loss of ventricular myocyte lineage specification leads to progressive
cardiomyopathy and complete heart block. Cell 2004; 117:373.

46. Gray B, Behr ER. New Insights Into the Genetic Basis of Inherited Arrhythmia
Syndromes. Circ Cardiovasc Genet 2016; 9:569.

47. Arnsdorf MF, Schreiner E, Gambetta M, et al. Electrophysiological changes in the

canine atrium and ventricle during progressive hyperkalaemia:
electrocardiographical correlates and the in vivo validation of in vitro predictions.
Cardiovasc Res 1977; 11:409.
48. Surawicz B. Relationship between electrocardiogram and electrolytes. Am Heart J
1967; 73:814.

49. Fisch C. Relation of electrolyte disturbances to cardiac arrhythmias. Circulation

1973; 47:408.

50. Hsu YJ, Lin YF, Chau T, et al. Electrocardiographic manifestations in patients with
thyrotoxic periodic paralysis. Am J Med Sci 2003; 326:128.

51. Roberts NK, Perloff JK, Kark RA. Cardiac conduction in the Kearns-Sayre syndrome
(a neuromuscular disorder associated with progressive external ophthalmoplegia
and pigmentary retinopathy). Report of 2 cases and review of 17 published cases.
Am J Cardiol 1979; 44:1396.

52. Sanyal SK, Johnson WW. Cardiac conduction abnormalities in children with
Duchenne's progressive muscular dystrophy: electrocardiographic features and
morphologic correlates. Circulation 1982; 66:853.

53. Komajda M, Frank R, Vedel J, et al. Intracardiac conduction defects in dystrophia

myotonica. Electrophysiological study of 12 cases. Br Heart J 1980; 43:315.

54. James TN, Carson DJ, Marshall TK. De subitaneis mortibus. I. Fibroma
compressing His bundle. Circulation 1973; 48:428.

55. den Dulk K, Brugada P, Braat S, et al. Myocardial bridging as a cause of

paroxysmal atrioventricular block. J Am Coll Cardiol 1983; 1:965.

56. Rosen KM, Heller R, Ehsani A, Rahimtoola SH. Localization of site of traumatic
heart block with His bundle recordings: electrophysiologic observations
regarding the nature of "split" H potentials. Am J Cardiol 1972; 30:412.

57. Zeltser D, Justo D, Halkin A, et al. Drug-induced atrioventricular block: prognosis

after discontinuation of the culprit drug. J Am Coll Cardiol 2004; 44:105.

58. Tisdale JE, Chung MK, Campbell KB, et al. Drug-Induced Arrhythmias: A Scientific
Statement From the American Heart Association. Circulation 2020; 142:e214.

59. Sanoudos G, Reed GE. Late heart block in aortic valve replacement. J Cardiovasc
Surg (Torino) 1974; 15:475.
60. Rosen KM, Mehta A, Rahimtoola SH, Miller RA. Sites of congenital and surgical
heart block as defined by His bundle electrocardiography. Circulation 1971;
44:833.

61. Furman S, Young D. Cardiac pacing in children and adolescents. Am J Cardiol

1977; 39:550.

62. Hofschire PJ, Nicoloff DM, Moller JH. Postoperative complete heart block in 64
children treated with and without cardiac pacing. Am J Cardiol 1977; 39:559.

63. Berdajs D, Schurr UP, Wagner A, et al. Incidence and pathophysiology of

atrioventricular block following mitral valve replacement and ring annuloplasty.
Eur J Cardiothorac Surg 2008; 34:55.

64. Murray LE, Smith AH, Flack EC, et al. Genotypic and phenotypic predictors of
complete heart block and recovery of conduction after surgical repair of
congenital heart disease. Heart Rhythm 2017; 14:402.

65. Roten L, Wenaweser P, Delacrétaz E, et al. Incidence and predictors of

atrioventricular conduction impairment after transcatheter aortic valve
implantation. Am J Cardiol 2010; 106:1473.

66. Kesek M, Lindmark D, Rashid A, Jensen SM. Increased risk of late pacemaker
implantation after ablation for atrioventricular nodal reentry tachycardia: A 10-
year follow-up of a nationwide cohort. Heart Rhythm 2019; 16:1182.

67. Thanopoulos BD, Rigby ML. Outcome of transcatheter closure of muscular

ventricular septal defects with the Amplatzer ventricular septal defect occluder.
Heart 2005; 91:513.

Topic 907 Version 23.0

GRAPHICS

Major causes of atrioventricular (AV) block

Physiologic and pathophysiologic

Increased vagal tone

Progressive cardiac conduction system disease

With fibrosis and/or sclerosis (Lenegre disease)

With calcification (Lev disease) 

Ischemic heart disease, including acute myocardial infarction

Cardiomyopathy

Infiltrative processes (eg, sarcoidosis, amyloidosis, hemochromatosis, malignancy, etc)

Other non-ischemic cardiomyopathies (eg, idiopathic, infectious, etc) 

Infections (eg, viral myocarditis, Lyme carditis)  

Congenital AV block 

Related to structural congenital heart disease 

As part of neonatal lupus syndrome 

Other

Hyperkalemia, severe hypo- or hyperthyroidism, trauma, degenerative neuromuscular diseases

Iatrogenic
Drugs

Beta blockers, calcium channel blockers, digoxin, adenosine, antiarrhythmic drugs

 Cardiac surgery

Post valvular surgery, post surgical correction of congenital heart disease 

Transcatheter aortic valve implantation

Catheter ablation of arrhythmias

Transcatheter closure of VSD

Alcohol septal ablation for HCM

VSD: ventricular septal defect; HCM: hypertrophic cardiomyopathy.

Graphic 62885 Version 6.0

Contributor Disclosures
William H Sauer, MD Grant/Research/Clinical Trial Support: Biosense-Webster [Catheter
ablation]; Boston Scientific [Catheter ablation]. Leonard I Ganz, MD, FHRS, FACC Equity
Ownership/Stock Options: Unequal Technologies [Protective equipment for sports, military];
Neoleukin Therapeutics [Oncology therapies]. Consultant/Advisory Boards: Abbott; St Jude
Medical [Cybersecurity]; Sanofi [Atrial fibrillation]. Speaker's Bureau: Pfizer; BMS
[Anticoagulation]; Lundbeck [Neurogenic orthostatic hypotension]. Gordon M Saperia,
MD Nothing to disclose
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