Bundle Branch Block, Right Clinical Presentation

  • Author: Glenn T Wetzel, MD, PhD; Chief Editor: Steven R Neish, MD, SM   more...
 
Updated: Jul 22, 2010
 

History

The history in children with right bundle branch block (RBBB) should include the following:

  • History of congenital heart disease
  • History of cardiac surgery
  • History of palpitations, general energy and activity level, exercise tolerance, dizziness, and/or syncope
  • Family history of known arrhythmias, including bundle branch block, complete heart block, and placement of a pacemaker or defibrillator
  • Family history of premature or sudden unexplained death, myocardial infarction in individuals younger than 45 years,[2] syncope, seizures, or fetal loss
Next

Physical

Upon physical examination, patients with right bundle branch block have a persistently split second heart sound with normal respiratory variation in the splitting interval. In addition, one should always evaluate for findings consistent with postoperative heart disease, such as murmurs or a thoracotomy scar.

Previous
Next

Causes

The following are causes of right bundle branch block:

Hereditary factors

Hereditary right bundle branch block was observed in 4 Lebanese families and has been mapped to chromosome 19.[3]

A subset of patients with Brugada syndrome have mutations in SCN5A, the gene that encodes for the voltage-gated cardiac sodium channel.[4, 5]

Risk factors

In children, most cases of right bundle branch block occur after intracardiac surgery, such as congenital heart surgery associated with repair of a ventricular septal defect (VSD) and cardiac transplantation. Right bundle branch block has also been described in patients undergoing transcatheter closure of perimembranous VSDs.

Right bundle branch block has been associated with cardiomyopathy, myocarditis, congestive heart failure, atrial septal defect (ASD), and Ebstein anomaly.

A transient form of right bundle branch may be observed in patients with premature atrial contractions (Ashman phenomenon) or supraventricular tachycardia (rate dependent right bundle branch block). This occurs when an early impulse is conducted from the atrioventricular (AV) node to the His bundle while the right bundle branch is still refractory but the left bundle is not. Conduction down the right bundle branch is therefore delayed or blocked, resulting in a transient right bundle branch block pattern on the ECG.

Right anterior hemiblock is described in children with perinatal exposure to human immunodeficiency virus (HIV) type 1.[6]

Associated syndromes

Duchenne muscular dystrophy is an X-linked myopathy characterized by early onset and rapid progression with muscular weakness and pseudohypertrophy seen in the second year of life. Cardiac findings include mitral valve prolapse, pulmonary flow murmur, and an S3 or S4 gallop. (See Muscular Dystrophy.)

Myotonic dystrophy is characterized by muscular dystrophy, myotonias, hypogonadism, frontal balding, and cataracts. Congenital muscular dystrophy manifests with neonatal hypotonia, paresis, and myotonia. The adult form of myotonic dystrophy is the most common muscular dystrophy seen in adults. ECG findings may include first-degree AV block, left anterior fascicular block, and intraventricular conduction delay. Patients may have arrhythmias, Stokes-Adams attacks, or both. (See Muscular Dystrophy.)

Kearns-Sayre Syndrome is a mitochondrial myopathy with the physical findings of ptosis, chronic progressive external ophthalmoplegia, and abnormal retinal pigmentation. Patients are at risk for heart block and sudden death. Rarely, patients present with dilated cardiomyopathy and heart failure.

Brugada syndrome is a channelopathy mediated by the SCN5A gene. The right bundle branch block pattern seen in patients with this syndrome is not actually right bundle branch block but is a function of the unusual repolarization abnormality. The ECG shows ST-segment elevation in leads V1-V3, and patients are at risk for sudden cardiac death. Cocaine consumption or the use of the antiarrhythmic drugs propafenone, ajmaline, flecainide or procainamide may reveal ECG findings consistent with Brugada syndrome.[7]

Right bundle branch block is one the diagnostic criteria for patients with arrhythmogenic right ventricular cardiomyopathy (ARVC).

Patients may have isolated right bundle branch block or right bundle branch block with a left anterior fascicular block.

Right bundle branch block has been associated with blunt chest trauma and polymyositis.

Right bundle branch block may disguise the characteristic QRS morphology associated with ventricular pre-excitation (see Supraventricular Tachycardia, Wolff-Parkinson-White Syndrome). In patients with known right bundle branch block (eg, tetralogy of Fallot) and Wolff-Parkinson-White syndrome with a left-sided accessory pathway, the presence of the pathway is often undetected. In patients with right bundle branch block and a right-sided accessory pathway, the QRS may appear relatively normal without the expected right bundle branch block pattern.

Previous
Proceed to Differential Diagnoses
 
 
Contributor Information and Disclosures
Author

Glenn T Wetzel, MD, PhD  Professor of Pediatrics, University of Tennessee College of Medicine; Director, Pediatric Arrhythmia Service, Le Bonheur Children's Medical Center

Glenn T Wetzel, MD, PhD is a member of the following medical societies: American Academy of Pediatrics, American Heart Association, and Society for Pediatric Research

Disclosure: Nothing to disclose.

Coauthor(s)

Kenneth R Knecht, MD  Fellow, Department of Pediatrics, Division of Pediatric Cardiology, University of Tennessee Health Science Center

Kenneth R Knecht, MD is a member of the following medical societies: American Academy of Pediatrics

Disclosure: Nothing to disclose.

Specialty Editor Board

Christopher Johnsrude, MD  Associate Professor of Pediatrics, Director of Electrophysiology, University of Louisville School of Medicine; Consulting Staff, Pediatric Cardiology Associates, PSC

Christopher Johnsrude, MD is a member of the following medical societies: American Academy of Pediatrics and American College of Cardiology

Disclosure: Nothing to disclose.

Mary L Windle, PharmD  Adjunct Associate Professor, University of Nebraska Medical Center College of Pharmacy; Pharmacy Editor, eMedicine

Disclosure: Nothing to disclose.

Alvin J Chin, MD  Professor of Pediatrics, University of Pennsylvania School of Medicine; Attending Physician, Cardiology Division, Children's Hospital of Philadelphia

Alvin J Chin, MD, is a member of the following medical societies: American Association for the Advancement of Science, American Heart Association, and Society for Developmental Biology

Disclosure: Nothing to disclose.

Gilbert Z Herzberg, MD  Assistant Professor, Department of Pediatrics, Section of Pediatric Cardiology, New York Medical College; Consulting Staff, Department of Pediatrics, Sound Shore Medical Center

Gilbert Z Herzberg, MD is a member of the following medical societies: American Academy of Pediatrics

Disclosure: Nothing to disclose.

Chief Editor

Steven R Neish, MD, SM  Director of Pediatric Cardiology Fellowship Program, Associate Professor, Department of Pediatrics, Baylor College of Medicine

Steven R Neish, MD, SM is a member of the following medical societies: American Academy of Pediatrics, American College of Cardiology, and American Heart Association

Disclosure: Nothing to disclose.

References

  1. Stein R, Nguyen P, Abella J, Olson H, Myers J, Froelicher V. Prevalence and prognostic significance of exercise-induced right bundle branch block. Am J Cardiol. Mar 1 2010;105(5):677-80. [Medline].

  2. [Guideline] Finnish Medical Society Duodecim. Myocardial infarction. In: EBM Guidelines. Evidence-Based Medicine [Internet]. Helsinki, Finland: Wiley Interscience. John Wiley & Sons; 2008 Apr 2. [Full Text].

  3. Stephan E, Chedid R, Loiselet J, Bouvagnet P. Clinical and molecular genetics of familial bundle branch block related to chromosome 19 [in French]. Arch Mal Coeur Vaiss. Dec 1998;91(12):1465-74. [Medline].

  4. Deschenes I, Baroudi G, Berthet M, et al. Electrophysiological characterization of SCN5A mutations causing long QT (E1784K) and Brugada (R1512W and R1432G) syndromes. Cardiovasc Res. Apr 2000;46(1):55-65. [Medline].

  5. Keller DI, Barrane FZ, Gouas L, et al. A novel nonsense mutation in the SCN5A gene leads to Brugada syndrome and a silent gene mutation carrier state. Can J Cardiol. Sep 2005;21(11):925-31. [Medline].

  6. Diogenes MS, Succi RC, Machado DM, et al. Cardiac longitudinal study of children perinatally exposed to human immunodeficiency virus type 1 [in Portuguese]. Arq Bras Cardiol. Oct 2005;85(4):233-40. [Medline].

  7. Daga B, Minano A, de la Puerta I, et al. Electrocardiographic findings typical of Brugada syndrome unmasked by cocaine consumption [in Spanish]. Rev Esp Cardiol. Nov 2005;58(11):1355-7. [Medline].

  8. Jain R, Dalal D, Daly A, et al. Electrocardiographic features of arrhythmogenic right ventricular dysplasia. Circulation. Aug 11 2009;120(6):477-87. [Medline].

  9. Becker AE, Anderson RH. Morphology of the human atrioventricular junctional area. In: Wellens JHH, Lie KI, Janse MJ eds. The Conduction System of the Heart. 1976:263-71.

  10. Brugada J, Brugada R, Brugada P. Right bundle-branch block and ST-segment elevation in leads V1 through V3: a marker for sudden death in patients without demonstrable structural heart disease. Circulation. Feb 10 1998;97(5):457-60. [Medline].

  11. Brugada P, Brugada J. Right bundle branch block, persistent ST segment elevation and sudden cardiac death: a distinct clinical and electrocardiographic syndrome. A multicenter report. J Am Coll Cardiol. Nov 15 1992;20(6):1391-6. [Medline].

  12. Brugada P, Brugada R, Brugada J. Sudden death in patients and relatives with the syndrome of right bundle branch block, ST segment elevation in the precordial leads V(1)to V(3)and sudden death. Eur Heart J. Feb 2000;21(4):321-6. [Medline].

  13. Colvin EV. Cardiac embryology. In: Garson A, Bricker JT, Fisher DJ, Neish SR, eds. The Science and Practice of Pediatric Cardiology. 2nd ed. Baltimore, MD: Williams & Wilkins; 1998:91-123.

  14. Dubin D. Rapid Interpretation of EKGs. 3rd ed. Tampa, FL: Cover; 1984:137-42.

  15. Esscher E, Hardell LI, Michaelsson M. Familial, isolated, complete right bundle-branch block. Br Heart J. Jul 1975;37(7):745-7. [Medline].

  16. Ewing L. Bundle-branch and fasicular blocks. In: Gillette PC, Garson A, eds. Pediatric Arrhythmias: Electrophysiology and Pacing. WB Saunders Co; 1990:319-21.

  17. Garson A. Interventricular conduction disturbances. In: The Electrocardiogram in Infants and Children: A Systematic Approach. Philadelphia, PA: Lea & Febiger; 1983:119-42.

  18. Garson A. Electrocardiography. In: Garson A, Bricker JT, Fisher DJ, Neish SR, eds. The Science and Practice of Pediatric Cardiology. 2nd ed. Philadelphia, PA: Lippincott, Williams & Wilkins; 1998:736.

  19. Udink ten Cate FE, van Heerde M, Rammeloo LA, Hruda J. Transientelectrocardiographic abnormalities following blunt chest trauma in a child. Eur J Pediatr. November 2008;167(11):1331-3.

  20. Horowitz LN, Alexander JA, Edmunds LH Jr. Postoperative right bundle branch block: identification of three levels of block. Circulation. Aug 1980;62(2):319-28. [Medline].

  21. Husson GS, Blackman MS, Rogers MC, et al. Familial congenital bundle branch system disease. Am J Cardiol. Sep 7 1973;32(3):365-9. [Medline].

  22. James TN. The connecting pathways between the sinus node and A-V node and between the right and the left atrium in the human heart. Am Heart J. Oct 1963;66:498-508. [Medline].

  23. Kehoe RF, Bauernfeind R, Tommaso C, et al. Cardiac conduction defects in polymyositis: electrophysiologic studies in four patients. Ann Intern Med. Jan 1981;94(1):41-3. [Medline].

  24. Komajda M, Frank R, Vedel J, et al. Intracardiac conduction defects in dystrophia myotonica. Electrophysiological study of 12 cases. Br Heart J. Mar 1980;43(3):315-20. [Medline].

  25. Krongrad E. Prognosis for patients with congenital heart disease and postoperative intraventricular conduction defects. Circulation. May 1978;57(5):867-70. [Medline].

  26. Li TC, Hu DY, Bian H, et al. Effects of transcatheter closure of perimembranous ventricular septal defects: intermediate and long-term follow-up of 68 cases [in Chinese]. Zhonghua Yi Xue Za Zhi. Oct 26 2005;85(40):2846-9. [Medline].

  27. Massing GK, James TN. Anatomical configuration of the His bundle and bundle branches in the human heart. Circulation. Apr 1976;53(4):609-21. [Medline].

  28. Ogura Y, Kato J, Ogawa Y, et al. A case of alternating bundle branch block in combination with intra-Hisian block. Int Heart J. Jul 2005;46(4):737-44. [Medline].

  29. Pickhoff AS. Electrophysiology; development and function of the cardiac conduction system. In: Allen HD, Gutgesell HP, Clark EB, Driscoll DJ, eds. Heart Disease in Infants, Children, and Adolescents: Including the Fetus and Young Adult. 6th ed. Philadelphia, PA: Lippincott Williams & Wilkins; 2000:414-24.

  30. Priori SG, Napolitano C, Gasparini M, et al. Clinical and genetic heterogeneity of right bundle branch block and ST- segment elevation syndrome: A prospective evaluation of 52 families. Circulation. Nov 14 2000;102(20):2509-15. [Medline].

  31. Rossi L. Histopathology of Cardiac Arrhythmias. 2nd ed. Philadelphia, PA: Lea & Febiger; 1979:1-75.

  32. Sanyal SK, Johnson WW. Cardiac conduction abnormalities in children with Duchenne''s progressive muscular dystrophy: electrocardiographic features and morphologic correlates. Circulation. Oct 1982;66(4):853-63. [Medline].

  33. Schaal SF, Seidensticker J, Goodman R, Wooley CF. Familial right bundle-branch block, left axis deviation, complete heart block, and early death. A heritable disorder of cardiac conduction. Ann Intern Med. Jul 1973;79(1):63-6. [Medline].

  34. Scheinman MM, Goldschlager NF, Peters RW. Bundle branch block. Cardiovasc Clin. 1980;11(1):57-80. [Medline].

  35. Silka MJ. Bundle branch block. In: Garson A, Bricker JT, Fisher DJ, Neish SR, eds. The Science and Practice of Pediatric Cardiology. 2nd ed. Philadelphia, PA: Lippincott Williams & Wilkins; 1998:2033-6.

  36. Simonsen EE, Madsen EG. Four cases of right-sided bundle-branch block and one case of atrioventricular block in three generations of a family. Br Heart J. Jul 1970;32(4):501-4. [Medline].

  37. Stephan E. Hereditary bundle branch system defect. A new genetic entity?. Am Heart J. Jun 1979;97(6):708-18. [Medline].

  38. Stephan E. Hereditary bundle branch system defect: survey of a family with four affected generations. Am Heart J. Jan 1978;95(1):89-95. [Medline].

  39. Sun XJ, Gao W, Zhou AQ, et al. Risk factors for arrhythmia early after transcatheter closure of perimembranous ventricular septal defects [in Chinese]. Zhonghua Er Ke Za Zhi. Oct 2005;43(10):767-71. [Medline].

  40. Sung RJ, Tamer DM, Garcia OL, et al. Analysis of surgically-induced right bundle branch block pattern using intracardiac recording techniques. Circulation. Sep 1976;54(3):442-6. [Medline].

  41. Tawara S. Das Reizleitungssystem des Saugetierkerzens. Jena: Gustav Fisher; 1906.

  42. Van Praagh R, Van Praagh S. Morphologic anatomy. In: Fyler DC, ed. Nadas' Pediatric Cardiology. Philadelphia, PA: Hanley & Belfus; 1992:23.

  43. Waller BF, Schlant RC. Anatomy of the heart. In: O'Rourke RA, Schlant RC, Roberts R, et al, eds. Hurst's The Heart. 8th ed. New York, NY: McGraw-Hill Medical; 1994:99-102.

  44. Walsh, EP. Electrocardiography and introduction to electrophysiologic techniques. In: Fyler, DC, ed. Nadas' Pediatric Cardiology. Philadelphia, PA: Hanley & Belfus; 1992:134.

  45. Yasui H, Yoshitoshi M, Komori M, et al. Cardiodynamic effects of experimental right bundle branch block in canine hearts with normal and hypertrophied right ventricles. Am Heart J. Jan 1985;109(1):69-77. [Medline].

 
Previous
Next
 
ECGs demonstrate a normal sinus rhythm and a sinus rhythm with a right bundle branch block.
Anatomy of the penetrating portion of the atrioventricular (AV) bundle.
ECG depicts electrophysiologic events of right bundle branch block. AV = atrioventricular.
Pathophysiology of right bundle branch block. AV = atrioventricular; LV = left ventricular; RV = right ventricular.
ECG depicts electrophysiologic sequence of events that occur in normal cardiac conduction. AV = atrioventricular.
ECG demonstrates a wide S wave.
 
 
 
All material on this website is protected by copyright, Copyright © 1994-2012 by WebMD LLC.
This website also contains material copyrighted by 3rd parties.

DISCLAIMER: The content of this Website is not influenced by sponsors. The site is designed primarily for use by qualified physicians and other medical professionals. The information contained herein should NOT be used as a substitute for the advice of an appropriately qualified and licensed physician or other health care provider. The information provided here is for educational and informational purposes only. In no way should it be considered as offering medical advice. Please check with a physician if you suspect you are ill.