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Pediatric Congenital Atrioventricular Block Clinical Presentation

  • Author: Monesha Gupta, MD, MBBS, FAAP, FACC, FASE; Chief Editor: P Syamasundar Rao, MD  more...
 
Updated: Mar 02, 2016
 

History

Congenital atrioventricular block (CAVB) may be identified during prenatal examinations, in the perinatal period, or during childhood or adulthood. Historical and other features widely vary and particularly depend on the timing of presentation and accompanying structural heart disease.

Isolated CAVB

Many of the mothers confirmed to have a fetus with autoimmune AV block may have no symptoms. The fetus in whom isolated CAVB is identified during the perinatal period usually presents with an incidental finding of bradycardia or hydrops fetalis.

Occasionally, these mothers may have a history of recurrent fetal loss. Fetuses of such mothers should be routinely evaluated for CAVB.

The mother is often completely asymptomatic. In addition to fetuses who are incidentally identified, siblings of children with known isolated heart block are at a higher risk with a recurrence rate for heart block of 17-22%.

In the perinatal period, fetal bradycardia may have myriad causes. However, when persistent bradycardia is identified in the third trimester, emergency delivery is sometimes inappropriately carried out without differentiating acute causes of bradycardia related to fetal distress from the more chronic condition of isolated CAVB.

Newborns with congenital heart block may present with a hydropic appearance secondary to fetal heart failure or may develop signs of low cardiac output within hours to days after birth. However, affected newborns often appear asymptomatic and may have accelerated ventricular rates approaching those of healthy newborns. Congenital heart block is not likely to be identified in many such infants until well after birth. An associated finding of neonatal lupus may be present.

In older infants or children, signs of low cardiac output due to bradycardia, such as pallor, mottling, lethargy, exercise intolerance, palpitations, dizziness, or syncope, may occur. Children may have sleep disturbances or be asymptomatic.

CAVB with structural heart disease

The diagnosis of a congenital heart defect in one child or first-degree relative is an indication for fetal echocardiography to check for cardiac malformations.

Children with structural heart defects may present with cyanosis, murmur, failure to thrive, or recurrent pneumonias or may be completely asymptomatic in childhood (such as children with L-transposition of the great arteries and intact ventricular septum).

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Physical Examination

The fetus may be monitored with ultrasonography for varying degrees of heart block and hydrops fetalis. In the newborn, the findings may range from asymptomatic to signs of congestive heart failure and low cardiac output.

The most typical physical finding is a low heart rate for age. Because the block is usually complete and the escape rhythm is usually junctional in origin, a regular rhythm at 60-80 beats per minute (bpm) is often found. Auscultation demonstrates a variable first heart sound caused by the AV asynchrony.

Congenital AV block may be associated with findings of low cardiac output or congestive heart failure. Low cardiac output may manifest with physical findings of irritability or lethargy, cool skin, mottling, or cyanosis.

Congestive heart failure may manifest with tachypnea and hepatomegaly. If congestive heart failure has been present prenatally, marked edema may be part of the complex of hydrops fetalis.

Children with neonatal lupus can present with rash, as well as with neurologic and hepatic manifestations. The rash can occur some days after birth and is worsened with sun exposure. Annular or elliptical erythematous plaques can be present on the skin of face, scalp, and extremities. It usually resolves without scarring but can be associated with residual hypopigmentation or, rarely, telangiectasias.

Children with structural heart disease may manifest symptoms and signs related to the specific congenital heart defect. Patients with heterotaxia syndrome may be cyanotic. Patients with L-transposition of the great arteries may be completely asymptomatic or may demonstrate a murmur.

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Contributor Information and Disclosures
Author

Monesha Gupta, MD, MBBS, FAAP, FACC, FASE Associate Professor of Pediatrics, Division of Pediatric Cardiology and Nephrology, Children's Memorial Hermann Hospital, University of Texas Medical School

Monesha Gupta, MD, MBBS, FAAP, FACC, FASE is a member of the following medical societies: American Academy of Pediatrics, American College of Cardiology, American Society of Echocardiography, Society for Pediatric Research, Society of Pediatric Echocardiography, Medical Council of India

Disclosure: Nothing to disclose.

Coauthor(s)

Robert Murray Hamilton, MD, MSc, FRCPC Electrophysiologist, Senior Associate Scientist, Physiology and Experimental Medicine, Labatt Family Heart Centre; Professor, Department of Pediatrics, University of Toronto Faculty of Medicine

Robert Murray Hamilton, MD, MSc, FRCPC is a member of the following medical societies: American Heart Association, Canadian Medical Association, Ontario Medical Association, Royal College of Physicians and Surgeons of Canada, Canadian Medical Protective Association, Heart Rhythm Society, Canadian Cardiovascular Society, Cardiac Electrophysiology Society, Pediatric and Congenital Electrophysiology Society, Society for Pediatric Research

Disclosure: Nothing to disclose.

Chief Editor

P Syamasundar Rao, MD Professor of Pediatrics and Medicine, Division of Cardiology, Emeritus Chief of Pediatric Cardiology, University of Texas Medical School at Houston and Children's Memorial Hermann Hospital

P Syamasundar Rao, MD is a member of the following medical societies: American Academy of Pediatrics, American Pediatric Society, American College of Cardiology, American Heart Association, Society for Cardiovascular Angiography and Interventions, Society for Pediatric Research

Disclosure: Nothing to disclose.

Acknowledgements

Charles I Berul, MD Professor of Pediatrics and Integrative Systems Biology, George Washington University School of Medicine; Chief, Division of Cardiology, Children's National Medical Center

Charles I Berul, MD is a member of the following medical societies: American Academy of Pediatrics, American College of Cardiology, American Heart Association, Cardiac Electrophysiology Society, Heart Rhythm Society, Pediatric and Congenital Electrophysiology Society, and Society for Pediatric Research

Disclosure: Johnson & Johnson Consulting fee Consulting

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.

Mary L Windle, PharmD Adjunct Associate Professor, University of Nebraska Medical Center College of Pharmacy; Editor-in-Chief, Medscape Drug Reference

Disclosure: Nothing to disclose.

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