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(Circulation. 1996;93:1588-1600.)
© 1996 American Heart Association, Inc.
Articles |
Complete Heart Block and Fatal Right Ventricular Failure in an Infant
From the World Health Organization Cardiovascular Center (T.N.J.) and the Departments of Medicine (T.N.J.), Pathology (T.N.J., M.M.N., P.L.D.), and Pediatrics (D.W.S., S.M.L.), University of Texas Medical Branch, Galveston.
Correspondence to Thomas N. James, MD, Office of the President, University of Texas Medical Branch, Galveston, TX 77555-0129.
Key Words: Uhl's anomaly • morphogenesis • heart block • heart failure • death, sudden
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Case Presentation |
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Drs David W. Sapire and Suzanne M. Lopez
Maternal History
The mother of this child was a 22-year-old primigravida in apparent good health. There was no clinical or biochemical evidence indicating use of ethanol, addictive drugs, or other possibly teratogenic substances. Fetal heart rate on ultrasonograms done early in her pregnancy was about 150 beats per minute. Near the end of her fifth month of pregnancy, she was referred to the John Sealy Hospital at the University of Texas Medical Branch (UTMB) because the fetal heart rate had decreased to 86 beats per minute. Although the atrial rate on subsequent examinations remained about 150 beats per minute, the ventricular rate ranged from 40 to 50 beats per minute. Two weeks before delivery, atrial and ventricular rates were 144 and 47 beats per minute, respectively. When the ventricular rate began to slow even further, it was decided that risk of intrauterine death was imminent, and a Caesarean section delivery was performed at approximately the 28th week of gestation.
There was no significant family history relevant to the case. The mother's serological studies for lupus erythematosus and antiphospholipid syndrome were negative.
Infant's Hospital Course
The baby was in no major distress just after delivery, but her
heart rate increased very little in response to increasing doses of
isoproterenol. Within hours after birth, it was decided that an
electronic pacemaker was advisable. During epicardial placement of
electrodes near the left ventricular apex, the surgeon
noted that the "infiltrated" right ventricular
myocardium was thin and that it did not respond to
electronic pacing. Over the following weeks,
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