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The Annals of Thoracic Surgery, Vol 50, 179-183, Copyright © 1990 by The Society of Thoracic Surgeons
PJ Horneffer, KG Zahka, SA Rowe, TA Manolio, VL Gott, BA Reitz and TJ Gardner
Between 1958 and 1977, 170 children aged 10 years or less underwent total
repair of tetralogy of Fallot at The Johns Hopkins Hospital. Follow-up data
were obtained on 128 (90%) of the 143 who survived the operation at 10 to
28 years postoperatively (mean follow-up, 18 years). All patients completed
an extensive questionnaire, and 59 returned for a thorough evaluation
consisting of a history and physical examination, electrocardiogram,
24-hour Holter monitoring, exercise stress testing, pulmonary function
testing, and two-dimensional and Doppler echocardiography. Late survival
was excellent with only two of four known late deaths due to
cardiac-related causes and with all 59 patients in New York Heart
Association class I or II. None had cyanosis or clubbing. Normal sinus
rhythm was present in 90%. One patient had complete heart block, and 75%
had right bundle-branch block on the electrocardiogram. Right ventricular
function was normal by echocardiography in 78%. Residual mild to moderate
pulmonary stenosis was noted by Doppler study in 8 patients. Pulmonary
regurgitation was present in 78%, but in only 11 patients was it graded as
moderate and in none was it severe. Stress testing documented the excellent
functional status of most patients, with 92% of predicted exercise time and
94% of maximum heart rate being attained. In the few who had impaired
cardiac performance, this correlated best with moderate pulmonary
regurgitation. Although the overall late functional status of patients
undergoing repair in the first decade of life was very good, these patients
need continued follow-up to assess the severity of pulmonary regurgitation
and the need of possible intervention.
ARTICLES
Long-term results of total repair of tetralogy of Fallot in childhood
Division of Cardiac Surgery, Johns Hopkins School of Medicine, Baltimore, Maryland.
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