Circulation, Vol 88, 1691-1698, Copyright © 1993 by American Heart Association
JJ Rome, JE Mayer, AR Castaneda and JE Lock
BACKGROUND. Patients with tetralogy of Fallot, pulmonary atresia, and
diminutive pulmonary arteries are a high-risk group for whom there is no
consensus on the correct approach to medical management. The purpose of
this report is to review a 14-year experience in the treatment of these
patients comparing management schemes. METHODS AND RESULTS. Between January
1978 and August 1988, 91 patients with tetralogy of Fallot and pulmonary
atresia had an adequate evaluation of their pulmonary artery anatomy before
any surgical management. Forty-eight of these patients had diminutive
pulmonary arteries (38 to 104 mm2/m2) supplied by aortopulmonary
collaterals and were managed in four different fashions. Of the 9 patients
repaired primarily, 7 died early and the two survivors had poor hemodynamic
outcome. Of the 9 patients conservatively managed with no intervention
before 5 years of age, 4 died and only 1 had a satisfactory hemodynamic
result after repair. Of 10 shunted patients, 3 died and 3 had satisfactory
repairs. Since 1984, we have rehabilitated pulmonary arteries with (1)
right ventricle to pulmonary artery surgical graft, (2) balloon dilation of
residual pulmonary artery stenoses and embolization of collaterals, and (3)
surgical closure of ventricular septal defect and repair of remaining
obstructions. Of 20 patients so managed, 7 died after various stages, but
10 of 20 had complete repairs. All repaired patients with subsystemic right
ventricular pressures had at least one successful pulmonary artery
dilation. CONCLUSIONS. A combined catheter-surgery approach begun at an
early age in patients with tetralogy of Fallot and pulmonary atresia with
diminutive pulmonary arteries appears to enhance the chances of
satisfactory complete repair.
ARTICLES
Tetralogy of Fallot with pulmonary atresia. Rehabilitation of diminutive pulmonary arteries
Department of Cardiology, Children's Hospital, Boston, MA 02115.
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