European Journal of Cardio-Thoracic Surgery, Vol 10, 433-438, Copyright © 1996 by European Association for Cardio-thoracic Surgery
Early and long-term results for correction of total anomalous pulmonary venous drainage (TAPVD) in neonates and infants
N Sinzobahamvya, C Arenz, AM Brecher, HC Blaschczok and AE Urban
Department of Pediatric Cardiothoracic Surgery, Johanniter Kinderklinik, Sankt-Augustin, Germany.
OBJECTIVE: To present our 17-year experience of surgical repair of total
anomalous pulmonary venous drainage (TAPVD) in 71 consecutive neonates and
infants, with particular emphasis on the role of preoperative pulmonary
venous obstruction (PVO), the management of postoperative pulmonary
hypertensive crises and the long-term results. PATIENTS AND METHODS: From
July 1977 to May 1994, 36 newborns and 35 infants, aged between 1 and 300
days, had repair TAPVD in our Department. Fifty-four patients had
decompensated heart failure, whereas three were moribund. The anomalous
drainage was supracardiac in 32 (45%), cardiac in 17 (24%), infracardiac in
17 (24%) and mixed in 5 infants (7%). Major associated cardiac anomalies
coexisted in nine cases. Forty-five (63%) exhibited signs of PVO. The
repair was performed under moderate hypothermia in 15 and deep hypothermic
arrest in 56 patients. In case of postoperative pulmonary hypertensive
crises, artificial hyperventilation with high oxygen concentration,
sedation and relaxation, inotropic support and afterload reduction with
phentolamine were applied selectively. The actual follow-up is based on
functional symptomatology and cardiac echo-doppler studies. RESULTS: Six
children (8.5%) died early: four perioperatively, two on the 2nd and 10th
postoperative day, respectively. Poor preoperative condition, operation
before 1987 and preoperative PVO were the main incremental risk factors for
death. No death occurred among the last 38 cases. There were complications
in 45 patients, in particular pulmonary hypertensive crises in 27. Two
patients died late, after 80 and 118 days, respectively, of progressive
intrapulmonary vein fibrosis: the second despite reoperation. The risk of
late death approaches zero after 6 months. Among the 63 survivors, 56 (86%)
are asymptomatic and without medication, 3 months-17 years after operation.
Five have mild symptoms with ordinary physical activity. Two are lost to
follow-up. CONCLUSIONS: Early repair of TAPVD with aggressive management of
pulmonary hypertensive crises carries low operative mortality nowadays.
Preoperative PVO as a risk factor has been neutralized since 1987. Long-
term results are gratifying: no late death after 6 months, no reoperation
and functional good results. Progressive pulmonary vein fibrosis remains an
unpredictable rare cause of death within the 1st year after surgery.
