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The Annals of Thoracic Surgery, Vol 57, 88-91, Copyright © 1994 by The Society of Thoracic Surgeons
MK Heinemann, FL Hanley, S Van Praagh, KN Fenton, RA Jonas, JE Mayer Jr and AR Castaneda
Children with visceral heterotaxy often present with total anomalous
pulmonary venous drainage (TAPVD) associated with univentricular congenital
heart disease. We reviewed our experience with the primary surgical
management of this lesion under these circumstances. Over a recent 10-year
span, 38 patients within the first 3 days of life were admitted to our
institution and underwent primary palliation. Twenty- one of them had
TAPVD, 18 to a systemic vein. Twelve (67%) of these 18 were seen with
obstruction of the anomalous connection and underwent emergency operation.
In 7 patients, repair of TAPVD was combined with a systemic-pulmonary
artery shunt because of additional obstruction of the pulmonary blood
supply, with two deaths. One patient had primary shunting and then repair
of TAPVD. Four patients underwent repair of TAPVD alone. Two of them then
showed signs of insufficient pulmonary blood flow, received a shunt in a
second procedure, and subsequently died. Early mortality in the group with
obstructed TAPVD was thus 4 (33%) of 12 patients. Statistical analysis of
all 38 patients (univariate analysis, chi 2 testing) showed that neither
the presence of TAPVD (p = 0.7) nor TAPVD repair alone (p = 0.8) or with
shunting (p = 0.8) was a definite risk factor for early death. The
performance of a shunt during the first operation, however, was associated
with lower early mortality (p = 0.03). Total anomalous pulmonary venous
drainage is a common finding in newborns with visceral heterotaxy. Its
presence and its subsequent early repair (requiring cardiopulmonary bypass)
do not increase the mortality risk. The need of a concomitant shunt in
obstructed TAPVD can initially be underestimated.
ARTICLES
Total anomalous pulmonary venous drainage in newborns with visceral heterotaxy
Department of Cardiac Surgery, Children's Hospital, Harvard Medical School, Boston, Massachusetts.
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