Because of the susceptibility for early development of pulmonary vascular obstructive
disease, early correction of Truncus Arteriosus (TA) is advised, which represents still a
surgical challenge.
We reviewed a series of 17 patients with TA, who were operated between February 1990
and September 1996. The median operative age was 21 days (5d - 16 years). Ten patients
(58.8 %) were operated at neonatal age and 12 patients (70.6 %) before the age of 3
months.
Morphologically 9 patients were classified into Type I and 8 patients into Type II TA.
Associated anomalies were: Interrupted Aortic Arch (2), truncal valve dysfunction (8),
ASD/PFO (6) and coronary anomaly (1). Total repair was performed in all children, using
insertion of 5 aortic (Ao) and 12 pulmonary (Pu) homografts (HG) with a mean diameter of
14 mm (8 - 25 mm). Two of them needed reduction to bicuspid valves. Only the oldest
patient had undergone previous palliative banding of both PAs. Hospital mortality was
23.5% (4 patients), essentially resulting from intractable pulmonary hypertension. Minor to
major morbidity was noticed in 9 of the 13 survivors (69.2 %): 3 delayed sternal closures,
a mean ventilation time of 18.5 d and a mean ICU stay of 26.6 d.
During the 5 to 149 month (mean 45.4) follow-up, 5 patients required reoperation without
mortality. Four HG in right position (3Ao; 1Pu) needed replacement as well as 1 incompetent
truncal valve. Associated plasty of a PA branch was performed in 3 cases. The remaining
unreplaced HG showed echographically moderate to severe insufficiency in 5 patients, a
gradient of 15 to 30 mm Hg in 3 patients and a distal HG PA gradient in 6 patients.
In conclusion, early TA repair can be performed with an acceptable mortality rate but with
a high morbidity rate, primarily relating to pulmonary hypertension. Furthermore,
pulmonary homografts should be preferred to reconstruct the RVOT, since most of the
aortic homografts were already replaced.
Although our number of patients is limited, a tendency to a higher reoperation rate is
noticed when TA repair happens before the age of 1 month.