Mid-term Results After Surgical Correction of Total Anomalous Pulmonary Venous Connection

Objective: To evaluate the surgical treatment
of total anomalous pulmonary venous
connection (TAPVC) and determination
of predictors for postoperative death.
Method & Material: Between 1995 and 2005,
80 patients aged from 1 month to 12 years
underwent surgical repair for supracardiac
(39), Cardiac (34), infracardiac (3) or
mixed (4) type of TAPVC. There were an
atrial communication in all patients, large
PFO in 23% and ASD in 77%. Systemic
pulmonary hypertension (PH) in 53.8%
of patients, half systemic PH in 26.3%
and mild PH (<40 mmHg) were found by
preoperative evaluations. Twelve patients
(15%) had some degrees of pulmonary
vein obstruction preoperatively.
Results: Seven patients (8.7%) died in
the operating room. Early postoperative
mortality (during 48 hours) occurred in 11
cases (13.7%) and nine patients died during
first hospitalization. We did not have late
mortality in survivors during follow-up
period; therefore the overall mortality
rate was 33.8%. Heart failure and low
cardiac output syndrome were the most
common causes of death. The incidence
of postoperative death was the highest in
infracardiac type (2/3). Approximately
two thirds of dead patients (21/27) had
presented with systemic PH and 89%
of them had at least half-systemic PH
preoperatively. Mortality rate in patients
with normal Pulmonary artery pressure
(PAP) was zero