Seventy-three patients with isolated total anomalous pulmonary venous connection-the patients associated with other major cardiac anomalies such as single ventricle, DORV (Double Outlet Right Ventricle) and large VSD (Ventricular Septal Defect)
were
excluded -were underwent surgical repair from January 1980 through October 1993.
There were 45 boys and 28 girls. The mean age at operation was 19.9 months (range 6 days to 24 years) and mean body weight was 7.1kg (range 2.6kg to 45kg). The anomalous locations of connection were supracardiac in 38, cardiac in 21, infracardiac
in 5,
and mixed in 9. In 38 patients (52%), the venous drainage was obstructed. The obstruction ratios according to the connection type were as follows :53% (20/38) in supracardiac, 52% (11./21) in cardiac, 100% (5/5) in infracardiac, 22% (2/9) in
mixed
type.
The operative mortality was 23%. The causes of death were pulmonary hypertensive crisis, perioperative myocardial failure, pneumonia with sepsis, arrhythmia and eto. The statistically significant factors in postoperative mortality were the
pulmonary
venous obstruction and age (p<0.01). The operative mortality was high in groups of age under 1 month and pulmonary venous obstruction.
The mean follow-up was 27.1 months. There were two late deaths. But all the other patients were in NYHA Fc Ⅰ and received no medications. The 5-year survival rate excluding early expired patients is 97.1*0.03%
In conclusion, although the operative mortality of total anomalous pulmonary venous connection was relatively high compared to other major cardiac anomalies, we could expect excellent long-term results by early surgical correction.
(Korean J Thorac Cardiovasc Surg 1995;28:565-70)
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