Truncus Arteriosus: Siriraj Experience

Abstract

Between January 1st, 1995 and December 31st, 2004, a total of 30 children (17 girls, 56.7% and 13 boys, 43.3%) were diagnosed to have truncus arteriosus at the Department of Pediatrics, Siriraj Hospital. The ages at the first diagnosis ranged from 1 day to 3 years (median 120 days). The predominant clinical presentations were congestive heart failure (53.3%), cyanosis (30%) and feeding problems (26.7%). All patients had heart murmur. Chest roentgenogram demonstrated cardiomegaly and increased pulmonary vascularity in 86.7% and 83.3%, respectively. Electrocardiogram showed a frontal plane QRS axis in a range of 0-90o in 70% of the patients, left ventricular hypertrophy and biventricular hypertrophy in 50% and 40%, respectively. Echocardiogram revealed type I anomaly in the majority of the patients (80%), and type II in the rest of patients. Ten percent of the patients had right-sided aortic arch. The majority of the patients received more than one medication for controlling heart failure. Sixteen patients (53.3%) were operated at the median age of 133 days and median weight of 3.8 kilograms. Thirteen percent of the patients (2 cases) had palliative surgery (pulmonary artery banding) and 87.5% of patients (14 cases) underwent total correction. All patients who had total repair had immediate complications, of which the majority (57.1%) were pulmonary hypertensive crisis. There were a total of 9 deaths (30%); 7 patients died immediately (<14 days) post total repair, 2 patients died preoperatively. During follow-up (median 57.9 months), all patients were asymptomatic except one patient (90.9%) who was re-operated on for conduit replacement due to severe stenosis and truncal valve repair due to severe regurgitation at 22 months after the first operation. Three patients have been waiting for surgery. In the present study, we could not definitely relate the associated risk factor with mortality such as the age at surgery, preoperatively high pulmonary vascular resistance or truncal valve abnormality. However, it seems that the older age at surgery may be the possible risk factor. Therefore, early diagnosis and surgical intervention for this anomaly should be considered.