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.
ABSTRACT
Fetal echocardiography has been accepted as a prenatal noninvasive diagnostic tool of cardiovascular diseases in fetuses for more than three decades. There are limited data in Thailand. A retrospective study of prenatal, natal and postnatal data of pregnant women who had fetal echocardiography at Siriraj Hospital, Bangkok, Thailand, from January 1999 to July 2005 was conducted. In total, there were 117 pregnant women who had fetal echocardiography under standard indications. Median age of pregnant women was 30.2 (17.3-44.2) years old. Median gestational age at the first fetal echocardiography was 29 (17-40) weeks. Median number of time the women had fetal echocardiography was 1 (1-10). Indications for fetal echocardiography were; obstetricians suspected of fetal cardiovascular diseases, multiple anomalies, family history of congenital heart diseases, chromosome anomalies, hydrops fetalis, and other indications (53.8%, 12%, 11.1%, 8.5%, 8.5% and 6.0% respectively). There were 49 (41.8%) cases who had abnormal cardiovascular problems, i.e., structural heart diseases 26 cases, rhythm disturbance 15 cases, and cardiac masses 8 cases. Upon comparison with postnatal echocardiography and/or autopsy findings, fetal echocardiography had a sensitivity of 96.9%, specificity of 90.6%, and accuracy of 92.8%. There was various degrees of risk to have cardiovascular problems for each of the indication taking fetal echocardiography and postnatal data into consideration. Fetal echocardiography had an impact on the management in 57.3% of cases. Conclusion: Fetal echocardiography is a reliable prenatal diagnostic tool for cardiovascular problems with high accuracy and has an impact on the management at prenatal, natal and postnatal period. Different indications for fetal echocardiography have an effect on the relative risk of fetal cardiovascular diseases.