Echocardiographic assessment of total anomalous pulmonary venous connections in pediatric patients.

BACKGROUND AND PURPOSE

Cardiac catheterization can be superfluous and risky for sick babies, infants, and young children with total anomalous pulmonary venous connection (TAPVC). This study assessed the accuracy of echocardiography in the clinical assessment of pediatric patients with TAPVC before cardiac surgery.

PATIENTS AND METHODS

A total of 15 consecutive patients with TAPVC treated between July 1, 1993, and December 31, 1999, were included in this retrospective study. Patients with TAPVC with heterotaxy syndrome were excluded. We assessed this cardiac anomaly preoperatively using plain chest roentgenograms, echocardiography, and magnetic resonance imaging. A combination of suprasternal, parasternal, subcostal, and apical four-chamber views and their tilting scans were employed for diagnosis and to trace the course of the anomalous pulmonary venous connection.

RESULTS

Interatrial right-to-left shunt via atrial septal defects were documented by two-dimensional echocardiography with color Doppler mapping in all 15 patients. Patent ductus arteriosus was found in six patients. Cardiomegaly with enlargement of the right atrium and the right ventricle could be seen on plain chest roentgenograms and by echocardiography in all but two patients with infracardiac TAPVC. In all patients, the left innominate vein, coronary sinus or right atrium, and portal vein were the draining sites of supracardiac, cardiac, and infracardiac TAPVC, respectively. A pattern of continuous flow without phasic variation, which is suggestive of stenosis of the pulmonary vein, was found in two patients with infracardiac TAPVC with obstruction. The surgical findings were reviewed and correlated well with those of the echocardiography.

CONCLUSIONS

In infants and children with TAPVC, the drainage sites and flow profiles of the pulmonary veins can be exactly determined by Doppler echocardiography preoperatively, which makes cardiac catheterization and angiocardiography unnecessary.