Catheterisation is the gold standard used to evaluate pulmonary blood flow in patients with a Blalock–Thomas–Taussig shunt. It involves risk and cannot be performed frequently. This study aimed to evaluate if echocardiographic measurements obtained in a clinical setting correlate with catheterisation-derived pulmonary blood flow in patients with a Blalock–Thomas–Taussig shunt as the sole source of pulmonary blood flow.

Chart review was performed retrospectively on consecutive patients referred to the catheterisation lab with a Blalock–Thomas–Taussig shunt. Echocardiographic parameters included peak, mean, and diastolic gradients across the Blalock–Thomas–Taussig shunt and forward and reverse velocity time integral across the distal transverse aorta. In addition to direct correlations, we tested a previously published formula for pulmonary blood flow calculated as velocity time integral across the shunt × heart rate × Blalock–Thomas–Taussig shunt area. Catheterisation parameters included pulmonary and systemic blood flow as calculated by the Fick principle.

18 patients were included. The echocardiography parameters and oxygen saturation did not correlate with catheterisation-derived pulmonary blood flow, systemic blood flow, or the ratio of pulmonary to systemic blood flow. As the ratio of reverse to forward velocity time integral across the transverse aorta increased, the probability of shunt stenosis decreased.

Echocardiographic measurements obtained outside the catheterisation lab do not correlate with catheterisation-derived pulmonary blood flow. The ratio of reverse to forward velocity time integral across the transverse aortic arch may be predictive of Blalock–Thomas–Taussig shunt narrowing; this finding should be investigated further.

The aim of our study was to compare the blood levels of adhesion molecules in children with different heart diseases and pulmonary flow rates.

In this study, we evaluated the levels of soluble intercellular adhesion molecule-1 and soluble vascular cellular adhesion molecule-1 in blood samples of 65 children with different congenital heart diseases. The patients were divided into four groups according to their pulmonary blood flow. The first group had increased pulmonary blood flow with pulmonary hypertension and left-to-right shunt. The second group had increased pulmonary blood flow without pulmonary hypertension and left-to-right shunt. The third group had decreased pulmonary blood flow with cyanotic congenital heart disease and the fourth group had normal pulmonary blood flow with left ventricle outflow tract obstruction and aortic stenosis.

The highest soluble intercellular and vascular cellular adhesion molecule-1 levels with the mean values of 420.2 nanograms per millilitre and 1382.1 nanograms per millilitre, respectively, were measured in the first group and the lowest levels with the mean values of 104.4 and 358.6 nanograms per millilitre, respectively, were measured in the fourth group. The highest pulmonary blood pressure levels were found in the first group.

Endothelial activity is influenced not only by left-to-right shunt with pulmonary hypertension, but also by decreased pulmonary blood flow in cyanotic heart diseases. Adhesion molecules are valuable markers of endothelial activity in congenital heart diseases, and they are influenced by pulmonary blood flow rate.

The bidirectional cavopulmonary shunt has become a mainstay in the palliation of patients with a functionally single ventricle. Despite its usefulness, there are a number of concerns following its construction, including issues of timing, the development of pulmonary arteriovenous fistulas, and growth of the pulmonary arteries.

Between January 1990 and April 1996, we constructed a bidirectional cavopulmonary shunt in 123 patients. Median age at surgery was 9.4 months, and ranged from 24 days to 43 years. In 25 patients (20%), it was the first operative procedure. An auxiliary source of pulmonary blood flow was included in 72 patients (58%).

Six patients (4.9%) died in the early postoperative period, and the overall early rate of failure (death or take-down) was 8.1%(n=10). By multivariate analysis, longer bypass time (p=0.012), age less than 1 month (p=0.03), and higher pulmonary vascular resistance (p = 0.043) were significant. Early reoperation was performed in 11 other patients to decrease (n=8) or increase (n=3) the amount of pulmonary blood flow. Early survivors were followed for a median of 15 months (range: 1 to 48 months), during which time 5 patients died and a Fontan circulation was completed in 30. Including early and late mortality, actuarial survival rates at 1 and 2 years were 91% and 88%, respectively. Among hospital survivors, the only significant predictor of poorer survival by Cox regression was age less than 2 months at the time of the initial cavopulmonary shunt (p=0.02). Atrioventricular valvar regurgitation was decreased after construction of the cavopulmonary shunt in 17 of the 28 patients (61%) in whom echocardiographic data were available to compare preoperative and postoperative status.

The bidirectional cavopulmonary shunt is a useful procedure in the early or intermediate term management of patients with a functionally univentricular heart. Much remains to be learned, nonetheless, about this unique physiologic system. The role of accessory pulmonary blood flow remains unclear, as does the use of the shunt as long-term palliation. Pulmonary arteriovenous fistulas are a serious concern, especially in young patients with heterotaxy syndrome (isomeric atrial appendages).