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Flow dynamics in the main pulmonary artery after the Fontan procedure in patients with tricuspid atresia or single ventricle.

We analyzed the flow velocity pattern in the main pulmonary artery after Fontan operation in patients with tricuspid atresia (n = 10) or with single ventricle (n = 10) by means of a catheter-mounted velocity probe. The area underneath the velocity signal of the forward flow was integrated, and ratios of the portions during atrial systole and during the diastolic phase to the total area (Fa and Fd) were calculated. The Fa was 0.54 +/- 0.09 in patients with tricuspid atresia and 0.45 +/- 0.05 in those with single ventricle (p less than .01). Cardiac output, obtained by the thermodilution method, was 2.45 +/- 0.48 liters/min/m2 in patients with tricuspid atresia and 2.75 +/- 0.72 liters/min/m2 in those with single ventricle. The forward flow during atrial contraction, calculated by multiplying Fa by cardiac output, was 1.32 +/- 0.35 liters/min/m2 in patients with tricuspid atresia and 1.23 +/- 0.33 liters/min/m2 in those with single ventricle. The diastolic forward flow, calculated from Fd and cardiac output, was 0.99 +/- 0.25 liter/min/m2 in patients with tricuspid atresia and 1.52 +/- 0.45 liters/min/m2 in those with single ventricle (p less than 0.005). The sum of cross-sectional areas of the right and left pulmonary arteries normalized by body surface area (PA index) was 282 +/- 85 cm2/m2 in patients with tricuspid atresia and 462 +/- 65 cm2/m2 in those with single ventricle (p less than .005). The Fa was inversely correlated with the PA index in the whole group (r = -.69) and also in the tricuspid atresia group alone (r = -.87).(ABSTRACT TRUNCATED AT 250 WORDS)

Blood Flow Velocity↗

Pulmonary vascular structure in patients dying after a Fontan procedure. The lung as a risk factor.

Pulmonary vascular structure was analysed in the lungs of 12 patients who had had a Fontan repair, 11 of whom died. Of the eight patients with a low pulmonary blood flow preoperatively, pulmonary vascular structure was almost normal in seven, but in the remaining patient many intra-acinar arteries contained organised occlusive thrombi. Of the four patients with an increased pulmonary blood flow immediately before operation, there was a significant increase in pulmonary vascular smooth muscle despite three of the patients having had a pulmonary arterial band. The findings suggest that it is more difficult for patients with an increased pulmonary blood flow to fulfil the Fontan criteria for repair, and even when the pulmonary vascular resistance is less than 4 units/m2 there may be significant pulmonary vascular abnormalities likely to increase right atrial afterload and prejudice the outcome of a Fontan repair.

Arteries↗

Successful modified Fontan procedure in an adolescent after left pneumonectomy.

We performed a Waterston's anastomosis on a patient with complicated tricuspid atresia when she was two months of age. At age 14 years she required left pneumonectomy for massive, idiopathic hemoptysis. Four months after the pneumonectomy we substituted a modified Fontan anastomosis for the Waterston anastomosis. Restudy when she was 17 years of age showed continued satisfactory function.

Adolescent↗

Magnetic resonance imaging of constant total heart volume and center of mass in patients with functional single ventricle before and after staged Fontan procedure.

It has been previously demonstrated that total heart volume (contents of pericardium) throughout the cardiac cycle varies by < 5% and location of center of mass by < 3 mm. The hypothesis has thus developed that for maximal efficiency, the heart should expend minimal energy in displacing extracardiac structures by maintaining a constant intracycle total heart volume and center of mass. This is achieved in the normal heart mainly by a piston-like movement of the atrioventricular valve plane toward the ventricular apex. As this has never been studied in the single ventricle heart or at various stages of Fontan reconstruction, it is conceivable that these patients may not exhibit the constancy of total heart volume and location of center of mass, which may lead to a poor outcome in some. The total heart volume and center of mass relationship was therefore evaluated in 25 children (0.4 to 237 months) with functional single ventricles at all stages of Fontan reconstruction using multiphase, multislice spin-echo or cine-magnetic resonance imaging. No significant difference was seen in variation between total heart volume and maximal volume between patients before bilateral cavopulmonary anastomosis (hemiFontan) (5.1 +/- 2.9%), after hemiFontan (3.6 +/- 1.6%) and after Fontan (8.2 +/- 7.7%); however, in 4 of 10, 1 of 8 and 5 of 7 patients, respectively, the total heart volume varied by < 5%. Across surgical subgroups, significant differences were found in the center of mass displacement (total, anteroposterior and superoinferior planes) but not in directionality of displacement.(ABSTRACT TRUNCATED AT 250 WORDS)

Analysis of Variance↗

Fontan procedure and surgical modification in tricuspid atresia.

Nineteen patients with tricuspid atresia and reduced lung perfusion (valvular-and/or subvalvular pulmonary stenosis, transposition of the great arteries and/or single atrium) were operated in the period 1975--1979. The surgical procedures employed varied according to the additional cardiac defects. The age of the patients was between 2 and 18 years. Eleven children received a contuit with a Hancock valve, 8 children a valveless conduit. In 9 patients it was possible to connect the conduit to the right ventricle using the pumping action of the right ventricle with an anatomically intact pulmonary valve. Six patients died (4 early deaths, 2 late deaths, early mortality rate 21%). In this study, the postoperative courses, which were complicated in several cases, are related to the hemodynamical findings. Thirteen children were examined between 1 and 38 months following the operation. Cardiac catheterization was performed in 10 patients. Of the 13, 10 had fully saturated arterial blood. A remnant atrial defect was demonstrated in one child, and 2 displayed intrapulmonary shunts attendant to Glenn anastomoses which had been in place for 10 to 12 years. Among the patients the right atrial pressure ranged from 10 to 20 mmHg with a mean value of 14.5 +/- 0.9 mmHg. An increase in size of the right ventricle was demonstrated angiographically in the case of 2 patients who had valve-bearing conduits to the right ventricle.

Adolescent↗