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Sixteen consecutive patients (12 with tricuspid atresia) underwent a Fontan procedure, with no operative deaths. There were three late deaths. Eleven of the survivors were electively catheterized 4-25 months postoperatively. Rest and exercise hemodynamics were measured in five patients, and resting hemodynamics alone were measured in three. All exercised patients were New York Heart Association class I Cardiac index was low at rest (2.3 +/- 0.61/min/m2) and during exercise (4.9 +/- 1.11/min/m2) due to a low stroke index both at rest (28 ml) and exercise (35 ml). Accordingly, mixed venous oxygen saturations were decreased (66% at rest and 31% during exercise). These values are significantly lower than those at rest and during exercise from 23 control patients of similar age and size. Heart rates, pulmonary vascular resistances, and left ventricular filling pressures appeared normal both at rest and during exercise. High right atrial pressure at rest (15 mm Hg) was associated with minimal conduit gradient (2 mm Hg). However, exercise increased the mean conduit gradient to 8 mm Hg, demonstrating significant functional conduit obstruction. As expected, the cardiovascular response to exercise is abnormal after the Fontan procedure, even in asymptomatic patients. This abnormal response may be exacerbated by conduit obstruction, and conduit obstruction may not be apparent during resting studies.

Rest and exercise hemodynamics after the Fontan procedure.