Imminent fetal cardiac tamponade by right atrial hemangioma.

A fetus presented with a large pericardial effusion caused by a right atrial transmural tumor. Correct prenatal diagnosis by use of targeted fetal echocardiography indicated that treatment was not required until the gestational age of 36 weeks. At that time, cesarean section was performed because early signs of imminent cardiac tamponade developed ("swinging heart"). At birth, the pericardial effusion was drained with a percutaneous drain. Elective surgical resection was performed on day 6 of life. Histologically, the tumor was a benign capillary hemangioma.

Fetal cardiac tamponade due to an intrapericardial teratoma.

A case of an intrapericardial tumor diagnosed in utero at 26 weeks of gestation is presented. The prenatal echocardiographic follow-up of an incipient hydrops fetalis determined the management and the emergency surgical treatment. Histologically, the tumor appeared to be a benign teratoma, grade I. In the postoperative period an unexpected mediastinal tumor was found and removed later. This tumor also appeared to be a benign teratoma, grade 0. Both teratomas were independent and therefore primary.

Extended aortic root replacement with pulmonary autografts: experience in 14 cases.

The surgical relief of complex multilevel left ventricular outflow tract obstruction remains a challenging problem. We present a new operation that combines the concepts of aortoventriculoplasty, extended aortic root replacement, and the use of a pulmonary autograft. Fourteen patients underwent this operation: 9 patients after previous attempts to relieve diffuse subvalvular stenosis and 5 patients who had excessive gradients over an outgrown aortic valve prosthesis. All patients except 1 survived the operation. Complete heart block developed in 1 patient after a septal infarction. One patient remained in congestive heart failure and died suddenly after 17 months. All other patients are in New York Heart Association class I after a mean follow-up of 20 +/- 12 months. All patients showed excellent function of the autograft and homograft valve at follow-up. This operation might present a more durable or even a definitive solution in the management of these complex left ventricular outflow tract obstructions.

Exercise responses in patients with congenital heart disease after Fontan repair: patterns and determinants of performance.

After a Fontan repair for congenital heart disease, 42 patients underwent graded supine bicycle exercise tests at levels relevant to normal daily activities. Results were compared with those of 28 age-matched normal control subjects. At rest, the cardiac index, stroke index and systolic blood pressure were comparable in both groups, but increases with exercise were smaller in the patients with a Fontan circulation. The heart rate at rest was higher in the Fontan group, but this difference disappeared as soon as exercise started. To determine whether there are limitations intrinsic to the Fontan circulation at these levels of exercise, the 10 best performers were compared with 10 age-matched control subjects; no differences were found in cardiac index, stroke index, heart rate or blood pressure at any exercise level. Analysis of the determinants of cardiac output showed that at the other end of the spectrum poor performance after a Fontan operation did not result from inadequate levels of heart rate, but from an inability to increase or maintain stroke volume. Multivariate analysis demonstrated that impairment of ventricular contractility, only when severe, predicted limited performance. There was no evidence of increased afterload, particularly in the poor performers. Therefore, ventricular filling, which is determined primarily by the pulmonary vascular bed, appears to be a major determinant of functional result after a Fontan repair.

Impact of Fontan operation on left ventricular size and contractility in tricuspid atresia.

Left ventricular dimensions and contractility were determined by echocardiography in 33 patients with tricuspid atresia in 1985 and again in 1988. Eight patients remained palliated throughout the 3-year period; neither the left ventricular end-diastolic diameter (153 +/- 15% of normal vs. 157 +/- 19%, p = NS) nor a load-independent index of contractility (rate-corrected velocity of shortening [VCFc]/end-systolic meridional stress [ESSM]) changed. Eleven patients underwent a Fontan operation during the study and were reevaluated at least 6 months after surgery; left ventricular dimension decreased (130 +/- 15% vs. 114 +/- 19%, p less than 0.001), and the contractility index VCFc/ESSM improved (p less than 0.05). Fourteen patients had undergone a Fontan operation 0.9-9.5 years (mean, 4.2 years) before initial examination in 1985. Over the 3-year period, left ventricular dimensions did not change (121 +/- 17% vs. 118 +/- 11%, p = NS), but the contractility index showed significant improvement (p less than 0.01). Eight additional patients were studied just before and after a Fontan operation to examine the early effects of surgery. Left ventricular dimensions decreased from 130 +/- 14% to 100 +/- 13% by 10 days p less than 0.001) with no further change at 2 months. An inappropriate degree of ventricular hypertrophy was observed in only the early postoperative period. Successful Fontan repair results in rapid reduction of left ventricular size, followed by regression of hypertrophy to a normal mass-to-volume ratio. Operating at more favorable dimensions and loading conditions results in an early increase in left ventricular contractility, which further improves in the medium term follow-up.