A case of restrictive cardiomyopathy presenting in fetal life with an isolated pericardial effusion.

We describe the evolution during pregnancy of a case of restrictive cardiomyopathy which first presented at 22 weeks' gestation with a large pericardial effusion. Measurements of cardiac function were normal and remained near normal until late in the third trimester, when pulsed and tissue Doppler data suggested impairment in ventricular relaxation. This disease progressed in postnatal life to symptomatic restrictive cardiomyopathy by 2 years of age necessitating cardiac transplant. To our knowledge, this is the first time this unusual association has been reported.

Perioperative respiratory compliance in children undergoing repair of atrial septal defects.

Children with atrial septal defects (ASD) have less respiratory compliance (Crs) than normal cohorts. There could be implications for anaesthetic management if these children also have decreased compliance during anaesthesia. To examine the changes in Crs before, during and following surgical correction of the atrial defect, Crs was measured in 29 infants and children, 15 undergoing correction of secundum type atrial septal defects, and a group of 14 children of similar weight undergoing non-thoracic surgery. During sedation, Crs was measured using the single breath technique (SBT) and during anaesthesia, both before and after the surgical procedure, an inflation technique was applied to determine Crs. To investigate the aetiology of the difference in Crs, the pulmonary to systemic flow ratio (Qp:Qs) was determined using echocardiography during sedation in the ASD patients. During sedation, Crs in the ASD group was 52.7 +/- 19.5% less than in the control group. The slope of the line of regression of Crs vs height for the ASD group was significantly less (P less than 0.05) than that of the control group during sedation. However, during anaesthesia, Crs in the ASD group was not significantly different from the control group either before or after surgery. The per cent decrease in Crs during sedation in the children with ASD, in comparison with the control group, did not correlate with the Qp:Qs ratio of the ASD group (r2 = 0.012,NS). We conclude that, in spite of lower Crs during sedation, infants and children with ASD do not have lower Crs during anaesthesia and cardiopulmonary bypass than normal controls undergoing non-thoracic surgery.(ABSTRACT TRUNCATED AT 250 WORDS)

Angiographic features associated with percutaneous balloon valvotomy for pulmonary valve stenosis.

Changes in the mobility of the pulmonary valve were determined by a retrospective review of right ventricular cineangiograms from 25 balloon pulmonary valvotomy (BV) procedures in 23 infants and children. The angiographic changes were compared with the post-BV catheter and Doppler pressure gradients across the right ventricular outflow tract. Angiographic features felt to indicate valve tearing were present following 17 of 25 procedures and included increased excursion or straightening of leaflets, localized change in leaflet motion (flail leaflet), and the presence of an additional contrast jet through the valve. There was no statistically significant relationship between any of the angiographic parameters and the pressure data. Most patients with marked increase in angiographic valve mobility had low residual right ventricular to pulmonary artery gradients. However, the absence of angiographic change was not always associated with a high residual gradient.

The morphology of the right ventricular outflow tract after percutaneous pulmonary valvotomy: long term follow up.

Twenty nine patients (19 male, mean (SD) age 6.25 (0.5) years (range 0.16-15 years] with typical pulmonary valve stenosis were treated by balloon dilatation of the pulmonary valve. They were studied by echocardiography before the procedure, immediately after it, and at follow up (mean (SD) 10.2 (5.6) months, n = 18). The morphology of the pulmonary valve, the right ventricular-pulmonary artery gradient, and ratio of the systolic to diastolic endocardial dimensions (infundibular ratio) were examined. No patient had pulmonary regurgitation before the study. The valve gradient was significantly reduced (47%) from a mean (SD) of 72 (31) to 37 (23) mm Hg with no short term change in cardiac index after dilatation with a balloon with a mean (SD) diameter that was 118 (10.8)% of the valve annulus. The infundibular ratio was unchanged by the procedure (0.49 (0.11) (n = 21) before dilatation and 0.47 (0.14) (n = 16) after dilatation). In twenty seven patients the commissure of the pulmonary valve was seen to be torn after dilatation. Two patients with bicuspid valves had flail leaflets. Doppler examination at follow up showed mild pulmonary insufficiency in all 29 patients; the mean (SD) valve gradient (31 (+/- 21) mm Hg) at follow up was no different from the gradient found immediately after the procedure and infundibular ratio (0.58 (0.15) was not abnormal. These data indicate that commissural tears are the primary mechanism of valve disruption and demonstrate that the dynamic right ventricular outflow tract obstruction relaxes and gradient reduction persists at follow up.

Partial absence of the left parietal pericardium with herniation of the left atrial appendage: diagnosis by cross-sectional echocardiography and contrast-enhanced computed tomography.

This report describes the use of cross-sectional imaging--echocardiography and contrast-enhanced computed tomography--to confirm the presence of a foramen-type defect of the left pericardium with herniation of the left atrial appendage.

Pulmonary valve morphology after balloon dilatation of pulmonary valve stenosis.

Percutaneous balloon valvuloplasty (BV) for pulmonic valve stenosis (PS) is increasingly becoming a nonsurgical alternative in patient management. However, the mechanism by which BV dilates the obstructive lesion has not been firmly established. We have had the opportunity to examine the effects of BV in the setting of PS and present two illustrative cases documenting the morphology after BV of the stenotic bicuspid and tricuspid pulmonary valve.