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Subarterial ventricular septal defect (VSD) is relatively common in Orientals. We reviewed the outcome of 214 patients (137 males) who were followed for 8.6 +/- 5.2 years (range 0.1 to 24.3) and addressed the issue regarding the necessity and optimum timing of closing subarterial defects before development of aortic valve deformities. Demographic data, transthoracic and transesophageal echocardiographic findings, cardiac catheterization results, and operative findings were reviewed. Kaplan-Meier actuarial analysis was performed to assess the development of aortic valve complications over time. Seventy-five patients with heart failure and pulmonary hypertension underwent surgical closure of VSD at the age of 2.4 +/- 2.9 years. No patient had aortic cusp prolapse before operation and none developed aortic cusp prolapse or aortic regurgitation (AR) on follow-up. In contrast, of the 139 asymptomatic patients managed conservatively, 102 (73%) developed aortic cusp prolapse, 78% of whom (80 of 102) developed AR. The prevalence of aortic cusp prolapse and AR at 1, 5, 10, and 15 years old was 8%, 30%, 64%, and 83%, and 3%, 24%, 45%, and 64%, respectively. Significant prolapse or AR prompted surgical closure of VSD with (n = 22) or without (n = 26) valvoplasty in 48 of 102 patients (47%). The size of the VSD was significantly larger in patients with heart failure (9.6 +/- 3.3 mm) or aortic cusp prolapse (11.7 +/- 4.1 mm) compared with those without heart failure (4.5 +/- 1.4 mm, p <0.001). All patients with aortic cusp prolapse and all but 1 with heart failure had a defect size of > or =5 mm. In conclusion, subarterial VSD of > or =5 mm should be closed as early as possible to prevent development of aortic cusp prolapse and AR. Asymptomatic patients with small defects <5 mm could be managed conservatively.

Accurate evaluation of secundum atrial septal defect (ASD) is important for the success of transcatheter closure. The purpose of this study was (1) to describe a quantitative evaluation by transthoracic (TTE) and transesophageal (TEE) echocardiography of the various morphologic features of ASD that might be important for patient selection for transcatheter closure, and (2) to assess the reliability of these 2 methods by surgical findings. Preoperative TTE and TEE were used to evaluate the ASD of 27 children undergoing surgical closure. Measurements included the diameters of ASD and the atrial septal lengths in the transverse and longitudinal axis, and the width of the superior, inferior, anteroinferior, and posterior septal margins. The shape and location of the ASD and the adequacy of the septal margins for anchoring occluding devices were determined. Echocardiographic data were compared with corresponding surgical measurements. No significant difference occurred in the means of all the parameters measured with the 3 methods, except for the transverse ASD diameter (p <0.05). Good agreement occurred between TTE and TEE and surgical data, except for the transverse ASD diameter, transverse atrial septal length, and the posterior septal margin on TTE. Intraclass correlations between TTE and TEE and surgical measurements of all parameters were good, except for the transverse ASD diameter. The accuracy in determining shape and location of the ASD by TTE and TEE were 100%, whereas that for determining the adequacy of septal margins was 98% and 97%, respectively. In conclusion, TTE and TEE are reliable methods for quantitative evaluation of ASD.

After major open heart surgery, a significant number of infants and children are dependent on peritoneal dialysis as a result of renal impairment. They often require broad-spectrum antibiotics for the treatment of ongoing infections or as prophylactic therapy and have an increased risk of fungal infection. Fluconazole is a new thiazole antifungal agent that has been widely used in adults, but its use in children with acute renal impairment requiring peritoneal dialysis has not been documented. The purpose of this investigation was to study the pharmacokinetics of fluconazole in infants and children who developed various degrees of renal impairment, with or without the need for peritoneal dialysis, after major open heart surgery. Between January 1992 and June 1995, 17 children ranging in age from 2 weeks to 3 years (mean, 6 months) who received fluconazole therapy intravenously (3 mg/kg per day for 2 to 3 weeks) after major open heart surgery were enrolled in a prospective study. They were divided into two groups--those who required peritoneal dialysis (PD group; n = 8) and those who did not (non-PD group; n = 9). Blood, urine, and peritoneal dialysate samples were collected for 4 days to determine the pharmacokinetics of the drug, and data were compared between the two groups. The two groups of children had similar demographic characteristics, and their plasma concentrations of fluconazole showed no statistically significant differences. For children dependent on peritoneal dialysis, fluconazole was excreted almost solely through dialysis. Despite a significantly longer terminal elimination half-life for this group of infants, they tended to have a marginally larger volume of distribution of the drug. This, coupled with the continuous hourly exchange of dialysate, provided a large sink volume to effectively remove flu- conazole from the circulation. Thus the plasma clearance and the accumulation ratio were comparable for the two groups of children. Continuous cycling peritoneal dialysis effectively removed fluconazole from the circulation and was the main mode of excretion of the drug in children dependent on dialysis.

To examine the impact on survival and clinical course of incorporating the morphologic classification of the right ventricle into the evolving management strategy for babies with pulmonary atresia and intact ventricular septum, the surgical results and follow-up status of the first 62 consecutive patients managed in this hospital between 1979 and 1990 were reviewed. Before 1984, all 23 babies from group I underwent primary right ventricular outflow reconstruction irrespective of right ventricular morphology and size. Since 1984, depending on the morphology and size of the right ventricle, 39 babies from group II had either closed transventricular pulmonary valvotomy (n = 31) or a shunt operation (n = 8). There were 10 hospital (43%) and 2 late deaths (total mortality 52%) in our group I patients. Three of the 11 long-term survivors had cyanosis at rest but none had any residual pressure gradient across the pulmonary outflow. Group II had 6 hospital (15%) and 4 late deaths (total mortality = 26%). Of the 29 long-term survivors, 9 had a second-stage right ventricular outflow reconstruction, 8 had balloon valvuloplasty and 2 had successful Fontan operation. At the latest follow-up, 5 children from this group have cyanosis at rest, 1 has a residual gradient (55 mm Hg) across the infundibulum, and 3 have right ventricular dysfunction. The hospital and total mortality for babies in group II was significantly lower than that in group I (p < 0.01). These data suggest that tailoring the treatment to the right ventricular anatomy results in a lower overall mortality although long term postoperative hemodynamic abnormalities are observed in both groups.

The clinical course was studied in 25 patients with ventricular septal defect (VSD) and aortic regurgitation (AR) who had undergone aortic valvuloplasty and VSD closure. Twelve patients had a doubly committed subarterial VSD and 13 had a perimembranous type of VSD. Preoperatively, progressive hemodynamic disturbance after the onset of AR occurred in 11 patients (44%). The follow-up period extended from 6 months to 23 years (mean 10 years). Four patients required prosthetic valve replacement after valvuloplasty. One of these had had initial improvement of the AR but required valve replacement 20 years later; the condition of the other 3 did not improve initially and their valves were replaced 1 month to 8.5 years later. Four other patients had no initial improvement as a result of valvular reconstructive surgery, but the AR did not progress and remained hemodynamically well tolerated. Hence, the overall success rate of the valvuloplasty, defined as improving or preventing progressive AR, was 21 of 25 (84%). Since initial and long-term improvement in aortic valve function can be expected in most patients after valvuloplasty and closure of the VSD, early surgical intervention is recommended in patients with a VSD and AR.