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Twenty-five neonates (16 boys and 9 girls) who had atrial flutter were identified. Diagnosis was made on or before the first day of life in 18 (72%). Heart failure were present in 9 patients, and hydrops fetalis was present in another 5. Atrial and ventricular rates did not differ between symptomatic and asymptomatic patients. Atrioventricular conduction was variable in 16 patients, and documented 1:1 conduction occurred in 5. Digoxin was the initial drug therapy given to 21 patients, with 7 reverting to sinus rhythm. Electrical cardioversion (pacing or synchronized shock) was attempted in 13 of the 14 cases in which digoxin was not successful and was attempted as the first treatment in 3 cases. Sustained sinus rhythm was achieved in 9. Two infants died of complications from prematurity but without having been successfully converted to sinus rhythm. No patient had atrial flutter during long-term follow-up (median 23 months). Neonatal atrial flutter has significant morbidity but an excellent long-term prognosis.

Objective: To compare the acceptability, compliance, and effectiveness of two forms of cholestyramine resin in the treatment of hypercholesterolemia in children., Study Design: Patients aged 10 to 18 years with familial hypercholesterolemia were enrolled in a randomized, crossover trial of two 8-week periods of either a pill or powder form of cholestyramine at a dose of 8 gm/day., Results: Of 40 children enrolled, 38 completed both medication periods, with a median age of 13 years (range, 10 to 18). At the end of the study, 82% preferred the pill form, 16% the powder form and 2% neither form. Mean (+/-SD) compliance as assessed by the amount of medication taken was significantly greater for pills (61% +/- 31%) than powder (50% +/- 30%, p = 0.01). The form of the medication increased compliance by at least 25% for 16 patients (42%), 13 in favor of pills and 3 in favor of powder. Compliance was not associated with patient attitudes and perceptions of hypercholesterolemia, demographics, family history, previous experience with lipid-lowering medication, or lipid profile parameters. Significant mean reductions in low-density lipoprotein cholesterol concentrations were noted for both pills (-10% +/- 20%, p = 0.006) and powder (-15% +/- 17%, p = 0.0001), with no significant difference between forms (p = 0.16)., Conclusions: A change in bile acid-binding resin formulation from powder to pills significantly increases acceptability and compliance in some children with hypercholesterolemia.

This study sought to assess pregnant diabetic women for the presence of fetal cardiac anomalies and to determine whether better diabetic control was associated with a reduced risk to the fetus. Between 1988 and 1995, pregnant type I and II diabetic women routinely underwent fetal echocardiography. Hemoglobin A1c values were used as an indicator of maternal diabetic control and any relation between congenital heart disease in the fetus and maternal hemoglobin A1c levels was sought. Cardiac defects were identified in 7 of 328 pregnancies assessed, for an incidence of congenital heart disease of 2.1% (95% confidence interval: 0.6-3.6%). A review of the postnatal cardiac database did not reveal any undetected major malformations. The mean hemoglobin A1c level was 7.6% +/- 2.0% obtained at a mean gestational age of 12 +/- 7 weeks. Hemoglobin A1c levels of mothers carrying a fetus with congenital heart disease did not significantly differ from those with a normal fetus: 8.1% +/- 3.4% versus 7.6% +/- 1.9% (p = 0.48). Mothers with an affected fetus demonstrated a wide range of HbA1c levels (4.1 to 13.7%). Thus, the incidence of significant fetal cardiac abnormalities is low and not significantly related to maternal diabetic control.

Objectives: Our purpose was to assess the risk factors for late mortality, loss of sinus rhythm and atrial flutter after the Mustard operation., Background: The Mustard operation provides correction of cyanosis with low surgical risk in transposition of the great vessels. However, right ventricular failure, loss of sinus rhythm, atrial flutter and death are frequent long-term complications., Methods: Records of 534 children who underwent the Mustard operation at a single center since 1962 were reviewed for demographic, anatomic, electrocardiographic and physiologic predictors and outcomes., Results: There were 52 early deaths (9.7%). Survival analysis was undertaken for 478 early survivors with a mean follow-up interval of 11.6 +/- 7.2 years. There were 77 late deaths (16.1%), with sudden death (n = 31) the most frequent cause. Survival estimates were 89% at 5 years and 76% at 20 years of age. Risk factors were an earlier date of operation, operative period arrhythmia and an associated ventricular septal defect. Risk (hazard) of late death declined in the first decade, with further peaks in the second decade. Sinus rhythm was present in 77% at 5 years and 40% at 20 years. Loss of sinus rhythm was associated with previous septectomy, postoperative bradycardia and late atrial flutter. Freedom from atrial flutter was 92% at 5 years and 73% at 20 years of age. Risk factors for atrial flutter were the occurrence of perioperative bradyarrhythmia, reoperation and loss of sinus rhythm during follow-up. Risk of atrial flutter demonstrates a late increase., Conclusions: Ongoing loss of sinus rhythm and late peaks in the risk of atrial flutter and death necessitate continued follow-up.

Objectives: This study sought to compare the immediate results and risk factors for suboptimal outcomes of percutaneous balloon angioplasty for native versus recurrent aortic obstruction., Background: Some cardiology centers have been reluctant to adopt balloon angioplasty for treatment of native aortic coarctation, while advocating balloon angioplasty over an operation for treatment of postsurgical or recurrent aortic obstruction., Methods: Acute results were analyzed from 970 procedures (422 native and 548 recurrent lesions) performed between 1982 and 1995 in 907 patients from 25 centers. An acute suboptimal outcome was defined as one or more of the following: residual systolic pressure gradient > or = 20 mm Hg, residual proximal to distal systolic pressure ration > or = 1.33 or a major complication (death, aortic transmural tear, stroke)., Results: Bal loon angioplasty significantly (p = 0.0001) increased lesion diameter fo r both native (mean [+/= SD] 128 +/= 94%) and recurrent aortic obstruction (97 +/= 87%), with a significantly greater increase in the native group (p = 0.0001). A reduction in systolic pressure gradients was significant in both groups (p = 0.0001), but slightly higher (p = 0.01) for native (-74 +/- 24%) versus recurrent obstruction (-70 +/- 31%). Death associated with angioplasty was reported in 0.7% of patients with native and in 0.7% of patients with recurrent lesions (p = 1.00). An acute suboptimal outcome was noted with angioplasty in 19% of native and in 25% of recurrent lesions (p = 0.04). Significant independent risk factors included higher preangioplasty systolic gradient (odds ratio [OR] 1.39/10-mm Hg increment; 95% confidence interval [CI] 1.28 to 1.50, p = 0.0001), earlier study date (OR 0.92/1-year increment, 95% CI 1.02 to 1.26, p = 0.02) and recurrent obstruction (OR 1.39 vs. native lesions, 95% CI 1.00 to 1.94, p = 0.05)., Conclusions: Acute results and complications of balloon angioplasty of native coarctation appear to be equivalent or slightly superior to those of recurrent aortic obstructions.

Background: Among adults who have a cardiac arrest outside the hospital, the survival rate is known to be poor. However, less information is available on out-of-hospital cardiac arrest among children. This study was performed to determine the survival rate among children after out-of-hospital cardiac arrest and to identify predictors of survival., Methods: We reviewed the records of 101 children (median age, two years) with apnea or no palpable pulse (or both) who presented to the emergency department at the Hospital for Sick Children in Toronto. The characteristics of the patients and the outcomes of illness were analyzed. We assessed the functional outcome of the survivors using the Pediatric Cerebral and Overall Performance Category scores., Results: Overall, there was a return of vital signs in 64 of the 101 patients; 15 survived to discharge from the hospital, and 13 were alive 12 months after discharge. Factors that predicted survival to hospital discharge included a short interval between the arrest and arrival at the hospital, a palpable pulse on presentation, a short duration of resuscitation in the emergency department, and the administration of fewer doses of epinephrine in the emergency department. No patients who required more than two doses of epinephrine or resuscitation for longer than 20 minutes in the emergency department survived to hospital discharge. The survivors who were neurologically normal after arrest had had a respiratory arrest only and were resuscitated within five minutes after arrival in the emergency department. Of the 80 patients who had had a cardiac arrest, only 6 survived to hospital discharge, and all had neurologic sequelae., Conclusions: These results suggest that out-of-hospital cardiac arrest among children has a very poor prognosis, especially when efforts at resuscitation continue for longer than 20 minutes and require more than two doses of epinephrine.

In the surgical repair of tetralogy of Fallot, monocuspid valves are sometimes inserted within a transannular patch to prevent pulmonary insufficiency. To determine whether this monocuspid valve prevents short-term postoperative pulmonary insufficiency and improves clinical outcome, we reviewed clinical data and preoperative and postoperative echocardiographic variables from 61 patients who underwent one of three different procedures for repair of tetralogy of Fallot between August 1992 and March 1994. We compared features from 24 patients who had undergone transannular patch repair with a monocuspid valve (patch-valve) with those from 17 patients who had undergone patch repair without a monocuspid valve (patch) and 20 patients who had undergone repair without a transannular patch (no patch). We used the ratio of pulmonary valve insufficiency jet width to pulmonary artery diameter, as measured by color-flow Doppler flowmetry, as an index of severity of pulmonary insufficiency. Moderate to severe pulmonary insufficiency was arbitrarily defined as a ratio of at least 0.50. We found no significant differences in ratios among the patch-valve group (0.73 +/- 0.25, mean +/- standard deviation), the patch group (0.79 +/- 0.20), and the no patch group (0.59 +/- 0.23). The percentages of patients with moderate to severe pulmonary insufficiency did not differ among the three groups (patch-valve 80%, patch 90%, no patch 64%). Clinical data (including mortality, number of reoperations, intensive care unit and hospital lengths of stay, and postoperative hemodynamics) were similar in the three groups. We conclude that insertion of a monocuspid valve in repair of tetralogy of Fallot does not prevent short-term postoperative pulmonary insufficiency and does not improve immediate postoperative outcome for these patients.

To compare characteristics of aortic regurgitation (AR), the results of 213 procedures (110 balloon aortic valvotomies [BAV] and 103 surgical aortic valvotomies [SAV]) for treatment of congenital aortic valve stenosis were reviewed. These procedures were performed in 187 patients from June 1981 to September 1993. Echocardiograms recorded immediately before, within 6 months afterward, and at latest follow-up were compared. Color Doppler was used to assess the degree of AR and was quantified as the ratio of the regurgitant jet width to valve annulus, the jet width ratio. Whereas BAV patients were older (median age 5.7 years vs 3 months; p = 0.0001), there was no significant difference in median follow-up interval (3.1 years [range 0.5 to 7.2] for BAV vs 3.6 years [range 0.6 to 10.4] for SAV; p = 0.44). The mean balloon-to-annulus ratio for BAV was 0.99 +/- 0.09. An open valvotomy was performed in 83% of surgical cases. Acute systolic gradient reduction and subsequent increase at late follow-up was similar for both groups. Acutely, the mean jet width ratio increased similarly (p = 0.84) for BAV (+9 +/- 15%; p = 0.0001) and SAV (+9 +/- 12%; p = 0.0003) and was not related to age at procedure. At late follow-up, mean jet width ratio further increased significantly in both groups, although there was no difference (p = 0.17) in amount of progression (BAV +10 +/- 12%; p = 0.0001, SAV +15 +/- 13%; p = 0.0002). Thus, BAV and SAV produce AR of similar severity with similar rates of progression.

To obtain normative data for the childhood signal-averaged ECG (SAECG), we obtained SAECGs in 155 volunteers, aged 5-15 years, using Frank leads and a Fourier transform filter. Unfiltered QRS duration (QRSDU) and filtered QRS duration (QRSDF) were significantly longer in males, and the root mean square of the terminal 40 ms (RMS40) was significantly higher in females. There were no gender differences in the duration of high frequency low amplitude signals < 40 microV (DHFLA). All SAECG variables were significantly related to body surface area (BSA). Regression models were established for SAECG variables. For males, the predicted mean + 1.96 standard deviations (SD) for QRSDF (97.5th centile) ranged from 114 ms at BSA 0.70 m2 to 123 ms at 1.90 m2. For females, the predicted mean + 1.96 SD for QRSDF ranged from 110 ms at BSA 0.70 m2 to 119 ms at 1.90 m2. For males and females, the predicted mean + 1.96 SD for DHFLA ranged from 34 ms at 0.70 m2 to 38 ms at 1.90 m2. For males, the predicted mean - 1.96 SD (2.5th centile) for RMS40 (based on natural logarithm model) ranged from 30 microV at 0.70 m2 to 15 microV at 1.90 m2. For females, the predicted mean -1.96 SD for RMS40 ranged from 42 microV at 0.70 m2 to 20 microV at 1.90 m2. In children 5-15 years of age, both gender and BSA need to be taken into account when interpreting the SAECG.

The clinical impact of transcatheter closure of the isolated secundum atrial septal defect was reviewed. Closure by echocardiographic evaluation was 23 +/- 14% at 6 months, 49 +/- 16% at 2 years, and 64 +/- 16% at 4 years, and right ventricular end-diastolic dimensions in patients without residual shunts did not differ significantly from those with residual shunts.

Scimitar syndrome was diagnosed in 27 patients seen between July 1974 and May 1993. All available echocardiograms taken before surgery or death were reviewed. Age at presentation ranged from 1 day to 14 years. Dextrocardia or mesocardia was noted in 70%, atrial septal defect in 70%, and increased right ventricular dimension in 70% of the patients. The ratio of the proximal and distal diameters of the right/left pulmonary arteries were reduced 0.68 +/- 0.17 and 0.66 +/- 0.17, respectively. "Blunting" of the right side of the left atrium was seen in all patients with total anomalous right pulmonary venous drainage and none with partial drainage. Anomalous right pulmonary venous drainage was characterized in 91% of echocardiograms with color flow mapping versus 14% without color flow mapping (p < 0.0002). Aortopulmonary collateral arteries were detected in all four cases in which color flow mapping was performed, but not detected otherwise.

Smaller patient series have not explored the independent relations between patient selection and technical factors that may impact on acute results of percutaneous balloon aortic valvotomy. To determine such relations, results from the Valvuloplasty and Angioplasty of Congenital Anomalies Registry were examined from 630 balloon aortic valvotomies in 606 patients from 23 institutions. Median age was 6.8 years (range 1 day to 18 years). The procedure could not be performed or completed in 4.1%. The left ventricular to aortic gradient was reduced by a mean +/- SD of 60 +/- 23% (p < 0.0001). Procedure-related mortality was 1.9%. A suboptimal outcome was defined arbitrarily as failure to perform the procedure, a residual gradient > or = 60 mm Hg or LV: aortic pressure ratio > or = 1.6, or major morbidity or mortality, and was noted for 17% of procedures. Independent risk factors from multiple logistic regression analyses included age < 3 months, higher preprocedure gradient, earlier procedure date, the presence of unrepaired aortic coarctation, and the use of undersized balloons. Detailed analysis revealed the optimal balloon: valve diameter ratio to be 0.9 to 1.0, with undersized balloons resulting in significant residual obstruction and oversized balloons resulting in aortic regurgitation complications. Improvements in results over time were related to increasing experience within individual institutions. Thus, percutaneous balloon aortic valvotomy provides adequate relief of obstruction, with minimal complications in most patients.

Objective: To determine the diagnostic accuracy of clinical assessment of heart murmurs in children and specific clinical features that are predictive of cardiac disease., Design: Concurrent case series with pretest-posttest assessment., Setting: Pediatric cardiology outpatient clinic., Participants: Five full-time academic pediatric cardiologists., Measures: For each of 222 consecutive patients who were seen for first-time evaluation of a heart murmur, the clinical findings and diagnostic impressions were recorded after clinical assessment. The results of electrocardiograms and echocardiograms were then reviewed, and changes in diagnostic impressions were recorded and compared with the original impressions., Results: The prevalence of cardiac disease was 33%. Clinical assessment differentiated those patients with pathologic murmurs with a sensitivity of 92%, specificity of 94%, positive predictive value of 88%, and negative predictive value of 96%. If diagnostic uncertainty was considered an indication for echocardiography, then sensitivity and specificity increased to 97% and 98%, respectively. Missed disease included only trivial or minor lesions. Clinical features that were independently predictive of the presence of disease included murmurs that were pansystolic (odds ratio [OR], 54.0), grade 3 or more in intensity (OR, 4.84), heard best at the left upper sternal border (OR, 4.24) and harsh in quality (OR, 2.37), and the presence of an abnormal second heart sound (OR, 4.09) and an early or midsystolic click (OR, 8.35)., Conclusions: Clinical assessment by a pediatric cardiologist is sufficient to distinguish pathologic from innocent heart murmurs. A genetic approach by using specific clinical features that are independently associated with disease may have some practical utility to noncardiologists.

Objective: To determine whether Aboriginal Canadians from Manitoba and Ontario have an increased incidence of isolated total anomalous pulmonary venous drainage (TAPVD) and to compare results obtained from two different data sources and time periods., Design: A nonconcurrent cohort study was undertaken. Incidence rates and relative risk from 'traditional' data sources (cases from medical records data; births from Census, Vital Statistics and Native Registry data for Manitoba and Ontario) from 1972-84 were derived and compared with those from computerized hospital abstract data from Manitoba for 1987-91., Results: Using traditional data sources an incidence of 0.282/1000 live births was noted in Aboriginals versus 0.062 in non-Aboriginals for a relative risk of 4.6 (95% CI = 2.7-7.7). For Manitoba only the relative risk was 5.8 (95% CI = 2.6-12.8). Using computerized administrative data from Manitoba the relative risk was 5.8 (95% CI = 1.3-25.8)., Conclusion: There is an increased incidence of isolated TAPVD in Aboriginal peoples from Manitoba and Ontario. Further epidemiological investigation is necessary to determine the nature of this association.

We reviewed 40 infants and young children with VT. Median maximum VT rate was 214 beats/min (range 152 to 375 beats/min). A cause was defined in 20 (50%), the most common being cardiomyopathy or myocarditis in 8 (20%). There were six deaths (15%) related to VT, three of which occurred at diagnosis and in patients less than 1 week old. In 5 of 6 deaths related to VT, a cause was defined. At follow-up, 31 (91%) of 34 survivors did not have VT. The presence of symptoms was a predictor of death related to VT. The outlook for asymptomatic patients and those who survived more than 6 months after diagnosis and who do not have progressive myocardial disease appears good.

Cardiac sequelae account for the major morbidity and mortality related to Kawasaki disease, and subclinical myocarditis is a frequent finding. The unusual finding of ventricular arrhythmia in a 2.5-year-old patient during the acute phase of Kawasaki disease is reported. The arrhythmia resolved during empirical treatment with oral steroids.

Background: The use of endovascular stents to relieve obstructions in the setting of non-balloon dilatable pulmonary artery stenosis has been encouraging. The benefits in management and the potential for restenosis, however, have not been defined. This study attempts to assess the impact of such implants on clinical outcomes and the pattern of stent incorporation within the vessel wall., Methods and Results: Fifty-five balloon-expandable stents were implanted in 42 patients 6.1 +/- 4.7 years of age. Patients were followed prospectively (median, 15 months) and recatheterized 1 year after implantation. Thirty-eight patients had the implants positioned percutaneously (49 implants), while 4 patients (6 implants) had intraoperative implantations. There was a diameter increase in the stenotic area of 109 +/- 79% (P < .0001) and a gradient reduction of 74 +/- 26% (P < .0001). Twelve stents straddled the orifice of side-branch pulmonary arteries and reduced flow to the branch vessel acutely in 7 patients. Twenty-nine patients underwent recatheterization, and various degrees and locations of acquired intraluminal narrowing were observed in all cases, particularly in areas of diameter mismatch between the stented and nonstented vessels. Eleven patients had further dilation with diameter improvement. Of the 38 patients who underwent percutaneous implantation, planned surgery for pulmonary artery stenosis was avoided in 33 and deferred in 4 patients. One patient who was considered inoperable had stent implantation as a palliative procedure. Symptomatic improvement was reported in 27 patients, and 15 patients remained asymptomatic., Conclusions: Endovascular stents have a role in the treatment of pulmonary artery stenoses and positively affect clinical care. The stenosis relief, however, may be tempered by the development of intraluminal stent obstruction, which may require redilation (15 of 55 stents) and mandates long-term follow-up.

Objectives: This study was undertaken to determine the accuracy of routine echocardiography in the detection of partial anomalous pulmonary venous drainage., Background: Although there are occasional case reports of the echocardiographic appearance of partial anomalous pulmonary venous drainage, no large series have addressed the accuracy of this technique in a large cohort of patients., Methods: Between January 1983 and December 1993, 50 patients with partial anomalous pulmonary venous drainage (with or without an associated atrial septal defect as the only other significant intracardiac defect) were identified from the data base at the Hospital For Sick Children, Toronto. Routine echocardiographic reports were reviewed, and the results were compared with surgical or catheterization findings. Risk factors related to diagnostic errors were sought using a Fisher exact test, chi-square analysis, t test and Kruskal-Wallis analysis of variance., Results: Confirmation of the diagnosis was available in 45 patients whose data were subsequently used for risk factor analysis. The median age at echocardiography was 4.1 years (range 1 month to 18 years). Right-sided drainage was present in 43 patients (86%), with left-sided drainage in 7 (14%). Thirteen patients had an intact atrial septum, 7 a patent foramen ovale and 30 a secundum atrial septal defect. Right ventricular dilation was observed in 46 patients. Two had normal dimensions (two not assessed). The diagnosis was missed by echocardiography in 15 (33%) of the 45 patients with a confirmed diagnosis. Year of study and use of color flow mapping were the only significant variables related to detection rate (7% missed diagnosis with vs. 62% without the use of color flow, p < 0.0005). The median year of missed diagnosis was 1985 versus 1990 (p < 0.002). Transesophageal echocardiography accurately defined the site of drainage in all three patients in whom it was utilized., Conclusions: Two-dimensional echocardiography in conjunction with color flow mapping is a valuable tool for the diagnosis of partial anomalous pulmonary venous drainage.

Objective: To gather information on which to base decisions about a general notification program for pediatric patients a decade after their receiving transfusions., Design: The physicians of a cohort of 1793 patients who underwent cardiac surgery were sent letters asking them to contact and counsel patients identified from cardiovascular surgery and blood bank databases about their risk for human immunodeficiency virus (HIV) infection. Questionnaires were used to collect data about physicians' HIV practices; telephone interviews were conducted to collect information about patients' and parents' knowledge and attitudes about HIV and transfusions. Because of unexpected media interest, questionnaires and interviews were modified to include questions about the source of information. The HIV-testing status of patients reported by physicians was anonymously cross-referenced with specimens received by the Laboratory Services Branch, Ontario Ministry of Health, Toronto., Setting: A large Canadian pediatric tertiary care hospital in Toronto., Participants: Seven hundred ninety-three patients undergoing cardiopulmonary bypass between 1980 and 1985., Results: The HIV Information Project successfully reached most (approximately 75%) of this cohort and, with the help of the media, many other at-risk transfusion recipients. The information was new for many; almost all informed wanted to undergo testing. The seroprevalence of this group that received multiple transfusions was, at minimum, 8.5 patients in 1000. Six previously unsuspected HIV-seropositive cases were diagnosed., Conclusions: Although we had assumed that most patients receiving transfusions would be aware of their risk for HIV infection, our results indicate that, even a decade after the transfusion, many recipients were not aware of the risk and wanted to undergo testing. Testing identified asymptomatic infected patients.

The development of renal failure necessitating peritoneal dialysis after cardiac operations is associated with a reported mortality greater than 50%. Improved fluid removal and nutritional support have been reported with the use of continuous arteriovenous hemofiltration and continuous venovenous hemofiltration techniques. We have compared our experience with all three techniques in managing children who required renal replacement therapy after cardiac operations in terms of efficacy (fluid removal, calorie intake, and clearance of urea and creatinine), complications, and outcome. Over a 5-year period renal replacement therapy was initiated in 42 children, and in 34 of them it was successfully established for more than a 24-hour period: 17 were managed with peritoneal dialysis, 8 with continuous arteriovenous hemofiltration, and 9 with continuous venovenous hemofiltration. A net negative fluid balance was achieved in only 6 (35%) patients treated with peritoneal dialysis compared with 50% of those treated with continuous venovenous hemofiltration and 89% of those treated with continuous venovenous hemofiltration. In terms of nutritional support, calorie intake increased by 43% after peritoneal dialysis was started compared with 515% and 409% in the arteriovenous and venovenous hemofiltration groups, respectively, (p < 0.005). The serum urea levels fell by 36% (p = 0.02) and 39% (p = 0.005) compared with pre-therapy levels with arteriovenous and venovenous hemofiltration, respectively, and the creatinine content was reduced by 19% and 33% (p = 0.003). Neither parameter was reduced in the peritoneal dialysis group. We conclude that the use of hemofiltration as a renal replacement therapy after surgical correction of congenital heart disease offers significant advantages over the more traditional approach of peritoneal dialysis. In addition, we suggest that a more aggressive approach to the management of fluid overload and nutritional depletion with hemofiltration may result in a decrease in the very high mortality seen in renal failure after cardiac operations.

This study sought to determine the extent of anxiety and misperceptions about heart murmurs for consenting parents of 182 children referred for first-time pediatric cardiology assessment (including echocardiography) of a heart murmur. From questionnaires completed before assessment, 22% of parents indicated that they were extremely concerned, and only 16% could define a heart murmur as a sound made by the heart. From 1-month follow-up questionnaires obtained from parents of children without heart disease at assessment, 10% continued to believe that their child had a heart problem. Cardiology assessment may not provide complete reassurance to all families and additional interventions may be necessary.

Objectives: The purpose of this study was to define the incidence and risk factors for atrial tachyarrhythmias after the Fontan operation., Background: Atrial tachyarrhythmias cause morbidity after the Fontan operation. Causative factors may be affected by the type of systemic to pulmonary connection., Methods: The Fontan operation was performed in 270 consecutive patients between 1982 and 1992. The mean age at operation was 7.0 +/- 4.3 years. Direct atriopulmonary connection was used in 138 patients (51%), total cavopulmonary connection in 94 (35%) and right atrial to right ventricular connection in 38 (14%)., Results: Atrial tachyarrhythmias were seen early postoperatively in 55 patients (20%), preoperative atrial tachyarrhythmia being the only risk factor. Follow-up was achieved for 228 early survivors (97%) at a mean interval of 4.4 years. There were 20 late deaths. Late atrial tachyarrhythmias were noted in 29% of patients who received an atriopulmonary connection, 14% of those who received a total cavopulmonary connection and 18% of those who received a right ventricular connection (p < 0.02). Significant risk factors as determined by univariate and multiple logistic regression analysis were atriopulmonary connection type (odds ratio 0.40 for total cavopulmonary relative to atriopulmonary connection [p < 0.05] and 0.37 for right ventricular relative to atriopulmonary connection [p = 0.08]), longer follow-up interval (odds ratio 1.32 for each consecutive year [p < 0.002]) and atrial tachyarrhythmia in the operative period (odds ratio 6.31 [p < 0.0001])., Conclusions: Early postoperative atrial tachyarrhythmias, length of follow-up and atriopulmonary connection are significant independent risk factors for the presence of late atrial tachyarrhythmias.

Objectives: This study attempted to determine whether early subaortic resection at lower levels of obstruction reduces the rate of recurrence of subaortic stenosis or reduces secondary damage to the aortic valve, or both., Background: Fibromuscular subaortic stenosis is a progressive condition, and at present it is unclear whether early operation reduces the recurrence rate along with decreasing the incidence of aortic insufficiency., Methods: Thirty-seven patients with fibromuscular subaortic stenosis and no other significant cardiac abnormality who underwent open subaortic resection were evaluated. The preoperative, early and late postoperative catheterization or echocardiographic findings as well as the operative reports were reviewed. The median age at operation was 6.4 years (range 1.1 to 17.3). The entire group has been followed up postoperatively for a median of 5.2 years (range 1.1 to 11). Mean systolic gradients across the left ventricular outflow tract were used for the purpose of this study., Results: There was a significant correlation between the preoperative mean systolic gradient and the incidence of preoperative aortic regurgitation and late postoperative aortic valve thickening as well as the incidence and degree of late postoperative aortic regurgitation. Late postoperative gradient and degree of aortic regurgitation correlated significantly with the follow-up interval. Aortic regurgitation was progressive in some patients despite subaortic resection. A preoperative mean gradient > 30 mm Hg provided a reasonable cutoff for the likelihood postoperatively of needing a reoperation, having a postoperative shelf, a thickened aortic valve, moderate aortic regurgitation or a gradient of > 10 mm Hg., Conclusions: Our results suggest that although early subaortic resection may not reduce the rate of recurrence of fixed subaortic stenosis, it is likely to reduce acquired damage to the aortic valve.

Background: This study was performed to determine independent predictors of long-term outcome after percutaneous balloon dilation of congenital pulmonary valve stenosis. Smaller follow-up series of patients after balloon pulmonary valvuloplasty have shown inconsistent results regarding the independent relation between prognostic factors and long-term outcome, as many patient selection and technical factors are correlated., Methods and Results: Follow-up data were obtained for 533 patients from 22 institutions at up to 8.7 years after an initial balloon pulmonary valvuloplasty. Patients were grouped based on defined long-term outcomes, and the independent effects of patient selection and technical factors were sought in multivariate statistical analyses. At follow-up, 23% of patients were noted to have an outcome judged to be suboptimal because of either a residual right ventricle to pulmonary artery peak systolic gradient of > or = 36 mm Hg or further treatment of pulmonary stenosis requiring repeat balloon pulmonary valvuloplasty or surgical therapy. Significant independent predictors of a suboptimal long-term outcome included an earlier study year of the initial valvuloplasty (adjusted odds ratio, 0.71 per consecutive year), a small valve hinge point diameter (0.81 per 1-mm increase), and a higher immediate residual gradient (1.32 per 10 mm Hg increase). A smaller ratio of balloon to valve hinge point diameter significantly predicted suboptimal outcomes for patients with valve morphologies classified as typical (0.52 per 0.1 increase in ratio) and complex (primarily postsurgical valvotomy, 0.43) but not for patients with dysplastic (0.95) or combined morphologies (dysplasia with commissural fusion, 1.01). Patient age, the presence of Noonan's syndrome or associated cardiac lesions, pre-balloon valvuloplasty hemodynamic parameters, and the use of a simultaneous double-balloon technique did not independently predict follow-up outcomes., Conclusions: Accurate prognostication after balloon pulmonary valvuloplasty depends on the careful determination of valvar anatomy. The use of an appropriate ratio of balloon to valve hinge point diameter in the setting of typical valve morphology will optimize the chance of long-term success.

Purpose: To evaluate the efficacy of gemfibrozil in men with primary isolated low high-density lipoprotein cholesterol (HDL-C) levels., Patients and Methods: Fourteen men with low levels of HDL-C but desirable total cholesterol levels received gemfibrozil in a randomized, double-blind, placebo-controlled, crossover trial. The men were placed on a National Cholesterol Education Program Step-Two Diet. They were randomly assigned to receive placebo and gemfibrozil each for 3 months, with a 1-month washout period between phases., Results: Overall, gemfibrozil increased the total HDL-C concentration by 9.2% (p = 0.001), reduced triglyceride (TG) levels by 38% (p < 0.01), and significantly lowered the total cholesterol:HDL-C ratio (p = 0.01). Those with fasting TG levels of 1.07 mmol/L (95 mg/dL) or greater had a significant elevation in the HDL-C level (14.6%, p = 0.005) and a reduction in TG levels (50%, p = 0.002) with gemfibrozil; those with fasting TG levels less than 1.07 mmol/L had a smaller increase in the HDL-C level (4.1%, p > 0.05) and a smaller reduction in TG levels (15%, p = 0.02). There were no significant differences in the plasma levels of low density lipoprotein-cholesterol, HDL2-C, apolipoproteins (apo) A-I and B, or Lp(a). HDL3-C and apo A-II levels rose slightly. The adverse effects attributable to gemfibrozil were minimal., Conclusion: In men with desirable total cholesterol levels, gemfibrozil raises HDL-C and lowers TG levels to a similar extent as reported for hyperlipidemic men in the Helsinki Heart Study. These lipid-altering effects were most pronounced in those with the highest fasting TG levels.

This study was undertaken to describe subspecialty characteristics and practices of the population of pediatricians given the ongoing controversy regarding a projected manpower oversupply of general pediatricians. A questionnaire was mailed to a national random sample of 1620 United States physicians listed in the American Medical Association's Physician Masterfile as being in office-based pediatric practice. The final response rate was 63%. Seventy percent of respondents designated their practices as "general pediatrics" versus 17% as "general pediatrics with a specific subspecialty interest" and 13% as "subspecialty practice." The general pediatricians with a specific subspecialty interest were intermediate in the proportion that had some training in a pediatric fellowship program (general pediatricians with a specific subspecialty interest, 63% versus general pediatricians, 14%, P < .0001, and pediatricians with a subspecialty practice, 92%, P < .0001) and that were certified in a pediatric subspecialty by the American Board of Pediatrics (general pediatricians with a specific subspecialty interest 16% versus general pediatricians, 2%, P < .0001, and pediatricians with a subspecialty practice, 62%, P < .0001). They were also intermediate in the proportion involved in various academic pursuits. Their practices, however, more closely resembled general pediatricians than pediatricians with a subspecialty practice in their location, setting, associates, and commitment to primary care. They were more likely than general pediatricians to utilize or provide specialized tests or procedures. A large percentage of pediatricians incorporate subspecialty elements into their general pediatric practices. Models of current and projected pediatric manpower supply need to be reassessed in light of this form of practice.

A 20-year-old woman with complex cyanotic congenital heart disease, including severe congenital mitral stenosis and intact atrial septum, who developed a left atrial ball thrombus and an embolic phenomenon, is presented. Increased vigilance in this unique setting is necessary for the antemortem detection of this rare complication.

We report the application of myocardial tagging by MR to define tissue planes and differentiate contractile from noncontractile tissue in a neonate with congenital cardiac rhabdomyoma. Using custom-written pulse programming software, six 2 mm thick radiofrequency (RF) slice-selective presaturation pulses (tags) were used to label the chest wall and myocardium in a star pattern in diastole, approximately 60 ms before the R-wave gating trigger. This method successfully delineated the myocardium from noncontractile tumor, providing information that influenced clinical management. This RF tagging technique allowed us to confirm the exact intramyocardial location of a congenital cardiac tumor.

Background: The objective of this study was to determine the long-term outcome of patients after percutaneous balloon pulmonary valvuloplasty (BPV) treatment of congenital pulmonary valve stenosis., Methods and Results: This study represents a case series with duration (mean +/- SD) of follow-up of 4.6 +/- 1.9 years. Forty-six patients with a median age of 4.6 years (range, 3 months to 56 years) had BPV at one academic institution between June 1981 and December 1986. Mean peak systolic pressure gradients from the right ventricle to the pulmonary artery were as follows: before BPV, 70 +/- 36 mm Hg; immediately after BPV, 23 +/- 14 mm Hg; at intermediate follow-up by cardiac catheterization or Doppler echocardiography at less than 2 years after BPV, 23 +/- 16 mm Hg (n = 33); and at long-term follow-up by Doppler at more than 2 years after BPV, 20 +/- 13 mm Hg (n = 42). BPV acutely reduced the gradient to less than 36 mm Hg for 41 of 46 (89%) patients. Available gradients at long-term follow-up were less than 36 mm Hg for 36 of 42 (86%) patients without additional procedures. A patient age of less than 2 years at the initial BPV was a significant risk factor for gradients over 36 mm Hg at follow-up., Conclusions: BPV provides long-term relief of pulmonary valvular obstruction in the majority of patients. Close follow-up of patients who require BPV at less than 2 years of age is warranted.

We have developed a model of cyclosporin A (CsA) central nervous system toxicity in the Munich-Wistar rat in which CsA, 20 mg/kg/day i.p., produces significant EEG abnormalities and mortality. In the present study we used cohorts of Munich-Wistar rats to assess effects of CsA on the threshold for tonic-clonic electroshock-induced seizures. Rat cohorts were begun on cremephore, CsA-10 mg/kg/day, or CsA-20 mg/kg/day. On day 7 and day 14 of the dosing protocol, cohorts of animals were exposed to maximal electroshock (MES) using a minimal staircase method within each cohort. Multiple logistic regression models were used to determine differences between groups on the relative odds of producing a MES-induced seizure while controlling for other variables. Seizure threshold was significantly affected by shock amperage and body weight, but not by SUN, creatinine, bilirubin, sodium, potassium, weight loss or day the shock was delivered. The odds ratios of seizure induction in the CsA-treated groups versus placebo group were 1.91 for CsA-10 mg/kg/day and 3.63 for CsA 20-mg/kg/d, both statistically significant. These results suggest that cyclosporine lowers seizure threshold and probably increases susceptibility to seizures, the etiology of which may be multifactorial clinically.

Henoch-Schönlein Syndrome (HSS) is characterized by the presence of purpuric skin lesions, colicky abdominal pain, renal manifestations, and arthritis. We report a case complicated by several unusual manifestations, including hydrops of the gallbladder and extreme thrombocytosis. Gallbladder hydrops has been known to occur in association with several conditions including Kawasaki syndrome, scarlet fever, and leptospirosis, but no previous report of this occurring in a patient with HSS could be found. The hydrops in our patient resolved spontaneously without specific medical or surgical treatment.