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Advances in the diagnosis and therapy of congenital heart disease in the past few decades have allowed the survival of young adults who are a fertile substrate for developing bacterial endocarditis. In addition to valvular involvement, young adults with congenital disease may develop infection on the ventricular septum, or in a vascular shunt, a patent ductus arteriosus, or coarctation of the aorta. The incidence of endocarditis in children and adults with congenital heart disease is higher than with rheumatic heart disease or mitral valve prolapse. Infecting organisms, diagnosis, and therapy are similar to acquired heart disease, but the physiologic variations and patient population often add a unique flavor to the management of this group.

Hypothesized that maternal perceptions would be more significant predictors of emotional adjustment than medical severity. Mothers of 99 children, between the ages 4-10 years, completed the Child Behavior Checklist, Parenting Stress Index, Parental Locus of Control Scale, and a measure of perception of medical severity. Assessed medical severity by number of hospitalizations, operations, catheterizations, hospital days, outpatient visits, and a cardiologist's rating of illness severity. Maternal perceptions were potent predictors of emotional adjustment. Approximately 33% of the variability in adjustment was accounted for by maternal perceptions, while the medical severity accounted for less than 3% of the variability. Severity of illness appears less critical to successful adaptation than the quality of the mother-child relationship.

Between 1971 and 1983, 201 patients received synthetic right heart conduits, predominantly porcine-valved Dacron conduits, at The Children's Hospital, Boston. There were 45 hospital deaths (22%). Follow-up has been achieved in 148 of 156 survivors (95%). Thirty-four conduits have been replaced, all because of conduit obstruction. The actuarial freedom from conduit replacement was 81% at 5 years, 61% at 7 years, and 0% at 10 years for valved conduits. There was no significant difference to 5 years in reoperation rate between patients with Carpentier-Edwards and those with Hancock conduits. Patients older than 18 years at the time of conduit insertion were 92% free of conduit replacement at 5 years. Those with nonvalved conduits were 100% reoperation free at 4 years. The actuarial survival of patients with valved conduits was 91% at 5 years and 83% at 10 years. The poor performance of porcine-valved tightly woven Dacron conduits warrants a change to use of an alternative conduit, particularly in smaller children. Possible alternatives include antibiotic-sterilized homografts and valved or nonvalved high-porosity knitted Dacron conduits appropriately pretreated with collagen impregnation or fibrin glue.

The case reports of 492 infants with critical congenital cardiac disease treated with prostaglandin E1 (PGE1) were reviewed to determine the nature and incidence of intercurrent medical events. Forty-three percent of the infants had at least one such event, but only half of these were related to PGE1 and the majority required only minor changes in management. Cardiovascular events were the most common (18% incidence), with cutaneous vasodilation and edema occurring more frequently during intraaortic infusion than during i.v. infusion. Central nervous system events were reported in 16% of the patients. Respiratory depression was reported in 12%, and was particularly common in infants weighing less than 2.0 kg at birth (42%). Hematologic, infectious and renal events appeared for the most part to be unrelated to PGE1. The overall mortality (excluding 19 patients with hypoplastic left-heart syndrome) was 31%; the mortality for the patients with critical coarctation or interruption of the aortic arch was nearly twice that for the cyanotic infants (50% vs 27%). No death was attributed to PGE1 administration. During infusion of PGE1, arterial blood pressure and respiratory activity should be monitored carefully and appropriate supportive steps taken if hypotension or respiratory depression occurs. The development of fever or jitteriness may require reduction of the infusion rate and, in view of the possible increased incidence of infections, the prophylactic use of antibiotics is recommended.

The technique of normothermic caval inflow occlusion was introduced primarily for relief of pulmonary valve stenosis and continues to be used for this purpose with excellent results. However, the technique may also be applied to a number of other lesions such as aortic valve stenosis in neonates and infants, for placement of outflow patches or valve excision for pulmonary atresia with intact ventricular septum, for atrial septectomy in children with restrictive atrial septum, and for other conditions wherein a period of up to 2 minutes of intracardiac exposure is sufficient. This report examines the early and long-term results in 140 children who underwent normothermic caval inflow occlusion at The Children's Hospital, Boston, Massachusetts, over the past 11 years. Ninety-four children underwent pulmonary valvotomy (early mortality 0%), 21 had aortic valvotomy (19%), 10 with pulmonary atresia and intact ventricular septum underwent various procedures (50%), 11 had atrial septectomy (9%), and there were four miscellaneous procedures (50%). Apart from avoiding many of the potential complications of cardiopulmonary bypass, inflow occlusion has provided as good or better short-term and long-term results as those obtained with cardiopulmonary bypass, particularly in infants and neonates, and also has proved more cost effective.

Dacron valved conduits or aortic allografts were placed between the right heart and the pulmonary artery for repair of various complex congenital cardiac anomalies in 56 patients (aged 15 days to 33 years; median, 11 years). Forty-four patients had a total of 56 previous palliative procedures, which contributed to postoperative morbidity and mortality. Six patients had a total of seven episodes of early or late sepsis involving the conduit. One patient, treated for early sepsis, again developed infection in the Hancock graft 1 year postoperatively and died. Three other patients, 2 with calcified allografts, developed infections 4 months to 7 years following repair and required graft replacement. Hemodynamic data 1 month to 5 years (mean, 1.6 years) following repair revealed mild to moderate obstruction (less than 45 mm Hg gradient) at the Hancock conduit valve ring in 13 of 19 patients, while 5 had large pressure gradients (greater than 75 mm Hg). All aortic allografts had severe obstruction and calcification necessitating graft replacement. It is anticipated that improved technique and appropriate timing of palliative and corrective operations will substantially reduce or eliminate these problems.

Although there is widespread acceptance that some children with congenital or acquired heart disease need exercise proscription, there is little data on which to base recommendations. In this article, the author, after reviewing the hemodynamic burdens of the common cardiac anomalies in children, gives his personal recommendations for recreational and sports activities.

The subject of this case report is an unusual patient whose right lung receives blood from the right heart and whose left lung receives blood from the left heart. Due to this unusual anatomy, it was possible to calculate the ratio of the outputs of the left and right hearts using standard techniques.

Cineurography is often performed after cineangiocardiography to look for occult congenital urinary tract disease. The accuracy of cineurography was investigated in 171 patients by comparing cineurograms with renal sonograms. One hundred fifteen cineurograms (67%) showed both kidneys well enough to allow assessment of renal structure and function and the results were confirmed in 112 by ultrasonography; 3 cineurograms yielded false-positive results. Limited or no information was obtained from cineurograms of 56 patients (33%) because of nonvisualization or poor visualization of 1 or both kidneys. Of the 11 patients (6%) with urinary tract disease, only 3 were correctly assessed by cineurography. Ultrasonography discovered all 11 renal abnormalities and produced only 1 false-positive result. These data indicate that cineurography is a poor screening test and should be abandoned. When uroradiologic screening is necessary for high-risk patients, sonography is recommended.

Simple and compound sagittally angled views of the heart obtained cineangiocardiographically with a parallelographic U-arm device were evaluated clinically in 132 patients with congenital heart disease. The principle advantage of the apparatus was the case with which axial and oblique projections were obtained without repositioning of the patient, although rotation and angulation of the image intensifier-X-ray tube unit resulted in some increase in scattered radiation. Certain angled views, particularly the 40 degree cranial and the compound 25 degrees cranial/70 degrees left oblique projections, were better than standard frontal and lateral views for visualization of pulmonary arteries in the mediastinum, in ventricular septal defects and anomalies of the left ventricular outflow tract.

Twenty-five children and young adults with complete heart block and 50 normal control subjects were maximally exercised on a treadmill and their electrocardiograms were analyzed for the presence and degree of ventricular ectopy activity. Sixty-eight % (17 of 25) of the heart block patients and significant ventricular ectopy (frequent unifocal ectopy or worse) on exercise compared to 2% (one of 50) of the controls (p = less than 0.01). In the patients with block there was a significant trend toward more frequent and more severe ectopy with increasing age (p = less than 0.05) independent of heart rate. More severe ventricular ectopy was also seen in those patients with QRS prolongation (p = less than 0.05). No significant differences were found between the 14 patients with isolated "congenital" heart block, the seven with associated defects, and the four with surgically acquired block. We believe that severe ventricular ectopy is common at exercise in patients with complete heart block and they may have an increased risk for sudden death.

The course and prognosis of 208 patients with an ascending aorta to pulmonary artery anastomosis is reviewed. Mortality rate during, or within one month, of surgery was 24 per cent (50/208) and late mortality rate, prior to repair, was 10 per cent (21/208). An additional 5 per cent (10/208) died during subsequent intracardiac repair. Congestive heart failure developed in 25 per cent (53/208), pulmonary artery hypertension in 17 per cent (12/72), and pulmonary vascular obstruction in 6 per cent (4/72). An increase in orifice size of the stoma with time was documented in eight patients. Additional subsequent palliative surgery was required in 22 per cent (45/208). Mortality rate was directly related to age at operation and was highest in neonates less than one week of age. In infants with tetralogy of Fallot, a preliminary comparison of mortality rate between palliative surgery and primary repair clearly suggests that the latter is the preferred method of treatment.