Tetralogy of Fallot: selective staged management.

One hundred twenty-four patients with tetralogy of Fallot have undergone either primary total repair (61), shunt and later repair (30), or an initial shunt (33). The mean ratio of pulmonary anulus to descending thoracic aorta increased from 0.80 +/- 0.25 before the shunt to 1.22 +/- 0.26 before the repair (p less than 0.0001). The mean ratio in the primary repair group was 1.23 +/- 0.25. A transannular patch was necessary in only six of 91 patients (6.6%). Postrepair right ventricular/left ventricular pressure ratio averaged 0.50 +/- 0.11 in the shunt plus repair group and 0.43 +/- 0.12 in the primary repair group. Only four patients had a right ventricular/left ventricular pressure ratio less than 0.65. A significant inverse linear relationship existed between this ratio and the pulmonary anulus size measured at operation and normalized for the patient's height (p less than 0.01). Postoperative complications occurred in 21% of patients after a shunt and 20% of patients after open heart repair. The early mortality was 0.8% (1/124). An initial shunt in patients with a small pulmonary anulus can result in an increased anulus size and better hemodynamic result with frequent avoidance of a transannular patch. Staged repair may result in improved overall mortality rates.

Intermediate-term results in pediatric aortic valve replacement.

BACKGROUND: Aortic valve replacement (AVR) in children is now more commonly performed with human tissue valves. METHODS: The results of 100 consecutive pediatric AVRs (50 mechanical, 50 human) were reviewed. RESULTS: There were five perioperative deaths in the mechanical group and one in the human group (p = 0.2). Late complications in the mechanical group included 4 late deaths, 2 cases of endocarditis, 3 thromboembolic complications, and 10 reoperations on the aortic valve. In the human group, there were no late deaths, 2 reoperations for allograft aortic valve deterioration (both in Marfan's patients), and 1 reoperation for allograft pulmonary valve stenosis. Four-year actuarial survival was 83% in the mechanical group and 98% in the human group (p = 0.02). Four-year actuarial survival free of all valve-related complications was 61% in the mechanical group and 88% in the human group (p = 0.008). CONCLUSIONS: Human valves in children requiring AVR provide superior intermediate-term survival and freedom from valve-related complications compared to mechanical valves. Marfan's syndrome may represent a rare remaining contraindication for human AVR in children.

D-2-hydroxyglutaric aciduria: hypotonia, cortical blindness, seizures, cardiomyopathy, and cylindrical spirals in skeletal muscle.

An infant girl was demonstrated to have D-2-hydroxyglutaric aciduria, the fifth case described and the first with muscle biopsy of this rare organic aciduria that differs clinically and genetically from the more common L-2-hydroxyglutaric aciduria. Her clinical features included mildly dysmorphic facies, developmental delay, generalized hypotonia, myoclonic seizures, cortical blindness, and dilated cardiomyopathy requiring treatment. Muscle biopsy demonstrated only excessive glycogen histochemically, but ultrastructural examination revealed subsarcolemmal cylindrical spirals and normal mitochondria. Because of the metabolism of D-2-hydroxyglutaric aciduria, we regard valproic acid as contraindicated in the treatment of epilepsy in this disease.

Pediatric critical care nurses' knowledge of cardiac dysrhythmias.

OBJECTIVE: To describe pediatric critical care nurses' knowledge of dysrhythmias in critically ill pediatric patients and relate this knowledge level to certain demographic variables (education, nursing experience, certification, supplemental training, area of employment and geographic region of residence). DESIGN: A descriptive survey. SETTING: American Association of Critical-Care Nurses' 19 geographic regions of the United States. PARTICIPANTS: Of 1000 questionnaires mailed to pediatric critical care nurses who were members of the American Association of Critical-Care Nurses in 1991, 356 responses were received (a response rate of 36%). INTERVENTION: A criterion-referenced, self-administered test regarding pediatric dysrhythmias and a demographic sheet randomly mailed to 1000 pediatric critical care nurses. Test results were analyzed and compared with demographic variables. RESULTS: The mean total test score was 66%. Significantly higher total test scores and selected subtest scores were demonstrated in relationship to the following variables: increased age; certification in pediatric advanced life support, advanced cardiac life support or adult critical care; increased years of adult critical care experience; advanced dysrhythmia courses and dysrhythmia self-study; and perceived knowledge level above that of the advanced beginner. CONCLUSIONS: Pediatric critical care nurses' overall knowledge of dysrhythmias was low. Knowledge strengths included recognition of basic and life-threatening dysrhythmias and calculation of basic ECG measurements. Knowledge deficits included importance of sinus bradycardia in the neonate, appropriate intervention for life-threatening dysrhythmias and calculation of an irregular heart rate. These deficits should be considered when planning continuing education programs for pediatric critical care nurses.

Comparison of human tissues and mechanical prostheses for aortic valve replacement in children.

BACKGROUND: Aortic valve replacement in children is problematic because of complications of mechanical valves and uncertain outcomes associated with human valves. The results of pediatric aortic valve replacements over 5 years were reviewed. METHODS AND RESULTS: Mechanical valves were used exclusively during the first part of this series (n = 26). Thereafter, 25 consecutive aortic valve replacements were performed with autografts (n = 19) or allografts (n = 6). Allografts were used for Marfan's syndrome patients or those with unusable pulmonary valves. Among autograft/allograft recipients, 16 patients underwent 27 prior operations. In the mechanical group, 18 patients underwent 19 previous operations. Three patients in each group underwent a previous mechanical aortic valve replacement. Operative complications included two mild strokes and one pacemaker in the autograft/allograft group and three deaths and two pacemakers in the mechanical group. One autograft recipient required reoperation for pulmonary allograft stenosis. In the mechanical group, late complications included six cases of nonstructural degeneration and two cases of endocarditis, with three reoperations. Reoperation-free survival was 96% at 2 years in the autograft/allograft group and 80% at 2 years and 75% at 3 years in the mechanical group. Event-free survival was 96% at 2 years in the autograft/allograft group compared with 67% at 2 years and 49% at 3 years in the mechanical group (P < .05). CONCLUSIONS: The frequency of reoperations for mechanical aortic valve replacement has been surprisingly high. Aortic valve replacement in children with only autografts or allografts achieves good early results.