Myocardial substrates in children with congenital heart disease: relationship to substrate supply, age, growth and desaturation.

AIM: The myocardial uptake of substrates in children has only been investigated on a small scale. The purpose of this study was to define myocardial substrate uptake in relation to the arterial supply of substrates, age, growth and oxygen saturation. METHODS: Thirty patients with congenital heart disease, aged 3 months to 16 years, were studied during cardiac catheterization. Arterial and coronary sinus blood was analyzed for the major fuel metabolites and amino acids. RESULTS: The uptake of all major substrates correlated significantly with the arterial supply: free fatty acids (r = 0.52, p = 0.004), beta-hydroxybutyrate (r = 0.74, p < 0.0001), lactate (r = 0.70, p < 0.0001) and glucose (r = 0.48, p = 0.01). Free fatty acids were the dominant substrate, irrespective of age, growth and saturation. With age, there was an increase in the uptake of lactate (r = 0.61, p = 0.0004) and a decrease in the uptake of beta-hydroxybutyrate (r =-0.41, p = 0.02). In multivariate analyses, these changes were explained by the arterial supply of the substrates, while age per se did not contribute significantly. CONCLUSION: The uptake of myocardial metabolites correlated with the arterial supply. Free fatty acids were the dominant substrate at all ages. The uptake of lactate and beta-hydroxybutyrate, although varying with age, was also determined by the arterial supply.

Myocardial metabolism is better preserved after blood cardioplegia in infants.

BACKGROUND: We have previously reported improved hemodynamic function after blood cardioplegia in comparison with crystalloid cardioplegia. Furthermore, lactate was released from the heart after crystalloid cardioplegia but not after blood cardioplegia. The purpose of this study was to determine whether the difference in substrate metabolism between the two cardioplegia methods was restricted to lactate, or whether the difference in metabolic derangement was more extensive. METHODS: Thirty consecutive infants with complete atrioventricular septal defects were included in this prospective, randomized, controlled study. Arterial and coronary sinus blood concentrations of substrates and amino acids were measured after weaning from bypass. RESULTS: After crystalloid cardioplegia, there was a myocardial uptake of glutamate (p = 0.003), leucine (p = 0.03), lysine (p = 0.003), and beta-hydroxybutyrate (p = 0.004), whereas lactate was released (p = 0.03). After blood cardioplegia, there was a myocardial uptake of free fatty acids (p = 0.01) but no uptake of amino acids and no release of lactate. CONCLUSIONS: There are differences in myocardial substrate metabolism between blood cardioplegia and crystalloid cardioplegia, which involve carbohydrates and amino acids. The differences may include lipids but our data in this respect are not conclusive.

Independent factors associated with mortality, reintervention, and achievement of complete repair in children with pulmonary atresia with ventricular septal defect.

OBJECTIVES: We described morphologic characteristics, particularly pulmonary anatomy, and determined the prevalence of definitive end states and their determinants in children with pulmonary atresia associated with ventricular septal defect (PAVSD). BACKGROUND: Pulmonary atresia associated with ventricular septal defect represents a broad morphologic spectrum that greatly influences management and outcomes. METHODS: From 1975 to 2004, 220 children with PAVSD presented to our institution. Blinded angiographic review (n = 171) characterized bronchopulmonary segment arterial supply. RESULTS: A total of 185 patients underwent surgery, and repair was definitive in 75%. Initial operations included systemic-pulmonary artery shunt in 57%, complete primary repair in 31%, or right ventricular outflow tract reconstruction in 12%. Based on angiographic review, 118 patients had simple PAVSD and 53 patients had PAVSD with major aortopulmonary collateral arteries (MAPCAs). Overall survival from initial operation was 71% at 10 years. Risk factors for death after initial operation included younger age at repair, earlier birth cohort, fewer bronchopulmonary segments supplied by native pulmonary arteries, and initial placement of a systemic-pulmonary artery shunt. Competing-risks analysis for initially palliated patients predicted that after 10 years, 68% achieved complete repair (with associated factors including later birth cohort and more bronchopulmonary segments supplied by native pulmonary arteries), 22% died without repair, and 10% remained alive without repair. Reoperations after complete repair occurred in 38 children (27%), with risk factors including older age at palliation, MAPCAs, and more segments supplied by collaterals. CONCLUSIONS: Outcomes in children with PAVSD have improved over time, and are better in completely repaired cases. Bronchopulmonary arterial supply is an important determinant of mortality, achievement of definitive repair, and post-repair reoperation.

Blood cardioplegia provides superior protection in infant cardiac surgery.

BACKGROUND: We hypothesized that blood cardioplegia preserves myocardial metabolism and function more effectively than St Thomas' crystalloid cardioplegia in infant cardiac surgery. METHODS: Thirty infants with atrioventricular septal defects were randomly allocated to either blood or crystalloid intermittent cold (4 degrees C) cardioplegia. Arterial and coronary sinus blood was analyzed for lactate and oxygen. Cardiac output (thermodilution) and left ventricular function (echocardiography) were evaluated. RESULTS: The lactate concentration in coronary sinus blood early after bypass was significantly higher after crystalloid cardioplegia than after blood cardioplegia (2.1 +/- 0.3 vs 1.3 +/- 0.1 mmol/L, p = 0.006), with a significant myocardial release of lactate after crystalloid but not after blood cardioplegia. Oxygen extraction (arterial-coronary sinus O2 content) was higher early after crystalloid cardioplegia (3.02 +/- 0.13 vs 2.35 +/- 0.22 mmol/L, p = 0.01), possibly reflecting a difference in oxygen debt. The cardiac index was higher after blood cardioplegia (4.9 +/- 0.3 vs 4.0 +/- 0.3 L/min(-1)/m(-2), p = 0.04) and echocardiographic grading of left ventricular function was better (4.1 +/- 0.17 vs 3.5 +/- 0.22 arbitrary units, p = 0.046). CONCLUSIONS: This study indicates that blood cardioplegia preserves myocardial metabolism and function more effectively than crystalloid cardioplegia in infant cardiac surgery. The clinical significance of this finding is uncertain, but the more than 20% increase in cardiac index in the critical phase during weaning from bypass may be advantageous.

[Limited value of prenatal risk group screening for heart abnormalities. Time for a general screening?]. / Begränsat värde av riktad prenatal screening för hjärtmissbildningar. Dags för generell screening?

544 fetuses at increased risk of cardiac malformations were examined by echocardiography from gestational week 14 to 40 (median 21), during the period January 1989-March 2002. A heart defect was diagnosed in 70 fetuses. Three false positive diagnoses and eight false negative diagnoses occurred (sensitivity 89 % and specificity 99 %). Seven were terminated (three with trisomy, two with concomitant diaphragmatic hernia and two with single ventricles) and eight died in utero. 22 of 52 liveborn infants died during the neonatal period. Fetal arrhythmia was diagnosed in 30 cases of which eight died prenatally. This risk group screening identified only 3.3 % of all infants with heart defects born during the study period. The ambition to diagnose a greater proportion prenatally would require a general screening program. In order to guarantee a high quality of specialized fetal cardiology service, referrals should be centralized to only a few units in the country.