Institutional transition from invasive to non-invasive imaging in children with univentricular heart defects: safety and cost savings.

OBJECTIVES: Patients with univentricular heart defects require lifelong imaging surveillance. Recent advances in non-invasive imaging have enabled replacing these patients' routine catheterisation. Our objective was to describe the safety and cost savings of transition of a tertiary care children's hospital from routine invasive to routine non-invasive imaging of low-risk patients with univentricular heart defects. METHODS: This single-centre cohort study consists of 1) a retrospective analysis of the transition from cardiac catheterisation (n = 21) to CT angiography (n = 20) before bidirectional Glenn operation and 2) a prospective study (n = 89) describing cardiac magnetic resonance before and after the total cavopulmonary connection in low-risk patients with univentricular heart defects. RESULTS: Pre-Glenn: The total length of CT angiography was markedly shorter compared to the catheterisation: 30 min (range: 20-60) and 125 min (range: 70-220), respectively (p < 0.001). Catheterisation used more iodine contrast agents than CT angiography, 19 ± 3.9 ml, and 10 ± 2.4 ml, respectively (p < 0.001). Controlled ventilation was used for all catheterised and 3 (15%) CT angiography patients (p < 0.001). No complications occurred during CT angiography, while they emerged in 19% (4/21) catheterisation cases (p < 0.001). CT angiography and catheterisation showed no significant difference in the radiation exposure. Pre-/post-total cavopulmonary connection: All cardiac magnetic resonance studies were successful, and no complications occurred. In 60% of the cardiac magnetic resonance (53/89), no sedation was performed, and peripheral venous pressure was measured in all cases. Cost analysis suggests that moving to non-invasive imaging yielded cost savings of at least €2500-4000 per patient. CONCLUSION: Transition from routine invasive to routine non-invasive pre-and post-operative imaging is safely achievable with cost savings.

Fate of fenestration in children treated with fontan operation.

OBJECTIVE: To review the operative history and clinical and catheterization data on patients treated with total cavopulmonary connection (TCPC) with baffle fenestration and to study whether it is possible to predict the fate of fenestration. BACKGROUND: A baffle fenestration may improve postoperative outcomes after Fontan operation but is later associated with cyanosis and thromboembolic risk. Fenestration may close spontaneously or it can be closed percutaneously in patients with favorable hemodynamics. METHODS: Patients were divided into three groups: those with spontaneous closure of fenestration (group S, n = 34) and those with patent fenestration and favorable (group F, n = 36) or unfavorable (group U, n = 20) response to fenestration test occlusion. Clinical records were reviewed for demographic and anatomical characteristics, previous surgeries, and catheterizations. RESULTS: Predominant ventricular morphology was right ventricle (RV)/left ventricle (LV)/undeterminate in 19/14/1 patients in group S, 14/22/0 in group F, and 14/6/0 in group U. No differences were detected between groups in pre-TCPC catheterization data. Type of baffle fenestration was window/tube in 20/14 in group S, 28/8 in group F, and 20/0 in group U. All tube fenestrations either closed spontaneously or could be closed percutaneously. Twenty-nine percent of patients with window-type fenestrations failed the test occlusion. CONCLUSIONS: Spontaneous closure and favorable result in test occlusion are more common in tube than in window fenestrations. Since most preoperative anatomic and hemodynamic factors were similar in all patient groups, we find it difficult to predict the fate of a window-type fenestration and the result of test occlusion. © 2015 Wiley Periodicals, Inc.

Atresia of proximal coronary arteries in pulmonary atresia with intact ventricular septum - fetal and neonatal findings.

Pulmonary atresia with intact ventricular septum (PA+IVS) is a rare congenital cardiac malformation which is associated with ventriculocoronary arterial communications from the right ventricle. We present a case of PA+IVS with a bilateral atresia of the coronary ostia, and thus, a completely right ventricular-dependent coronary circulation followed up by fetal echocardiography. Eventually the infant died of myocardial infarction at 2 days of age.

Fetal growth restriction in preterm infants and cardiovascular function at five years of age.

OBJECTIVES: We have previously reported an increased cardiac workload in newborn preterm small (SGA) infants, but not in infants appropriate for gestational age (AGA). We hypothesized that these cardiovascular changes will persist at follow-up at 5 years of age. STUDY DESIGN: We assessed blood pressure, echocardiography, and skin perfusion with laser Doppler flowmetry in 22 SGA (821 +/- 248 g, 28.5 +/- 2.5 gestational weeks) and in 25 AGA (1065 +/- 241 g, 27.6 +/- 0.8 weeks) preterm children at age 5 years. Laser Doppler flowmetry also was used in 13 control children (3982 +/- 425 g, 40.4 +/- 1.8 weeks). RESULTS: The preterm children in both the SGA and AGA groups had similar higher systolic blood pressures, increased interventricular septum thicknesses, and smaller left ventricular end-diastolic diameters compared with population reference values. Maximal endothelium-independent perfusion to sodium nitroprusside was higher and maximal endothelium-dependent perfusion to acetylcholine reached a plateau earlier in the AGA preterm group than in the control group. CONCLUSIONS: Prematurity may impair cardiovascular function independently of intrauterine growth restriction. Altered cardiac dimensions and differences in perfusion responses may reflect increased cardiac afterload.

Long-term outcome in fetuses with cardiac arrhythmias.

OBJECTIVE: The objectives of this follow-up study of 292 fetuses with various cardiac arrhythmias were to estimate the incidence of structural heart defects and fetal compromise, to investigate the effects of antiarrhythmic medication, and to evaluate perinatal mortality and morbidity and long-term outcome. METHODS: The arrhythmias were classified into atrial extrasystoles (n = 200), atrial tachycardias (n = 35), atrioventricular block (n = 36), sinus bradycardia (n = 14), and ventricular extrasystoles (n = 7), and outcome of the infants was analyzed. RESULTS: The incidence of cardiac anomalies was 12% in the study population. In utero cardiac failure was noted in 11%. Among fetuses with atrial extrasystoles, 1% developed supraventricular tachycardia after birth. During antiarrhythmic therapy, sinus rhythm was achieved in 92% of nonhydropic and in 63% of hydropic fetuses. The latter had higher mortality and risk for neurologic morbidity than did nonhydropic fetuses; 38% versus 3.7% and 40% versus 12%, respectively. Among fetuses with atrioventricular block only, the survival rate was 82%, with a heart defect, prognosis was poor: 50% survived. Sinus bradycardia and ventricular extrasystoles were associated with survival rates of 75% and 67%. In the follow-up of the whole study population lasting a median 5 years, 93% are alive and 3% have a neurologic disorder. CONCLUSION: All fetal arrhythmias except atrial extrasystoles were associated with a moderately high risk for fetal distress. In cases of compromise, fetal and neonatal prognosis was poor and was an indication for perinatal medication. After the newborn period, the prognosis has been good. However, the risk for neurologic morbidity must be taken into consideration.

Pharmacokinetics of levosimendan in pediatric patients evaluated for cardiac surgery.

OBJECTIVE: The objective of the study was to evaluate the pharmacokinetics, hemodynamic effects, and safety of levosimendan in children with congenital heart disease. DESIGN: Open, one group, single-dose study. SETTING: Cardiac catheter laboratory in a pediatric cardiology department of a university hospital. PATIENTS AND TREATMENTS: Thirteen children between the ages of 3 months and 7 yrs coming for preoperative cardiac catheterization were enrolled into this study. All children received 12 microg/kg levosimendan as an intravenous infusion given over 10 mins during the catheterization. MEASUREMENTS: Concentrations of levosimendan and its metabolites were determined at specified time points before and after infusion (0-4 hrs). Invasive hemodynamics was evaluated up to 25 mins after the start of the infusion and echocardiography up to 2 hrs after the start of the infusion. MAIN RESULTS: The mean maximum concentration of levosimendan was 59 +/- 23 ng/mL in children older than 6 months of age. Levosimendan was rapidly distributed, with a mean half-life of 0.24 +/- 0.07 hrs. Mean terminal elimination half-life was 1.6 +/- 0.80 hrs. Total plasma clearance for the 10-min infusion was 3.6 +/- 1.3 mL/min/kg. Terminal elimination half-life in children aged 3-6 months was slower than in older children, i.e., 2.3 hrs vs. 1.6 hrs. Values of other pharmacokinetic variables were on the same level between the two age groups. The changes in hemodynamic variables were not statistically significant. There were no serious adverse events or unexpected adverse drug reactions during the study. CONCLUSIONS: The pharmacokinetic profile of levosimendan in children with congenital heart disease is similar to that in adult patients with congestive heart failure. The minimal hemodynamic efficacy after the 12 microg/kg levosimendan bolus was probably due to a small dose relative to body surface area.

Serum Levels of Natriuretic Peptides in Children before and after Treatment for an Atrial Septal Defect, a Patent Ductus Arteriosus, and a Coarctation of the Aorta-A Prospective Study.

Background and Objectives. We evaluated and compared the influence of treatment for atrial septal defect (ASD), patent ductus arteriosus (PDA), and coarctation of the aorta (CoA) on serum levels of N-terminal proatriopeptide and N-terminal probrain natriuretic peptide. Correlations between peptide levels and echocardiographic measurements were calculated. Patients and Methods. Peptide levels were measured and echocardiography performed before and 6-12 months after treatment in 21 children with ASD, 25 with PDA, 15 with CoA, and 76 control children. Results. ANPN levels were higher than in controls at baseline in all patient groups, and NT-proBNP in patients with ASD and PDA. Both peptide levels were elevated 6 months after treatment and decreased thereafter. Peptide levels were higher in patients with volume than pressure overload. They correlated with echocardiographic measurements. At the 6-month follow-up, dimensions of the originally overloaded ventricle had normalized only in patients with PDA. Conclusions. After intervention, peptide levels decrease but normalization takes over 6 months. The type of correlation between peptide levels and echocardiography varies according to the loading condition. Measurement of peptide levels can be used for monitoring the course of a patient's heart disease.

Left ventricular hypertrophy persists after successful treatment for coarctation of the aorta.

OBJECTIVES: To evaluate cardiac size and function in patients with coarctation of the aorta (CoA) before and after treatment. DESIGN: Ventricular size and function were examined by 2- and 3-dimensional echocardiography, and concentrations of natriuretic peptides measured in 15 paediatric patients before repair, and one, 6, and 12 months thereafter. Controls comprised 15 children. RESULTS: Before repair, mitral inflow velocities and left ventricular (LV) size and wall thickness were higher in patients. Thicknesses of interventricular septum and LV posterior wall decreased after repair but increased to initial level one year thereafter. The LV end-diastolic diameter remained larger than in controls despite successful repair. The size of right ventricle increased and levels of natriuretic peptides decreased during follow-up. Levels of natriuretic peptides correlated with the smallest diameter of CoA segment and diastolic indices of LV function. CONCLUSION: LV hypertrophy persists and LV size remains larger than in controls after successful repair even in normotensive patients with normal growth of CoA segment. This may be due to remodelling of ventricles and the aorta caused by CoA.

Hemodynamic improvement is faster after percutaneous ASD closure than after surgery.

OBJECTIVES: Hemodynamic effects of surgical and percutaneous closure of atrial septal defect (ASD) were evaluated. BACKGROUND: ASD causes volume overload of right ventricle (RV) and is associated with distortion and dysfunction of left ventricle (LV). The amount and timing of hemodynamic changes after ASD closure are not well known. METHODS: The study group consisted of 7 children treated surgically and 17 treated in the catheterization laboratory. In the control group, there were 51 healthy children. RV size and LV end-diastolic and systolic dimensions, volumes, and function were examined by two- and three- dimensional echocardiography and serum concentrations of natriuretic peptides measured prior to ASD closure, and 1, 6, and 12 months thereafter. RESULTS: In all children with ASD, during the 1-year follow-up, the z score of RV end-diastolic diameter decreased from a median 5.00 SD to 2.25 SD (P < 0.001). Dilatation of RV did not resolve entirely during 1-year follow-up in either treatment group. End-diastolic LV diameter increased from -1.50 to -0.50 SD (P < 0.001). LV size increased slower in the surgical subgroup but reached control levels in both groups. Concentrations of natriuretic peptides increased during the first month after ASD closure and normalized thereafter in patients treated percutaneously but remained higher than in controls in patients treated surgically. CONCLUSIONS: During 1-year follow-up after ASD closure, RV size decreases but does not normalize in all patients. The size of the LV normalizes after ASD closure but the increase in LV size is slower in patients treated surgically. Serum levels of ANPN and proBNP are elevated prior to ASD closure but decrease thereafter to control levels in patients treated with the percutaneous technique but not in those treated surgically.

The influence of percutaneous closure of patent ductus arteriosus on left ventricular size and function: a prospective study using two- and three-dimensional echocardiography and measurements of serum natriuretic peptides.

OBJECTIVES: We aimed to evaluate the effect of percutaneous closure of patent ductus arteriosus (PDA) on left ventricular (LV) hemodynamics. BACKGROUND: Today, most PDAs are closed percutaneously. Little is known, however, about hemodynamic changes after the procedure. METHODS: Of 37 children (ages 0.6 to 10.6 years) taken to the catheterization laboratory for percutaneous PDA closure, the PDA was closed in 33. Left ventricular diastolic and systolic dimensions, volumes, and function were examined by two-dimensional (2D) and three-dimensional (3D) echocardiography and serum concentrations of natriuretic peptides measured before PDA closure, on the following day, and 6 months thereafter. Control subjects comprised 36 healthy children of comparable ages. RESULTS: At baseline, LV diastolic diameter measured >+2 SD in 5 of 33 patients. In 3D echocardiography, a median LV diastolic volume measured 54.0 ml/m2 in the control subjects and 58.4 ml/m2 (p < 0.05) in the PDA group before closure and 57.2 ml/m2 (p = NS) 6 months after closure. A median N-terminal brain natriuretic peptide (pro-BNP) concentration measured 72 ng/l in the control group and 141 ng/l in the PDA group before closure (p = 0.001) and 78.5 ng/l (p = NS) 6 months after closure. Patients differed from control subjects in indices of LV systolic and diastolic function at baseline. By the end of follow-up, all these differences had disappeared. Even in the subgroup of patients with normal-sized LV at baseline, the LV diastolic volume decreased significantly during follow-up. CONCLUSIONS: Changes in LV volume and function caused by PDA disappear by 6 months after percutaneous closure. Even the children with normal-sized LV benefit from the procedure.

Outcome of structural heart disease diagnosed in utero.

OBJECTIVE: The aim of the study was to review the outcome of fetuses with structural heart disease detected by echocardiography. METHODS: A total of 99 fetuses with different types of cardiac defects, diagnosed at a median gestational age of 28.4 weeks (range 16-41 weeks), were included. The inclusion criteria were a structural heart defect confirmed postnatally or at post-mortem examination, and a complete, long-term follow-up in utero and after birth in the Hospital for Children and Adolescents, Helsinki, Finland from 1983 to 1999. RESULTS: Of 99 fetuses with in utero diagnosed cardiac anomalies, 6 (6%) showed normal cardiac status postnatally. Thirty-five percent of fetuses (n = 10) with heart disease diagnosed before 24 weeks of gestation were terminated. Of 83 fetuses, 7 (8%) with a heart defect died in utero at a median gestational age of 33 weeks. Chromosomal abnormality was found in 28% of cases. Fetuses with normal chromosomes had extracardiac anomalies in 40% of cases. Mortality due to chromosomal abnormality was 73% and from extracardiac anomaly 48%. Intrauterine heart failure was detected in 27% of fetuses and was frequently associated with univentricular heart (UVH) and intracardiac tumors, in 36 and 67%, respectively; 12 fetuses (13%) were found to have associated arrhythmia; 4 of these died. Of 76 live births (median gestational age 38 weeks, birth weight 2878 g), a total of 37 (49%) neonates died. Twenty-four neonates (32%) underwent cardiac surgery or invasive procedure; six infants (25%) died after the procedure. Neonatal mortality was highest in fetuses with hypoplastic left heart syndrome (HLHS), ventricular septal defect (VSD), and UVH (87, 64, and 50%, respectively). In long-term follow-up (median 3.8 years), 34 children of 76 live births (45%) were alive, 59% of them were without symptoms. CONCLUSION: Our data indicate that despite elective, planned delivery the prognosis for fetuses with in utero diagnosed heart defect was poor. The outcome was largely attributable to associated extracardiac malformations and chromosomal abnormalities.

Etiology and outcome of fetuses with functional heart disease.

BACKGROUND: The aim of this study was to assess the etiology and outcome of fetuses with functional heart disease as detected by echocardiography. METHODS: In total, 51 fetuses (median gestation age of 28.6 weeks) were included. The inclusion criteria were hydrops (n = 14), pericardial effusion (PE; n = 9), tricuspid valve regurgitation (TR; n = 8), hypertrophic cardiomyopathy (HCM; n = 7) and dilated cardiomyopathy (DCM; n = 7). Antenatal management was performed for 17 of 51 fetuses (33%): two abortions, nine digoxin administrations, three thoracocenteses, one pericardial puncture, one blood transfusion and one ascites centesis. RESULTS: The etiology of functional heart disease was twin pregnancy in 18, fetal lung lesions in five, maternal diabetes in five, fetal anemia in four, extracardiac or chromosomal abnormalities in three, infection in three, teratoma or arteriovenous malformation in four, indomethacin administration in two, endocardial fibroelastosis in two, maternal anaphylaxia in one, idiopathic arterial calcification of infancy (IACI) in one, pregnancy-induced hypertension (PIH) in one, and unknown in two fetuses. There was no significant difference between fetuses with and without treatment (53% vs. 79%; p = 0.06). There were two stillbirths and 12 postnatal deaths (29%). Among 35 surviving infants, 85% were free of symptoms in the follow-up (mean 3.9 years). CONCLUSIONS: These findings indicate that a functional heart disease in utero is associated with very varying etiology and high mortality. Improved understanding of the hemodynamic findings may lead to treatment that is more successful.

Haemodynamic effects of erythrocyte transfusion in preterm infants.

UNLABELLED: The aim of the study was to assess the short-term cardiorespiratory effects of a standard red cell transfusion in very low birth weight (< 1500 g) infants undergoing intensive care. A total of 37 infants (birth weight 920 +/- 230 g, gestational age 27.8 +/- 2.1 weeks, age at study 6.1 +/- 3.9 days) with indwelling arterial lines were studied when 10 ml/kg of packed donor red cells were transfused based on clinical judgment. Infants with patent ductus arteriosus and/or inotropic treatment were excluded from the study. Oxygen saturation, left ventricular output, stroke volume, systolic, diastolic and mean arterial pressure, heart rate, and capillary refill time were assessed immediately prior to the transfusion and within an hour after the transfusion was completed. Capillary refill time after the transfusion was significantly shorter than prior to the transfusion (2.1 +/- 0.9 versus 2.4 +/- 1.0 s, P = 0.033). Left ventricular output, stroke volume and arterial pressures remained unaltered. Oxygen saturation after the transfusion was lower than before the transfusion (94.0 +/- 3.8 versus 95.3 +/- 2.5%, P = 0.014) despite unaltered oxygen supply. CONCLUSION: the data suggest that although a red cell transfusion of 10 ml/kg may marginally improve peripheral perfusion, it does not influence cardiac output and arterial blood pressure in normotensive preterm infants. It may, however, cause a transient decrease in oxygen saturation.

Cardiac hypertrophy and altered hemodynamic adaptation in growth-restricted preterm infants.

The objective was to elucidate hemodynamic adaptation in very low birth weight (<1500 g) infants after intrauterine growth retardation. 31 growth-retarded (SGA, birth weight <-2 SD) and 32 appropriate for gestational age (AGA, birth weight within +/- 1 SD range) infants were enrolled. In SGA infants, the diastolic diameters of the interventricular septum and the left ventricle were increased, and serum brain natriuretic peptide (BNP) was elevated. Left ventricular output (LVO) of the AGA infants increased from 150 +/- 28 to 283 +/- 82 mL/kg/min during the study (p < 0.01). The SGA infants had a higher initial LVO than the AGA infants (243 +/- 47 versus 150 +/- 28 mL/kg/min, p < 0.05), but did not show further LVO increase during the study period. Red cell (RCV) and blood (BV) volume were assessed by Hb subtype analysis, when packed donor red cells were transfused. RCV and BV did not differ between the groups initially, but RCV increased by 18% and BV by 29% in the AGA group during the first 3 d. On day 3, AGA infants had larger BV than the SGA infants (88 +/- 5 versus 73 +/- 12 mL/kg, p < 0.05). In conclusion, cardiac hypertrophy, elevated initial LVO and BNP of the SGA infants suggest increased cardiac workload after intrauterine growth retardation. Based on the BV and RCV data, blood volume regulation may also be impaired. The data suggest that SGA preterm infants may be exposed to an increased risk of circulatory failure during early adaptation.