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Differentiation between post-operative inflammation and bacterial infection remains an important issue in infants following congenital heart surgery. We primarily assessed kinetics and predictive value of C-reactive protein for bacterial infection in the early (days 0–4) and late (days 5–28) period after cardiopulmonary bypass surgery. Secondary objectives were frequency, type, and timing of post-operative infection related to the risk adjustment for congenital heart surgery score.
This 3-year single-centre retrospective cohort study in a paediatric cardiac ICU analysed 191 infants accounting for 235 episodes of CPBP surgery. Primary outcome was kinetics of CRP in the first 28 days after CPBP surgery in infected and non-infected patients.
We observed 22 infectious episodes in the early and 34 in the late post-operative period. CRP kinetics in the early post-operative period did not accurately differentiate between infected and non-infected patients. In the late post-operative period, infected infants displayed significantly higher CRP values with a median of 7.91 (1.64–22.02) and 6.92 mg/dl (1.92–19.65) on days 2 and 3 compared to 4.02 (1.99–15.9) and 3.72 mg/dl (1.08–9.72) in the non-infection group. Combining CRP on days 2 and 3 after suspicion of infection revealed a cut-off of 9.47 mg/L with an acceptable predictive accuracy of 76%.
In neonates and infants, CRP kinetics is not useful to predict infection in the first 72 hours after CPBP surgery due to the inflammatory response. However, in the late post-operative period, CRP is a valuable adjunctive diagnostic test in conjunction with clinical presentation and microbiological diagnostics.
Direct hepatic veins-to-hemiazygos connection offers the balanced distribution of hepatic venous blood to both lungs, not requiring anticoagulation. We report a 13-year follow-up after this type of off-pump Fontan completion. Patient’s hepatic veins-to-hemiazygos confluence increased with growth to allow for unobstructed flow. This unique technique can be recommended in heterotaxy patients, if atrial hepatic venous drainage and hemiazygos vein are in close proximity.
A 25-year-old patient with signs of cirrhosis on ultrasound and CT presented with portal vein thrombosis on routine follow-up examinations; retrograde hepatic wedge angiography demonstrated only the right-sided portal vein branch. Development of a portosystemic collateral vessel to the left-sided renal vein prevented signs of hypersplenism. This unique complication of portal vein thrombosis should be considered during long-term surveillance.
Chronic paediatric heart disease is often associated with residual symptoms, persisting functional restrictions, and late sequelae for psychosocial development. It is, therefore, increasingly important to evaluate the health-related quality of life of children and adolescents with chronic heart disease. The aim of this study was to determine medical and socio-demographic variables affecting health-related quality of life in school-aged children and adolescents with chronic heart disease.
Patients and methods
The Pediatric Cardiac Quality of Life Inventory was administered to 375 children and adolescents and 386 parental caregivers. Medical information was obtained from the charts. The socio-demographic information was provided by the patients and caregivers.
Greater disease severity, low school attendance, current cardiac medication, current parental employment, uncertain or limited prognosis, history of connection to a heart–lung machine, number of nights spent in a hospital, and need for treatment in a paediatric aftercare clinic independently contributed to lower health-related quality of life (self-report: R2=0.41; proxy-report: R2=0.46). High correlations between self-reports and parent-proxy reports indicated concordance regarding the evaluation of a child’s health-related quality of life.
Beyond medical treatment, integration into school is important to increase health-related quality of life in children and adolescents surviving with chronic heart disease. Regular screening of health-related quality of life is recommended to identify patients with special needs.
Although clinically silent in the majority of cases, enlarged bronchial arteries or systemic-to-pulmonary collateral arteries may complicate congenital heart disease in infants, causing significant left-to-right shunting with subsequent pulmonary congestion and respiratory compromise. So far, pulmonary haemorrhage, a well-known complication in older patients with cyanotic congenital heart disease, has not been described in infancy. We describe the case of a 6-month-old girl with tetralogy of Fallot and absent pulmonary valve who developed haemoptysis with severe respiratory distress following corrective surgery of the cardiac malformation. High-resolution computed tomography of the thorax followed by selective angiography revealed a systemic-to-pulmonary collateral artery originating from the left internal mammary artery. Pulmonary haemorrhage stopped immediately following coil occlusion of the collateral. A second episode of pulmonary haemorrhage occurred at the age of 9 months during mechanical ventilation for treatment of pneumonia. Repeat angiography revealed two more collateral vessels. Again coil occlusion resulted in prompt resolution of pulmonary haemorrhage. According to our experience, enlarged bronchial arteries or systemic-to-pulmonary collateral arteries should be considered in infants with cyanotic heart disease with unexplained pulmonary congestion or prolonged respiratory problems.
Neonatal interventions for critical aortic coarctation may be associated with considerable morbidity and mortality if the patient is extremely premature. We report the successful treatment of critical coarctation in a 25-week, 740-gram infant using initial clipping of the duct until continued prostaglandin E1 infusion delayed end-to-end anastomosis 7 weeks later.
The procedure of total cavopulmonary connexion includes trans-section and closure of the pulmonary trunk, the systemic venous blood flow being directed to the pulmonary vascular bed by an anastomosis of the superior caval vein with the right pulmonary artery. Following this procedure, there remains a small residual pulmonary trunk. The purpose of our study was to obtain information about the fate of this residual trunk and its contained pulmonary valve. Using transthoracic echocardiography, we examined 29 patients (mean age 7 years 4 months) after a total cavopulmonary connexion (mean interval 17 months). We were able to obtain adequate visualization of the pulmonary valve and the pulmonary trunk in 23 of the patients. Pulmonary regurgitation was demonstrated by colour Doppler echocardiography in 13 pts (57%). Formation of thrombus in the residual pulmonary trunk was detected in one patient (4%) 6 months after the operation. One cerebrovascular embolic event occurred in a patient who had pulmonary regurgitation without formation of thrombus in the residual trunk 3 months after the total cavopulmonary connexion. Our findings show that pulmonary regurgitation is frequently present in patients after total cavopulmonary connexion. Although formation of thrombus in the residual pulmonary trunk seems to be less common, we recommend surgical closure of the pulmonary valve during this procedure so as to exclude the residual pulmonary trunk as a possible source of systemic emboli.
Despite a good haemodynamic result, many children have amildly decreased arterial oxygen saturation following a total cavopulmonary connection. Our study was performed to determine possible mechanisms of right-to-left shunting in these patients. We performed elective cardiac catheterization in 19 children at a mean interval of 3.6 years following a total cavopulmonary connection. The intrapulmonary right-to-left shunt, the intracardiac right-to-left shunt and the total right-to-left shunt were calculated under mechanical ventilation with 100% oxygen. The intrapulmonary right-to-left shunt was 10.8±3.5% of the pulmonary blood flow, and the total right-to left shunt accounted for 18.9±5.2% of the systemic blood flow. The intracardiac right-to-left shunt in patients with no relevant venovenous collaterals or leaks in the atrial tunnel was calculated at 6.4±3.0% of the systemic blood flow, while the intracardiac right-to-left shunt in patients with relevant collaterials or leaks accounted for 13.0±5.9% of the systemic blood flow. Since intrapulmonary arteriovenous fistulas were not demonstrated angiographically in any of our patients, the intrapulmonary right-to-left shunt is probably due to low ratios of perfusion to ventilation in some pulmonary segments. The intracafdiac right-to-left shunt was due to leaks across the interatrial baffle, collaterals between stystemic and pulmonary veins, and to the coronary sinus draining to the pulmonary venous atrium.
To investigate cardiopulmonary performance in patients after a Fontan procedure, comparing it to patients following a Senning operation.
We studied 21 children, with a mean age of 11.1 years, after a total cavopulmonary anastomosis, comparing them to 13 with complete transposition after a Senning procedure, having a mean age of 11.8 years, and 21 control subjects with a mean age of 11.2 years. All were tested on a bicycle ergospirometer.
Peak consumption of oxygen, maximal work rate, peak oxygen pulse and endexpiratory pressure of carbon dioxide at a work rate of 1.5 Watt/kg were lowest in patients with a modified Fontan procedure, and highest in the control group (p ≤ 0.0278). Production of carbon dioxide, and minute ventilation at a work rate of 1.5 Watt/kg, was highest in the patients after Fontan procedure, and lowest in the healthy subjects (p ≤ 0.0163). Production of carbon dioxide per single breath was lower in those having a Fontan procedure (28.9 ml) than in the two other groups (35.1 ml; p = 0.0243). The tidal volume showed no significant differences between the three groups.
The reaction to exercise was identical qualitatively in both groups of patients, and comparable to the behaviour of patients with chronic heart failure. Quantitatively, the results of the patients following a Senning procedure lay between those of control subjects and those who had undergone a Fontan operation. The only exception was dead space ventilation, where the patients after a Fontan procedure differed from the two other groups because of their increased ventilation-perfusion mismatch.
Introduction: Recurrent but infrequent syncopes represent a diagnostic challenge, since they frequently remain unexplained despite extensive investigations. This applies specifically for patients who carry an increased risk of potentially lifethreatening arrhythmias, either due to congenital cardiac disease or primary electrical disorders. Implantable loop recorders permit long-term electrocardiographic monitoring. Experience with these devices is still limited in children. Patients and methods: Between January 1999 and August 2005, 12 patients underwent implantation of a loop recorder in our tertiary referral centre. The mean age was 10.9 years, with a range from 2 to 17 years. Of the patients, 6 had structural disease, 3 had primary electrical abnormalities, and 3 had no cardiovascular disease. Results: Resyncope occured in 9 of the 12 patients. Arrhythmic origin of the syncope was diagnosed in 4 of these patients. The events recorded were ventricular fibrillation in 2, intermittent asystole in 1, and pacemaker-syndrome in the other patient. Malignant arrhythmia was ruled out in the remaining 5 patients. There were no complications related to implantation of the loop recorder, and the mean duration until explantation was 8.3 months. Conclusions: Based on our experience, we suggest that implantation of a loop recorder represents an additional tool for a selected group of children. Due to its invasive nature, it should be restricted to patients at high risk, or those in which there is substantial clinical suspicion of the likelihood of serious arrhythmias when conventional testing has been inconclusive. In this cohort, implantation of the loop recorder either helps to establish the correct diagnosis, or to exclude an arrhythmic event, thus avoiding unnecessary escalation of therapy and providing reassurance for the family.
We report a 17 year old boy with a large fistula from the right coronary artery to the right ventricle. The fistula consisted of two major branches. The smaller branch could be embolised with three detachable platinum microcoils without problems. The high flow of blood in the larger branch, however, prevented conventional occlusion using coils. Interventional occlusion of this branch was achieved in a second attempt. Following creation of an arterio-venous wire-loop, we advanced a balloon catheter into the distal end of the fistula. Under temporary occlusion of the fistula, complete closure was achieved by delivery of 7 detachable platinum microcoils. Thus, even large coronary arterial fistulas can be closed safely with these new platinum detachable microcoils. The procedure, however, requires temporary control of the flow of blood by balloon occlusion.
We report the rare combination of a severely hypoplastic left ventricle and discordant ventriculoarterial connections, with associated subpulmonary obstruction of the left ventricular outflow tract. The long tortuous arterial duct originated at an acute angle from the aorta, suggesting that the subpulmonary obstruction developed early in fetal life. Residual flow via the hypoplastic left ventricle to the pulmonary circulation after insertion of an aortopulmonary shunt resulted in haemodynamic deterioration, which was instantly reversed by closing the pulmonary trunk.
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