Double aortic arch with dominant left arch and right ligamentum arteriosum.

This case report describes a rare example of double aortic arch with a dominant left aortic arch, patent minor right aortic arch, left descending aorta, and right ligamentum arteriosum causing tracheobronchial compression in a twin baby girl with DiGeorge syndrome. She also had large right subclavian artery arising from right-sided diverticulum of Kommerell, aplastic thymus, T cell lymphopenia with normal immunoglobulin, hypocalcemia, and hypomagnesemia. The diverticulum of Kommerell was resected and minor right aortic arch, right ligamentum arteriosum, and right subclavian artery were divided through right posterolateral thoracotomy. Aortopexy was performed under bronchoscopic guidance to relieve the airway compression. We strongly suggest a right-sided approach in this type of vascular arrangement for easy access and better outcome.

Intractable tinnitus and sensorineural deafness cured by surgical correction of coarctation of aorta.

Tinnitus is a challenging condition to manage with no effective treatment. We report a case of lifelong tinnitus present in a patient who was cured with the surgical repair of his coarctation of aorta. The patient was a fit 37-year-old with coarctation of aorta, senserineural deafness and intractable tinnitus. He underwent a thoracotomy and tube by-pass of his coarctation of aorta. A complete relief of his tinnitus and improvement in his hearing was noted postoperatively and confirmed on audiograms. This is the first reported case of tinnitus due to coarctation of aorta relieved by surgical correction of the coarctation.

Vacuum-assisted closure in the paediatric patient with post-cardiotomy mediastinitis.

Mediastinitis has a high mortality and is a major cause for concern in the neonatal cardiac surgical population. Vacuum-Assisted Closure (V.A.C.) is a newly established technique for expediting healing in the management of wounds resistant to established treatments; this includes the treatment of post-cardiotomy mediastinitis in the adult cardiac surgical patient. We describe the previously unreported use of the V.A.C. device for the successful treatment of post-cardiotomy mediastinitis in an infant. The device also improved the mechanics of respiration. We discuss potential risks and benefits of V.A.C. and suggest guidelines for its use.

Assessment of mortality rates for congenital heart defects and surgeons' performance.

BACKGROUND: In the absence of reliable national data, we have collected results of all operations for congenital heart defects from five departments to assess mortality rates and compare them among surgeons and departments. METHODS: Data relating to all operations (2,718) carried out at the five centers during a period from April 1, 1997 through March 31, 1999. Clearly defined criteria were agreed for the classification of patients into various subgroups. RESULTS: The overall hospital mortality was 4.4% (95% confidence intervals 3.7%-5.3%). Mortality for open-operations was 12.6% in neonates, 5.1% in infants, and 3.5% in children. Mortality rates were 1.1% for coarctation, 0.4% ventricular septal defect, 4.1% atrioventricular septal defect, 2.9% Fallot, 0.9% switch, and 15.6% truncus arteriosus. Although individual surgeons' mortality rates ranged from 1.8% to 7.5%, none of the 12 surgeons' data were above 95% confidence intervals. For individual surgeons, the change in mortality rates between the 2 years ranged between -3.3% and +3.8%. CONCLUSIONS: With 2 years' data available, estimates of mortality rates are more precise as reflected by tighter confidence intervals. There were relatively small data sets for individual hospitals and surgeons, which made statistical evaluation difficult. For setting standards, data from more departments for a longer period will be required. Statistical methods alone cannot be used as a sole arbiter of what is considered acceptable performance.

Mortality rates after surgery for congenital heart defects in children and surgeons' performance.

BACKGROUND: A public inquiry into surgery for paediatric congenital heart defects in Bristol, UK, underscored the need for reliable data on overall mortality rates, which would allow assessment of individual surgeons' performance. We aimed to gather and report such data for 1 year to provide information for clinicians, researchers, policy makers, and the general public. METHODS: We collected data on all operations (1378) for congenital heart defects done by 11 surgeons in five departments in the UK between April 1, 1997, and March 31, 1998. These operations represented about 36% of all operations done in the UK during that time. Clearly defined criteria were agreed to classify operations into subgroups. FINDINGS: The overall mortality rate for all operations was 4.0% (95% CI 3.0-5.2). No deaths occurred for 67 arterial-switch operations. Mortality rates for coarctation, ventricular septal defect, atrioventricular septal defect, Fallot, and truncus arteriosus operations were 1.1%, 0.6%, 3.6%, 2.3%, and 28.6%, respectively. Although overall mortality rates between surgeons varied (1.6-6.9%), no surgeon's were higher than the 95% CI. The numbers of operations done by individual surgeons were small, which led to wide confidence intervals and made the detection of differences in performance difficult. INTERPRETATION: The participating departments seemed to reach high standards of care for children with congenital heart defects, although more data would be needed to assess performance of individual surgeons. The development of quality standards will be difficult because of the complexity of defects, the different types of operations, and few patients in each subgroup. Collection of larger sets of data for more patients and centres are needed.

Coronary ostial patch angioplasty in children.

Patch angioplasty for coronary artery ostial stenosis was first reported in adults in 1952 and only sparsely used until 1983 when Hitchcock reviewed the technique in adults. We present two cases that highlight our use of this technique in inflammatory diseases of the aorta in children. Patch angioplasty restores physiologic perfusion of the coronary artery tree, preserves conduit material for further procedures later in life, and allows subsequent percutaneous transluminal coronary angioplasty of stenosis. For these reasons we believe that patch angioplasty holds undoubted advantage over the available alternatives.

Left atrial myxoma in a preschool child.

A case of left atrial myxoma successfully removed using cardiopulmonary bypass in a 5-year-old child is presented. Review of the literature emphasizes the rarity and clinically aggressive behavior of this tumor in this age group.

Acute-phase responses to cardiopulmonary bypass in children weighing less than 10 kilograms.

BACKGROUND: Cardiopulmonary bypass induces a systemic inflammatory response. This study investigated, in a pediatric population, cytokine-induced responses and their potential modification by intraoperative steroid administration. METHODS: Markers of the acute-phase response were measured perioperatively in 24 children weighing less than 10 kg undergoing cardiac operations. Those having operations with cardiopulmonary bypass were randomized to receive either no steroid (group I, n = 8) or 10 mg/kg methylprednisolone in the pump prime (group II, n = 10); patients undergoing nonbypass procedures were controls (group III, n = 6). RESULTS: In all groups, plasma interleukin-6 level was elevated (p < 0.01) above baseline throughout the post-operative period, peaking earlier in group I. Levels of C-reactive protein peaked at 48 hours, and postoperative core temperature was raised in all groups. Levels of interleukin-6 from 2 to 6 hours and C-reactive protein at 24 hours postoperatively were greater (p < 0.05) in group I than in group II. Maximum interleukin-6 level, C-reactive protein level, and temperature were all significantly greater in group I than in group III. Maximum interleukin-6 level correlated with maximum C-reactive protein level in group I only (rs = 0.76; p < 0.05) and showed no association with temperature. Duration of bypass did not correlate with levels of interleukin-6. CONCLUSIONS: This study demonstrated a marked acute-phase response to operation; the greater response to procedures with cardiopulmonary bypass was abrogated by intraoperative steroid administration. The importance of interleukin-6 as an inducer of acute phase proteins after bypass is supported by its association with C-reactive protein levels, but other factors must be important in the induction of pyrexia.

Role of ECMO in neonatal myocardial infarction.

A neonate with myocardial infarction who failed to respond to conventional treatment was supported by extracorporeal membrane oxygenation (ECMO). Severe mitral valve regurgitation necessitated mitral valve replacement while receiving ECMO following which the infant was successfully weaned. ECMO should be considered for potentially treatable causes of catastrophic heart failure.

The acute phase response to cardiopulmonary bypass in children.

The endocrine phase of the stress response to cardiopulmonary bypass in children is known to be subtly different from that seen in adults. The aim of this investigation was to determine whether there are similar differences in the acute phase response. Thirteen children were studied (mean age 2.65 years). Each child had congenital heart disease and underwent corrective cardiac surgery. Blood samples taken two days prior to operation and at 6, 9, 12, 24, 48 and 120 hours after were analysed for C-reactive protein, albumin, caeruloplasmin, zinc and copper concentrations. Metal:carrier protein molar ratios were also calculated. Results demonstrate changes which, although similar to those seen in adults, differed both quantitatively and qualitatively. This is explained by the concept of immaturity leading to a generally poor capacity for protein synthesis and a relative inability to respond to altered circumstances.

Effects of dopamine on liver blood flow in children with congenital heart disease.

BACKGROUND: A reduction in liver function is common after cardiac operations, particularly in children with preexisting cardiac failure. The etiology is multifactorial, but the redistribution of organ blood flow that occurs during cardiopulmonary bypass implicates ischemia as one of the principal causes of injury. Dopamine hydrochloride is known to have specific effects on the renal circulation, and the aim of this study was to investigate its effects on hepatic perfusion. METHODS: Eight children with congenital heart disease were studied 6 hours after the end of cardiopulmonary bypass when they were fully rewarmed and hemodynamically stable. Using noninvasive auricular densitometry, we determined the percent disappearance rate of indocyanine green as an index of liver blood flow both before and 1 hour after commencing an infusion of dopamine at 4 micrograms.kg-1.min-1. RESULTS: Results showed an increase of approximately 31% in the percent disappearance rate of indocyanine green with the addition of low-dose dopamine (4 micrograms.kg-1.min-1) (p < 0.01). CONCLUSIONS: Dopamine may have a therapeutic role in increasing hepatic perfusion and minimizing any loss in liver function.

Total body water in children with congenital heart disease, before and after cardiac surgery.

The aim of this study was to measure total body water in children with congenital heart disease before and after cardiac surgery and to compare the results of deuterium and 18oxygen dilution methods. Seventeen children (aged 4 to 33 months) were given aliquots of isotopically labeled water 1 week before and 6 hours after cardiac surgery. Isotope equilibration and analysis of the declining enrichment of daily urine samples allowed calculation of the total body water content. Before operation, total body water was significantly elevated (p < 0.001, Wilcoxon test); after operation it fell to approximately normal values. This finding is in contrast to those of previous reports, but may be explained in that the method used for calculation depended on measurements taken over a 7-day period rather than on a single measurement of isotope dilution as used elsewhere. Nevertheless, these results do suggest that surgery can correct the preoperative fluid overload. Comparison of deuterium and 18oxygen dilution methods showed a 2% to 2.5% overestimation of the total body water content with deuterium sampling.

The validation of auricular densitometry for indocyanine green clearance measurement of hepatic blood flow during and after cardiopulmonary bypass in children.

The clinical measurement of hepatic perfusion is complicated by a dual blood supply and the invasive nature of the majority of techniques available. The aim of this study was to validate indocyanine green clearance and noninvasive auricular densitometry as a measure of hepatic perfusion in the context of paediatric cardiac surgery. The effects of different dye concentrations on densitometer recording were assessed in vitro and found to have a linear relationship. Similarly, variations in haematocrit, within the range 21-47%, also had little effect on accuracy. Comparison of densitometry and direct blood sampling with plasma spectrophotometry in six postoperative, normothermic children showed no significant difference between the noninvasive and invasive techniques (r = 0.968; p > 0.05, t-test). Comparison in 10 hypothermic children during cardiopulmonary bypass also showed no significant difference between the two methods, provided that no further cooling or rewarming took place (r = 0.83; p > 0.05, Wilcoxon test). Noninvasive auricular densitometry can, therefore, provide a reliable assessment of hepatic perfusion in children undergoing cardiac surgery.

The effects of congenital heart disease and cardiac surgery on liver blood flow in children.

Children with congenital heart disease may have some degree of hepatic impairment, with further impairment developing shortly after surgical correction of the cardiac defect. The redistribution of organ blood flow that occurs during cardiopulmonary bypass implicates ischaemia as one of the principal causes of injury. The aim of this study was to measure liver blood flow in children with congenital heart disease and to determine both the effects of cardiopulmonary bypass and the consequences of corrective surgery. Indocyanine green clearance and auricular densitometry, were used in 31 children. In 83% we demonstrated a reduced liver blood flow, with a mean percentage disappearance rate (PDR) of 12.9% (SEM +/- 1.2). This finding was unrelated to the patient's age, the type of congenital heart defect or the presence or absence of cyanosis. During cardiopulmonary bypass, hepatic perfusion was further reduced in 77% of children, by an average of 67%, out of proportion with the iatrogenic reduction in total body flow. Six hours after surgery, liver blood flow had increased significantly above preoperative levels (p < 0.001; t-test) to approximately normal values with a mean PDR of 20.4% (SEM +/- 1.5).