Systemic inflammation has been suggested to underlie in part the elevated risk of arrhythmias and myocardial dysfunction during the first weeks after cardiac surgery. Recent transthoracic Doppler studies from our centre indicated increased basal coronary arterial flow in children 5 days after cardiopulmonary bypass surgery. In these children, we investigated whether the inflammatory mediator, C-reactive protein, could influence this association.

The peak flow velocity, velocity time-integral in diastole and systole, and basal blood flow in the proximal part of the left anterior descending artery, were assessed by transthoracic Doppler echocardiography 1 day before, and 5 days after, cardiac surgery in 17 children with ventricular and atrioventricular septal defects whose mean age at surgery was 6 months. Levels of C-reactive protein in the plasma were measured at both time-points.

Prior to surgery, all children had levels of C-reactive protein under the limit for detection, that is less than 0.8 milligrams per litre. The levels of the protein had increased significantly by the second day, when the median value was 25, and the range from 20 to 142 milligrams per litre. They remained elevated on the fifth day after surgery, when the median was 11, and the range from 3 to 20 milligrams per litre. On the fifth day, the percentage increase in velocity time integral corrected for left ventricular mass was significantly lower in those patients with C-reactive protein greater than or equal to 10 milligrams per litre than in the remaining patients. Also, both the velocity time integral and the velocity of diastolic peak flow correlated inversely with log C-reactive protein, r being equal to −0.54 and p less than 0.02 and r equal to −0.74 and p less than 0.01, respectively, particularly among those patients in whom clamping of the aorta lasted for more than 1 hour, r for this statistic being equal to −0.8.

The postsurgical increase in the velocity of coronary arterial flow in children is inversely associated with rising levels of C-reactive protein. The duration of the aortic cross-clamping during surgery strengthens the association between levels of C-reactive protein and the microcirculatory changes.

With improvements in technology and surgical technique, paediatric cardiologists are challenging surgeons to repair balanced atrioventricular septal defects in smaller patients. Early repair minimizes aggressive medical therapy to prevent heart failure, maintains growth, and limits exposure to elevated pulmonary pressures. We compare the outcomes of repair among different-sized children.

From December 2002 to July 2005, 92 patients underwent repair of an atrioventricular septal defect with common atrioventricular valvar orifice and balanced ventricles. We reviewed operative and postoperative data. We excluded patients weighing more than 10 kilograms, but included those who underwent concomitant closure of a patent oval foramen or atrial septal defect, or ligation of a patent arterial duct. Those requiring other concomitant procedures were excluded from the analysis.

The median weight at repair was 4.9 kilograms, with a range from 2.93 to 7.9 kilograms, and the median age was 5.1 months, with a range from 0.39 to 9.6 months. Operative data included the time required for cardiopulmonary bypass, aortic cross-clamping, and the overall procedure. These times were not significantly affected by decreasing weight. Postoperative continuous data included duration of ventilation and length of intensive care unit and hospital stay. Stay in intensive care (p = 0.006) and hospital (p = 0.007) both increased significantly with decreasing weight. Postoperative categorical data included presence of residual ventricular septal defects, regurgitation across the left atrioventricular valve, and complications. While there was no difference in residual defects (p = 0.166) or valvar regurgitation (p = 0.729), there was a significantly higher presence of complications with decreasing weight (p = 0.0043). There was no mortality, and no persistent heart block requiring placement of a permanent pacemaker.

Our data shows that, with the exception of a slightly longer and more complicated postoperative course, early surgery for symptomatic patients with atrioventricular septal defects and common atrioventricular valvar orifice can be undertaken safely and effectively in smaller children with excellent outcomes.

Children born with heterotaxy syndromes have poorer outcomes compared with children born with comparable cardiac lesions requiring similar surgical palliation. Heterotaxy has been reported as a separate risk factor for mortality and increased morbidity in a series of Fontan operations reported from single centres. Little is known, however, about the functional state of surviving patients with heterotaxy following a Fontan operation.

In the multicentric cross-sectional study carried out by the Pediatric Heart Network of 546 survivors of the Fontan procedure, the patients, aged from 6 to 18 years, underwent evaluation by echocardiography, exercise testing, electrocardiography, magnetic resonance imaging, and functional health status questionnaires compiled by the patients and their parents. Heterotaxy was identified in 42 patients (8%). Medical and patient characteristics were compared between those with heterotaxy and the remaining 504 patients who did not have heterotaxy.

Patients with heterotaxy had their Fontan procedure performed at a later age, with a median of 3.9 years versus 2.8 years (p = 0.001) and had volume-unloading surgery performed later, at a median age of 1.4 versus 0.9 years (p = 0.008). These patients had significantly different ventricular and atrioventricular valvar morphology, as well as a higher incidence of systemic and pulmonary venous abnormalities. They had a higher incidence of prior surgery to the pulmonary veins, at 21 versus 0.4%. The type of Fontan procedure was different, but no difference was detected in length of stay in hospital, or the number of postoperative complications. Sinus rhythm was less common, at 44 versus 71%, (p = 0.002), and history of atrial arrhythmias more common, at 19 versus 8%, (p = 0.018) in those with heterotaxy. Echocardiography revealed a greater degree atrioventricular valvar regurgitation, lower indexed stroke volume, and greater Tei index. Exercise performance, levels of brain natriuretic peptide in the serum, and summary and domain scores from health status questionnaires, were not different from those not having heterotaxy.

The study illustrates a profile of characteristics, medical history, functional health state, and markers of ventricular performance in patients with heterotaxy after the Fontan procedure. Despite obvious anatomic differences, and some differences in echocardiography and heart rhythm, there were no important differences in exercise performance or functional health state between these patients and other survivors of the Fontan procedure.

To analyze symptoms, associated anomalies, diagnostic approach, and surgical procedures in patients with partial fenestrations of the coronary sinus, a rare congenital disorder.

Partial fenestrations of the walls that usually separate the coronary sinus from the left atrium, also known as partial unroofing of the coronary sinus, may result in increased flow of blood to the lungs, cyanosis, or bidirectional shunting. The diagnosis is important, but difficult to confirm.

We reviewed retrospectively the clinical, echocardiographic, operative, and follow-up data on 25 patients with partial fenestration of the coronary sinus. The patients had a median age of 8 years, and underwent cardiovascular surgery at Mayo Clinic between 1958 and 2003.

The initial diagnosis of a fenestration of the coronary sinus was made by the surgeon at repair of other congenital cardiac anomalies, by cardiac catheterization, or by echocardiography. In 14 patients, fenestration was missed during previous cardiovascular surgery. The most common associated cardiac lesions were atrial septal defects within the oval fossa, persistent left or right superior caval veins, and pulmonary or tricuspid atresia. In 7 patients, the symptoms were at least partially attributable to the fenestration, and included dyspnea, cerebral abscess, transient ischaemic attacks, and cyanosis. The fenestration was addressed surgically in 23 patients, and consisted of closure of the mouth of the coronary sinus, creation of an intra-atrial baffle, or direct repair of the fenestration. Death occurred in 1 patient due to complications unrelated to the repair. In the 24 early survivors, who have been followed up for a median of 85 months, 1 patient has died after a third reoperation for complex congenital cardiac disease.

Fenestrations of the coronary sinus are often missed in the preoperative evaluation of congenitally malformed hearts. When associated with right heart hypoplasia, atrial septal defect, or persistent superior caval vein, fenestrations of the coronary sinus should be considered as a possible additional finding. Once the diagnosis has been made, repair is straightforward.

To evaluate the rate of hospitalization for acute respiratory tract infection in children less than 24 months with haemodynamically significant congenital cardiac disease, and to describe associated risk factors, preventive measures, aetiology, and clinical course.

We followed 760 subjects from October 2004 through April 2005 in an epidemiological, multicentric, observational, follow-up, prospective study involving 53 Spanish hospitals.

Of our cohort, 79 patients (10.4%, 95% CI: 8.2%–12.6%) required a total of 105 admissions to hospital related to respiratory infections. The incidence rate was 21.4 new admissions per 1000 patients-months. Significant associated risk factors for hospitalization included, with odds ratios and 95% confidence intervals shown in parentheses: 22q11 deletion (8.2, 2.5–26.3), weight below the 10th centile (5.2, 1.6–17.4), previous respiratory disease (4.5, 2.3–8.6), incomplete immunoprophylaxis against respiratory syncytial virus (2.2, 1.2–3.9), trisomy 21 (2.1, 1.1–4.2), cardiopulmonary bypass (2.0, 1.1–3.4), and siblings aged less than 11 years old (1.7, 1.1–2.9). Bronchiolitis (51.4%), upper respiratory tract infections (25.7%), and pneumonia (20%) were the main diagnoses. An infectious agent was found in 37 cases (35.2%): respiratory syncytial virus in 25, Streptococcus pneumoniae in 5, and Haemophilus influenzae in 4. The odds ratio for hospitalization due to infection by the respiratory syncytial virus increases by 3.05 (95% CI: 2.14 to 4.35) in patients with incomplete prophylaxis. The median length of hospitalization was 7 days. In 18 patients (17.1%), the clinical course of respiratory infection was complicated and 2 died.

Hospital admissions for respiratory infection in young children with haemodynamically significant congenital cardiac disease are mainly associated with non-cardiac conditions, which may be genetic, malnutrition, or respiratory, and to cardiopulmonary bypass. Respiratory syncytial virus was the most commonly identified infectious agent. Incomplete immunoprophylaxis against the virus increased the risk of hospitalization.

Myocardial dysfunction due to coronary arterial lesions is an important complication after Kawasaki syndrome in childhood. Tissue Doppler echocardiography, and strain rate imaging, have shown their value in detecting regional myocardial dysfunction in coronary arterial disease. We aimed to examine the diagnostic value of these methods in patients with coronary arterial lesions after Kawasaki syndrome.

We assessed regional myocardial function in 17 asymptomatic patients with coronary arterial lesions. Follow-up coronary angiographies were available in all cases. Tissue Doppler echocardiography, and strain rate imaging, were performed at rest and during bicycle exercise. Examination included peak systolic and diastolic velocities, peak systolic strain and strain rate. We enrolled 17 age- and gender-matched persons to serve as a control group.

Segmental left ventricular longitudinal function did not significantly differ between the groups with respect to peak systolic velocity, strain, and strain rate. Diastolic abnormalities were identified in segments supplied by coronary arteries with stenotic lesions. Peak diastolic velocity decreased significantly during exercise in those areas, from 77 plus or minus 34 to 59 plus or minus 56 millimetres per second, p smaller than 0.05. Under exercise, a peak diastolic velocity value under 90 millimetres per second enabled us to identify coronary arterial stenosis with a sensitivity of 75 percent and specificity of 64 percent.

After Kawasaki syndrome, diastolic impairment develops in segments supplied by stenotic coronary arteries before systolic dysfunction is detectable. Exercise tissue Doppler echocardiography has the potential to detect these subtle abnormalities, and help monitor progression of the disease.

A problem facing doctors treating adults with congenitally malformed hearts is that a significant number of these patients are lost for follow-up. The purpose of our study is to describe the medical history and clinical findings in a group of such adults that was lost for follow-up.

The Danish press ran a front-page story about adults with congenitally malformed hearts who were lost for follow-up. These patients were strongly advised to contact a center for congenital cardiac disease, and we examined all responding patients within four-weeks.

We carried out a structured interview, a clinical examination, echocardiography, and measured levels of N-terminal pro brain natriuretic peptide.

The number of responders was 147. Based on the diagnosis and the findings, further follow-up was scheduled for 52 (35.4%), either because of significant residual lesions, found in 32, or the risk of late complications, judged to be present in 20. Symptoms were present in 36.5% of patients scheduled for follow-up. The presence of a heart murmur was highly predictive of the need for further follow-up but the sensitivity was too low to recommend the use of auscultation as a screening test.

A large proportion of adults with congenitally malformed hearts who are lost for follow-up require regular assessment according to a modern standard. Symptoms, signs, and measurement of natriuretic peptide cannot replace full cardiological assessment. It is a challenge for centres treating adults with congenital heart disease to find the lost group of patient with significant cardiac malformations.

Our purpose was to evaluate the effect of a treatment over six months with bisoprolol on the surrogate parameters of N-Terminal-pro brain natriuretic peptide, subsequently to be described as brain natriuretic peptide, peak uptake of oxygen, and ventricular function assessed by magnetic resonance imaging in grown ups and adults who had undergone surgical correction of tetralogy of Fallot.

We designed a prospective, randomized, double-blind, placebo controlled trial. We enrolled 33 patients, aged 30.9 plus or minus 9.5 years in either class 1 or 2 of the grading of the New York Heart Association class with both levels of brain natriuretic peptide greater than 100 pg/ml and a reduced peak uptake of oxygen less than 25 ml/kg/min. During treatment with Bisoprolol, the levels of brain natriuretic peptide increased significantly from 206 plus or minus 95 to 341 plus or minus 250 pg/ml (p< 0.05), and those of atrial natriuretic peptide from 4117 plus or minus 1837 to 5340 plus or minus 2102 fmol/ml (p = 0.0005). These measures remained unchanged in the group of patients receiving the placebo. Peak uptake of oxygen did not differ significantly in either group, nor did treatment have any significant effect on right and left ventricular volumes and ejection fractions as determined by magnetic resonance imaging. The clinical state as judged within the grading system of the New York Heart Association was also unchanged by beta-blockade.

Beta blockade with Bisoprolol seems to have no beneficial effect on asymptomatic or mildly symptomatic patients with right ventricular dysfunction secondary to repaired tetralogy of Fallot with residual pulmonary regurgitation and/or stenosis.