To investigate whether accurate estimation of the diameter of the patent ductus arteriosus can be obtained by colour Doppler echocardiography.

The minimum and maximum diameters of the patent ductus arteriosus were measured by colour Doppler echocardiography and compared with its angiographic size.

We studied 40 patients, aged between 4 months and 18 years. The measured pulmonary side diameter in echocardiography was 33.6% larger than that in angiography, and the aortic side was 7.8% smaller. We found a correlation between the measurements of the minimum and maximum size of the patent ductus arteriosus (r = 48.8 and 52.6) by colour Doppler echocardiography and angiographic findings, respectively.

Colour Doppler echocardiography significantly overestimates the minimum size of the patent ductus arteriosus; therefore, reconsideration of the respective size is suggested.

To examine early outcomes for pulmonary atresia with intact ventricular septum undergoing single-ventricle palliation and to determine risk factors for mortality.

Retrospective observational study.

Tertiary paediatric critical care unit.

Risk factors for mortality were sought for infants after the primary intervention whether surgical shunt or ductal stent.

We reviewed outcomes of 19 infants with pulmonary atresia with intact ventricular septum undergoing single-ventricle palliation between July, 2000 and July, 2008. Echocardiograms, cardiac catheterisation findings, anaesthesia, and critical care management, as well as autopsy reports were reviewed. We modelled survival after surgery and looked for predictors of early mortality. A total of 19 infants underwent single-ventricle palliation and seven of these died. The risk of death was increased by a lower arterial pH at induction of anaesthesia (p = 0.01), a lower systolic blood pressure (p = 0.01), and technical problems during surgery (p = 0.03). On admission to the critical care unit, a lower mixed venous saturation (p = 0.02) and presence of tachyarrhythmia (p = 0.02) were associated with the need for mechanical support within the first 48 hours.

There is a high early mortality for those who undergo single-ventricle palliation. It is higher for those who are haemodynamically compromised before surgery; technical problems, and haemodynamic instability during surgery also increase mortality.

We aimed to assess the ability of the multiplanar review modality of three-dimensional echocardiography to examine the dynamic morphology and the functional characteristics of malformed tricuspid valves in patients previously identified as having Ebstein’s malformation. Based on these characteristics, we attempted to differentiate Ebstein’s malformation from tricuspid valvar dysplasia.

Using three-dimensional multiplanar review, analysed with either Qlab 6.0 or Tomtech Image Arena 3.0, we studied 23 patients, aged from 1 day to 70 years, previously diagnosed using cross-sectional echocardiography as having Ebstein’s malformation.

Using the features of rotational abnormality, and the orientation, of the effective tricuspid valvar orifice as diagnostic features of Ebstein’s malformation, we reclassified 11 patients (48 per cent) as exhibiting tricuspid valvar dysplasia. In addition, we studied the dynamic morphology as well as the function of the tricuspid valve. Surgical treatment was undertaken on 10 patients, revealing good correlation with the findings obtained using three-dimensional multiplanar review. In those with Ebstein’s malformation, we found varying degrees of rotation, with the effective valvar orifice always directed towards the right ventricular outflow tract. The opening of the orifice of dysplastic tricuspid valves, in contrast, was towards the apex of the right ventricle. The degree of delamination, and abnormalities of subcordal apparatus, were similar in the two groups.

Three-dimensional multiplanar review permits accurate definition of the dynamic morphology of Ebstein’s malformation, permitting clear differentiation from tricuspid valvar dysplasia.

Acute rheumatic carditis is still an important cause of cardiac failure in developing countries. B-type natriuretic peptides, especially N-terminal segment of its prohormone are now recognised as essential parts of cardiologic evaluation. Increased plasma concentrations of B-type natriuretic peptide and its prohormone are markers of cardiac failure and hypoxia in adults.

To measure the prohormone levels in children with acute rheumatic carditis and to determine whether its concentrations correlate with clinical and laboratory findings.

A total of 24 children with acute rheumatic carditis and 23 age and sex-matched healthy subjects were entered in the study. Transthoracic echocardiography was performed in all patients to assess the severity of the valve insufficiency and cardiac dysfunction. The prohormone plasma levels were tested for correlation with cardiac dysfunction and other biochemical markers, such as C-reactive protein, erythrocyte sedimentation rate, and anti-streptolysin-O titter.

The prohormone plasma concentrations were significantly higher in children with acute rheumatic carditis than in control subjects at the time of diagnosis. A significant decrease of the plasma level was detected among patients after treatments (6–8 weeks).

We found increased plasma prohormone levels in children with acute rheumatic carditis in the acute stage of illness compared with healthy subjects. Another result is increased plasma prohormone levels as acute rheumatic carditis are reversible.

The main cause of long-term morbidity and mortality after the arterial switch operation for transposition of great arteries is complication at the coronary arteries. Myocardial perfusion magnetic resonance imaging represents a relatively novel and appealing tool for detecting myocardial ischaemia but with little experience in paediatric patients. The purpose of this paper is to report a single centre experience with myocardial perfusion magnetic resonance imaging for detecting ischaemia after the arterial switch operation for transposition of great arteries.

Twenty-eight patients aged 13–16 years with an arterial switch operation for transposition of great arteries were included in the study. Coronary pattern, operative and postoperative complications, and long-term follow-up events were reviewed. Patient functional evaluation included clinical examination, electrocardiogram and echocardiogram. Every patient underwent magnetic resonance imaging perfusion scanning at rest and under adenosine-induced stress.

All patients were symptom free with no ischaemic signs on the electrocardiogram. All magnetic resonance imaging examinations were generally well tolerated with minor adenosine secondary effects in 36% of the patients. Two stress myocardial perfusion magnetic resonance studies were excluded from analysis for technical reasons. No perfusion stress defects were detected at the remaining 26. Myocardial delayed enhancement was performed in all 28 patients. In five subjects, a subendocardial late enhancement consistent with patch tissue for septal defect closure at the time of repair was indentified.

Magnetic resonance imaging evaluation of myocardial perfusion and viability is feasible in paediatric patients long after arterial switch operation. No signs of myocardial ischaemia or necrosis were documented in this young asymptomatic population. Further studies including coronary angiography correlation are needed to validate magnetic resonance imaging results.

Mitral valvar prolapse is the most common anomaly of the mitral valve apparatus throughout childhood. Fibrillin is one of the structural components of the elastin-associated microfibrils found in the mitral valve. A case-controlled study has performed to investigate the relationship between fibrillin 1 gene intron 56 polymorphism and risk of mitral valvar prolapse in Turkish children.

A total of 77 patients with mitral valvar prolapse diagnosed by clinical evaluation and echocardiography and 89 normal children of same age and sex were studied. The fibrillin-1 gene intron 56 polymorphism was identified by the polymerase chain reaction-based restriction analysis.

There was a significant difference in the distribution of fibrillin-1 gene intron 56 genotypes (p = 0.0001) and allelic frequency (p = 0.0001) between the cases and the controls.

Patients with mitral valvar prolapse have higher frequencies of fibrillin-1 gene intron 56 GC genotypes. Healthy children have higher frequencies of fibrillin-1 gene intron 56 CC genotypes. We speculate that the higher frequency of fibrillin-1 gene intron 56 G-allele increases the risk of mitral valvar prolapse.

Many studies have reported on the estimation of the development of left ventricular dimensions in children by using two-dimensional echocardiography and formulated equations to evaluate normal cardiac dimensions. We reported that height is the most simple and useful index to evaluate the normal dimensions of the left ventricle in children. Disparities have been detected between the “normal” dimensions estimated from these formulae and real dimensions in infants less than 1 year of age and neonates, by using body weight or body surface area as an index. Our objective is to assess the normal size of left ventricular end-diastolic dimension in infants less than 1 year of age and neonates, including premature neonates, by two-dimensional echocardiography by using height as an index.

The study group consisted of 243 infants, 123 males, and 120 females without congenital cardiac disease. The regression equation for the relationship between left ventricular end-diastolic dimension (Y) and height was Y (millimetre) = 0.352 × height (centimetre) + 1.86 in mature neonates and infants with height of less than 75 centimetres, whereas that in premature neonates was Y (millimetre) = 0.495 × height (centimetre) − 5.43. No significant differences were observed in the infants on the basis of sex.

The regression equations reported in the previous studies cannot be applied to data obtained from infants. In addition, three different formulae, one for each group of infants by using height as an index can be usefully applied for practical purposes.

Anomalous origin of one pulmonary artery from the ascending aorta is a rare cardiac anomaly in which the pulmonary artery abnormally arises from the ascending aorta. Physiologically, most patients develop signs of cardiac failure due to high flow to both lungs, with systemic or supra-systemic pressures in the normally connected lung. The purpose of this study is to present our experience with this rare anomaly, in which early anatomic repair lead to rapid physiologic correction.

Retrospective case review of all patients with anomalous origin of one pulmonary artery from the ascending aorta at Schneider Chidren’s Medical center of Israel between 1986 and 2007. All clinical operative and echocardiographic charts were analysed.

Twelve patients were diagnosed as anomalous origin of one pulmonary artery from the ascending aorta. In 10 patients, the right pulmonary artery rose from the ascending aorta, while in two an anomalous origin of the left pulmonary artery was associated with a right aortic arch. Initial diagnoses was made with two-dimensional echocardiography in all patients. In six patients, diagnostic cardiac catheterisation was performed in order to confirm the diagnosis. Age at diagnosis ranged from 5 to 180 days with a median of 15 days, and patient weight ranged from 780 grams to 5 kilograms, with a median of 3 kilograms. Initial echocardiographic evaluation showed systemic (four patients) or supra-systemic (seven patients) pressures in the right ventricle and normally connected lung. All underwent surgical repair. There was no operatrive mortality. All reconstructed patients achieved normal right ventricular pressures within days after surgery. The flow pattern in both pulmonary arteries was normalised.

Early surgical repair of anomalous origin of one pulmonary artery from the ascending aorta is feasible and safe even in newborn and premature babies with complete resolution of the pulmonary hypertension and normalisation of pulmonary vascular resistance.

Neonates and infants with congenital and acquired cardiac disease often require placement of central venous lines for extended intravenous therapy. It may be advantageous to avoid the larger venous vessels of the head and neck and lower extremities in order to preserve these for future interventions and therapies. We evaluated the results of a nursing led peripherally inserted central catheter team in our congenital cardiac centre.

Bedside peripherally inserted central catheter the insertion procedures were evaluated for success, complications, and completion of therapy.

A total of 125 peripherally inserted central catheters were successfully placed in 105 patients. The mean age at the time of placement was 13.5 plus or minus 19.1 days; median age was 7 days; mean weight was 3.5 plus or minus 1.1 kilogram. Cyanotic cardiac disease accounted for 76% of the diagnoses. Central placement of these lines was successful in 78% of patients. Complications during insertions were limited to inadvertent arterial access in five (3%) infants and oxygen desaturations during sedation for the procedure in two (1%) patients. None of the infants suffered long-term compromise from arterial access; none required intubation for the desaturations. The team was able to respond to 90% of their requests within 24 hours. Median catheter dwell time was 14 days.

In a population of infants with cardiac disease, a nursing staffed peripherally inserted central catheter team achieved an insertion rate of 78% with few complications and a rapid response time. Reliance on bedside insertion permits continuous critical care monitoring and may eliminate the need for fluoroscopy.

To assess the general health and activity levels of 4- and 5-year-old children after intervention for congenital cardiac disease.

Health behaviour outcomes were assessed in 91 children who had surgery or catheter intervention for congenital cardiac disease. The children were classified into four groups according to severity. The main parameters of classification were the presence of residual symptoms, frequency of visits to general practitioner or the Accident and Emergency Department, and ability to participate in physical activity according to a calculated “activity score”.

Children had very few residual symptoms after “corrective surgery”. Those with complex congenital cardiac disease post-Fontan-type repair still had symptoms on average 18.2 days per month. Surprisingly, the complex group had fewer days “sick” from non-cardiac causes and had fewer visits to general practitioner or Accident and Emergency Departments. Regression analysis indicates that three variables had significant relevance to the general practitioner or Accident and Emergency visits: complex congenital cardiac disease, fewer visits; Townsend score – more deprivation – more visits; and maternal worry – higher maternal worry score – more visits. Regression analysis indicates that lower activity score is significantly related to complex cardiac disease and higher maternal worry score.

The majority of this group of 4- and 5-year-old children had few residual symptoms and had good exercise tolerance. Maternal worry is a significant factor in influencing both activity levels and frequency of unscheduled health service demands – general practitioner or Accident and Emergency visits.

Heterotaxy syndrome, which is often associated with atrioventricular valvar regurgitation, has been considered a risk factor for the surgical repair for Fontan candidates. The results of atrioventricular valve repair in this challenging patient population remain largely unknown.

From July, 1994 to January, 2007, 25 patients with the heterotaxy syndrome consisting of 22 right isomerism and three left isomerism presented to the Shizuoka Children’s Hospital Japan with significant (3–4+) atrioventricular valvar regurgitation necessitating atrioventricular valve repair. The clinical and Doppler/echocardiographic data were retrospectively reviewed to determine the efficacy of the repair and patient outcome.

Patients were divided into two groups on the basis of atrioventricular valvar regurgitation at the most recent follow-up: those with a successful late outcome, (0–2) and those with a poor outcome (3–4). There were 17 (67%) patients with a successful outcome and 8 (33%) with a poor outcome. The repair technique including leaflet apposition was predictive of a successful outcome (p = 0.003). Overall survival was 64% (16/25). Survival was 88.2% (15/17) for patients with a successful result versus 12.5% (1/8) for those with a poor outcome (p = 0.0007). Of the 15 survivors, 13 have reached final completion of the Fontan circulation, and two currently remain at the bi-directional Glenn shunt stage.

Atrioventricular valve repair can be accomplished in this challenging patient population with excellent results. The combination of the leaflet apposition technique and the Kay suture can be performed with an excellent outcome in the majority of patients with heterotaxy syndrome, even with significant atrioventricular valvar regurgitation.

Parents of children with congenital cardiac disease suffer from psychological stress and financial burdens. These costs have not yet been quantified.

In cooperation with paediatricians, social workers, and parents, a questionnaire was devised to calculate direct non-medical and indirect costs. Direct non-medical costs include all costs not directly related to medical services such as transportation. Indirect costs include lost productivity measured in lost income from wages. Parents were retrospectively queried on costs and refunds incurred during the child’s first and sixth year of life. The questionnaire was sent out to 198 families with children born between 1980 and 2000. Costs were adjusted for inflation to the year 2006. Children were stratified into five groups according to the severity of their current health status.

Fifty-four families responded and could be included into the analysis (27.7%). Depending on severity, total direct non-medical and indirect costs in the first year of life ranged between an average of €1654 in children with no or mild (remaining) cardiac defects and an average €2881 in children with clinically significant (residual/remaining) findings. Mean expenses in the sixth year of life were as low as €562 (no or mild (remaining) cardiac defects) and as high as €5213 (potentially life-threatening findings). At both points in time, the highest costs were lost income and transportation; and day care/ babysitting for siblings was third.

Families of children with congenital cardiac disease and major sequelae face direct non-medical and indirect costs adding up to €3000 per year on average. We should consider compensating families from low socioeconomic backgrounds to minimise under-use of non-medical services of assistance for their children.

To examine the overall morbidity of patients who underwent surgery for congenital cardiac defect during childhood.

A congenital cardiac defect treated with surgery is seldom totally cured. The incidence of residua, sequelae, and comorbidity is quite high. The morbidity has not been thoroughly examined.

Medication was used as an indicator of morbidity. Data from the Finnish Research Registry of Paediatric Cardiac Surgery were linked to data from the medication registry of Finland’s Social Insurance Institution. This study includes 5116 patients with a mean age of 33.5 (ranged from 14.7 to 64.8) years, who had undergone surgery for congenital cardiac defect between 1953 and 1989. The use of medicines among patients in 2004 was compared with 10232 age- and sex-matched control subjects.

The overall use of medicines was frequent; 62% of patients and 53% of controls had purchased at least one prescribed medicine (risk ratio: 1.2, 95% confidence interval: 1.1–1.2). The number of patients using cardiovascular medicines (17%) and anti-thrombotic agents (5%) was higher than that of control subjects (risk ratio: 2.2 and 8.4). In addition, the patients needed medicinal care for epilepsy (3%), asthma (7%), and psychiatric diseases (10%) more often than did controls (risk ratio: 2.2, 1.5, and 1.3, respectively).

Patients operated on for congenital cardiac defect had more chronic diseases and used more medicines than did controls.