Providing appropriate care for adults with congenital heart disease requires the evaluation of their current situation. There is limited research in Iran about these patients, particularly in relation to gender differences in the demographic/socio-economic and lifestyle factors, as well as disease parameters.

The sample consisted of 347 congenital heart disease patients in the age group of 18–64 years, including 181 women, assessed by an analytical cross-sectional study. The patients were recruited from the two major heart hospitals in Tehran. Data were collected using questionnaires.

The mean age of the patients was 33.24 years. Women were more often married and more often had offspring than men (p < 0.001). Educational level and annual income were similar between women and men. Unemployment was higher among women (p < 0.001), but financial strain was higher among men (p < 0.001). Smoking, alcohol, and water-pipe use was higher among men than among women (p < 0.001). Cardiac factors, for example number of cardiac defects, were similar among women and men, except that there were more hospitalisations owing to cardiac problems, for example arrhythmia, among men. Disease was diagnosed mostly at the hospital (57.4%). Most medical care was provided by cardiologists (65.1%). Only 50.1% of patients had knowledge about their type of cardiac defect.

Gender differences exist in the socio-economic and lifestyle characteristics of adults with congenital heart disease, in some cases related to the disease severity. Our findings also point to the need for interventions to increase patients’ knowledge about, and use of, healthier lifestyle behaviours, irrespective of gender. Furthermore, providing appropriate jobs, vocational training, and career counselling may help patients to be more productive.

To assess behavioural problems in adolescents with congenital and acquired heart disease in comparison with healthy controls. The perception of behavioural problems by the patients’ parents was also assessed and compared.

A cross-sectional study was carried out in 130 adolescents with congenital and acquired heart disease and 246 healthy controls. The second part of the Youth Self-Report was applied to the patients and controls, and the Child Behavior Checklist to the patients’ parents.

Male patients showed significantly fewer behavioural problems compared with male controls. No significant difference was found in the female gender. Healthy male adolescents scored significantly higher in the Internalising, Externalising, and in the Total Problems scales. Patients scored significantly higher only on the Social Problems subscale. Female patients in middle and late adolescence and male patients in early adolescence displayed more problems. No significant difference was found between the diagnostic groups. Operated patients did not differ from the non-operated ones. Patients scored significantly lower than did their parents.

Male adolescents with cardiac disease reported fewer behavioural problems when compared with healthy controls, but no difference was observed in the female gender. Patients also reported fewer behavioural problems than did their parents. Adolescents with cardiac disease scored higher than did controls only on the Social Problems subscale. Analysing the patients’ behavioural profile, female patients in middle and late adolescence and male patients in early adolescence were the most problematic ones. No difference was observed between the diagnostic groups, nor between operated and non-operated patients.

Women with congenital heart disease are often considered to be restricted in their obstetrical life and even their marital life. Our single-centre study aimed to determine the real-life situation of these women with regard to successful family life and any pregnancy complications they may experience.

From our database of adults with congenital heart disease, 160 of 178 women completed a questionnaire and had their files reviewed. They were classified into three groups according to their pregnancy risk – “good condition” group, no pregnancy restriction; “at-risk” group, pregnancy allowed with close follow-up at a tertiary centre; and “contraindicated” group, pregnancy inadvisable.

The proportion of women in a relationship was 46% with no difference between the three groups. In the groups where pregnancy was allowed, 55% of women conceived a child. The total incidence of spontaneous abortion was 21%. The rate of caesarean section was 15%. The incidence of cardiac failure was 4.7%, arrhythmia 1.2%, endocarditis 1.2%, hypertension 2.4%, and preeclampsia 1.2%. Foetal complications included prematurity and/or low birth weight (9.5%) and one foetal malformation (0.82%).

Women with severe congenital heart disease are willing to start a family and are successful in this enterprise. Although the complication rate during pregnancy in congenital heart disease remains high, with good monitoring these pregnancies occur without severe complications and a low rate of medical abortion or caesarean section.

It is unclear how autografts grow and dilate after the Ross operation in children. We analysed autograft growth and dilatation in children who underwent the Ross operation and examined the relationship of these factors to autograft failure.

From our institutional database, we retrospectively identified 33 children who underwent the Ross operation without aortic root reinforcement (mean age 9.9 years) and had normal body measurements and echocardiographic data throughout follow-up.

Autograft insufficiency developed in 10 patients 5.1 years after the Ross operation. The average Z score at the development of autograft insufficiency was −0.1 (range from −2.0 to 6.1). The proportions of patients who remained free of autograft insufficiency at 5 and 10 years were 87.2% and 55.7%, respectively. A consistent trend in the time course of Z score was not found in any age group studied.

Autograft growth and dilation after the Ross operation varied widely among patients, and the incidence of autograft insufficiency was independent of annulus size.

To study coronary artery haemodynamics in adolescents with homozygous familial hypercholesterolaemia and aortic supravalvular stenosis.

Patients diagnosed with familial hypercholesterolaemia who were younger than 16 years and who had undergone transthoracic echocardiography from 2007 to 2010 were included in this study. We included patients with homozygous familial hypercholesterolaemia and aortic supravalvular stenosis and those with heterozygous familial hypercholesterolaemia. All patients underwent stress echocardiography, and left anterior descending coronary artery flow was successfully detected. Coronary flow velocity reserve was calculated as the ratio of hyperaemic mean diastolic flow velocity after injection of adenosine to basal mean diastolic flow velocity. Changes in coronary haemodynamics and the relationship between lipid concentrations were determined.

A total of 11 patients with homozygous familial hypercholesterolaemia were enrolled in this study. Lipid concentrations were measured, and the mean coronary flow velocity reserve was 1.97 plus or minus 0.51. Seven children were included in the group of patients with heterozygous familial hypercholesterolaemia. In these children, the mean coronary flow velocity reserve was 3.08 plus or minus 0.84.

The coronary flow velocity reserve of homozygous familial hypercholesterolaemic patients is lower than that of heterozygous familial hypercholesterolaemic patients, and it is associated with a high concentration of low-density lipoprotein cholesterol. Aortic stenosis and plaques compromised the ostia of the coronary artery and caused increased basal mean diastolic coronary velocity with blunted increase in peak velocity, which decreased the coronary flow velocity reserve.

The objective was to report two new patients with the diagnosis of alveolar capillary dysplasia and congenital heart disease, to describe the associated cardiac defects seen in these cases and in the literature, and to consider recent genetic advances concerning the FOX transcription factor gene cluster in chromosome 16q24.1q24.2.

We retrospectively analysed the records of all patients with congenital heart disease and alveolar capillary dysplasia seen in the Pediatric Cardiology Department between 2005 and 2010. We reviewed all literature published in the English language relating to cases of alveolar capillary dysplasia and congenital heart disease.

Two infants with alveolar capillary dysplasia and cardiac malformation were identified: one had an atrioventricular septal defect and a de novo balanced reciprocal translocation t(1;16)(q32;q24), the second infant had a ventricular septal defect. Analysis of 31 cases of the literature including these new cases showed a predominant association of alveolar capillary dysplasia with obstructive left heart disease (35%), as well as an atrioventricular septal defect (29%). FOX gene cluster defects were identified in eight of these patients.

Genetic background of alveolar capillary dysplasia is discussed in the light of the balanced reciprocal translocation t(1;16)(q32;q24) identified in the first child of this report. Alveolar capillary dysplasia should be suspected in neonates with congenital heart disease and unexpectedly elevated pulmonary vascular resistances, especially in cases of obstructive left heart disease or atrioventricular septal defect. Detecting FOX gene cluster defects should be considered in infants with alveolar capillary dysplasia with or without congenital heart disease.

To evaluate exercise performance and body mass index in healthy children over an 8-year period in order to determine whether cardiorespiratory fitness has decreased relative to changes in body mass index in our patient population.

This study is a retrospective review of anthropometric and cardiorespiratory fitness data from our hospital's Exercise Physiology Laboratory's database on 1201 healthy children (6–18 years) with structurally normal hearts who performed one cycle ergometry test between 1999 and 2006. Subjects were stratified by gender and age. Body mass index was compared between 1999–2002 (Early Group) and 2003–2006 (Late Group) and with national averages. Exercise outcome variables were maximal oxygen consumption, anaerobic threshold, physical working capacity indexed to body weight. Analysis of variance was used to detect differences between groups for each outcome variable.

Body mass index was not significantly different from national data (p-value equals 0.79) or between groups. A negative trend (p-value greater than 0.15 less than 0.20) was observed among most indices of exercise performance between Early and Late Groups. Significantly lower anaerobic threshold was found among males between Early and Late Group (25.7 plus or minus 0.9 versus 23.4 plus or minus 1.6 millilitres per kilogram per minute, p-value less than 0.01), and in physical working capacity among females between Early and Late Groups (2.9 plus or minus 0.1 versus 2.7 plus or minus 0.1, p-value less than 0.05).

Cardiorespiratory fitness in males and working capacity in females declined over time independent of body mass index. This suggests that other measures may need to be evaluated for promotion of overall cardiovascular health.

The aims of this case–control study were to (a) compare the caries experience and oral hygiene, and (b) quantify the persistence of a delay in the dental age in children with cardiac disease and a group of healthy children.

The study population comprised a group of 268 3- to 16-year-old children and adolescents with a cardiac disease and a group of 268 age- and sex-matched healthy children and adolescents. Specifically, the decayed, missed, and filled teeth indices, simplified oral hygiene index, and the dental ages of the two groups of children were calculated and then compared.

Although the oral health of the children with either a congenital or an acquired heart disease was the same as that of the healthy children, there were significant differences in the decayed, missed, and filled teeth indices. Dental ages of the children with a congenital heart disease were significantly lower than those of healthy children. The findings showed that complex univentricular heart diseases had the highest negative impact on dental development (−1.1), followed by complex biventricular (−0.9), simple surgical (−0.5), and mild (−0.4) heart disease patients.

Once thorough knowledge of the child's cardiac status is gained, a definitive dental treatment plan for the child with a cardiac disease can be established.

This study was designed to investigate the value of history taking in identifying children with cardiac syncope, and to improve diagnostic efficiency and accuracy in children with cardiac syncope.

We compared the characteristics of a group of children and adolescents with cardiac syncope at the Pediatric Syncope Unit of five hospitals in China with those with typical vasovagal syncope. We included a cohort of 275 patients in Pediatric Syncope Unit. A cardiac cause of syncope was established in 31 patients, autonomic-mediated reflex syncope in 214, non-syncopal attacks in 15, and in the remaining 15 the cause of syncope remained unexplained. Cardiac syncope was triggered by exercise, whereas vasovagal syncope by prolonged standing, warm-crowded place, and fear or pain emotion. Syncopal spells occurred at various positions in cardiac syncope. Children who had prodromal symptoms with cardiac syncope were significantly fewer than those with vasovagal syncope. Most children with cardiac syncope had history of abnormal electrocardiogram findings when compared with children suffering from vasovagal syncope. On multivariable analysis, history of abnormal electrocardiogram findings and exercise-triggered syncope were independent predictors of cardiac syncope.

Children and adolescents with a history of abnormal electrocardiogram findings and exercise-related syncope spells were at high risk for cardiac syncope.

To describe our experience in the management of coronary artery anomalies both in an adult and in a paediatric population and to compare the two groups for finding out differences in terms of angiographic incidence and treatment.

Databases at the Department of Cardiology of San Martino Adult's Hospital and of Gaslini Children's Hospital were searched for all patients with a diagnosis of coronary artery anomaly who underwent coronary angiography between 1994 and 2006.

Coronary anomalies were diagnosed in 76 (1%) adult patients. Anomalous left circumflex artery was the commonest coronary anomaly (25%). Anomalous left coronary artery from pulmonary artery and myocardial bridges were the only anomalies responsible for angina-like symptoms. No patients except the one with anomalous left coronary artery from pulmonary artery needed surgical intervention. In the paediatric population, we found 28 (0.9%) patients with coronary anomalies. Anomalous left coronary artery from pulmonary artery was the most common anomaly (48%) and always required emergency surgical treatment; in addition there were two patients with stenosis of the left main coronary artery.

Coronary artery anomalies may be associated with very acute, even life-threatening symptoms in children, whereas they are usually clinically silent and detected by accident on coronary angiography in adults. Recognition of coronary artery anomalies enables early treatment or close follow-up in children, whereas it could be useful in case of cardiac surgery in adults.

Patient satisfaction with care has received little attention within the field of congenital heart disease. Our objective was to examine patient satisfaction with the care received when undergoing open-heart surgery in order to identify the best and worst aspects of peri-operative care. Moreover, we examined whether having contact with a specialised nurse in addition to usual care is associated with higher patient satisfaction levels.

Patient satisfaction was measured by the Satisfaction with Hospital Care Questionnaire, evaluating nine aspects of care by answering individual items and giving overall grades. A top 10 of the best and worst items was selected. Linear regression analyses were used to examine the relationship between having contact with a specialised nurse and patient satisfaction (9 grades), independent of patient characteristics – sex, age, educational level, and health status.

Data were available for 75 patients. Grades ranged from 6.74 for “discharge and after care” to 8.18 for “medical care”. In all, 21% of patients were dissatisfied with the clarity of the information about lifestyle adjustments given by the surgeon. However, patients who had contact with a specialised nurse were more satisfied with the provided information (B-coefficient is 0.497, p-value is 0.038), independent of patient characteristics.

Patients were satisfied with the received care, although there is room for improvement, especially in discharge and after care and the clarity of the information provided by the surgeon. This gap in care can be compensated for by specialised nurses, as patients who were counselled by a specialised nurse were more satisfied with the provided information.

Shunt thrombosis after implantation of systemic-to-pulmonary shunts in paediatric patients is common. Acetyl salicylic acid is used for anti-thrombotic treatment; however, the effect is rarely monitored, although it is known that the response varies. The aim was to determine the effects of acetyl salicylic acid medication on platelet aggregation in children with systemic-to-pulmonary shunts.

A total of 14 children – median age 12 days; ranging from 3 to 100 days – were included in a prospective observational longitudinal study. All children were treated with oral acetyl salicylic acid (3–5 milligrams per kilogram once daily) after shunt implantation. Acetyl salicylic acid-dependent platelet aggregation in whole blood was analysed with impedance aggregometry (Multiplate®) after addition of arachidonic acid. Analyses were carried out before the primary operation, before and 5 and 24 hours after the first acetyl salicylic acid dose, and after 3–6 months of treatment. The therapeutic range for acetyl salicylic acid was defined as a test result less than 60 units.

Acetyl salicylic acid reduced the arachidonic acid-induced platelet aggregation in all but one patient. Of the patients, 93% were in the therapeutic range 5 hours after acetyl salicylic acid intake, 86% were in the range after 24 hours, and 64% after 3–6 months.

Acetyl salicylic acid reduces platelet aggregation after shunt implantation in paediatric patients, but a considerable percentage of the children are outside the therapeutic range. Monitoring of platelet aggregation has the potential to improve anti-platelet treatment after shunt implantation by identifying children with impaired acetyl salicylic acid response.

To describe the haemodynamic response of children who receive arginine vasopressin for haemodynamic instability after cardiac surgery and to identify clinical variables associated with a favourable response.

We reviewed patients less than or equal to 6 years undergoing open heart surgery in our institution between January, 2009 and July, 2010 who received arginine vasopressin during the first 7 days post operation. Favourable responders were defined as those in whom blood pressure was increased or maintained and catecholamine score was decreased, or blood pressure was increased by greater than or equal to 10% of baseline and catecholamine score was unchanged at 6 hours following arginine vasopressin initiation.

Of the 34 patients identified, 17 (50%) patients responded favourably to arginine vasopressin. At 6 hours, the mean blood pressure was increased by 32.2% in responders as compared with 4.6% in non-responders, with a p-value less than 0.001. The mean catecholamine score decreased by 30.1% in responders and increased by 7.6% in non-responders, with a p-value less than 0.001. Anthropometric, demographic, and intra-operative variables were similar in both groups, as was maximum dose of arginine vasopressin. The median time after arrival to the intensive care unit at which arginine vasopressin was initiated, however, was later in those who responded, 20 hours as compared with those who did not, 6 hours, with a p-value equal to 0.032.

Arginine vasopressin therapy led to haemodynamic improvement in only half of the children in this study, and improvement was more likely to occur if arginine vasopressin was initiated after the post-operative night.