Data assessing the effect of altitude on Fontan haemodynamics are limited to experimental models and case reports. Both suggest a detrimental impact. This study describes exercise performance in patients with Fontan circulation and matched controls at a low altitude versus at sea level. We sought to assess the impact of increasing altitude on functional capacity in patients with Fontan palliation.

A retrospective review of 22 patients at low altitude (1602 metres) and 119 patients at sea level with Fontan circulation, as well as age-, gender-, and altitude-matched controls, underwent maximal cardiopulmonary exercise testing. Linear regression models were created to determine the influence of altitude on differences in exercise variables between Fontan patients and their matched controls.

Peak oxygen consumption was 28.4 millilitres per kilogram per minute (72% predicted) for the sea-level cohort and 24.2 millilitres per kilogram per minute (63% predicted) for the moderate altitude cohort. The matched case–control differences for patients at moderate altitude were greater for peak oxygen consumption (−29% against −13%, p = 0.04), anaerobic threshold (−36% against −5%, p = 0.001), and oxygen pulse (−35% against −18%, p = 0.007) when compared with patients living at sea level. When compared to institution-matched controls, the same parameters fell by 3%, 8.9%, and 4.2%, respectively, for each increase of 1000 feet in residential altitude (p = 0.03, p = 0.001, and p = 0.05, respectively).

Patients with Fontan circulation at a higher altitude have impairment in aerobic capacity when compared with patients at sea level. Reduction in exercise capacity is associated with a reduction in stroke volume, likely related to increased pulmonary vascular resistance.

Although most Kawasaki disease with giant coronary aneurysms is asymptomatic, conventional investigations might not identify previous lesions, or all Kawasaki disease with giant aneurysms at risk of future myocardial lesions. We evaluated the long-term histopathology of the myocardium, especially of intramural small vessels in asymptomatic Kawasaki disease with giant aneurysms.

The initial study comprised 16 consecutive Kawasaki patients – male-to-female ratio was 12:4 – aged from 2 to 12 years, and in the subsequent study, the same patients were aged from 4.9 to 16 years. Endomyocardial biopsies were histopathologically evaluated. Microangiopathies, mitochondrial abnormalities, and loss or disarray of myofibrils were compared by electron microscopy.

The incidence of histopathological abnormalities such as degeneration, hypertrophy, and inflammatory cell infiltration was quite high in the initial study, and inflammatory cell infiltration, interstitial fibrosis, and disarray were very noticeable at follow-up biopsies. The area of fibrous tissue was significantly higher in patients administered with intravenous immunoglobulin at follow-up biopsies. Electron microscopy showed microangiopathies including microthrombi within intramural small vessels in some patients at follow-up biopsies. The sites of the coronary aneurysms did not seem to have an impact on the biopsy findings, suggesting that the underlying pathophysiology is related to the original disease process.

Whether the abnormalities were due to direct myocardial injury, chronic ischaemia, repeated small-vessel thrombosis, or other problems associated only with biopsies, is difficult to determine. However, this subgroup had residual abnormal lesions in the myocardium. Follow-up should be more aggressive in this group of patients to identify myocardial damage that could be asymptomatic.

Complications, such as secondary hypertension, probably related to the loss of arterial elasticity, frequently arise in Cushing’s syndrome, and may persist even beyond cure. This study aimed at demonstrating that arterial compliance, evaluated by automated recording of the QKd interval, was lower in children after a successful surgery for Cushing’s syndrome than in a control group of healthy subjects.

In all, 23 young girls aged between 11 and 18 years who had undergone a surgical cure for Cushing syndrome – 18 with a pituitary adenoma, three with a primary adrenal disease, and two suffering from ectopic adrenocorticotrope hormone secretion – were enrolled. Arterial stiffness was measured by the standardised non-invasive QKd 100-60 method. A 24-hour ambulatory blood pressure monitoring and a transthoracic echocardiography were also performed.

The children operated for Cushing’s syndrome showed disadvantageous differences in 24-hour ambulatory blood pressure monitoring and in QKD 100-60 value, with p less than 0.01, compared with the control group.

In spite of its successful surgical cure, Cushing’s syndrome results in a significantly decreased arterial distensibility when compared with the control group, which might explain these differences in blood pressure levels. It underlines a significantly higher cardiovascular risk, notwithstanding both the normalisation of cortisol secretion and the very early age of the patients.

β-blockers improve the prognosis of patients with cardiac failure due to left ventricular systolic dysfunction. The aim of this study was to assess the efficacy of β-blockers in patients with dysfunctional systemic right ventricle.

Fourteen patients with systemic right ventricle following a Mustard or a Senning operation for the transposition of the great arteries, or congenitally corrected transposition were included in the study. All had a decreased systemic right ventricular ejection fraction despite having standard cardiac failure therapy. Quality of life, New York Heart Association class, aerobic capacity, and systemic right ventricular function were assessed before treatment with β-blockers and at the end of the follow-up period, mean of 12.8 months with a range from 3 to 36 months.

Change in New York Heart Association class was significant (p = 0.016). Quality of life improved significantly throughout the study from a median grade 2 with a range from 1 to 3 to a median grade 1 with a range from 1 to 2 (p = 0.008). Systemic right ventricular ejection fraction assessed by radionuclide ventriculography improved significantly from a median of 41% (range: 29–53%) to 49% (range: 29–62%; p = 0.031). However, the change in thee ejection fraction assessed by magnetic resonance imaging was not significant from a median of 29% (range: 12–47%) to 32% (range: 22–63%; p = 0.063).

In patients with cardiac failure due to systemic right ventricular dysfunction, β-blockers improve New York Heart Association class, quality of life, and systemic right ventricular ejection fraction assessed by radionuclide ventriculography.

Functional health is becoming an important part of outcome assessment following congenital heart surgery.

The Child Health Questionnaire was used to evaluate self-reported functional health in a cohort of children operated on for congenital heart disease between 1996 and 2002, now aged 10–20 years. A total of 288 schoolchildren served as controls. The association between demographic and clinical factors such as the Risk Adjusted Classification for Congenital Heart Surgery, the Aristotle Basic Complexity Score, physical and psycho-social domains was explored by multivariate analysis.

In total 239 children who were operated on (response rate 68%, mean age at assessment 13.1 years, 50% male children) participated. There were no differences between children operated on for congenital heart disease and controls in nine out of thirteen domains. In multivariate analysis, male gender was positively associated with physical, mental and general health. Higher education of the parents was also associated with better scores for family activities, physical, emotional and general health. In contrast, living with a single parent was negatively associated with mental health. Category 4 in the Risk Adjusted Classification for Congenital Heart Surgery was associated with worse scores in all behaviour domains. The Aristotle Basic Complexity Score was not associated with any domain.

Functional health in children operated for congenital heart disease was overall similar to other children of the same age. Male gender of the child, education of the parents, living with a single parent, and category 4 in the Risk Adjusted Classification for Congenital Heart Surgery were important factors for functional health.

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.

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.

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.

Improvements in long-term survival of children undergoing the Norwood staged procedure and the arterial switch operation have resulted in the need to prepare these at-risk children for each stage of their developmental trajectory, including school readiness. This study describes and compares functional outcomes following the Norwood staged procedure and arterial switch operations.

This prospective inception cohort study comprised a sample of 73 children (71% boys) who had the Norwood staged procedure (n = 28) or the arterial switch operation (n = 45) at the age of 6 weeks or younger at the Stollery Children’s Hospital, Edmonton, Alberta, between 2002 and 2005. We excluded children who had chromosomal abnormalities or cerebral palsy. When children were 18–24 months of age, parents completed the Adaptive Behavioral Assessment System II. Standard scores for the domains are mean 100, standard deviation (15); skill area scaled scores, 10 (3). Student’s t-test with Bonferonni correction was used to compare groups.

This population has greater than four times the number of children delayed on the General Adaptive Composite than the normative group. Functional outcomes were similar in the two groups other than those of home living (Norwood: 8.8 (2.8) compared with arterial switch: 11.2 (3.1), t = 3.389, p = 0.001) and self-care (Norwood: 5.9 (3.5) versus arterial switch: 8.1 (2.6), t = 3.140, p = 0.002).

These survivors are at increased risk for delayed functional abilities. Self-care, necessary for independence and confidence as children reach school age, was particularly low in the Norwood group. Reasons for low self-care abilities require further study.

To determine the outcome of dilated cardiomyopathy presenting in childhood and the features that might be useful for prognostic stratification.

Retrospective study of 41 consecutive children affected by dilated cardiomyopathy – aged 0–14 years; median 33.4 plus or minus 49.25 – between 1993 and 2008. We reviewed the medical history to determine age at diagnosis, family history, previous viral illness, aetiology, symptoms and signs at presentation, treatment, and outcome. The diagnosis was made on the basis of cardiomegaly and evidence of poor left ventricular function by echocardiography. We also carried out a metabolic evaluation including blood lactate, pyruvate, carnitine, amino acids, urine organic acids, assessment of respiratory chain enzymes, and analysis of histopathological material. Survival curves were constructed by the Kaplan–Meier method.

Follow-up ranged from 10 days to 162 months – median 45.25 plus or minus 41.15 months. Freedom from death or cardiac transplantation was 68.3% at 5 years. The primary end-point of death/cardiac transplantation was associated with the need for intravenous inotropic support. A trend towards a poorer prognosis was found for age at diagnosis of more than 5 years and for a metabolic aetiology of dilated cardiomyopathy. For the children affected by cardiomyopathy as part of a multi-system involvement, mortality was 50%.

In children, dilated cardiomyopathy is a diverse disorder with outcomes that depend on cause, age, and cardiac failure status at presentation. Overt cardiac failure at presentation is a major prognostic factor for death or cardiac transplantation. Older age at presentation and metabolic aetiology may be associated with a poorer prognosis.

The goal of this study was to see whether the open anastomosis technique using vacuum-assisted venous drainage at the time of the Fontan procedure was associated with decreased post-operative pleural effusion.

We analysed a subgroup of patients with a functional single ventricle who underwent non-fenestrated total cavopulmonary connection completion with the insertion of an extracardiac conduit as the sole or predominant procedure conducted by a single surgeon at a single institute, using either an open or closed anastomosis technique.

Median age and weight were 2.3 years, with a range from 1.3 to 27.6 years and 11.4 kilograms, with a range from 9.7 to 43 kilograms, respectively. The open anastomosis technique was associated with a shorter bypass run (p = 0.015), decreased surgical duration (p = 0.032), fewer pleural effusion days (p = 0.049), and lesser pleural effusion (p = 0.013) than closed anastomosis. Correlation analysis demonstrated a significant relationship between the amount of pleural effusion and surgical duration (correlation efficient, 0.535; p = 0.033). A logistic regression model showed that the open technique was associated with a 20-fold increase in the likelihood of having a total chest tube discharge of less than 300 millilitres (p = 0.027).

The open anastomosis technique shortens operative duration and bypass run, which in turn might contribute to decreased pleural effusion soon after the modified Fontan procedure.