Serial cardiorespiratory exercise testing in patients with congenital heart disease.

Aerobic capacity of patients with different forms of congenital heart disease was serially evaluated in 79 patients and the evolution was correlated with the lesion and the level of daily activity. The patients were divided into six groups: patients with a small ventricular septal defect (VSD) with mini shunt (n = 14), mild pulmonary valve stenosis with gradient < 40 mm Hg (PS) (n = 12), mild to moderate aortic valve stenosis (gradient 36 +/- 17 mmHg) (AS) (n = 12), patients 4.7 +/- 2.

Reliability of subjective estimates of exercise capacity after total repair of tetralogy of Fallot.

Sixty-nine patients (age 10 +/- 3.5 years, median 9.7 years) operated on for tetralogy of Fallot, underwent exercise testing 5.

Cardiorespiratory exercise performance after Senning operation for transposition of the great arteries.

Objective: To assess the cardiorespiratory response to graded exercise in patients after the Senning operation for transposition of the great arteries.

Design: Cross sectional study. Exercise performance was assessed by determination of the ventilatory anaerobic threshold.

Apparent Greig cephalopolysyndactyly and sinus node disease.

We present the clinical findings and follow-up data from birth to 10.5 years in a boy with Greig cephalopolysyndactyly who, in addition, presents sinus node disease ("sick sinus syndrome"). The significance of the concurrence of Greig cephalopolysyndactyly syndrome, an autosomal dominant condition mapped at 7p13, and sinus node disease is discussed.

Abolishment of chronic volume overload. Implications for diastolic function of the systemic ventricle immediately after Fontan repair.

Background: In patients with a univentricular heart, the chronic ventricular volume overload is acutely abolished by the creation of a Fontan circuit. This results in an immediate reduction of the ventricular cavity size but also in an inappropriate degree of ventricular wall thickness, at least in the early postoperative period. The implications for the diastolic properties of the ventricle are poorly understood.

Evaluation of breath-by-breath measurement of respiratory gas exchange in pediatric exercise testing.

In adults, breath-by-breath analysis has been used for measuring respiratory gas exchange during exercise. The present study evaluates the validity and reproducibility of this method in children. In 21 patients with various types of congenital heart disease, steady state exercise testing was performed on a motor-driven treadmill.

Rheologic genesis of discrete subvalvular aortic stenosis: a Doppler echocardiographic study.

To determine whether morphologic structures or abnormal flow patterns predispose to pathologic proliferation of subvalvular tissue, 26 patients (mean age 19.8 +/- 10.3 years) were studied greater than or equal to 6 months after operation for isolated discrete subvalvular aortic stenosis.

Cardiorespiratory exercise capacity after surgical closure of atrial septal defect is influenced by the age at surgery.

To study the influence of age at the time of the operation on long-term functional performance in children undergoing surgery for atrial septal defect (ASD) of the secundum type, exercise tolerance was assessed in 24 patients and values were compared with those of normal subjects. Patients were divided into two groups: 11 patients had surgery before the age of 5 years (group 1) and 13 patients had surgery at a later age (group 2). There were no significant differences between groups 1 and 2 with regard to the pulmonary-to-systemic flow ratio, pulmonary artery pressure, and the interval between surgery and exercise testing.

Hypertrophic retromedullary venous drainage in spinal cord tumours: MR visualisation.

Pseudoangiomatous dilatation of retromedullary veins at myelography was noted in three cases of intramedullary spinal cord tumours. This concerned two haemangioblastomas at levels Th8 and L1, respectively, and an ependymoma of the conus terminalis. MRI accurately demonstrated the tumoural mass, together with the enlarged and tortuous draining perimedullary veins.

Results from an international survey of Kawasaki disease in 1979-82.

Kawasaki disease is a febrile illness of unknown etiology affecting mainly children younger than five years, 20% of whom develop coronary artery aneurysms. The disease was first reported in Japan, but case reports have come from several countries; epidemics apparently occur every two to three years. A committee of the International Society and Federation of Cardiology (ISFC) collaborated on an international, retrospective survey in mid-1983 through 1984, asking about Kawasaki disease cases seen in 1979-82 and their outcomes.

Cardiorespiratory response to exercise in congenital complete atrioventricular block.

In 8 patients aged 8 to 17 years with congenital complete atrioventricular (AV) block, exercise testing was performed on a treadmill. Cardiorespiratory endurance performance was assessed by determination of the ventilatory threshold. The mean value for ventilatory threshold was significantly lower (p less than 0.

Transient neonatal tricuspid regurgitation: a Doppler echocardiographic study of three cases.

Three patients with normal hearts and no pulmonary abnormality had neonatal tricuspid regurgitation causing cardiorespiratory distress and cyanosis. The signs of tricuspid regurgitation resolved over a few weeks. In the acute phase echocardiography showed gross dilatation of the right atrium and ventricle.

Cardiorespiratory exercise testing after venous switch operation in children with complete transposition of the great arteries.

In 14 children who underwent a venous switch operation for complete transposition of the great arteries, exercise testing was performed 3 to 15 years (mean 8) after the operation. Exercise performance capacity was assessed by the determination of the ventilatory threshold during exercise and by the oxygen uptake (VO2) reached at a heart rate of 170 beats/min (VO2,170). The ventilatory threshold was defined as the highest oxygen uptake (VO2) at which the pulmonary ventilation (VE) stops to increase linearly when related to VO2.

Cardiac chamber growth pattern determined by two-dimensional echocardiography.

Two-dimensional echocardiographic measurements of the right and left ventricles from the parasternal long-axis and apical four-chamber views and an estimated right ventricular-left ventricular dimensional ratio were obtained in 173 normal subjects, ranging from 1 day to 15 years of age. The end-diastolic dimensions of both the right and left ventricles increases in proportion to the logarithmic function of body surface area. The right ventricular-left ventricular dimensional ratio decreases immediately after birth and continues to decrease during the first months of life.

Cross sectional echocardiographic assessment of great artery diameters in infants and children.

The pulmonary trunk and aortic root were measured on cross sectional echocardiograms in 173 normal subjects aged from one day to 15 years. Fifteen neonates were reexamined 3-6 days later. The great vessels were visualised in the parasternal long axis and short axis views.

Interrupted right aortic arch in DiGeorge syndrome.

The clinical and necropsy findings in four cases of interrupted right aortic arch and right descending aorta associated with DiGeorge syndrome (congenital absence or hypoplasia of the thymus and parathyroids) are described. All patients had a mirror image of type B interruption, namely a right aortic arch with reversed branching pattern and an interruption between the right common carotid and right subclavian artery. In two patients there was a doubly committed subarterial ventricular septal defect and in the two other patients there was a perimembranous septal defect.

Exercise testing after correction of tetralogy of Fallot: the fallacy of a reduced heart rate response.

Thirty-nine patients, 5 to 19 years of age, were studied 1 to 10.5 years (mean 4.5) after surgical correction of tetralogy of Fallot (TF).

Infections caused by Kingella kingae: report of four cases and review.

Kingella kingae, formerly known as Moraxella kingae, is a fastidious, non-motile, coccobacillary, fermentative Gram-negative rod that has been chiefly associated with two types of infections in man: bone and joint infections, and endocarditis. We describe four patients with K. kingae infections, one with septicaemia, two with endocarditis, and one with osteoarthritis.

Ventilatory anaerobic threshold for evaluating exercise performance in children with congenital left-to-right intracardiac shunt.

The ventilatory response to graded treadmill exercise was studied in 50 children with congenital heart disease (CHD), a left-to-right intracardiac shunt, and compared to that of age and sex-matched healthy children. In all these children, a breakpoint in the minute ventilation (VE) in relation to increasing oxygen uptake (VO2) was detected. Because of the reported coincidence of the nonlinear increase in VE and lactate with increasing VO2, this threshold has been named "ventilatory anaerobic threshold" (VAT), expressed in ml O2 X min-1 X kg-1.

Usefulness of echocardiographic assessment of right ventricular and pulmonary trunk size for estimating magnitude of left-to-right shunt in children with atrial septal defect.

M-mode and 2-dimensional echocardiographic studies were performed in 42 patients, aged 1 to 16 years (mean 6), with a secundum or sinus venosus type atrial septal defect (ASD) and normal pulmonary artery pressure. Twenty normal children served as a control group. In patients with ASD the echocardiographic variables were correlated with the magnitude of the left-to-right shunt (Qp/Qs) calculated by the Fick principle.

Pacing in children.

Fourteen children had a permanent pacemaker implanted between May 1967 and July 1983. Postoperative complete heart block was the indication in nine cases, congenital complete heart block in three, and sick sinus syndrome in two. Two patients died, one suddenly and one after aortic valve replacement.

Ventilatory anaerobic threshold in healthy children. Age and sex differences.

The ventilatory anaerobic threshold (VAT) during graded exercise was defined as the oxygen uptake (VO2) immediately below the exercise intensity at which pulmonary ventilation increased disproportionally relative to VO2. Since VAT is considered to be a sensitive and noninvasive measure for evaluating cardiorespiratory endurance performance, the purpose of the present study was to determine normal values in children. We examined 257 healthy children (140 boys and 117 girls) varying in age from 5.

Long-term echocardiographic assessment of dilated cardiomyopathy in children.

Left ventricular (LV) dimensions and function were assessed by echocardiography in 22 children with dilated cardiomyopathy. They had survived an initial episode of congestive heart failure in infancy for greater than or equal to 2 years. At the time of echocardiography, when they were 3 to 16 years old, 8 patients (Group 1) still had signs of dilated cardiomyopathy and 14 (Group 2) had lost all roentgenographic and electrocardiographic evidence of heart disease.