Of the 12 children who represent the complete cohort of Norwegian patients surviving the complete palliative operative programme for hypoplastic left heart syndrome, 10 were studied by a pediatric cardiologist, a pediatric neurologist, a neuropsychologist and a child psychiatrist. We found significant morbidity. Hemodynamic function was relatively uncompromised in eight of the patients, but one had significant cyanosis due to use of an operative technique subsequently abandoned and one had obstruction of the flow of blood from the left to the right atrium. Five children had disturbances of rhythm, with one requiring insertion of a pacemaker. One child had epilepsy. Delayed motor development, most often to mild degree, was found in seven children, while six had attention problems. Mental retardation was found in two children and autistic traits in one. Despite these various problems, the parents were seemingly satisfied with their own lives, and the quality of life of their children.

Definitive repair of tetralogy of Fallot was performed on 74 patients under two years of age, including 40 under the age of one year and 13 less than 6 months of age. In six patients, there had been previous construction of a systemic-to-pulmonary arterial shunt. Patching across the ventriculo-pulmonary junction was required in 46 patients, placement of conduits in six, and procedures producing unifocaliza-tion in three. Three patients died during the 60-day postoperative period of observation. Of the 71 survivors, 64 recovered without complications. One patient had neurologic sequels, one required prolonged mechanical ventilation, two reoperation, and one balloon dilation of residual left pulmonary arterial stenosis. Morbidity and mortality were not significantly higher in those patients undergoing surgery at earlier than 6 months of age, although transjunctional patching was more common in that age group. Previous construction of a shunt was associated with a higher prevalence of distal stenosis and distortion of the pulmonary arteries, which needed surgical repair in two out of six patients (33%). Since the outcome of definitive repair is favourable in patients with tetralogy of Fallot younger than six months of age, we now advocate definitive surgery for all young symptomatic patients by the age of six months.

The traditional approach to repair of tetralogy of Fallot involves a right ventriculotomy for closure of ventricular septal defect. During the past two decades, reports of progressive right ventricular dilation and dysfunction, and late occurrence of ventricular arrhythmias, have led investigators to re-evaluate this approach and advocate instead the transatrial-transpulmonary approach, hoping to preserve global right ventricular function. We studied the short term effects on right ventricular function of either of the two approaches through a prospective randomised study, involving two comparable groups of patients operated in the same time frame.

Between June 1993 and February 1994, 40 patients having tetralogy of Fallot with comparable preoperative characteristics, were assigned randomly to each of two groups for surgical correction.

In 20 patients, correction was achieved via the transatrial-transpulmonary route. In the other 20 patients, transventricular correction was the chosen option. Six months after surgery, patients were evaluated clinically, by Doppler echocardiography, cardiac catheterisation, first pass radionuclide angiography and by 24 hours ambulatory electrocardiographic monitoring, taking note of hemodynamics, abnormalities in rhythm, and global right ventricular function.

There were no early deaths or morbidity in either group. Mean immediate postoperative ratio between peak right ventricular and systemic pressures was 0.62 ± 0.22 after transatrial and 0.70 ± 0.007 after transventricular correction. All patients were in functional class I. Six months after surgery the mean ratio between peak ventricular pressures was similar in the two groups (transatrial group: 0.37 ± 0.02, transventricular group: 0.38 ( 0.01), but significantly lower than that measured in the operating room. There were no significant arrythmias in either group. Mean right ventricular ejection fraction was nearly the same in both groups (transatrial group versus transventricular group; 44.83 ± 5.65% versus 42.37 ± 8.70%). Significant global hypokinesia of the right ventricle was documented in three patients, and mild hypokinesia in another three, undergoing repair through the transventricular route while in the group undergoing transatrial repair only one patient had mild hypokinesia.

We conclude that comparable hemodynamic results are obtained on short term follow-up after repair of tetralogy of Fallot by either the transatrial or transventricular route. Although more patients in the transventricular group were found to have global hypokinesia of the right ventricle, longer follow-up is necessary to establish the clinical relevence of these findings.

This study was designed to determine, first, the stiffness of the pulmonary arteries and, second, the relationship between the stiffness of the pulmonary arteries and the success rate of balloon angioplasty in patients with complete transposition after an arterial switch operation. Indexes of pulmonary arterial wall stiffness, percent change in the radius during a cardiac cycle and the pressure elastic modulus, were calculated from the pulmonary arterial pressure and radius measured from a cineangiogram. Of the patients, 13 had no significant stenosis, while 25 had significant stenoses and, therefore, underwent balloon angioplasty. In all, we dilated 33 stenotic lesions. In the presence or absence of postoperative pulmonary stenosis, the percent change in the radius of the pulmonary trunk was significantly less than the normal value. The pressure elastic modulus of the trunk and its branches was significantly greater than normal, and increased significantly with time after the operation. In patients without pulmonary stenosis, the systolic pulmonary arterial pressure increased concomitantly with the pressure elastic modulus. Balloon dilation was successful at 17 locations and unsuccessful at 16 locations. The percent change in radius was significantly less for the failures than for the successes. Prior to 3.5 years after the operation, the pressure elastic modulus was < 400 g/cm2 at most locations and balloon dilation was successful in 88%. More than 3.5 years after the operation, the pressure elastic modulus was < 400 g/cm2 in 11 of 15 locations, and balloon dilation was successful in only one location. The increased stiffness of the pulmonary artery may result in increased pulmonary systolic pressure in patients without pulmonary stenosis, and result in a low success rate for balloon angioplasty in patients with pulmonary stenosis.

We report our initial experience in three children with percutaneous transvenous closure of significant atrial septal defects. A newly developed one-piece nitinol device is delivered through a long venous sheath of 7 French calibre under fluoroscopic and transoesophageal echocardiographic monitoring. Complete closure of all defects was verified by echocardiography on the morning after the procedure. No complication occurred. In the first two patients, the device slipped sideways into the defect. This was easily discovered and corrected. Our experience shows that the Amplatzer® device is reliable, easy to implant, and presents very clearly on transoesophageal echocardiography and fluoroscopy. This makes implantation a controlled procedure. Until release, the device can easily be retracted into the sheath at any time and removed. The results suggest that closure of an atrial septal defect with this device is feasible, rapid, and safe.

Some patients with so called “secundum” atrial septal defect within the oval fossa show a large defect on the echocardiogram, although they do not have all the classical clinical features. Until now, a large atrial septal defect was thought to be characterized by a large shunt (functional defect). Experience indicates, nonetheless, that such large defects in size (anatomical defect) are not always accompanied by large shunts. In order to assess how often the classical clinical features of an atrial septal defect exist, and to investigate whether the surgical indication for closure of the defect are anatomical or functional, we carried out a retrospective study. We evaluated the records of 161 patients, with birth dates from 1973 to 1994 (age between 0 and 21 years), so as to study the classical clinical features (history, physical examination, electrocardiogram, chest X-ray, echo-Doppler studies, cardiac catheterization, surgery). Only patients with an atrial septal defect found in isolation were included.

We discovered that the classical clinical features are often not present when there is a large defect, be it functional or anatomical. The anticipated features are present in roughly two-thirds of the patients. There is also a discrepancy between the anatomical and the functional sizes of the defect. This finding creates a fundamental problem for the future, namely the indications fot closure of atrial septal defects within the oval fossa. Further studies are needed to answer this important question

Cross-sectional echocardiography was performed on 108 healthy children (7 days – 17 years old) and 55 children (6 months - 16.5 years old) with interatrial communication. Right ventricular end-diastolic volume, end-systolic volume, stroke volume, ejection fraction, muscle volume, and the ratio of muscle to cavity were calculated on the basis of outlined cavity and myocardium of an apical fourchamber view.

In the normal subjects right ventricular end-diastolic volume, end-systolic volume, stroke volume and muscle volume correlated with body surface area (end-diastolic volume: y=12.5x+7.8x2, r=0.99; end-systolic volume: y=4.8x+3.6x2, r=0.98; stroke volume: y=7.7x+4.2x2, r=0.98; muscle volume: y=14.1x+2.9x2, r=0.97), muscle/cavity ratio (0.85±0.17) and ejection fraction (58.9 ± 6.2%) were unrelated to body surface area. In the subjects with interatrial communication, the right ventricular volumes were significantly larger (p<0.001) than the normal values with a linear relationship to the ratio of pulmonary to systemic flows.

Right ventricular volumes can be determined in normal children with acceptable repeatability using a standard apical four-chamber view. The growth related normal values provide a basis for future quantitative studies.

The structural complexity of the right ventricle has made quantitative evaluation difficult. Conventional cross-sectional echocardiographic methods are limited by geometric assumptions and the position of the planes used for imaging. Previous reports have demonstrated accurate three-dimensional echocardiographic quantitation of the right ventricle in-vitro and in experimental animals. We adapted a previously described method for three-dimensional reconstruction of the left ventricle to compute right ventricular volume and ejection fraction in a clinical setting.

We examined 29 patients aged from 2 to 42 years with pulmonary hypertension, by three-dimensional echocardiography and resonance imaging. Correlation and agreement were calculated for volumes and ejection fractions. Three-dimensional echocardiographic reconstruction, when compared to resonance imaging, yielded r values of 0.95 and 0.93, and mean differences (bias) of 31% ± 19% and 33% ± 18%, for systolic and diastolic volumes respectively. Interobserver variability was low (12.9% and 8.0%). Ejection fraction as calculated by three-dimensional echocardiography showed close agreement with resonance images (bias=l% ±7%). Three dimensional echocardiography is now a method of measuring right ventricular ejection fraction in the clinical setting which produces results comparable to those of resonance imaging. Volume measurements correlated well for systole and diastole, but consistently underestimated values produced from resonance images.

Anomalous origin of the coronary arteries is a well-known phenomenon, but is less well reported in world literature. We present our findings in a one year old boy in whom there was bifid, or “y”, origin of the right coronary artery, co-existing with a fistula from the right coronary artery which opened into the right ventricle.

We describe a case of anomalous origin of the right subclavian artery from the right pulmonary artery detected by cross-sectional echocardiography in an infant with type B interruption of the aortic arch. Preoperative recognition and surgical reimplantation of the right subclavian artery are important to prevent subsequent subclavian steal syndrome.

A patient with aortitis presented in infancy with dermatitis gangrenosa. Coarctation of the aorta was subsequently produced by granulomatous tissue. We report 30 years of medical observation.

Previous studies have demonstrated the individual association of subpulmonary tissue tags, dissection of the pulmonary trunk and primitive neuroectodermal tumors with congenital heart disease. We are reporting a case of double inlet left ventricle with all these associations as it is exceptionally rare for all features to be manifest in one individual.

We describe an unusual case of congenitally corrected transposition in which left-sided obstructions included a supravalvar tricuspid ring, a parachute deformity of the valve itself, and coarctation of the aorta. Surgical correction was performed in 3 stages. Initially we repaired the coarctation and banded the pulmonary trunk. Then we removed the band and, finally, attempted unsuccessfully to replace the tricuspid valve. When coarctation of the aorta occurs in congenitally corrected transposition, it is always wise to search carefully for abnormalities of the tricuspid valve.