[Transesophageal electrophysiological study in non sedated children younger than 11 years with a Wolff-Parkinson-White syndrome]. / Etude électrophysiologique par voie transoesophagienne chez des enfants de moins de 11 ans, non sédatés, ayant un syndrome de Wolff-Parkinson-White.

UNLABELLED: The electrophysiological evaluation of Wolff-Parkinson-White syndrome (WPW) is recommended in children aged more than five years to detect a risk of life-threatening arrhythmia. The purposes of the study were to determine the feasibility of transesophageal EPS in a child between six and 10 years in out-patient clinic. METHODS: Electrophysiological study (EPS) was indicated in 22 children, aged six to 10 years, with a manifest WPW either for no documented tachycardia (n=7), unexplained dizziness (n=2) or for a sportive authorization in 10 asymptomatic children. Two of the last children had a history of permanent tachycardia after the birth but were asymptomatic since the age of one year without drugs. RESULTS: EPS was performed in all children. The main difficulty lied in passing the catheter through the mouth. Programmed stimulation at cycle length of 380 ms was performed in all children to avoid high rates of pacing when the conduction through the accessory pathway (AP) and normal AV system was evaluated. Isoproterenol was not required in five children, because they developed a catecholaminergic sinus tachycardia. The AP refractory period was determined in all children between 200 and 270 ms. Orthodromic reentrant tachycardia (RT) was induced in 11 children, three asymptomatic children (27%), seven complaining of tachycardia and one with syncope. Rapid antidromic tachycardia was induced in this last child with dizziness. Atrial fibrillation was never induced. CONCLUSIONS: Esophageal EPS can be performed without sedation in a young child six to 10-year-old with a shortened protocol of stimulation, which was capable to clearly evaluate the WPW-related risks.

Interest of non-invasive and semi-invasive testings in asymptomatic children with pre-excitation syndrome.

AIMS: To determine the feasibility and the results of exercise testing (ET) and electrophysiological study (EPS) in outpatient asymptomatic children with a Wolff-Parkinson-White (WPW) syndrome. METHODS AND RESULTS: Exercise testing and transesophageal EPS were performed in 55 outpatient asymptomatic children aged 6 to 19 years old (14 +/- 3) with WPW. Wolff-Parkinson-White persisted during maximal exercise. Isoproterenol was not required in five children younger than 10 years old, because they developed a catecholaminergic sinus tachycardia. Maximal rate conducted through accessory pathway (AP) was higher in children younger than 16 years old than in teenagers (P < 0.05). Atrioventricular re-entrant tachycardia (AVRT) was induced in six children; atrial fibrillation (AF) in 12 children. The induction of tachycardias and the dangerous forms (18%) were not influenced by age. After 5 +/- 1 years, one child, 12 year old with inducible rapid AF, had a sudden cardiac arrest; two children became symptomatic after ablation. CONCLUSIONS: Transesophageal EPS was required to determine the prognosis of asymptomatic WPW in children. The maximal rate conducted in AP was higher in children younger than 16 years old than in teenagers; other data did not differ. AVRT was rare; 71% of children had no inducible arrhythmia and were authorized to resume physical activities.

[Prenatal detection and management of congenital heart disease]. / Détection et prise en charge prénatales d'une cardiopathie.

Antenatal diagnosis of congenital heart diseases rests on a two steps procedure. 1) A first intended fetal echocardiography which takes place during the two first trimesters of gestation, either early oriented by the familial or maternal history or the finding of fetal developmental abnormalities (intra uterine growth retardation, defects), or systematically performed as part of the fetal echography of the second trimester. 2) A second echocardiography which is part of a specialized cardiologic evaluation; its aims are to define the cardiac phenotype, to specify the hemodynamic tolerance of the heart defect, to orientate the genetic diagnosis, and finally to evaluate the cardiac prognosis.

[Atresia or congenital stenosis of the left coronary ostium. Myocardial revascularization in 5 children]. / Atrésie ou sténose congénitale de l'ostium coronaire gauche. Revascularisation myocardique chez 5 enfants.

Five patients, 3 months to 13 year old with atresia (4) or stenosis (1) of the left coronary artery underwent myocardial revascularisation at Marie Lannelongue Hospital. The preoperative symptoms were dominated in one case by cardiac failure due to myocardial infarction and in the other cases by effort angina with syncope. The operative technique of revascularisation in one case was angioplasty with enlargement of the left main coronary artery with a patch and, in the others, bypass graft of the left coronary artery with the left internal mammary artery. All 5 patients survived surgery and are asymptomatic with a follow-up of 6 months to 7 years. Control coronary angiography confirmed the patency of the different procedures of myocardial revascularisation. In 3 children, exercise electrocardiography was normal. Myocardial revascularisation of a child with a congenital stenotic abnormality of the left coronary network is possible, the technique of which depends on the diameter of the left main stenosis when the vessel is absent or atresic, left internal mammary artery bypass graft is the only solution. When the left main coronary is stenosed but patent, direct reconstructive surgery is an interesting alternative.

Aortic arch thrombosis in the neonate.

Two cases of neonatal aortic arch thrombosis are reported. One patient, who had ascending aortic thrombosis, died preoperatively. The other had reoperation and is alive and well at 6 months' follow-up. No obvious cause was found.

[Extracardiac pulmonary bypass in the treatment of tricuspid atresia]. / Dérivation bicavo-pulmonaire extracardiaque dans le traitement de l'atrésie tricuspide.

Six patients aged 1.5 to 4 years with type 13 tricuspid atresia underwent extracardiac bicavopulmonary repair. This corrective procedure comprises control of palliative aorto-pulmonary anastomosis, the construction of an anastomosis between the superior vena cava and right pulmonary artery and the interposition of an extracardiac tube of autologous pediculated pericardium between the inferior vena cava and the main pulmonary artery. This tube, fashioned from a rectangular flap, remains attached along its right border, conserving its vascular pedicle with the pericardium. Its diameter is calibrated to that of the inferior vena cava. The postoperative course was uncomplicated: all children survived. Assisted respiration was discontinued 24 to 36 hours after surgery, and the pleural drains withdrawn after 48 to 72 hours. The rhythm remained sinusal. Central venous pressure ranged from 8 to 12 mmHg. Peripheral arterial situation was over 95%. At follow-up at 3 to 15 months, the children were very active, reflecting good exercise capacity. Holter monitoring was normal. Saturation was 94 to 97%. Doppler echocardiography showed laminar blood flow. This technique insures good venous drainage without any prosthetic thrombogenic material. In addition, the pediculated pericardium conserves a potential for growth, justifying its use in small children. Long-term evaluation is essential but the initial results are encouraging and encourage perseverance with this method.

Changes of right ventricular size and function in neonates after valvotomy for pulmonary atresia or critical pulmonary stenosis and intact ventricular septum.

Right ventricular end-diastolic and stroke volumes were calculated from orthogonal subcostal echocardiographic images in 24 neonates (mean weight +/- SD 3.4 +/- 0.4 kg) with pulmonary atresia (n = 18) or critical pulmonary stenosis (n = 6) and intact ventricular septum before and at an average of 5 days and then 19 days after pulmonary valvotomy. The preoperative echocardiographic volume determinations were compared with the respective angiographic determinations. In addition, the endocardial area outlines of the left and right ventricles were obtained by planimetry from an end-diastolic frame taken in the apical four-chamber view. End-diastolic and stroke volumes calculated by the echocardiographic method (y) correlated closely with those calculated by the angiographic method (x); the regression equations were y = 1.02 x -0.13 (r = 0.95, SEE +/- 0.45 ml) and y = 1.16 x -0.15 (r = 0.89, SEE +/- 0.38 ml), respectively. All except one infant had right ventricular hypoplasia before valvotomy with an end-diastolic volume of 16.6 +/- 6.4 ml/m2 (44.5 +/- 17.3% of normal). Right to left ventricular area ratio was 0.56 +/- 0.09 (normal 0.95). Five days after valvotomy, right ventricular end-diastolic volume decreased to 10.6 +/- 4.6 ml/m2 (p less than 0.05) and stroke volume decreased from 8.3 +/- 3.5 to 5.5 +/- 2.8 ml/m2 (p less than 0.05). Nineteen days after valvotomy, right ventricular end-diastolic volume and right to left ventricular area ratio had increased to their respective preoperative values; right ventricular stroke volume had increased further to 10.4 +/- 3.9 ml/m2 (p less than 0.05).(ABSTRACT TRUNCATED AT 250 WORDS)

Two-dimensional echocardiographic determination of ventricular volumes in the fetal heart. Validation studies in fetal lambs.

The determination of ventricular volumes in the fetal heart from two-dimensional echocardiography (2DE) may give a better estimate of fetal ventricular size than simple diameter measurements, but the accuracy of this method has not been established. In fetal lambs, we tested whether ventricular volume calculations from 2DE using a biplane Simpson's rule algorithm are accurate. Calculations of left and right ventricular end-diastolic volumes from 2DE were compared with cast volumes of these ventricles. Also, at different levels of left atrial pressure, left ventricular stroke volumes calculated from 2DE were compared with stroke volumes measured simultaneously by an electromagnetic flowmeter. There was a good correlation between volumes determined from 2DE (y axis) and from casts (x axis) for both the left (r = 0.92; y = 0.2 + 1.1x; SEE = 0.19 ml) and right ventricle (r = 0.90; y = 0.7 + 0.9x; SEE = 0.21 ml). Left ventricular stroke volumes calculated from 2DE correlated well with those measured by the electromagnetic flowmeter (r = 0.87; y = 0.2 + 0.9x; SEE = 0.27 ml). Thus, calculation of fetal ventricular volumes from 2DE images using a biplane Simpson's rule method is feasible and accurate.

Doppler echocardiographic measurement of low velocity motion of the left ventricular posterior wall.

A new noninvasive method using pulsed Doppler echocardiography was developed to assess left ventricular (LV) posterior wall motion dynamics. Seventeen normal subjects and 23 patients undergoing cardiac catheterization were prospectively studied. The sample volume was placed within the LV posterior wall endocardium just apical to the mitral valve sulcus using a posteriorly angulated low parasternal view. The wall filter was set at 100 Hz to record the low velocities of the LV posterior wall motion. The Doppler signal was morphologically similar to the rate of change of the LV posterior wall endocardium excursion obtained by a digitized M-mode echocardiogram, and showed 3 major waves: a systolic wave (S), an early diastolic wave (E) and a late diastolic wave (A). The peak velocities of LV posterior wall endocardium excursion were also determined by M-mode echocardiographic technique. We found a significant linear correlation between peak E-wave velocity and M-mode peak diastolic endocardial velocity (r = 0.90, p less than 0.001) and between peak S-wave velocity and M-mode peak systolic endocardial velocity (r = 0.81, p less than 0.001). M-mode peak systolic endocardial velocity showed an important overlap between control subjects and patients with normal and patients with abnormal LV posterior wall motion on the angiogram. In contrast, peak S-wave velocity was a better discriminator, and a peak S-wave velocity less than 7.5 cm/s was associated with abnormal LV posterior wall motion with an 83% sensitivity, 100% specificity and 95% accuracy. In patients with coronary artery disease but normal systolic LV posterior wall motion and normal global systolic LV function, peak S-wave velocity was not different when compared to control subjects. Peak E-wave velocity and E/A were significantly lower than in control subjects (p less than 0.01) and peak A-wave velocity was greater (p less than 0.01). In conclusion, these data suggest that pulsed Doppler echocardiography can be used for the direct analysis of LV posterior wall instantaneous low velocities and appears to be more informative than M-mode technique for systolic measurements. Thus, detection of abnormal LV posterior wall diastolic motion by pulsed Doppler echocardiography may, upon additional confirmation, be used as a new noninvasive method to gain insight into global LV diastolic performance.

Assessment of right ventricular performance by pulsed Doppler echocardiography in patients after intraatrial repair of aortopulmonary transposition in infancy or childhood.

The purpose of this study was to determine whether measurement of aortic blood flow velocity and acceleration by pulsed Doppler ultrasound can be used to assess the global performance of the systemic right ventricle noninvasively in young patients who have undergone intraatrial repair of aortopulmonary transposition. The effect of age at surgery on right ventricular performance in these patients was also studied. Pulsed Doppler velocity profiles of aortic blood flow were analyzed at a mean age of 5.5 years in 24 patients who had undergone intraatrial repair of aortopulmonary transposition either in early infancy (mean age 3.4 +/- 1.3 weeks) or later (mean age 8.5 +/- 6.5 months). Velocity and acceleration variables in these patients were compared with the same variables in 24 age-matched normal subjects and with the performance of their own right ventricle as assessed by two-dimensional echocardiographic measurement of ejection fraction. The 12 patients who underwent early repair had a higher ejection fraction than did the 12 who underwent later repair (mean +/- SD 0.60 +/- 0.07 versus 0.42 +/- 0.10; p less than 0.001). Aortic flow velocity was similar in all patients. Aortic acceleration was normal in patients after early intraatrial repair of aortopulmonary transposition (20.8 +/- 2.3 m/s2), but was abnormally slow in patients after late repair (11.1 +/- 1.8 m/s2; p less than 0.001), thus suggesting that the latter group had diminished right ventricular performance. Concomitantly, acceleration time and ratio of acceleration time to ejection time were increased in patients after late repair.(ABSTRACT TRUNCATED AT 250 WORDS)

[Percutaneous transluminal valvuloplasty in aortic valve stenosis in children]. / Valvuloplastie transluminale percutanée dans les sténoses aortiques valvulaires de l'enfant.

Percutaneous transluminal balloon valvuloplasty has been used in children since 1983 to treat tight aortic valve stenosis. Although more than 100 cases have been published, its indications compared with those of surgical valvulotomy have not yet been clearly defined. Twelve patients with tight aortic valve stenosis underwent transluminal balloon valvuloplasty: 3 were infants whose ages ranged from 1 month and 24 days to 3 months and 23 days (mean 80 +/- 13.7 days), and 9 were children aged from 4 to 17 years (mean 10.44 +/- 4.61 years). Aortic regurgitation grade I (5) or grade III (1) was present in 6 cases. An 8-year old child had previously undergone surgical aortic valvulotomy simultaneously with repair of aortic coarctation. Other associated malformations were congenital grade III mitral regurgitation in 1 case and Turner's syndrome in 1 case. Dilatation was performed with one or two successive balloons in 11 patients and with two balloons concomitantly in 1 patient. The diameter of the balloon (or the sum of those of the two balloons) was 77 to 112 percent of the diameter of the aortic annulus, but in one case this figure rose to 119 percent after failure of dilatation with a smaller balloon. One infant died at the end of the dilatation, due to perforation of the left ventricle. Another infant developed inferior myocardial infarction which followed a favourable course and left minimal sequelae.(ABSTRACT TRUNCATED AT 250 WORDS)

[Abnormal origin of the right pulmonary artery from the ascending aorta. Echocardiographic diagnosis apropos of 2 cases]. / Naissance anormale de l'artère pulmonaire droite à partir de l'aorte ascendante. Diagnostic échocardiographique à propos de deux observations.

Anomalous origin of the right pulmonary artery from the ascending aorta is a rare congenital malformation. Formerly identified at angiography and during surgery, it can now be diagnosed by two-dimensional echocardiography, as was done in the two cases reported here. The first case was a neonate in heart failure in whom echocardiography revealed the anomalous origin of the right pulmonary artery; this diagnosis was confirmed at post-mortem examination. The second case was a 4-month old infant in heart failure; echocardiography displayed the malformation which was confirmed at angiography and surgery. In both cases echocardiography showed discontinuity between the pulmonary trunk and the right pulmonary artery and continuity between the pulmonary trunk and the left pulmonary artery. There also was continuity between the right pulmonary artery and the ascending aorta. Doppler ultrasound showed a systolic and a diastolic flow in the right pulmonary artery and only a systolic flow in the left pulmonary artery. A colour-coded doppler examination in one case confirmed the anomalous origin of the right pulmonary artery and the absence of any other abnormality. Two-dimensional echocardiography combined with colour-coded doppler ultrasound therefore is the method of choice to diagnose anomalous origin of the right pulmonary artery from the ascending aorta.

Determination of pulmonary to systemic blood flow ratio in children by a simplified Doppler echocardiographic method.

Doppler echocardiographic determination of the pulmonary to systemic blood flow (Qp/Qs) ratio requires calculation of pulmonary artery and aortic luminal areas and integrals of the Doppler systolic velocity curves. To simplify the Doppler calculation of the Qp/Qs ratio, the square of the ratio of pulmonary to aortic luminal diameters, substituted for the ratio of vessel areas, was multiplied by the ratio of pulmonary to aortic peak flow velocities, substituted for the ratio of velocity time integrals. The Qp/Qs ratios were calculated by both the conventional and the simplified Doppler method in 42 children, 1 month to 16 years of age. Fifteen children had no evidence of shunt and 27 had an intracardiac left to right shunt at the atrial or ventricular level, or both. In children with a shunt, the Qp/Qs ratio obtained at cardiac catheterization by oximetry ranged from 1.4:1 to 4.3:1. Both the conventional and the simplified Doppler Qp/Qs ratios correlated well with the oximetric Qp/Qs ratios (r = 0.94 and 0.93, respectively). Since the pulmonary to aortic luminal area ratio and velocity time integral ratio varied in each patient, neither measurement alone correlated well with the oximetric Qp/Qs ratio and, therefore, neither alone could be used as an estimate of the Qp/Qs ratio. The simplified Doppler calculation of the Qp/Qs ratio, which requires less time and no computer facilities, provides an excellent estimate of the Qp/Qs ratio in children.

[Cardiovascular dysplasia in Noonan's syndrome. Apropos of 64 cases]. / La dysplasie cardio-vasculaire du syndrome de Noonan. A propos de 64 observations.

The multiple malformation syndrome with characteristic facies described by Jacqueline Noonan in 1963 is one of the most commonly encountered syndromes in paediatric cardiology. The series presented here comprises 64 cases, almost a quarter of which were familial (6 families). The morphotype (Turner phenotype with hypertelorism) can easily be recognized, but the variations, progressivity and resemblance to some similar syndromes imply a detailed analysis of a wide range of discriminant features. Short stature and webbed neck are less frequent than in Turner's syndrome, while mental debility and, mostly, cardiovascular lesions are much more frequent (the latter are present in 50-60% of the cases). The most typical cardiovascular lesion is pulmonary valve stenosis with dysplastic leaflets, or "atypical pulmonary stenosis syndrome", characterized by a curious electrical axis and above all, by left ventricular myocardiopathy with very peculiar deformations at ventriculography and 2D-echocardiography. In our series, myocardiopathy was even more frequent than dysplastic pulmonary valve stenosis (71.8% versus 64%). The dissemination of dysplastic lesions must be emphasized. They involve the aorta much more often than is usually reported (one-third of the cases); lesions of the lymphatic system are less frequent but may be severe. Occasionally, entirely different heart diseases, such as Fallot's tetralogy, are encountered. Owing to the relationship between its lesions and those of other multiple malformation syndromes, notably those of the phakomatosis group, and to the possibility of borderline cases with these syndromes, or even with other dystrophies, such as Williams-Beuren dystrophia, the cardiovascular dysplasia of Noonan's syndrome must be classified within the vast group of histodysplasias which are embryonic diseases of the "layers" or "neuro-ecto-mesodermoses" of unknown genetic mechanism.

[Current role of radionuclide imaging in pediatric cardiology]. / Place actuelle des isotopes en cardiopédiatrie.

Three main nuclear medicine methods are used in paediatric cardiology: sequential first-pass radionuclide imaging of the cardiac cavities, radionuclide equilibrium ventriculography and radionuclide myocardial imaging. Valuable functional information is obtained, and invasive explorations can be avoided in an ever increasing number of cases. Of particular interest is left-to-right shunt measurement which indicates that atrial septal defects must be surgically corrected when the pulmonary/systemic flows ratio (QP/QS) is above 2. This technique is also useful to evaluate the tightness of repairs in ventricular and atrial septal defects. Radionuclide studies of the right and left ventricles may detect dysfunction in one or the other cavity. The left ventricular ejection fraction is reduced in myocardiopathy an in aortic or mitral valve diseases seen at a late stage. The right ventricular function is often abnormal, notably during exercise, after repair of the tetralogy of Fallot and after atrial correction of complete transposition of the great arteries. An altered ejection fraction in patients with single ventricle is also a sign of deterioration. Right ventricular diastolic overload evaluated by radionuclide equilibrium ventriculography correlates with the QP/QS ratio value in atrial septal defects and with the inducibility of ventricular tachycardia by endocavitary pacing in repaired tetralogy of Fallot. Thallium 201 myocardial imaging provides information on myocardial ischaemia, notably that associated with congenital abnormalities of the coronary arteries. Its use had now been extended, albeit with some limitations, to the evaluation of right ventricular systolic overload. Other radionuclide techniques are being developed with new tracers: Kryton 81m for studies of the right ventricle, short-lived radionuclides for first-pass studies, Iodine 123-labelled fatty acids for myocardial imaging. More recently, some substrates, such as deoxyglucose, have been labelled with positron emitters permitting in vivo metabolic studies.

[Hemodynamic effects of an alpha-blocking vasodilator in cardiac insufficiency caused by left-right shunt in children]. / Effets hémodynamiques d'un vasodilatateur alpha-bloquant dans l'insuffisance cardiaque par shunt gauche-droite de l'enfant.

The acute haemodynamic effects of an alpha-blocking vasodilator, nicergoline, observed during cardiac catheterisation were studied in 9 babies and 1 infant (mean age 11 months) with severe cardiac failure due to a large left-to-right interventricular shunt. Nicergoline was administered intravenously at a dose of 0.05 mg/kg/mn to 0.2 mg/kg/mn to lower mean systemic blood pressure by at least 10 mmHg. No significant changes in heart rate or in right and left atrial pressures were observed. On the other hand, mean systemic and pulmonary arterial pressures fell by 16% (p less than 0.001) and 13% (p less than 0.01) respectively. The ratio of pulmonary and systemic flow (Qp/Qs) decreased in 8 patients by an average of 21% (p less than 0.002). This fall was accompanied by a parallel reduction in oxygen concentrations of pulmonary arterial blood (16%) compared with mixed venous blood. However, the Qp/Qs ratio increased in the other 2 patients by over 50%. In the group of 8 patients in which the left-to-right shunt decreased, the ratio of pulmonary to systemic resistance (Rp/Rs) increased by 33% (p less than 0.002) whilst this value fell by 36% in the 2 patients in whom the volume of the shunt increased. There were no discriminatory parameters between the two groups with regards to age, pulmonary artery pressures, the volume of the shunt (Qp/Qs) or level of pulmonary resistances (Rp/Rs) to explain the variability of the therapeutic response on the left-to-right shunt.(ABSTRACT TRUNCATED AT 250 WORDS)

[Measurement by Doppler echocardiography of the pulmonary arterial pressure in children with ductus arteriosus. Simultaneous Doppler and hemodynamic study]. / Mesure par échocardiographie Doppler de la pression artérielle pulmonaire chez l'enfant atteint d'un canal artériel. Etude hémodynamique et Doppler simultanée.

The aim of this study was to evaluate Doppler echocardiography in the non invasive assessment of pulmonary artery pressures in children with patent ductus arteriosus. Systolic pulmonary artery pressure was measured simultaneously at cardiac catheterisation and by pulsed Doppler in 11 children (mean age 1.8 +/- 2 years) with patent ductus arteriosus alone (6 cases) or associated with a malformation of the heart or great arteries (5 cases). Doppler assessment of systolic pulmonary artery pressure was performed by subtracting the value of the maximal pressure gradient between the aorta and pulmonary artery from the systolic systemic pressure measured simultaneously by sphygmomanometry. The maximal pressure gradient between the aorta and pulmonary artery was calculated using the modified Bernouilli formula and the maximal velocity (v) of the shunt flow (gradient = 4 v2). Ductal flow was recorded from the suprasternal approach by direct interrogation of the patent ductus visualised by 2D echocardiography. The systolic pulmonary arterial pressure measured by catheterisation ranged from 21 to 82 mmHg (mean 58 +/- 21 mm Hg) and by pulsed Doppler from 20 to 89 mm Hg (mean 56 +/- 24 mm Hg) (correlation r = 0.94). This study illustrates the value of Doppler echocardiography in the assessment of systolic pulmonary artery pressures in children with patent ductus arteriosus.

Is the aorta truly dextroposed in tetralogy of Fallot? A two-dimensional echocardiographic answer.

The embryogenesis of tetralogy of Fallot is still much debated. In particular, the dextroposition of the aorta is not considered by all pathologists as a genuine abnormality in this congenital heart disease but rather as a false impression due to an exaggeration of the normal overriding caused by dilatation of the aorta secondary to abnormal hemodynamics. We used two-dimensional echocardiography to examine the spatial position of the aortic root in 22 patients with tetralogy of Fallot (aged 5 days to 24 years, mean 6.4 years) and in 23 normal subjects (aged 1 month to 27 years, mean 7.6 years). Using the parasternal short-axis view, we determined the percent rightward displacement of the aortic root in relation to the plane of the atrial septum, and the relationship between the aortic cusps and the atrial septum. We measured the value of the angle luminal diameter, which was defined as the angle between the plane of the atrial septum and the plane of the left coronary-noncoronary commissure and leaflet appositional plane. In the control group, the aortic root was displaced to the right by only 23.6 +/- 7.6%; the atrial septum crossed the posterior aspect of the aortic root at the middle (n = 19) or at the right half of the posterior cusp (n = 4), and the angle luminal diameter had a value of 43.3 +/- 8.8 degrees. In the 22 patients with tetralogy, the percent rightward shift of the aortic root was augmented to 55.5 +/- 9% (p less than .001) and the atrial septum was related to the posterior commissure in 14 patients.(ABSTRACT TRUNCATED AT 250 WORDS)