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A follow-up study was performed to assess the prevalence of scoliosis in 160 patients in whom aortic coarctation had been treated operatively through a left posterolateral incision in the fourth intercostal space. Scoliosis had not been seen in any patient before the thoracotomy, but a scoliosis of 10 degrees or more was observed in thirty-five patients (22 per cent) at the follow-up examination, which was performed an average of seven years after the thoracotomy. The scoliosis was a thoracic curve and was directed to the left in twenty-six of the thirty-five patients. Most of the curves were mild (between 10 and 20 degrees) and hardly progressed during the follow-up period. The high-risk period for the onset of scoliosis began about three years after the operation. The prevalence of scoliosis did not correlate significantly with the age or sex of the patients.

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After resection of the coarctation in a 3-year-old child with end-to-end anastomosis, a second membrane was found 1.5 cm lower. After a successful reoperation with an aortoplasty using Gore-Tex weak femoral pulses persisted. At catheterization, an area of irregular narrowing of the abdominal aorta just distal to the superior mesenteric artery was found with hypoplasia of the iliac and femoral arteries. In addition, this child had a two-sided cheilo-gnatho-palatoschisis, vesicoureteral reflux and absence of gall bladder and hepato-duodenal ligament. It is worthwhile considering that a second or third aortic abnormality can be hidden by a coarctation of the aorta.

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The M-mode echocardiographic diagnosis of overriding tricuspid valve rests on the ability to demonstrate simultaneously two atrioventricular valves with no intervening septal echo [6, 13]. When scanning inferiorly toward the body of the ventricles, a distinct septal echo at the level of the midportion of the tricuspid valve can be detected. Here we report a case of Ebstein's anomaly, pulmonary stenosis, and ventricular septal defect (VSD), in which the echogram falsely indicated an overriding tricuspid valve.

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Apical displacement of the septal tricuspid valve leaflet is considered the most reliable criterion to diagnose Ebstein's anomaly. This feature is best assessed using 2-dimensional echocardiography. However, the anatomy in Ebstein's anomaly is highly variable; therefore, the problem arises as to how to distinguish between the abnormal displacement in borderline cases of Ebstein's disease and the lowered septal offsetting of the tricuspid valve in normal persons. To solve this problem the minimal and maximal differences in offsetting of the tricuspid and mitral valves have been studied, both anatomically and echocardiographically, in fetuses, infants, children and adults. In fetuses in the first trimester of pregnancy it was impossible to measure a difference in offsetting of the 2 atrioventricular valves. Thereafter, a gradual increase occurred with age. In normal hearts the most significant separation was usually recorded in anteriorly angulated 4-chamber views, whereas in hearts with Ebstein's anomaly maximal separation appeared to posteriorly angulated views. The anatomic and echographic measurements showed a constant relation. When the minimal distances in offsetting were measured, an overlap was found between cases with and those without Ebstein's anomaly. The maximal values, however, clearly discriminated between the 2 conditions. The critical difference in children was 15 mm, and in adults the discriminating value was 20 mm.

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In 23 patients with Ebstein's anomaly of the tricuspid valve, the functional class of the patients has been related to the echocardiographic parameters generally used to diagnose this disorder. These are the extent of apical displacement of the tricuspid valve and the delay in tricuspid valve closure time related to that of the mitral valve. In addition, the functional class of the patients has also been evaluated in relation to the severity of tricuspid valve insufficiency and to the presence or absence of associated cardiac abnormalities. The study revealed that the echocardiographic parameters i.e. the degree of apical displacement of the tricuspid valve and the delayed closure time of the tricuspid valve have no predictive value for the patient's clinical condition. On the other hand, both tricuspid valve insufficiency and the presence of additional anomalies have a direct correlation with the well-being of the patient.

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In this paper we report the sequelae of a patient with an anomalous right coronary artery (RCA) originating from the pulmonary artery (PA) in association with a normal heart, operated upon at the age of 13 years. Three years after the end-to-side reimplantation of the RCA, with a rim of the PA, into the aorta, the surgical result has been evaluated by cineangiography. Before operation both coronary arteries were tortuous and increased in size. Afterwards the left coronary artery showed a normalized calibre, although the RCA remained tortuous with no decrease of the internal diameter. The notable postoperative changes in shape and size of the LCA may be due to the disappearance of the steal phenomenon. The lack of involutive changes in the RCA could be explained by its thinner wall. Left ventricular wall motion, evaluated under resting conditions and during an atrial pacing stress test, was found to be normal.

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A rapid, simple and low-cost method is presented for intracardiac left-to-right shunt quantification in children with congenital heart disease. The percentage of shunt is calculated from data obtained by continuous PO2 measurement during oxygen inhalation, using a nondisposable intravascular PO2-electrode cardiac catheter. These values are compared with those obtained by the dye-dilution method (correlation coefficient r = 0.89) and the Fick method (correlation coefficient r = 0.98). The PO2 measurement proved reliable for small, medium, and large sized shunts.

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Over a period of 3 years, 21 infants under 3 months of age were operated upon for coarctation of the aorta by subclavian flap aortoplasty. Associated intracardiac defects were present in 13 patients (62%), VSD being the most frequently encountered (10 patients, 48%). In 8 patients coarctation of the aorta, with or without patent ductus arteriosus, was the only cardiovascular malformation. The indication for surgical treatment was intractable congestive heart failure despite intensive medical treatment. Eight patients were on mechanical ventilatory support prior to surgery. There was no early operative mortality. One patient died in congestive heart failure due to valvular aortic stenosis 3 months after surgery for correction of the coarctation. During a follow-up of from 2 months to 3 years, 5 patients underwent a second operation for correction of intracardiac defects. Nineteen of the surviving 20 patients are in good clinical condition. One patient has clinical evidence of residual coarctation; a blood pressure difference of more than 10 mmHg between right arm and leg is presented in 4 patients. Early subclavian flap aortoplasty is recommended for patients with coarctation of the aorta, with or without associated intracardiac defects, who remain in congestive heart failure despite medical therapy.

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Cross-sectional echocardiographic studies were used to measure and compare the internal diameter of both aortic root and pulmonary artery in 104 patients with normally related great arteries. Six groups of patients were assessed: normal, with an intracardiac shunt, with tetralogy of Fallot, with pulmonary stenosis, with aortic stenosis, and with atresia of the right atrioventricular valve orifice. In addition, a postmortem study was carried out in normal heart specimens and in specimens with an atrioventricular septal defect. It appeared that the echocardiographically studied ratio between the aortic and pulmonary artery diameter varied considerably. In normal subjects the pulmonary artery diameter tends to be slightly larger than the aortic root diameter: this observation was confirmed by the postmortem data. In other instances the pulmonary artery diameter appeared to be smaller than the aortic root diameter, for example patients with tetralogy of Fallot and those with tricuspid atresia, whereas the reverse was noted in patients with an intracardiac shunt. This two dimensional study has shown that functional adaptations of the calibre of the great arteries can be measured and identified in patients with congenital heart disease.

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Clinical and echocardiographic findings of a six-month-old female with the cardiac variety of glycogen storage disease II type (Pompe's disease) are described. Obviously thickened right and left ventricular walls were detected with both M-mode and two-dimensional echocardiography. Echocardiographic features were similar to those seen in extreme hypertrophic cardiomyopathy. In combination with the clinical and biochemical features, this echocardiographic technique may be helpful in the early diagnosis of cardiac involvement in generalized glycogenosis.

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Two siblings were born with congenital heart block, and one suffered from severe hydrops fetalis, although maintaining a higher heart rate than the other. The association of hydrops fetalis with disorders of cardiac rhythm is unlikely to be simply a manifestation of congestive cardiac failure in the fetus.

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Necrotising enterocolitis occurred in 3 infants after angiography at cardiac catheterisation. It is suggested that hypertonic contrast medium might have been responsible and that this complication could be avoided by the use of nonionic contrast media.

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The aim of this study was to determine the accuracy of range gated pulsed Doppler (RGPD) echocardiography for detecting obstruction to the pulmonary outflow tract in children with d-transposition of the great vessels (d-TGV). Twenty-one children were randomly selected for those available with d-TGV and were studied by precordial and suprasternal RGPD echocardiography. Three were excluded, leaving a population of 18 subjects. The exclusive criterion used to judge the RGPD results was the output of the time interval histogram (TIH). Coherence of the TIH was considered to represent laminar flow. Dispersion of the TIH was considered evidence of flow disturbance and obstruction to the outflow tract. With the range gating feature, the first site of disturbance could be localized. Information was handled by a technique that decreased bias. RGPD results were then compared to diagnoses of the outflow tract established at cardiac catheterization or operation. Comparison of these results indicated that all seven children with obstruction were correctly identified by RGPD study, and the level of the first obstruction was correctly identified. With one exception, all children without pulmonary obstruction were correctly identified by the examination.

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The primary purpose of this study was to attempt to select, by examination of the time interval histogram (TIH) output of a range gated pulsed Doppler (RGPD), all children with left-to-right shunt at the atrial level from a pool of 57 children. Fifty-four of the children had various forms of acyanostic cardiac disease. A secondary purpose was to identify any associated lesions in those children with atrial defects. Examiners were unfamiliar with the children and their diagnoses. Results were interpreted independently by two examiners. Detection of diastolic TIH dispersion was used when studying the right atrial outflow tract to separate children with atrial left-to-right shunts from control children. All controls were judged negative by this technique, and 13 of 14 children with atrial shunts were detected by both examiners; the 14th was detected by one examiner. Of a total of 308 TIH decisions on the atrial shunt group, 298 were made identically by both examiners for a 97.7% agreement, demonstrating the objectivity of the method. This study demonstrated the usefulness of the TIH evaluation, indicating that continued investigation and equipment improvements are warranted.

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Idiopathic infantile arterial calcification is a disorder of unknown etiology manifested by widespread arterial calcification. This usually leads to early death from coronary artery occlusion. In 12 of the 75 cases in the literature, radiographs were taken and it was possible to make the diagnosis in them all. We present two patients, siblings, in whom the diagnosis was established radiologically. The nature of the calcium deposits was studied in one of the infants and proved to be calcium hydroxyapatite. Therapy with diphosphonate was apparently successful in the other child.

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