Michael Joseph

Embolisation procedures in congenital heart disease

Eight therapeutic embolisation procedures were performed by the transcutaneous catheter technique in seven patients with congenital heart disease. After surgical correction of tetralogy of Fallot (four patients), catheter embolisation was used to occlude two large aortopulmonary collaterals (one patient), three small aortopulmonary collaterals (one patient), and two Blalock-Taussig shunts (two patients). In two patients congenital coronary anomalies were occluded--a coronary arteriovenous malformation and a coronary artery/bronchial artery anastomosis. In one patient a pulmonary arteriovenous malformation was embolised. Detachable balloons were used to occlude six large arteries, the three small arteries were occluded with small gelfoam fragments, and the pulmonary arteriovenous malformation was occluded with multiple steel coils and large gelfoam pieces. Successful occlusion was achieved in all cases. No complications were encountered and the procedure was well tolerated even in the two patients receiving postoperative intensive care. Therapeutic embolisation in suitable cases is a safe and effective alternative to surgery and the detachable balloon technique is effective in occluding high flow vessels.

Surgical risk factors in total anomalous pulmonary venous connection

Eighty-three patients underwent surgical correction of total anomalous pulmonary venous connection (TAPVC) between 1973 and 1986. There were 46 boys and 37 girls. Median age at operation was 60 days (1 to 240) and median weight 3.9 kg (1 to 22). The anatomic types encountered included infracardiac connection (16 patients), supracardiac connection (32) and pulmonary venous drainage connected directly to the coronary sinus (27). Mixed anomalous drainage or pulmonary venous return connected directly to the right atrium occurred in 8 patients. Diagnosis was established by cardiac catheterization and angiography (56 patients), clinical examination (3) and cross-sectional echocardiography alone in 24 of the last consecutive 28 patients. Pulmonary hypertension was present in 26 (55%) of those who underwent cardiac catheterization. The median pulmonary vascular resistance was 4.2 units/m2 (body surface area) for all the patients, whereas in those with infracardiac pulmonary venous connection the median value was 10 units/m2. The median interval between admission and operation was 72 hours. Surgical correction was performed using profound hypothermia and circulatory arrest in 68; for the remainder, conventional cardiopulmonary bypass with profound to moderate hypothermia was used. Ten patients developed 1 or more pulmonary hypertensive crises during the early postoperative period. These were diagnosed in 8 by direct pulmonary artery pressure measurement and in 2 by clinical examination. Late reoperation was necessary in 6 patients (10%). Analyses of risk factors for 30-day survival for all patients showed that the risk of early death was associated with the type of anomaly (infradiaphragmatic), occurrence of pulmonary hypertensive crises, year of the operation, set of the patient and pressure of preoperative congestive heart failure.(ABSTRACT TRUNCATED AT 250 WORDS)

Cardiac rhythm and conduction before and after Mustard's operation for complete transposition of the great arteries.

Between 1973 and 1978, 120 patients with transposition ofthe great arteries had a modification of Mustards operation designed to avoid damage to the sinus and atrioventricular nodes. Of these, 88 had an atrial baffle alone from which nine hospital and four late deaths occurred (total mortality 14.8%); 32 had additional closure of ventricular septal defect and/or relief of left ventricular outflow tract obstruction from which six hospital and three late deaths occurred (28%). On the standard electrocardiogram, 101 of 105 patients (96%) discharged from hospital were in sinus rhythm, but of the four patients with arrhytbmias two had conduction abnormalities before operation. A further two patients with complex procedures had acquired bifascicular block. Twenty-fourhour electrocardiograms in 19 patients who had both preand postoperative recordings showed acquired arrhythmias in one of 13 who had simple Mustards operation and in two of six who had additional procedures. Of a further 39 patients who had only postoperative 24-hour recordings, three of 30 with a simple operation and three of nine with additional surgical procedures had arrhythmias that were thought to be acquired. Rhythm patterns and rates previously considered to be surgically acquired were found before operation on 24-hour electrocardiograms in patients with complete transposition and have now been reported to occur in normal infants and children. There is thus no evidence to support the view that such episodes with the electrocardiographic patterns of Wenckebach, 2:1 or complete sinuatrial block, atrial premature beats of less than 12 per hour, or intermittent junctional escape rhythms represent acquired cardiac arrhythmias. Seven of the 22 deaths occurred after leaving hospital and included five which were sudden and unexpected, no adequate cause being found at necropsy. Four of these had conduction disorders or arrhythmias on standard and 24-hour electrocardiograms including three with complex procedures. Our results indicate that a modified Mustards operation may reduce the frequency of surgically acquired arrhythmias.

Two dimensional echocardiographic categorisation of the univentricular heart. Ventricular morphology, type, and mode of atrioventricular connection.

Most univentricular hearts have two chambers in their ventricular mass, only one of which possesses an atrioventricular connection. Categorisation into univentricular heart of right, left, or indeterminate type, using two dimensional echocardiography has been successfully achieved in 122 out of 132 patients to whom this technique has been applied. Thus, right ventricular rudimentary chambers in 84 univentricular hearts of left ventricular type were shown to be anterosuperior and either to the right or left. In contrast, left ventricular rudimentary chambers in 25 univentricular hearts of right ventricular type were posteroinferior and to the right, left, or directly posterior. Thirteen univentricular hearts of indeterminate morphology were characterised by absence of rudimentary chamber on angiography and echocardiography. The trabecular pattern of both main and rudimentary chambers were separately identified in some of the patients with univentricular hearts of right and left ventricular type and two dimensional echocardiography also illustrated the mode of atrioventricular connection, either via two atrioventricular valves, a common valve, or a valve straddling or overriding the trabecular septum. Finally it was also possible to distinguish absent atrioventricular connection from an imperforate valve in 10 patients.

Recognition of imperforate atrioventricular valves by two dimensional echocardiography.

The majority of hearts in which selective right or left atrial angiography shows no direct communication between one atrium and a ventricular chamber are considered to be examples of atresia of the right or left atrioventricular valves. Most patients presenting with the clinical features of tricuspid atresia have an absent right atrioventricular connection, while those with features of mitral atresia and a normal aortic root frequently have an absent left atrioventricular connection. By studying 67 patients using two dimensional echocardiography we have identified 12 in whom there was an imperforate atrioventricular valve and not an absent atrioventricular connection. Thus, in 44 hearts with the angiographic features of tricuspid atresia, 36 had absence of the right atrioventricular connection and six had an imperforate right atrioventricular valve. In the latter, four were examples of atrioventricular concordance and two were univentricular hearts of left ventricular morphology in which the mode of atrioventricular connection was through one perforate and one imperforate valve. Similarly, 17 out of 23 cases of mitral atresia had absence of the left atrioventricular connection. The remaining six had an imperforate left atrioventricular valve which was associated with atrioventricular concordance in two patients and with double inlet univentricular heats in four patients.

Eye Defects in Children with Congenital Heart Lesions: A Preliminary Study

Eighty‐five children aged 6 years and over, with various types of congenital heart lesion were given a full ophthalmic examination. The incidence of the types of eye defect found is tabulated and discussed, and the conclusion is reached that children with cyanotic heart disease have more eye defects of all sorts than those with obstructive heart lesions or left to right shunts.

An assessment of a stereophotogrammetric technique for the study of facial morphology in the child.

An assessment has been made of a stereophotogrammetric technique (Beard and Burke, 1967) for measuring and contouring the face. Its contouring capabilities depend on the skin surface texture of the subject. Adults have good skin texture and accurate contours can be drawn. Young children cannot be contoured satisfactorily because they lack adequate skin texture, and only linear measurements can be obtained with precision. The disadvantage of incomplete facial coverage was eliminated by using a pair of oblique exposures instead of the conventional frontal exposure. The need for mechanical head positioning was avoided by use of a sighting device incorporated in the system to orientate each exposure to a common datum.

SEQUELAE OF INFANTILE HYPERCALCAEMIA

Fig. 1. Effect of overacting hamstrings on the tibia during walking. The crouch position is caused by the hip and knee flexion, which must be corrected if walking and posture are to be improved. a, quadriceps femoris; b, hamstring; c, soleus. (Figs. 1 and 2 are reproduced,from the Journal of Bone and Joint Surgery by kind permission of the publishers.) Fig. 2. When the hamstrings are transplanted to the femoral condyles they no longer flex the knees, and the quadriceps muscle, released from the retinacula, extends the knee-joint. With the knee extended, the transplanted hamstrings rotate the pelvis, extend the hip, and permit a more nearly normal erect posture. The heel is in an improved walking position.

A Numerical Evaluation Of A Facial Pattern In Children With Isolated Pulmonary Stenosis

A facial contouring technique, using light sectioning by Coob was modified by Ainsworth and Joseph and used in a numerical study of children with isolated pulmonary stenosis (PS) to test the hypothesis that the facial pattern in this condition differs from the normal. Measurements were compared between a group of 20 normal children, and a group of 20 children with PS between the ages of 6 and 10.5 years. A distinctive facial pattern has emerged. Many anteroposterior measurements were significantly greater in the PS group, indicating that the tissues are more prominent in the maxillary region. Twenty-nine of the measurements showed significant differences between the two groups (P <.05). Discriminant analyses were carried out to discover which, if any, might be used to predict the group to which an individual should belong. Depending on the variables chosen, between 34 and 37 individuals from the total of 40 were assigned to their correct group, PS or control.

A light-sectioning technique for contouring and measuring a child's face.

A contouring device for measuring the face (Cobb, 1972) was adopted and developed to make it suitable for use with children. Modifications were made to overcome incomplete facial coverage and lack of common head orientation by incorporating a method of optical head positioning which had been invented during the assessment of a stereophotogrammetric technique (Ainsworth and Joseph 1976). The precise alignment of a series of exposures with a common datum enables a set of contour maps to be processed by computer to produce three-dimensional measurements and sections of the complete face.