Biomedical subjects
Publications and source records attributed to M de Leval.
In 3 infants, severe airways obstruction was caused by mediastinal lesions which were not evident on the antero-posterior chest radiograph. Their presence was demonstrated by barium swallow examination. Each infant had thoracotomy carried out urgently. Duplication cysts (without associated cervicodorsal vertebral anomalies) were present in 2 patients and neuroblastoma in the third.
The long-term follow-up of six patients operated on for aorto-left ventricular communication has been reviewed in detail. All had residual aortic regurgitation after the initial repair of the defect. It was severe in four and required repeated reoperation in three with ultimate aortic valve replacement. The failure of early repair to solve the haemodynamic problem has provoked a reconsideration of the basic anatomy, of the surgical approach, and of the postoperative physiology of this anomaly. The so called "tunnel" is not a tunnel with length but should be considered as a localised breach at the insertion of the right coronary cusp. The localised aortic root dilatation at the site is a weakness that remains after closure of the tunnel leaving a poorly supported aortic valve and a weak root. Thus, the initial repair of the aorto-left ventricular communication must not only close the communication but reinforce, strengthen, and support the right aortic sinus in order to maintain cusp competence.
Between January 1965 and December 1979, in our Unit 53 patients underwent Mustard's operation for TGA, intact ventricular septum and LVOTO. There were 35 males and 18 females, ranging in age from 27 days to 12 years (mean = 26 months) and in weight from 3.7 to 26 kg (mean = 9.6 Kg). Twenty-one infants had undergone previous palliative procedures. The degree of LVOTO was mild in 26 cases, moderate in 10 and severe in 17 patients. In 28 cases no distinct anatomical obstructions were identified. In the other 25 patients the obstruction occurred at various level and it was determined by several anatomical structures. Subvalvular fibromuscular narrowing, redundant mitral valve, valvular stenosis and subvalvular fibrous shelf were the commonest forms encountered. The LVOTO was managed in several different ways. In 28 patients in whom the gradient was judged to be functional, no surgical intervention at the level of the LVOT was attempted. In 8 cases, all operated on at the beginning of this experience, an anatomic obstruction was present but was considered too difficult to be resected and, therefore, was left untreated. In 9 patients the LVOT was inspected either through the pulmonary valve or from below, through a left ventriculotomy. Pulmonary valvotomy was carried out in 5 of these cases, resection of the subvalvular fibrous shelf in 3 and subvalvular fibromuscular tunnel resection in 1. In 6 patients a left ventricle to pulmonary artery conduit was used to bypass the obstruction. A fibromuscular tunnel type of obstruction was present in 5 of these cases, while in one the obstruction was mainly due to a redundant mitral valve.(ABSTRACT TRUNCATED AT 250 WORDS)
Renal function was studied in 36 children who were randomly allocated to receive either gentamicin and cloxacillin or cephalothin as prophylactic antibiotic cover for complex cardiopulmonary bypass surgery. Both groups of children developed a similar degree of impairment of glomerular function with significant elevations in plasma creatinine concentrations and urine albumin excretion compared to preoperative levels which tended to resolve by the 5th postoperative day. The urine excretion of N-acetyl-glucosaminidase increased in both groups postoperatively but remained significantly elevated only in those children who had received gentamicin. These findings suggest that whilst gentamicin exerts a demonstrable nephrotoxic effect on the proximal renal tubules it does not contribute significantly to either the incidence or severity of postoperative renal glomerular impairment.
Between August 1980 and July 1982, 70 children were operated on for congenital heart defects without the use of cardiac catheterization and/or angiocardiography (7.5% of the total of 929 operations). The diagnosis was established by clinical examination, chest x-ray, electrocardiography, and comprehensive cross-sectional echocardiography. The following lesions were repaired: coarctation of the aorta in infancy (n = 28), total anomalous pulmonary venous drainage (n = 6), aortic valvular/subvalvular lesions (n = 11), mitral and tricuspid valve lesions (n = 4), simple transposition of the great arteries (n = 3), vegetations (n = 3), persistent truncus arteriosus (n = 2), and others (n = 13). Three diagnostic errors occurred, and in four children the initial diagnosis was not complete. No child died as a consequence of an error or incomplete diagnosis, and in only one instance was an inappropriate operation carried out (abdominal coarctation of the aorta). We conclude that operation on selected patients can be safely performed on the basis of noninvasive investigation.
Five patients with isolated clefts in the anterior leaflet of the mitral valve, unassociated with atrioventricular septal defects, are described. All had significant mitral regurgitation, with the cleft being the only abnormality in three. Two patients had an associated ventricular septal defect, one with a straddling right atrioventricular valve. Angiocardiography in four showed moderate regurgitation, but was not able to delineate the aetiology. Two dimensional echocardiography showed a constant defect in the anterior leaflet, pointing towards the left ventricular outflow tract. This differed from 30 cases with atrioventricular septal defects where the "cleft" pointed towards the interventricular septum and was situated between the anterior and posterior bridging leaflets. All cases with isolated clefts had surgical correction, with minimal residual regurgitation on follow-up examination in two cases. Our current policy in patients with uncomplicated isolated cleft involves non-invasive assessment of these children and surgical correction if the regurgitation is significant.
A 3-day-old infant investigated for cyanotic heart disease was found to have partial DiGeorge syndrome and pulmonary atresia with ventricular septal defect. The only source of pulmonary blood flow was a right-sided persistent ductus arteriosus which originated from an aberrant right subclavian artery. Such a vascular ring has not been described previously. Surgical treatment included a left-sided prosthetic shunt in infancy followed by resection of the ligamentum arteriosum and right subclavian artery during the second year of life.
To determine the sensitivity and specificity of cross-sectional echocardiography in diagnosing anomalous systemic venous return we used the technique in 800 consecutive children with congenital heart disease and whom the diagnosis was ultimately confirmed by angiography. Cross-sectional echocardiography was performed without prior knowledge of the diagnosis in all but 11 patients, who were recalled because of a known abnormality of atrial situs. The sensitivity of cross-sectional echocardiographic detection of various structures was as follows: right superior vena cava 792/792 (100%); left superior vena cava 46/48 (96%); bilateral superior vena cava 38/40 (95%); bridging innominate vein with bilateral superior vena cava 13/18 (72%); connection of superior caval segment to heart (coronary sinus or either atrium) (100%); absence of suprarenal inferior vena cava 23/23 (100%); azygos continuation of the inferior vena cava 31/33 (91%); downstream connection of azygos continuation, once seen, 21/21 (100%); partial anomalous hepatic venous connection (one hepatic vein not connected to the inferior vena cava) 1/1 (100%); total anomalous hepatic venous connection (invariably associated with left isomerism) 23/23 (100%). The specificity of each above diagnoses was 100% except in one infant with exomphalos in whom absence of the suprarenal inferior vena cava was incorrectly diagnosed. Thus cross-sectional echocardiography is an extremely specific and highly sensitive method of recognizing anomalous systemic venous return. It is therefore of great value of planning both cardiac catheterisation and cannulation for open heart surgery.
Sixty patients with pulmonary atresia and intact ventricular septum (PA:IVS) presenting from 1970 to 1980 are reviewed. Three groups of patient are discussed: those with tripartite right ventricles, those with no trabecular portion to the cavity, and those with neither trabecular nor infundibular portions. The decrease in early mortality for neonates with PA:IVS since 1977 (one death in 15 patients) supports our current management policy of preoperative prostaglandin E1 infusion with transpulmonary valvotomy (for patients with an infundibular cavity) combined with a left modified Blalock-Taussig shunt using a Gore-Tex prosthesis. Tricuspid valve growth, estimated by serial angiograms in 12 patients, was greater if right ventricle-to-pulmonary artery continuity was established. Later definitive repair was attempted in nine patients, with two early deaths; five underwent right ventricular outflow tract reconstruction and four had modified Fontan procedures. Neonates with critical pulmonary stenosis are also discussed. Their neonatal mortality (nine deaths in 20 patients) was similar to that of comparable patients with PA:IVS, but their actuarial survival at 5 years (55%) was superior (36% at 5 years).
Closing the ventricular septal defect in patients with atrioventricular discordance with sutures placed through the defect onto the morphologically right septal surface should avoid production of complete heart block. To discover whether this procedure otherwise affects conduction, standard electrocardiogram (ECG) were compared preoperatively and postoperatively in 11 such patients. Operation had lengthened the PR interval in one and shortened it in another. The QRS interval remained unchanged in 5 patients thought 2 of these showed minor changes in QRS configuration. Four patients showed QRS prolongation (increase greater than 20 msec) with delayed conduction towards the right ventricle and the initial QRS vector preserved, i.e., morphologically right bundle branch block. Two patients showed QRS prolongation with initial QRS vector alteration and delayed conduction towards the left ventricle, i.e., morphologically left bundle branch block. Repair through the morphologically left ventricle invariably caused bundle branch block. Repair through a right atriotomy caused bundle branch block in only 3 of 8 patients. Therefore closure of a ventricular septal defect by this method, particularly transatrially, need not affect conduction. Morphologically left ventriculotomy rarely if ever causes morphologically left bundle branch block. When present, morphologically right bundle branch block presumably results from interruption of the proximal right bundle.
The left pulmonary artery and ductus arteriosus were ligated in 14 pigs at birth. Animals were sacrificed at intervals from 2 to 24 weeks of age. In the right lung the pulmonary artery and in the left, either the distal pulmonary artery, bronchial arteries or both were injected. The fixed lung specimens were studied by arteriography, dissection and microscopic examination of serial and random sections of lung tissue. The bronchial arterial circulation to, and within the right lung appeared normal and was similar to that described in the human lung. In the left lung, the bronchial arterial circulation hypertrophied rapidly during the first 2 weeks, and large anastomoses between pulmonary and systemic circulations were found at the same sites as in the normal pig lung. The position and structural characteristics of the anastomosing arteries is described in the different types of broncho-pulmonary connection. In most animals aged 16 weeks or more, peripheral bronchial arteries immediately proximal to the anastomotic sites, developed intimal and medial proliferation. The left lung continued to grow although in all animals it was small. The axial pulmonary artery and its branches became smaller with age. These findings help explain how the lung is perfused and grows in children with congenital heart disease and an acquired collateral pulmonary arterial circulation.
Although it is a common congenital cardiac lesion, secundum atrial septal defect (ASD) rarely presents in infancy. Its natural history in the first two decades of life is generally benign. Reports of symptomatic ASD in early childhood stress the place of conservative management, and recommend that the operation should be deferred until school age. Few accounts of the indications for surgery in infancy exist. We present our experience of 6 patients who failed to respond adequately to conservative treatment and required operation for intractable heart failure due to isolated secundum ASD in the first year of life. As their presentation and physical signs were not typical of ASD seen in later childhood, they presented problems both of diagnosis and management.
In nine patients with pulmonary atresia, ventricular septal defect, and major aortopulmonary collateral arteries, the effect of a systemic-pulmonary anastomosis on the lung was studied by comparison of pre- and postoperative cine angiograms. Selective injections into the collateral arteries were performed in all patients and the source of blood supply to each bronchopulmonary segment was sought. Central pulmonary arteries were present in six children and absent in three, as confirmed at thoracotomy. After insertion of a shunt, central pulmonary arteries increased in size, but the intrapulmonary vessels with which they connected remained abnormally small and were frequently stenosed. In addition, in each case the central pulmonary arteries increased flow to only five to 11 segments of lung. In the absence of central pulmonary arteries, shunting to a hilar or a lobar pulmonary artery increased perfusion to five to nine segments of lung per case. In all these three cases the pulmonary arteries at lobar level showed aneurysmal dilatation proximal to a severe stenosis. Sixty-eight per cent of collateral arteries were stenosed. The findings suggest that in this anomaly, when the central pulmonary arteries are hypoplastic, the intrapulmonary branches are also hypoplastic, emphasising the need for early surgical intervention to increase blood flow while the lung still has growth potential. Further, one collateral artery may connect with at least as many bronchopulmonary segments as does a central pulmonary artery, and the peripheral intrapulmonary arteries with which it connects may appear at least as normal angiographically as do vessels connected to central pulmonary arteries. These observations suggest that segments of lung connected to collaterals alone should, and sometimes can, be connected to central pulmonary arteries, the aim being to produce a unifocal blood supply as a prelude to total correction.
Formalin infiltration of the ductus arteriosus was performed in 13 neonates with pulmonary atresia (three with ventricular septal defect, two with tricuspid atresia, and eight with intact ventricular septum, one of whom had Ebstein's anomaly) in an attempt to maintain duct patency. Nine patients had an additional retrograde pulmonary valvotomy and one a Blalock-Taussig shunt. The mean preoperative systemic PO2 was 31 mmHg and rose significantly to 46 mmHg after operation. However, the PO2 rose in only two of the four patients who had formalin infiltration alone. There were five early deaths and three had clinical evidence of duct closure. Four patients needed further surgery (systemic-pulmonary artery shunt) within eight days. Seven of eight surviving patients were shown to have a closed duct at repeat cardiac catheterisation (two to 13 months after operation). The eighth died before restudy. There were four late deaths, one following late reoperation and the other three within five months of early reoperation. These results suggest that formalin infiltration cannot ensure long-term duct patency. As short-term patency can be maintained more reliably by prostaglandin administration, we have abandoned the operation.
The left pulmonary artery and ductus arteriosus were ligated in 14 newborn pigs. Animals were put to death at intervals between 2 and 24 weeks of age. In each animal a cardiac catheterization was performed before sacrifice. Pulmonary arterial development in both lungs was studied by applying quantitative morphometric techniques to the infected and inflated specimens. In the right lung, pulmonary arterial pressure and resistance fell to normal after birth; however, structurally, muscularity remained high in arteries less than 75 mu in diameter. Size and number of intra-acinar arteries increased normally, not excessively, with age despite the abnormally high flow. After 16 weeks in all animals, muscularity increased significantly in arteries of all sizes; mean pulmonary arterial pressure was 20 to 35 mm Hg, the pulmonary--systemic pressure ratio was 0.21 or more, and total pulmonary vascular resistance was 0.16 to 0.4 torr (ml/min/kg) (normal, 0.08). In the left lung, the axial pulmonary artery and its branches were small, decreasing in size with age, and its elastic wall structure was disorganized. Small preacinar arteries showed obliterative change in the majority of animals put to death at 16 weeks or more. Within the acinus, however, the bronchial arterial blood supply ensured almost normal postnatal pulmonary arterial development, the vessels increasing in size and number although muscularity was reduced. Thus failure to perfuse one pulmonary artery in the immature animal changes growth and development of both lungs, and the findings are discussed in relation to the hyperperfused and hypoperfused lung in childhood.
A 13-year-old girl presented with infective endocarditis of the tricuspid valve related to a residual ventricular septal defect. Antibiotic therapy produced a satisfactory initial improvement, however, subsequent deterioration with uncontrolled infection, in spite of alterations in the antibiotic regime, necessitated surgical intervention. Because the endocarditis was localized to the septal leaflet, management by excision of this leaflet and annuloplasty was possible and resulted in a rapid and sustained clinical improvement with no evidence of significant residual valvar incompetence. The medical management of infective endocarditis requires bacteriological assessment to ensure that the drugs and dosages selected are appropriate and adequate for the causative organism. Early operation may be required for uncontrolled infection or cardiac decompensation; in tricuspid endocarditis secondary to a ventricular septal defect, the operation described permits removal of infective tissue while retaining valvar competence.
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Since 85 to 90% of children with d-TGA die in the first year of life, treatment of this malformation is mandatory before age one. Even after Rashkind septostomy, 50% of the patients die before 2,5 years of age. In Great Ormond Street Hospital for Sick Children all the patients with d-TGA have a balloon septostomy performed at birth. Then at 4-5 months of age all patients are evaluated with cardiac catheterisation and angiography. If the patient is doing well, surgery at atrial level (Mustard or Senning operation) is performed between 6 and 12 months of age. But, if despite Rashkind atrioseptostomy, the child remains cyanotic the authors do not perform Blalock Hanlon operation and prefer to do a repair at atrial level. If the child presents with d-TGA and PDA the ductus is ligated during the neonatal period and the repair at atrial level is performed before 6 months of age. In children with VSD associated with d-TGA, the authors prefer to do a Mustard or a Senning operation with closure of the VSD, and so before 6 months of age. In patients with left ventricular outflow tract obstruction, the authors do a Blalock Taussig shunt in the cases with VSD and try a repair at atrial level in the cases with intact septum. The authors discuss the results, not only in term of mortality but also in terms of post-operative complications.