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Biomedical subjects

M R de Leval

Publications and source records attributed to M R de Leval.

At least 19 recordsLinked to original sources

Pulmonary artery sling.

Eighteen patients who underwent surgical treatment of pulmonary artery sling at this institution since 1969 were reviewed. Four of them had associated tracheal stenoses produced by complete cartilaginous rings (stovepipe trachea). All patients underwent operation. The median age at operation was 180 days (range, 27 days to 54 months). In the 14 patients with isolated pulmonary sling, the operation consisted of division of left pulmonary artery and reimplantation into the main pulmonary artery anterior to the trachea. In the 4 patients with stovepipe trachea, the stenotic tracheal segment was resected on cardiopulmonary bypass and the left pulmonary artery was brought anterior to the trachea before the latter was reanastomosed to the main pulmonary artery. There were no early deaths. One patient died late. The pulmonary artery anastomosis was patent in all 14 patients investigated postoperatively. Three patients have residual tracheobronchial problems. One patient is mentally retarded and is institutionalized. All other patients are symptom-free and growing normally and have normal chest radiographs.

Child, Preschool

Early and late arrhythmias after the Fontan operation: predisposing factors and clinical consequences.

OBJECTIVE: To study the incidence, predisposing factors, and clinical significance of arrhythmias early and late after the Fontan operation for congenital heart disease. PATIENTS AND METHODS: All 104 consecutive patients undergoing Fontan repair from 1975 to 1988 were studied retrospectively. Hospital records were reviewed for perioperative arrhythmia. Clinical information and annual electrocardiograms were available for all 78 hospital survivors during a follow up of up to 13 years (mean 3.7 years). Ambulatory electrocardiographic monitoring was performed in 67 patients (81%). RESULTS: Eleven patients (10.6%) developed a perioperative tachycardia (eight, atrial flutter; three, His bundle tachycardia). Multivariate analysis showed that raised preoperative mean pulmonary artery pressure and low aortic saturation were significant risk factors for the development of atrial flutter (r2 = 0.32, p = 0.0001) but not for His bundle tachycardia. Despite intensive medical treatment 10 of these 11 patients died. At the last visit 72 (92%) of the 78 patients were in sinus rhythm on their standard 12 lead electrocardiogram. Junctional rhythm was present in three patients, two patients had atrial flutter, and one had a paced rhythm. Ambulatory monitoring did not show important bradycardia or ventricular arrhythmias. Actuarial survival free of supraventricular arrhythmia was 82% at eight years after operation. Multivariate analysis identified older age, increased right atrial size, and raised mean preoperative pulmonary artery pressure as risk factors for arrhythmia during intermediate follow-up (r2 = 0.46, p less than 0.001). Late tachycardias, in contrast to those occurring in the perioperative period, were not associated with an increased mortality. CONCLUSIONS: Except for his bundle tachycardia in the perioperative period, early and late arrhythmias after a Fontan operation seem to be a consequence of adverse preoperative and postoperative haemodynamic function. The perioperative outcome is therefore poor even when the patient can be restored to sinus rhythm. Medical and surgical modifications to improve the haemodynamic disturbances associated with arrhythmias are therefore indicated.

Analysis of Variance

Current surgical technique to repair Fallot's tetralogy with absent pulmonary valve syndrome.

The experience with surgical repair of Fallot's tetralogy and absent pulmonary valve syndrome is reviewed. Twenty-two patients aged 1 day to 8 years were treated between 1982 and 1989 using one surgical technique. This consisted of resection of the main pulmonary artery and large parts of the anterior wall of the right and left pulmonary artery. The ventricular septal defect was closed with a patch, and an aortic or pulmonary homograft (size, 8 to 24 mm) was interposed between the right ventricle and the pulmonary artery. Two of the 8 infants operated on died; there was one late death. All 14 older children survived the operation. All survivors are well up to 7 years follow-up (mean follow-up, 3.6 years). We recommend early treatment of infants; older children can be treated electively. The technique used in our series gives excellent results.

Child

Pulmonary hypertensive crises following surgery for congenital heart defects in young children.

In this clinical study, 20 high risk infants and neonates were monitored to identify and characterize pulmonary hypertensive crises following surgery for congenital cardiac defects. Monitoring included right ventricular or pulmonary artery pressure catheters and transcutaneous oximetry. Eleven patients also had continuous analog recording of hemodynamic data so that antecedents of crises and the sequence of events following treatment could be reconstructed. Eleven of the 20 patients had one or more crises. Six of these ultimately died whereas 5 patients survived with aggressive vasodilator therapy. Four patients without crises but with episodic pulmonary hypertension benefitted from pulmonary vasodilator therapy to ease weaning from ventilatory support. Typically, each crisis was associated with a stress event. Crises were difficult to ablate if not rapidly treated and multiple crises would often cluster following an initial event. High dose narcotic (fentanyl) analgesia was found to be important in the postoperative management. Tolazoline and oxygen were the most consistently useful vasodilators, but isoproterenol and nitrates also played a role. Five of the children who died were examined post mortem: histologically, there was increased pulmonary arterial muscularization in 2, in none were there changes of fixed pulmonary vascular disease. The postoperative management must be individualized on the basis of monitored responses of pulmonary circulation.

Biopsy

Surgical treatment of aortico-left ventricular tunnel.

Thirteen children (age range 6 h-11 years, median 4 months) operated upon for aortico-left ventricular tunnel since 1971 are reported. All patients had systolic and diastolic murmurs, 4 of 5 neonates were in severe congestive heart failure. Twelve children had left ventricular hypertrophy on ECG with an ischaemic pattern in the neonates. Chest X-ray revealed cardiomegaly with a broad upper mediastinal shadow from the enlarged aorta. Echocardiography (2DE) revealed a tubular communication between the aorta and the left ventricle bypassing the aortic valve and massive regurgitation through this tunnel demonstrated by Doppler sonography. In 8 patients, the diagnosis was confirmed by angiography. The tunnel arose from the right coronary sinus in 12 and from the left coronary sinus in 1 patient. The tunnel was closed at both ends in 8, at the aortic end only in 3 and at the ventricular end in the remaining 2 cases. One 6-day-old infant, operated upon in 1971 died postoperatively. There was 1 non-cardiac related late death due to a brain tumour. Echocardiographic analysis of left ventricular size and function was carried out in 9 children up to 39 months after surgery. Mild to moderate aortic incompetence was detected during the follow-up period in 2 of 12 patients. All surviving children are leading normal lives.

Aorta

Arrhythmias after the Fontan procedure. Comparison of total cavopulmonary connection and atriopulmonary connection.

Arrhythmia was compared between 40 consecutive patients (pts) undergoing atriopulmonary connection (APC) and 40 undergoing total cavopulmonary connection (TCPC), between 1986 and 1990. Patients were not randomized, and those undergoing APC predominantly had tricuspid atresia (57.5% versus 15%) compared with pts undergoing TCPC who had more varied and complex cardiac defects. Before surgery there was no significant arrhythmia in either group. Hospital mortality was 15/40 and 6/40 for APC and TCPC, respectively (p less than 0.05). Early after APC nine pts developed atrial flutter (AFL) and eight died, while after TCPC only two developed AFL, and neither died (p less than 0.01). Fatal junctional ectopic tachycardia occurred in one pt in each group. During follow-up (38 +/- 8.5 months after APC versus 20 +/- 10 months after TCPC), new arrhythmia was uncommon (two AFL after APC with one death versus two AFL and one supraventricular tachycardia after TCPC with no deaths). Ambulatory ECG during follow-up showed arrhythmia in two APC and six TCPC pts (p = NS). The incidence of early arrhythmia and mortality associated with early arrhythmia appears to be less after TCPC compared with APC.

Arrhythmias, Cardiac

Heart and lung transplantation for terminal cystic fibrosis. A 4 1/2-year experience.

From among 112 patients with cystic fibrosis who were assessed for heart-lung transplantation, 83 were accepted. Twenty-six died while awaiting heart-lung transplantation and 32 had the operation. The management and the outcome of these 32 patients is reported. Survival, infection, and rejection rates among these patients were compared with those of 61 patients without cystic fibrosis who underwent heart-lung transplantation between 1984 and 1990. The cumulative survival rate was 72.29% +/- 94.91% at 1 year and 55.59% +/- 7.50% at 3 years. The mortality rate was slightly higher in the group with cystic fibrosis during the first year after the operation but it was lower at 3 years. The difference, however, could have been due to chance alone (p = 0.308). The same was true for the prevalence of rejection (up to 6 months: chi 2 = 1.8141, p = 0.17), and infection (up to 6 months: chi 2 = 2.20, p = 0.14), between the two groups. It is concluded that cystic fibrosis does not constitute an additional risk in terms of survival and morbidity after heart-lung transplantation.

Adolescent

Long-term results of homograft valves in extracardiac conduits.

Between 1971 and 1986, 335 patients received various extracardiac valved conduits between one of the heart chambers and the pulmonary arteries. The group of patients who received aortic homograft conduits and survived the operation were analysed in detail. The age varied between 9 days and 18 years (mean 7.1 +/- 0.7 years), weight 2.4 kg-63.5 kg (mean 17.8 +/- 10.8 kg). The diameter of the conduit used was 10-30 mm (mean 20.8 mm). Multivariate analysis revealed a highly significant model (P less than 0.005) which showed that the time interval between harvesting and use of the homograft (P less than 0.02) and the earlier date of operation (P less than 0.05) were the major risk factors for obstruction. Homografts used within 3 weeks of harvesting had freedom from obstruction of 79% at 8 years; homografts used between 3-6 weeks had freedom from obstruction of only 55% at 8 years. Homografts used alone performed significantly better than those extended with woven Dacron tubes. At 10 years, 93% of homografts used alone were free of obstruction compared to 52% of homografts extended with a Dacron tube. We conclude that aortic homografts used within 3 weeks of harvesting provide a reasonably durable conduit for a period of 12 years. Longer storage, and extension of the homograft with a woven Dacron tube should be avoided.

Adolescent

Surgical management of doubly committed subarterial ventricular septal defects.

The clinical presentation, diagnosis, and surgical treatment of 63 patients with doubly committed subarterial ventricular septal defects (DCVSD) were analyzed retrospectively. The patients were divided into three groups. Thirty-one patients had severe congestive heart failure in infancy and presently have a large ventricular septal defect that has no tendency to close or to produce aortic valve regurgitation (Group 1). Ideally, these defects should be closed in infancy, and the transpulmonary approach is recommended to achieve closure. In the first group, there was one death in a patient with a hypoplastic right ventricle. In Group 2, nineteen patients had aortic valve prolapse or aortic valve regurgitation. The DCVSD were moderately large or small. A number of DCVSD in Group 2 patients had maintained the normal offsetting of the arterial valves. These defects must be closed by the time mild aortic valve regurgitation has occurred. In Group 2, there was one late death in a patient who developed subacute bacterial endocarditis. The two patients who had severe aortic valve regurgitation required aortic valve replacement and underwent multiple surgical procedures to replace calcified bioprostheses. Group 3 comprised 13 patients who were diagnosed with tetralogy of Fallot. All of these patients had a large DCVSD with aortic valve overriding. All Group 3 patients survived radical repair of the defect, which required a transannular patch in most cases (76%).

Adolescent

Surgical unifocalization in pulmonary atresia and ventricular septal defect. A realistic goal?

Between 1979 and 1986, 26 patients with pulmonary atresia, ventricular septal defect, and multifocal pulmonary blood supply underwent surgery (median age, 2 years) to reconnect major aortopulmonary collateral arteries to other collateral arteries or to the central pulmonary arteries in an attempt to prepare the pulmonary circulation for complete repair. In four patients, central pulmonary arteries were found to be absent at exploratory thoracotomy, and therefore, the surgery aimed for optimal palliation. Four (15%) patients died perioperatively. The survivors had symptomatic improvement. At restudy 1 week-110 months (median time, 25 months) postoperatively, modest growth of central pulmonary arteries had occurred [right plus left pulmonary artery diameters/descending aortic diameter from 0.64 +/- 0.61 (mean +/- SD) preoperatively to 0.95 +/- 0.60 postoperatively (p less than 0.01)], with a greater number of bronchopulmonary segments connected to central pulmonary arteries [from 6.3 +/- 4.8 preoperatively to 10.0 +/- 4.5 postoperatively (p less than 0.001)]. However, only seven (27%) patients met the established criteria for repair, of whom three (12%) have undergone definitive surgery. Thirteen (50%) patients are unsuitable for repair, and two (8%) await restudy. Failure of anastomoses involving major aortopulmonary collateral arteries (13 of 33) was higher than for grafts from a systemic artery to the central pulmonary artery (1 of 22, p = 0.003). Failure was not related to the number of anastomoses per operation, suggesting that poor distal runoff may have been responsible. This surgical approach provided reasonable palliation but was rarely effective as a prelude to complete repair.(ABSTRACT TRUNCATED AT 250 WORDS)

Adolescent

Total cavopulmonary connection: a logical alternative to atriopulmonary connection for complex Fontan operations. Experimental studies and early clinical experience.

To understand better the contribution of a right atrium in a valveless atriopulmonary connection, we performed some basic hydrodynamic studies. Pulsation of a valveless chamber in a simple continuous flow circuit was found to generate turbulence and thereby to increase resistance to net forward flow. Visualization of flow through cavities and around corners and measurements of energy losses across nonpulsatile cavities, corners, and stenoses indicated the importance of streamlining. These studies suggested ways in which hydrodynamic designs of the Fontan circulation might be improved. In parallel with these in vitro studies, we have developed a modified approach to Fontan reconstruction that entails exclusion of most or all of the right atrium (total cavopulmonary connection). The operation consists of three parts: (1) end-to-side anastomosis of the superior vena cava to the undivided right pulmonary artery; (2) construction of a composite intraatrial tunnel with the use of the posterior wall of the right atrium; and (3) use of a prosthetic patch to channel the inferior vena cava to the enlarged orifice of the transected superior vena cava that is anastomosed to the main pulmonary artery. The operation was performed in 20 patients between March 1987 and March 1988. The diagnoses were double-inlet ventricle (11 patients), hypoplastic systemic or pulmonary ventricle (seven patients), and absent right atrioventricular connection (two patients). There were two early deaths and one late death. None of the deaths was related to the actual procedure but rather to increased pulmonary vascular resistance (two patients) or systemic ventricular failure (one patient). Total cavopulmonary connections have the following advantages: (1) They are technically simple and reproducible in any atrioventricular arrangement and are away from the atrioventricular node; (2) most of the right atrial chamber remains at low pressure, which reduces the risk of early or late arrhythmias; (3) reduction of turbulence prevents energy losses and should minimize the risk of atrial thrombosis; (4) postoperative cardiac catheterization performed in 10 patients confirmed these favorable flow patterns with minimal gradients throughout the connections. These encouraging early results support the continuing use of total cavopulmonary connection, at least for patients with a nonhypertrophied right atrium.

Anastomosis, Surgical

Assessment of residual right ventricular outflow tract obstruction following surgery using the response to intravenous propranolol.

The response to intravenous administration of propranolol hydrochloride was studied in 24 children undergoing operation for isolated or complex right ventricular outflow tract obstruction. Ten had pulmonary valve stenosis, 4 had pulmonary valve stenosis plus ventricular septal defect, 8 had tetralogy of Fallot, and 2 had complex lesions. Propranolol (0.01 to 0.2 mg per kilogram of body weight) was given to patients with a residual right ventricular to left ventricular systolic pressure ratio greater than 0.75. Four children did not respond to propranolol and required further surgical intervention. Twenty patients responded, 2 of whom died. Fifteen were restudied by cardiac catheterization 3 weeks to 27 months postoperatively. The right ventricle-pulmonary artery gradient was 25 mm Hg or less in 13 patients. We conclude that a small dose of propranolol given intraoperatively can identify patients in whom a reduction in the right ventricle-pulmonary artery gradient to acceptable levels will occur in the months following operation. This may reduce the need for placement of a subannular or transannular patch in some patients.

Blood Pressure

Evaluation of long-term results of homograft and heterograft valves in extracardiac conduits.

Between 1971 and 1982, 249 patients received right heart extracardiac conduits. The 173 patients who survived 30 days were reviewed retrospectively; 72 had antibiotic-sterilized aortic homografts, 97 had xenografts of various types, and four had valveless tubes. Reoperation for conduit obstruction was required as early as 13 months after the original operation, but actuarial analysis showed no differences in overall performance of homograft and heterograft conduit groups with respect to late death or survival free of conduit obstruction. More important risk factors for late death or the need for reoperation were the severity of the underlying cardiac lesions and the influence of early postoperative complications. When analyzed statistically, the performance of conduits bearing homografts was disappointing, influenced sometimes by complications in the Dacron extension tubes. Repairs dispensing with conduits entirely should be evaluated.

Actuarial Analysis

Baffle obstruction.

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Cardiac Surgical Procedures