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Biomedical subjects

M de Leval

Publications and source records attributed to M de Leval.

At least 19 recordsLinked to original sources

Cardiac ECMO for biventricular hearts after paediatric open heart surgery.

OBJECTIVE: To delineate predictors of hospital survival in a large series of children with biventricular physiology supported with extracorporeal membrane oxygenation (ECMO) after open heart surgery. RESULTS: 81 children were placed on ECMO after open heart surgery. 58% (47 of 81) were transferred directly from cardiopulmonary bypass to ECMO. Hospital survival was 49% (40 of 81) but there were seven late deaths among these survivors (18%). Factors that improved the odds of survival were initiation of ECMO in theatre (64% survival (30 of 47)) rather than the cardiac intensive care unit (29% survival (10 of 34)) and initiation of ECMO for reactive pulmonary hypertension. Important adverse factors for hospital survival were serious mechanical ECMO circuit problems, renal support, residual lesions, and duration of ECMO. CONCLUSIONS: Hospital survival of children with biventricular physiology who require cardiac ECMO is similar to that found in series that include univentricular hearts, suggesting that successful cardiac ECMO is critically dependent on the identification of hearts with reversible ventricular dysfunction. In our experience of postoperative cardiac ECMO, the higher survival of patients cannulated in the operating room than in the cardiac intensive care unit is due to early effective support preventing prolonged hypoperfusion and the avoidance of a catastrophic cardiac arrest.

Cardiopulmonary Bypass↗

Vacuum-assisted venous drainage in complex congenital cardiac surgery.

Here, we report on a case of temporary neurological damage following conduit replacement from the right ventricle to pulmonary artery during which vacuum-assisted venous drainage was used. We will discuss the benefits and risks of this technique with potential arterial air embolism despite the use of arterial line filters. We will also make recommendations for avoiding such complications.

Adult↗

Scientific, ethical, and logistical considerations in introducing a new operation: a retrospective cohort study from paediatric cardiac surgery.

OBJECTIVE: To review the initial impact on mortality of infants with congenital heart disease of a new surgical technique that is now taken for granted. DESIGN: Retrospective cohort study. SETTING: A tertiary paediatric cardiology centre. SUBJECTS: 325 consecutive neonates with simple transposition of the great arteries admitted before, during, and after the preferred management changed from the Senning operation to the arterial switch (1978-98); and 100 consecutive neonates requiring a different neonatal open heart operation that did not change in that period. MAIN OUTCOME MEASURES: Mortality before and early after operation reconstructed sequentially as the series evolved and retrospectively once the series was complete; actuarial survival associated with the different treatment strategies. RESULTS: For both the transposition and the comparison group, early mortality in 1998 was lower than in 1978. During that period, however, there was a phase temporally related to the adoption of the switch operation in which early mortality for transposition increased. Actuarial survival of recent patients with "intention to treat" with arterial switch is superior to those with intention to treat with the Senning operation, as predicted when the switch operation was first adopted. CONCLUSIONS: A period of increased hazard for individual patients may occur when a specialist community, a particular unit, and an individual surgeon are all learning a new technique concurrently. Obtaining informed consent during this time of uncertainty is helped by clarity about the objectives of treatment and availability of relevant local and international data.

Child↗

The effect of prelung transplant clinical status on post-transplant survival of children with cystic fibrosis.

The aim of this study was to determine whether transplanting paediatric cystic fibrosis (CF) patients later in the course of their disease was detrimental to their post-transplant survival. Data was collected from 51 children with CF undergoing lung or heart-lung transplantation May 1988-March 1999. The following risk factors were tested by Cox proportional hazards modelling: age at transplant; sex; donor/recipient sex mismatch; donor/recipient cytomegalovirus (CMV) mismatch; cold and warm graft ischaemic times; and donor age. Pretransplant forced expiratory volume in one second (FEV1), minimum oxygen saturation obtained during 12 min walk (Sa,O2min), and a survival probability score (SP) calculated from FEV1, age adjusted resting heart rate, age, sex, blood haemoglobin (Hb), and serum albumin were then added to the model. None of the risk factors were significantly correlated with death during the study period. No evidence that clinical status prior to transplant has any effect upon the post-transplant survival of children with cystic fibrosis was found.

Adolescent↗

Lung transplantation and life extension in children with cystic fibrosis.

BACKGROUND: Lung transplantation has been available as therapy for end-stage lung disease since the early 1980s, but survival after transplantation remains poor, with continued controversy as to the survival benefit from the procedure. We examined the effect of lung or heart-lung transplantation on the survival of a cohort of children with cystic fibrosis and severe lung disease. METHODS: Between May, 1988, and May, 1998, 124 children with cystic fibrosis were accepted for lung transplantation. 47 received transplants, 68 died while they awaited organs, and nine remained on the active waiting list. We constructed a proportional-hazards model that used variables of prognostic significance in this population. By including transplant status as a time-dependent covariate, we were able to calculate a hazard ratio for transplantation. Date of entry into the study was the date when children were added to the list for transplantation, and measurements were taken at this time. Children were accepted for transplantation if they had a life expectancy of 2 years or less, a poor quality of life, and no contraindications to transplantation. FINDINGS: After 1 year, 35 (74%) children were still alive; after 5 years 12 (33%) children were alive. The univariate hazard ratio for transplantation was 0.41 (95% CI 0.23-0.74; p=0.003). Transplantation remained significantly associated with survival after correction for differences in age, sex, height-corrected forced expiratory volume in 1 s, minimum oxygen saturation during a 12 min walk, haemoglobin concentration, albumin concentration, and age-corrected resting heart rate (hazard ratio 0.31 [0.13-0.72]; p=0.007). INTERPRETATION: If centres follow our criteria for accepting patients for transplantation, and achieve similar survival after transplantation, they could expect a survival benefit for their patients in line with our results.

Adolescent↗

The modified Norwood procedure for hypoplastic left heart syndrome: early to intermediate results of 120 patients with particular reference to aortic arch repair.

BACKGROUND: Classic first-stage Norwood repair of hypoplastic left heart syndrome uses a homograft patch enlargement to obtain an unobstructed aorta and coronary arteries. Because of possible disadvantages of the homograft, such as lack of growth, degeneration and calcification, and availability, we have tried to repair the aorta without patch supplementation. METHODS: Between February 1993 and September 1997, 120 patients, aged birth to 47 days (median 4 days) and weighing 1.7 to 4.4 kg (median 3.1 kg), underwent first-stage palliation for hypoplastic left heart syndrome. The diameter of the ascending aorta ranged from 1.5 to 8.0 mm (median 3.0 mm). Eight patients had an aberrant right subclavian artery arising from the descending thoracic aorta. In 95 patients (group I), all duct tissue was excised and the descending aorta was anastomosed to the aortic arch, which had been opened back into the ascending aorta. Then to this confluence was anastomosed the proximal main pulmonary artery. In the remaining 25 patients (group II), continuity of the aortic arch was maintained and the repair was performed with a Damus-Kaye-Stansel anastomosis. The size of the systemic-to-pulmonary shunt was 3 mm in 48 patients, 3.5 mm in 70, and 4.0 mm in 2. RESULTS: Circulatory arrest time ranged from 19 to 105 minutes (median 54 minutes). A homograft patch was necessary for the arch reconstruction in 18 patients (15%); 9 group I patients (10%) and 9 group II (36%) (P =.001). There were 82 hospital survivors (68%); 69 group I patients (73%) and 13 group II (52%) (P =.04), 71 patients without a patch (70%) and 11 with a patch (61%) (P >.2). By multiple logistic regression, the aberrant right subclavian artery was a significant risk factor for hospital death (P =.008). There were 6 late deaths. Sixteen of 71 patients (23%) who underwent second-stage palliation had a neoaortic arch obstruction develop, with a peak gradient greater than 10 mm Hg; 14 group I patients (23%) and 2 group II (22%) ( P >.2), 15 without a patch (23%) and 1 with a patch (17%) (P >.2). Overall survivals were 57% at 1 year and 55% at 2 years. CONCLUSION: The modified Norwood procedure for first-stage palliation of hypoplastic left heart syndrome is possible in the majority of patients without the use of exogenous materials and does not result in an increased incidence of neoaortic arch obstruction. Repair of the aorta without patch supplementation may improve the potential for long-term growth of the new aorta.

Anastomosis, Surgical↗

Urgent homograft aortic root replacement for aortic root abscess in infants and children.

OBJECTIVE: To assess the results of early homograft aortic root replacement in infants and children with an aortic root abscess. DESIGN: Descriptive study of all patients with an aortic root abscess during 1987-97, identified by retrospective review of the echocardiographic and surgical registries. SETTING: A tertiary referral centre. PATIENTS: Five patients (age 0.6 to 13 years; two female) were identified with an aortic root abscess. Four had no known pre-existing congenital heart abnormality. Three had a misleading presentation and were referred to our hospital with non-cardiac diagnoses (fulminant hepatic failure; adult respiratory distress syndrome; cerebrovascular accident). The other two presented with septicaemia and a murmur, respectively. Blood cultures identified Staphylococcus aureus (n = 3) and Streptococcus pneumoniae (n = 2). Aortic root abscess was diagnosed by transthoracic echocardiography. INTERVENTIONS: Homograft aortic root replacement with coronary reimplantation was performed urgently (median one day after diagnosis). RESULTS: Four patients survived. The youngest died following multiorgan failure, multiple aortic fistulae, three valve involvement, and extensive tissue destruction preventing mitral valve replacement (S pneumoniae). Two of the four survivors have required further surgery: mitral valve replacement (0.3 years later), and pulmonary autograft replacement of the homograft (8.3 years later). All survivors remain in sinus rhythm and New York Heart Association functional class I. CONCLUSIONS: Infective endocarditis should be considered in any child with severe septicaemia or embolic phenomena. Echocardiographic diagnosis of an aortic root abscess indicates uncontrolled infection and impending haemodynamic collapse. Homograft aortic root replacement can be performed successfully in critically ill children with active infection.

Abscess↗

Fate of subpulmonary homograft conduits: determinants of late homograft failure.

PATIENTS AND METHODS: Between 1971 and 1993, 656 conduits were placed in the subpulmonary position. Patients receiving heterografts or valveless conduits and patients dying within 90 days of insertion were excluded; thus 405 homograft conduits were studied. There were 293 aortic homografts, 94 pulmonary, and 18 of unknown type. The end point of conduit failure was defined by conduit replacement for whatever reason, balloon dilation of the conduit, or death of the patient with the conduit in place. The following factors were analyzed: aortic versus pulmonary homograft, antibiotic preservation versus cryopreservation, ABO and Rh compatibility, type of material used for conduit extension, age at operation, size of the conduit, diagnosis, and reoperations. Conduit number (1 to 405) in the series was included in the multivariable model. RESULTS: First conduits and conduits inserted earlier in the series appeared to last longer than second and subsequent conduits and those inserted later in the series (p = 0.001 and 0.003, respectively). Overall survival of conduits at 5, 10, and 15 years was 84% (95% CL, 80% to 88%), 58% (95% CL, 50% to 66%), and 31% (95% CL, 19% to 43%). Corresponding figures for the first conduits were 88% (95% CL, 84% to 92%), 65% (95% CL, 56% to 73%), and 34% (95% CL, 20% to 47%). The longest surviving homograft conduit in our series lasted 22.7 years. Regarded univariately, reoperation (redo worse), order number (recent worse), type of conduit (pulmonary worse than aortic), preservation (cryopreserved worse than antibiotic preserved), and age at operation (older patients worse) were statistically significant. However, in multivariable analysis, including all the above in the model, only reoperation and order number had significant predictive power. When patient survival was considered, patients operated on more recently survived longer despite the fact that their conduits were being replaced earlier. Overall, survival of patients at 5 and 15 years was 95% (95% CL, 93% to 98%) and 85% (95% CL, 77% to 92%), respectively. CONCLUSIONS: Pulmonary and aortic homografts, both cryopreserved and preserved in nutrient antibiotic solution, give similar results. All conduits will probably have to be replaced during the lifetime of the patient. In view of the worse performance of replacement conduits, techniques of repair that avoid the use of conduits should be further explored. Despite gradual deterioration of homograft conduits, they remain an important tool in the correction of many complex lesions with excellent 15-year patient survival.

Adolescent↗

Early age at repair prevents restrictive right ventricular (RV) physiology after surgery for tetralogy of Fallot (TOF): diastolic RV function after TOF repair in infancy.

OBJECTIVES: To assess diastolic right ventricular (RV) physiology after tetralogy of Fallot repair in infancy. BACKGROUND: Restrictive RV physiology after tetralogy of Fallot repair is related to type of repair, pulmonary regurgitation, and late arrhythmias. METHODS: Forty-seven patients were investigated, 27 and 20 patients in Lund and London, respectively. Median age at repair was 0.78 years (0.08-0.99) and median follow-up was 3.0 years (0.08-10.4). Restrictive RV physiology was assessed by Doppler echocardiography. RESULTS: Thirteen patients (28%) had restrictive RV physiology at follow-up, three of 19 patients (16%) with transatrial repair and 10 of 28 patients (32%) with transventricular repair, respectively (p=0.1). Ten percent of the patients repaired before 6 months of age were restrictive at follow-up, increasing to 38% with repair after 9 months. Transannular patch (TAP) repair was performed in 55% of the patients, including eight of 10 patients (80%) with repair before 6 months of age. Thirty-one percent of the patients with TAP repair were restrictive. These restrictive patients had more severe preoperative pulmonary stenosis (p < 0.05), were older at repair (p < 0.05), and had shorter duration of pulmonary regurgitation (p < 0.001) at follow-up. CONCLUSIONS: Restrictive RV physiology is inversely related to age at repair and independent of type of outflow tract repair. Since TAP repair is more common in early repair, and restriction seems to be less frequent, long-term follow-up to assess adverse effects of pulmonary regurgitation is mandatory.

Age Factors↗

Intracardiac repair of lesions associated with atrioventricular discordance.

OBJECTIVE: Discordant atrioventricular (AV) connection is a rare congenital cardiac anomaly. Associated cardiac defects modify the physiology, clinical presentation, and surgical management of these patients. We have reviewed our overall experience with 90 patients operated for lesions associated with AV discordance between 1975-1990. METHODS: 90 patients, consecutively operated between 1975-1990, were reviewed. Patients' charts, angiograms and echocardiograms were studied. Follow-up was obtained from our records or was requested from referring cardiologists/paediatricians. It was completed in December 1992. For the analysis of risk factors of early death we used P values by chi-squared and Fisher's exact test. For the analysis of the triangulated events, we used the Kaplan-Meier method. Variables associated with P value over 0.20 were eliminated. RESULTS: 90 patients aged 6 months-30 years (mean 9.1 year) underwent repair of cardiac anomalies associated with AV discordance. Three patients had total cavopulmonary connection; the remainder received biventricular repair. Most important associated malformations were ventricular septal defect (77), subpulmonary obstruction (68) and tricuspid valve anomaly (21). 59 patients received extracardiac valved conduit, 10 had tricuspid valve replacement. Thirteen patients died in hospital (14%). One year and 10 year actuarial survival rate was 84% and 70% respectively. The most significant factors for early death were: anatomically abnormal tricuspid valve (P < 0.0001), tricuspid valve regurgitation (P < 0.002), date of operation (P < 0.012), preoperative or perioperative complete heart block (P < 0.015), and tricuspid valve surgery (P < 0.05). Complete heart block developed in 17 (20%) out of 85 patients who did not have it preoperatively. During the follow-up, 22 intracardiac reoperations were necessary (4 died). 63 patients of 73 survivors (86%) remain in NYHA Class 1, 6 in Class 2 and 4 in Class 3. CONCLUSIONS: The operative mortality was 14%. Twenty per cent of patients developed complete heart block. These results improved in the later part of the series (1985-1990); mortality decreased to 5% and incidence of heart block to 14%. Cardiac anomalies associated with AV discordance still present a surgical challenge. The data reported in our study should serve as a useful baseline for evaluation of newer surgical techniques, such as the "double switch" operation.

Adolescent↗

Closure of muscular ventricular septal defects through a left ventriculotomy.

OBJECTIVE: To evaluate the results of closure of muscular ventricular septal defects through a left thoracotomy. METHODS: Records of 23 children operated consecutively between 1972 and 1990 were studied. Age of patients was 2.8 +/- 3 years (2 months-10 years), weight 8.9 +/- 5.7 kg (2.6-22 kg). Ten patients (43%) had undergone one and 4 patients (17%) two previous cardiac operations. Late follow-up was obtained from direct examination of patients or from reports of their referring physicians. Bypass time was 89 +/- 28 min (66-167 min). The aorta was cross-clamped for 44 +/- 15 min (21-66 min). Until 1977 operations were performed with moderate hypothermia and intermittent aortic cross-clamping. After 1978 deep hypothermia (20-25 degrees C) and cold crystalloid cardioplegia was used. Ventricular septal defects not accessible from other approaches were closed through a small fish-mouth incision in the apex of the left ventricle. Patients' data were sampled and stored in a computerised database. Risk factors were evaluated by stepwise logistic regression. RESULTS: Four patients died in the hospital (17%); two died later. Two required reoperation for residual/recurrent defects. All patients, except two from abroad, were available for follow-up, which ranged from 36 months to 18 years (mean 11.3 years). All were in NYHA class I. Only two risk factors were identified: the number of ventricular septal defects (P < 0.05) and associated atrial septal defect (P < 0.02). Early echocardiographic evaluation showed good LV size and function in all except one patient, who had a perioperative septal infarction. Late echocardiography performed in six patients demonstrated normal LV shortening without evidence of regional wall abnormality. CONCLUSIONS: Left ventriculotomy is a useful approach for closure of low muscular ventricular septal defects in selected patients.

Cardiac Catheterization↗

Paediatric incidence of acute rejection and obliterative bronchiolitis: a comparison with adults.

Obliterative bronchiolitis (OB) continues to be a major cause of morbidity and mortality following heart-lung transplantation. We compared the incidence of death from obliterative bronchiolitis in 19 children and 72 adults following heart-lung transplantation at our institutes. The incidence of death from OB at 2 years was 38% for children compared with 17% for adults, this difference was significant (Cox-Mantel Z value = 2.243, P < 0.05). The frequency of acute lung rejection and persistent lung rejection, previously described as risk factors for OB in adults, were significantly more common in children, P = 0.004 and P = 0.001, respectively. Average forced expiratory volume in 1 s was lower in children than in adults for each 3-month period after transplantation (P < 0.001). In conclusion, identified risk factors for the development of OB were more common, and the risk of death from OB was greater in children than in adults following heart-lung transplantation.

Adolescent↗

Outcome measures for the neonatal management of pulmonary atresia with intact ventricular septum.

The outcome in 135 patients with pulmonary atresia with intact ventricular septum was reviewed in terms of actuarial survival and "suitability" for eventual definitive repair (defined as having a tricuspid valve diameter larger than 2.4 standard deviations below the mean normal beyond the neonatal period). Of patients who underwent an initial closed valvotomy, 50% were dead, 22% were alive and suitable, and the remaining 28% were alive but unsuitable for definitive repair at 5 years. None of 66 patients who underwent primary shunt alone achieved suitability and 52% were dead at 5 years. No mode of neonatal closed valvotomy was consistently satisfactory: in only 10 of 26 survivors with serial measurements did the neonatal valvotomy alone achieve decompression to subsystemic pressures. Only half of the decompressed ventricles achieved growth of the tricuspid valve disproportionate to somatic growth as a result of the neonatal procedure. Strategies for the neonatal treatment of pulmonary atresia with intact ventricular septum should not rely on closed operations on the pulmonary valve to optimized right ventricular growth.

Actuarial Analysis↗

Ventricular expression of atrial natriuretic peptide after orthotopic cardiac transplantation.

In order to determine if atrial natriuretic peptide is produced by the human ventricle after transplantation, specimens were obtained from 54 separate right ventricular endomyocardial biopsies from 14 transplant recipients (age range 10-19 years) and were analyzed with simultaneous plasma samples. Radioimmunoassay techniques were used to determine plasma and tissue levels of atrial natriuretic peptide in each biopsy specimen, and blot hybridizations with the atrial natriuretic peptide c deoxyribonucleic acid probe were performed once for each patient. Mean plasma levels of atrial natriuretic peptide were obtained from the right ventricle (106 +/- 12 pg/ml); there was a positive correlation with end diastolic pressure (r = 0.7, P < 0.0001). Plasma levels, however, were not significantly increased during rejection episodes. Atrial natriuretic peptide was detectable in all ventricular biopsy specimens--mean 989 +/- 164 pg/mg soluble protein. Mean tissue level during rejection episodes was 1163 +/- 17 and following treatment of rejection was 411 +/- 147--however, this difference was not significant (P = 0.55). In 6 patients biopsied within 1 month of transplantation initial myocardial levels were significantly higher than those obtained at the following biopsy (P < 0.05). Despite these initially high tissue levels no correlation was found with time posttransplantation. Ventricular tissue levels positively correlated with systolic ventricular pressure, r = 0.3, P < 0.05, but there was no significant correlation with other hemodynamic parameters. The normalized atrial natriuretic peptide messenger ribonucleic acid signal from the slot blot hybridization was 59 +/- 6 mm (peak height of signal intensity) range 20-110 mm. The expression of atrial natriuretic peptide gene was also confirmed by Northern blot analysis. The ventricular messenger ribonucleic acid signal contained a single hybridizing band of approximately 920 nucleotides. Thus, we have demonstrated that atrial natriuretic peptide production by the human ventricle is independent of innervation. The consistent atrial natriuretic peptide gene expression and detection by radioimmunoassay in apparently healthy ventricles was unexpected but may reflect the use of immunosuppressive drugs, or perhaps subclinical graft dysfunction.

Adolescent↗

Technique and use of transbronchial biopsy in children and adolescents.

Since July 1988, a total of 92 transbronchial biopsies (TBB) have been performed in 18 patients (aged 3-16 years). Twelve patients (67%) were heart-lung transplant (HLT) recipients undergoing surveillance for pulmonary graft rejection and infection. The remainder included immunocompromised patients at risk of opportunistic infections (n = 4), patients with fibrosing alveolitis (n = 1) and a collagen vascular disorder with suspected lung involvement (n = 1). TBB was performed through either a fiberoptic (n = 50) or a rigid (n = 41) bronchoscope, all under general anesthesia. On one occasion a cardiac bioptome was used through an endotracheal stent. The sensitivity of TBB for diagnosing acute and chronic rejection in HLT patients was 88% and 60%, respectively (specificity, 91% and 100%). Definitive diagnoses were made in 4 (67%) of the non-HLT group. Bronchoalveolar lavage (BAL) was performed during each procedure for microbiological and cytological examination. Thirty-four pathogenic organisms including Pseudomonas aeruginosa (16/34), Staphylococcus aureus (8/34), and Candida albicans (5/34) were isolated from BAL culture. Complications included pneumothorax (8%), transient pyrexia (7%), and dyspnea (2%).

Acquired Immunodeficiency Syndrome↗

Heart-lung transplantation for cystic fibrosis. 2: Outcome.

From March 1988 to March 1990, 11 children with cystic fibrosis (age 5-15 years) underwent combined heart-lung transplantation at our institutes. Maintenance immunosuppression consisted of cyclosporin and azathioprine with corticosteroids and antithymocyte globulin used perioperatively and during rejection episodes. Six patients (55%) survive from 1.5-23 months all of whom have improved life quality. Actuarial survival to 1 year was 55%. At six months after transplant, mean forced expiratory volume at one second was 73.5% of predicted normal, compared with 25% before transplant. There was one perioperative death, three later deaths associated with obliterative bronchiolitis at two, eight, and nine months, and one from mediastinitis at four months. Of the 15 children accepted for transplantation but not receiving grafts, 10 have died (eight within four months of being placed onto the transplant list). Early postoperative problems included acute reversible rejection (n = 10), meconium ileus equivalent (n = 3), and pancreatitis (n = 1). There was a high incidence of later pulmonary rejection with a mean of 5.7 episodes per patient in the first six months. Pulmonary infection occurred relatively infrequently, with Pseudomonas aeruginosa being the most common pathogen. Persistent diabetes mellitus requiring insulin occurred in four and systemic hypertension developed in one.

Adolescent↗