Pulmonary valve incompetence. I. Evaluation using electromagnetic flow velocity catheters and a new valve insufficiency analyser.

In animal experiments regurgitant flow through competent and incompetent pulmonary valves was evaluated with electromagnetic flow velocity catheters by using the ratio of the separately integrated backward and forward flow velocity signals. This ratio was obtained with a special purpose analog computing circuit set by an in-vitro zero flow reference signal, which was in good agreement with in-vivo zero flow signals.

Pulmonary valve incompetence. II. Application of electromagnetic flow velocity catheters in children.

During cardiac catheterization in 49 children regurgitant flow through competent and incompetent pulmonary valves was evaluated with electromagnetic flow velocity catheters using the ratio of the separately integrated backward and forward flow velocity signals (vb/vf). When the pulmonary valve was normal, vb/vf was less than or equal to 0.10. After operation for pulmonary valve stenosis or tetralogy of Fallot larger values were often found. In patients with an outflow patch across the pulmonary valve ring vb/vf values of up to 0.54 were found.

The postvalvulotomy propranolol test to determine reversibility of the residual gradient in pulmonary stenosis.

We advocate the use of propranolol in very low dosage at the end of operation to determine a prognosis of the long-term results to be expected after valvulotomy in diverse types of pulmonary stenosis.

More than thirty-five years of coarctation repair. An unexpected high relapse rate.

Between 1953 and 1985, 109 consecutive patients (17% with isolated coarctation) younger than 3 years of age underwent resection of aortic coarctation. These patients had nonelective operations because of congestive heart failure or severe systemic hypertension not responding to medical therapy. Special surgical techniques were used to lower the prevalence of restenosis. Hospital mortality was 32% (35 patients). Logistic regression analysis proved that age at operation, pulmonary artery banding, and type of repair were independent predictors of hospital death. Late mortality occurred in 9 patients. Associated cardiac anomalies were an independent prognostic factor for late mortality. The operation rate for recoarctation was low (5.8%). However, the follow-up study revealed that 30 patients (41%) had recoarctation. The Kaplan-Meier estimate of recoarctation is 86% after 30 years' follow-up in patients undergoing classic end-to-end anastomosis with silk sutures (n = 48). None in the group with an "extended" anastomosis and polypropylene sutures (n = 26) had recoarctation. The Cox analysis revealed age at operation under 6 months to be prognostic for recoarctation. Because of the shorter period of follow-up, the extended anastomosis with polypropylene sutures proved not to be a significant prognostic factor for recoarctation. In the late postoperative period (mean follow-up 16.7 years), blood pressure was elevated in 49% of the patients. At last follow-up 27 (36%) of the 74 survivors had aortic valve disease. Patients operated on for coarctation of the aorta under the age of 3 years need lifelong follow-up for detection of restenosis, hypertension, and valvular disease. Reoperation-free rate is not a good criterion to judge the outcome of operation for coarctation. Longer follow-up will be needed to investigate whether or not the use of the extended anastomosis technique with polypropylene sutures lowers the prevalence of recoarctation.

Long-term results of atrial correction for transposition of the great arteries. Comparison of Mustard and Senning operations.

Few data exists on the differences in long-term outcome between Mustard and Senning operations. We reviewed available data of all hospital survivors of these operations and assessed risk factors for late death and sinus node dysfunction. Of those patients undergoing the Mustard operation, 60 were hospital survivors (46 simple transposition, 14 complex); of those patients undergoing the Senning operation, 62 were hospital survivors (43 simple, 19 complex). Median duration of follow-up was 16 years (maximum 25 years) for Mustard operation, 11 years (maximum 20 years) for Senning operation. No reoperations were done, except for pacemaker implantation. No differences were found between the two groups with regard to baffle-associated problems, right ventricular failure, sudden death (6% in both groups), and functional status at final follow-up (New York Heart Association class I or II, except for four patients). For patients undergoing the Mustard operation, survival at 16-year follow-up was 91% with simple transposition and 60% with complex transposition (p = 0.027); for both groups of patients undergoing the Senning operation, survival at 16-year follow-up was 78%. Survival in the absence of rhythm disturbance at 16-year follow-up was 18% for Mustard operation and 53% for Senning operation (p < 0.001). In multivariate analysis, significant independent risk factors for late death turned out to be complex transposition (versus simple) and active arrhythmias. The only significant risk factor for the occurrence of sinus node dysfunction was the Mustard operation. We conclude that apart from the difference in the loss of sinus rhythm, no differences were found in the long-term clinical results of the two types of operations.

The neural crest as a possible pathogenetic factor in coarctation of the aorta and bicuspid aortic valve.

Patients (n = 109) operated on for coarctation of the aorta were analyzed for occurrence of associated cardiac and noncardiac anomalies. Attention was also paid to the prevalence of cardiac anomalies in the relatives of these patients. Of the patients with coarctation of the aorta, 57 (52%) had a bicuspid aortic valve. Forty-three (39%) of the 109 patients had one or more noncardiac anomalies. In 29 (27%) patients the noncardiac anomaly involved the head/neck structures. Noncardiac anomalies were much more prevalent in patients with coarctation and bicuspid aortic valve, especially anomalies involving the head/neck structures: 44% compared to 8% of patients with a normal aortic valve. Congenital cardiac malformations were present in relatives in the first or second degree of 18% of the patients. Bicuspid aortic valve was more prevalent in patients with an affected relative (75%) than in patients with unaffected relatives (47%). Recent studies showed that the neural crest plays an important role in the development of cardiac and a variety of noncardiac structures. The cardiac structures derived from the neural crest involve the outflow tract of the heart and the aortic arch system. Maldevelopment of neural crest cells could therefore be responsible for the combined occurrence of outflow tract (e.g., bicuspid aortic valve), aortic arch (e.g., coarctation), and noncardiac anomalies. This study supports the concept that some anomalies of the aortic arch system, including aortic coarctation, are cardiovascular manifestations of a spectrum of anomalies involving the head and neck region that may be due to a genetic-environmental disorder of the neural crest.

The arterial switch operation. An eight-year experience.

Sixty-six patients (23 neonates with transposition of the great arteries and intact ventricular septum, 33 infants and children with transposition and a large ventricular septal defect, and 10 with double-outlet right ventricle with a subpulmonary ventricular septal defect have received an arterial switch repair since 1977. Eight (one with transposition and intact ventricular septum, six with transposition and ventricular septal defect, and one with double-outlet right ventricle with subpulmonary ventricular septal defect) died in the hospital. All other patients have had follow-up as of June, 1985. Including the hospital deaths, the 11 month actuarial survival rate for the entire group was 81%, and no deaths have occurred among the 33 patients alive at that time and traced as long as 8 years. The hazard function for death has only a single early phase, and its 70% confidence limits overlap the hazard function of a matched general population by 12 months after the operation. Incremental risk factors for death included low birth weight (but not weight or age at operation), transposition of the great arteries with large ventricular septal defect, double-outlet right ventricle with subpulmonary ventricular septal defect, and presence of a patent ductus arteriosus. Coronary artery morphology and position of the great arteries were not risk factors. Long aortic cross-clamp time was possibly (p = 0.11) a risk factor. Early date of operation was a risk factor (p = 0.004); thus, predicted 1 year survival rate, including hospital deaths, after the arterial switch operation in 1985 is 99.9% (70% confidence limits 99.0%-100%) for neonates with transposition and intact ventricular septum and 99.7% (98.4%-99.9%) for those with transposition and a large ventricular septal defect or double-outlet right ventricle. The late functional status was excellent, and the rhythm was sinus in 96% of the 55 surviving patients. A formal comparison with the results of the atrial switch repair indicates that the arterial switch repair is superior.

Tricuspid atresia. Morphology of the outlet chamber with special emphasis on surgical implications.

An anatomic study of 28 heart specimens with tricuspid atresia showed 15 hearts with ventriculoarterial concordance and a right-sided anterior outlet chamber. Of these 15 hearts, 12 showed evidence of restricted pulmonary blood flow. In eight of these 12 specimens, an anterior deviation of the outlet septum, relative to the primary septum, was present. This deviation caused a narrowing inside the outlet chamber and functioned as infundibular pulmonary stenosis. The outlet chambers of the 15 hearts with ventriculoarterial concordance were compared with those of 13 hearts with tricuspid atresia and ventriculoarterial discordance and also a right-sided anterior outlet chamber. In the latter group of 13 hearts, five showed evidence of restricted pulmonary blood flow, at least partially caused by posterior deviation of the outlet septum relative to the primary septum. A clear difference in outlet chamber morphology was found without exception between specimens with and without ventriculoarterial concordance. Clinical data in three long-term survivors of the Fontan procedure demonstrate that the special morphology of the outlet chamber can pose surgical problems. Especially in patients with ventriculoarterial concordance, when the outlet chamber is incorporated into the pulmonary circuit, the surgeon must give special attention to the specific morphology to prevent undesirable sequelae.

Takedown and reconstruction of cavopulmonary anastomosis.

Takedown and reconstruction of a previous Glenn anastomosis at the time of repair was performed in 1 child with pulmonary atresia and ventricular septal defect and in 3 children with transposition of the great arteries, ventricular septal defect, and pulmonary stenosis. All 4 survived and showed excellent clinical results. The surgical technique and postoperative hemodynamic and lung scintigraphic data are presented.

Behavioral analysis of the effects of benzodiazepine receptor ligands in the conditioned burying paradigm.

In the conditioned burying paradigm, 10 mutually exclusive behavioral sequences were encoded in order to characterize the effects of various benzodiazepine receptor ligands. It was found that each ligand produced a specific behavioral pattern, the elements of which were consistent with results obtained in other experimental situations. Whereas anxiolytic (diazepam, Ro 16-6028) and 'anxiogenic' ligands (FG 7142) both decreased the mean duration of burying, these two classes of drugs largely differed when approaches to the prod and escape movements away from the prod were considered. The benzodiazepine receptor antagonist Ro 15-1788 was devoid of effects on all of the behavioral sequences considered. The main characteristics of these behavioral profiles persisted during an extinction test, although the animals were no longer treated. It is proposed that additional behavioral measures, or better, an ethological analysis of the behavior may increase the potency of the conditioned defensive burying paradigm.

Surgical correction of unusual double-outlet right ventricle.

This paper presents the case history of an 8-year-old girl who had total situs inversus and double-outlet right ventricle with pulmonary stenosis and severe tricuspid insufficiency in the presence of dextrocardia with ventricular discordance. A successful repair was performed using the Rastelli technique in conjunction with replacement of the tricuspid valve with a Bjork-Shiley prosthesis. The postoperative course was uneventful, and follow-up catheterization revealed a good operative result. However, the patient died suddenly during an emotionally upsetting period about two months after the operation. Postmortem examination revealed only signs of moderately severe cardiac decompensation. Some anatomical and embryological comments are made.

Tricuspid atresia with overriding imperforate tricuspid membrane: an anatomic variant.

An anatomical study of 34 specimens presenting with tricuspid atresia, in which particular emphasis was devoted to the atrioventricular junctional area, revealed 5 cases with an imperforate valve. In 1 heart the valve was completely committed to the right ventricle. In the other 4, however, the membrane was related not only to the right ventricle but also to the left ventricle. A hypoplastic right ventricular inlet portion was found in these 4 specimens. In 2 cases the tricuspid membrane overrode an inconspicuous inlet septal defect. In the other 2 hearts, as studied macroscopically, no defect was present underneath the membrane, which was connected to both right and left ventricles. These 4 specimens also had other ventricular septal defects. In classical tricuspid atresia, characterized by absence of the inlet portion of the right ventricle, the inlet septum is absent. In contrast, these 4 hearts did have an hypoplastic inlet septum.

One stage repair of aortic arch anomalies and intracardiac defects.

Between August 1985 and May 1990, 27 neonates and infants underwent combined correction of intracardiac and aortic arch anomalies through a median sternotomy. Coarctation (CoA) was combined with VSD (6), AVSD (2), Taussig-Bing (TB) heart (5), transposition of the great arteries (TGA) (1), TGA + VSD (2), congenitally corrected TGA + VSD (1) and VSD + myxoid stenotic outlet valves (1). Interrupted aortic arch (IAA) was combined with VSD (10) and TB heart (1). Two patients had IAA type B as well as CoA. Age at operation varied from 2 to 243 days with a mean age of 51 days. Twenty patients (70%) were younger than 30 days. One TGA + VSD and all TB hearts had maligned outlet septum and right ventricular outflow tract obstruction (RVOTO). Posterior outlet septum deviation and left ventricular outflow tract obstruction (LVOTO) occurred in 8 patients with malalignment VSD and IAA (7) or CoA (1). Aortic arch reconstruction was performed using extended end-to-end anastomoses. In 3 patients, arch hypoplasia necessitated patch implantation. VSDs were closed through the right atrium unless the anatomy dictated otherwise. One TB heart was reconstructed with a Kawashima procedure. All other TB hearts and all TGAs were corrected with arterial switch operation. Obstructing outlet septum was resected whenever necessary. Follow-up was complete and included echo-Doppler control. Eleven patients had postoperative heart catheterisation. Early mortality was 18.5% (5 patients). Persisting LVOTO or RVOTO was responsible. There was no late mortality. Five patients were reoperated upon: 3 for stenotic anastomoses and 2 for a subaortic membranous stenosis. Successful balloon dilatation of recoarctation was performed four times.(ABSTRACT TRUNCATED AT 250 WORDS)

A comparative study of Powell's and Downhill Simplex algorithms for a fast multimodal surface matching in brain imaging.

Multimodal images registration can be very helpful for diagnostic applications. However, even if a lot of registration algorithms exist, only a few really work in clinical routines. We developed a method based on surface matching and compared two minimization algorithms: Powell's and Downhill Simplex. We studied the influence of some factors (chamfer map computation, number and order of parameters to determine, minimization criteria) on the final accuracy of the algorithm. Using this comparison, we improved some processing steps to allow a clinical use, and selected the simplex algorithm which presented the best results.

[Childhood psychoses and organic pathology: results of a study of 144 cases]. / Psychoses infantiles et pathologie organique: résultats d'une étude sur 144 cas.

The records of 144 patients of Child Psychiatry Units of Alsace (France), with childhood psychosis (CP) or pervasive developmental disorders (PDD) have been systematically screened for previous or associated pathological events. Half of the children studied have been or are still affected by severe somatic disorders, but none of the diagnostic subcategories (referring to DSM III or CFTMEA) appeared significantly more frequently affected. In our population, the severity of organic disorders was positively correlated with: the age of the mother: more severe cases were reported when the mother was younger than 20 or older than 40 at the moment of childbirth; pathological events during pregnancy; early mother-child separation during the first year of life. The most frequent associated disorders however (neonatal pathology 45% of the cases, epilepsy 17% of the cases, neurological or neurosensorial pathology 15% of the cases) were associated neither with a specific diagnostic nor with a clinical and social specific pattern. The only statistically significant correlation was found between neurological pathology and a relatively low level of cognitive and social functioning. All these results were confirmed by multivariate statistical analysis. A main component analysis integrating all quantified data concerning organic pathology was performed: it emphasizes the independence of the different pathological events reported. The factorial analysis including the clinical, diagnostical and somatic event-related data failed to show any statistical profile associating functional features of the children with any particular previous or existing somatic disorders. Our results suggest that a history of organic pathological events is frequent not only in autistic disorders but in any kind of PDD or early CP - associated with moderate to severe mental retardation, in most cases of our study. However, this does not demonstrate that this type of pathological events constitute the direct and unique cause of PDD and CP: the concept of the aetiology of these severe diseases must take account of other factors - such as relational disruption -, also frequently seen in these children.

[Consensus heart and vascular diseases and sports]. / Consensus hart- en vaatziekten en sportbeoefening.

A consensus meeting on persons with a cardiovascular disease engaging in sports was held on 25 November 1988. The purpose was to arrive at uniformity in counselling and attendance. For these purposes, the exercise tolerance of the cardiovascular patients has to be weighed against the expected work load. The recommendations should be based on examinations suitable to the nature of the disorder. Guidelines for performance- and recreation-directed athletic activities are presented for a number of cardiovascular diseases. In counselling and attendance the medication used should also be taken into account. Expertise in counselling and attendance is of great importance for prevention of injuries. This is mostly achieved by team work with the sports physician coordinating.

[Closure of the persistent ductus arteriosus (Botalli) using a catheter procedure; the initial 50 patients treated in The Netherlands]. / Sluiting van de persisterende ductus arteriosus (Botalli) door middel van catheter-interventie; de eerste 50 in Nederland behandelde patiënten.

Experience gained with non-surgical catheter occlusion of patent ductus arteriosus in 50 patients (1-65 years) is described. Embolisation of the Rashkind device into the right pulmonary artery occurred in 1 patient. In another patient the device was removed because the prongs of the proximal umbrella could not be visualized. In 4 patients a considerable residual shunt was successfully treated with implantation of a second device. In 1 of these 4 patients this resulted in disappearance of considerable haemolysis as well. Five patients, however, still have a--haemodynamically insignificant--residual shunt. In 43 patients catheter occlusion of the ductus arteriosus was 100% successful. The advantages and disadvantages of this method are briefly compared with those of surgical treatment.

[The effect of the anthracycline derivative doxorubicin on myocardial function in children]. / Invloed van het antracyclinederivaat doxorubicine op de hartspierfunctie van kinderen.

Echocardiograms of 47 children treated with doxorubicin in combination with other cytostatic agents in the period 1979-1984 were reviewed. The authors compared the first and the last echocardiographic registration of every individual patient, analysing the left ventricular dimensions and the shortening fraction which is the indicator of myocardial contractility. Statistical analysis revealed a significant dilation of the left ventricle with a decrease of contractility in the whole group of patients during the therapy. Seven out of 9 patients who had an abnormal shortening fraction at the last measurement died of malignancy. Three children, all in remission of their malignancy, developed a dilated cardiomyopathy that was fatal in two. The possibilities for prevention of this serious complication by means of serial echocardiography during treatment with anthracycline derivatives are discussed.