Neonatal diaphragmatic hemangioma.

The first neonatal case of a hemangioma of the diaphragm in a neonate is reported. After 25 months the patient is well with no signs of recurrence. Diaphragmatic tumors should be considered in the differential diagnosis of neonatal thoracic masses.

[Physiopathologic findings and surgical treatment in transposition of great vessels: our experience]. / Rilievi fisiopatologici e trattamento chirurgico della transposizione dei grossi vasi: nostra esperienza.

Transposition of great vessels (TGV) is the most frequent neonatal cyanotic malformative cardiopathy. The Authors report their experience in surgical anatomical correction of transposition of the great arteries in 55 patients, 44 male and 11 female. Minimum age was 2 days, maximum 6 months. TGV was simple in 38 cases and associated with ventricular defect in 12 cases, with double outlet right ventricle in 3 cases, and with complex cardiopathy in 2 cases. There have been 11 deaths. The cause of death was: cardiac failure in 3 patients, myocardial infarct in 4 patients, respiratory insufficiency in 2 patients, and sepsis in 2 patients. There was one death by myocardial infarction and 1 asymptomatic ostial left coronary stenosis during follow-up (from 1 to 104 months). There were neither anastomotic pulmonary stenosis nor aortic valve incompetence. The authors review the literature on functional and anatomic correction of TGV and underline the importance of precocious anatomic together with early diagnosis, percutaneous atrioseptostomy and pharmacologic (PGE1) therapy in determining further reduction of mortality and to improve late outcome.

[Indications for percutaneous atrioseptostomy. Comparison of echocardiographic and fluoroscopic monitoring]. / Indicazioni alla atriosettostomia percutanea. Confronto fra monitorizzazione ecocardiografica e fluoroscopica.

Balloon atrial septostomy (BAS) is of established value in the management of several congenital heart diseases in the neonatal period. This procedure, which leads to creation of a tear in the membrane of the Foramen Ovalis using a balloon catheter, may be undertaken under fluoroscopic monitoring in catheter laboratory as well as under echographic control, even bedside. In this study we present our experience and discuss the indications to these two techniques. 91 neonates underwent to BAS; in 14 of them this was carried out under two-dimensional echocardiographic control in the intensive care unit. In all the patients BAS had good result, with clinical improvement in the majority of cases (97%) and a low rate (6%) of minor complications (such as transient supraventricular arrhythmias), without differences between fluoroscopic and ultrasound monitoring. The higher rate of mortality in the fluoroscopic monitoring group (2/91 = 2.2%) was thought to depend on an extremely critical presentation of the neonates. The echographic monitoring does not seem to offer any real advantage from the technical point of view, but the necessity of a prompt treatment of very ill patients emphasizes the advantages of a quickly and easily feasible procedure. Thus, we recommend the use two-dimensional echocardiographic imaging only in the very ill neonates in whom the septostomy can be more safely performed in intensive care unit bedside.

[Clinical-therapeutic aspects and surgical treatment of aortic coarctation associated with hypoplasia of the aortic arch in less than 3-month-old patients]. / Aspetti clinico-terapeutici e trattamento chirurgico della coartazione aortica (CoAo) associata ad ipoplasia dell'arco aortico sotto i tre mesi di vita.

The surgical treatment of the CoAo plus Hypoplastic Aortic Arch is debated. The point is whether to treat or not the associated tubular hypoplasia performing an extended Resection and "End to End" Anastomosis (ERETE) or a subclavian flap angioplasty (Waldhausen). According to some report in the literature we called an arch hypoplastic if its diameter in the first or second portion was less than the patient body weight plus 1. Between January 1988 and December 1992 in our Institution 38 patients less than 3 months underwent aortic coarctation repair. In 14 cases we were able to consider the aortic arch as hypoplastic. These patients were aged between 3 days and 45 days (median 15 days), the body weight was between 2.6 and 3.9 kgs (median 3.2 kgs). 6 patients had CoAo as isolated lesion, in 3 cases there was Ventricular Septal Defect (VSD) associated. In 5 patients major intracardiac anomalies were associated (1 DORV, 1 TGA, 2 SV, 1 SV+Arterio-Ventricular discordance). In 7 cases in order to treat the arch an ERETE was performed. The other 7 patients had a Waldhausen procedure. In 5 cases a Pulmonary Artery Banding (PAB) was associated, in 1 case a palliative arterial switch operation. The hospital death was 43% (3 patients) in the ERETE group, and 28% (2 patients) in the Waldhausen group. The echocardiography and or angiography performed during the follow-up period showed a nice growth of the arch in both groups. Although from our present data it's impossible to get definitive conclusions, the ERETE doesn't give better result in terms of hospital death and long term aortic arch growth.(ABSTRACT TRUNCATED AT 250 WORDS)

[A rare cause of a continuous murmur. Our experience with a newborn infant with a congenital coronary fistula]. / Una rara causa di soffio continuo. Nostra esperienza in un lattante con fistola coronarica congenita.

Congenital coronary artery fistulas are very rare. The most common connect one of the two coronary arteries with the right cardiac chambers, in particular the right ventricle. So, a left to right shunt appears; furthermore ischemic problems are possible. Notwithstanding in the pediatric age subset the patients are usually free of symptoms. The most important clinical sign is a continuous murmur in the precordium. We report a case of a newborn with a fistula between the right coronary artery and the right ventricle. He presented with the typical murmur; ECG and chest-ray were considered as normal. Echocardiography did not do the diagnosis, and the baby was submitted to a cardiac catheterization. There was a mild left to right shunt (QP/QS = 1.5/1), without pulmonary hypertension. The diagnosis was done by selective coronary angiography. We decided not to close the fistula, and the baby was discharged. Three months later he is still well. In this discussion we consider indications for surgery, and hypothesize a wider application in the future of transcutaneous embolization.

[Stenosis of the pulmonary veins in total anomalous pulmonary venous drainage. Associated or iatrogenic abnormality?]. / Sténose des veines pulmonaires dans les retours veineux pulmonaires anormaux totaux. Anomalie associée ou iatrogène?

Obstruction of total anomalous pulmonary venous drainage (TAPVD) is sometimes observed in neonates, usually at the site where the collector drains into the caval system. Stenosis of the pulmonary veins themselves before joining the collector is less common. This was observed in 6 cases of intra- and supracardiac TAPVD; the prognosis is usually very bad due to postoperative pulmonary hypertension (PHT) which is difficult to treat. Three of our cases had TAPVD into the coronary sinus with obstruction presenting at birth. Conventional surgery did not reduce the PHT of the first 2 children because the congenital stenosis of the pulmonary veins was not corrected. In the first case, the pulmonary veins resembled fibrous cords and in the second case, the collector was stenosed at its junction with the coronary sinus. These two children died despite surgery. The third child, however, was cured because the obstruction was diagnosed preoperatively and successfully treated when the TAPVD was corrected. In the other three cases, TAPVD to the superior vena caval system was not obstructed but the pulmonary veins retracted progressively after surgery causing PHT and right ventricular failure. After unsuccessful percutaneous dilatation, reoperation revealed obstruction due to exuberant scar tissue with retraction at the site of anastomosis and (or) reconstruction of the pulmonary veins by incision or enlargement with a pericardial patch. One of these children survived after pneumonectomy of the obstructed lung which relieved reflex PHT of the contralateral lung.(ABSTRACT TRUNCATED AT 250 WORDS)

[Results of the surgical treatment of tetralogy of Fallot before 6 months of age. A consecutive series of 62 cases with 49 complete repairs]. / Résultats du traitement chirurgical appliqué aux tétralogies de Fallot régulières avant l'âge de 6 mois.

From January 1980 to July 1988, 62 infants aged under 6 months with an uncomplicated Tetralogy of Fallot (single ventricular septal defect, normal coronary arteries, no localised pulmonary artery branch stenosis) underwent 64 surgical procedures. The indications for surgery were increasing cyanosis and/or anoxic spells. Fourteen systemic-pulmonary shunts (21.5%), 49 complete repairs (75.4%) and one enlargement of the right ventricular outflow tract and of the main pulmonary artery without closure of the ventricular septal defect, were performed. The results of palliative shunts are preoccupying: cumulative mortality of 36 per cent; high rate of early reoperation for complete repair: 14 per cent. Complete repair was associated with an operative mortality of 14 per cent. Only one child had to be reoperated. There was no late death after complete repair compared with 2 late deaths after shunt. Ultimate results of complete repairs are good. Some risk factors were statistically significantly associated with complete repair: age (2.5 months or less), weight (4,500 g or less), measurements of the pulmonary arteries estimated by the diameter of the right pulmonary artery (5 mm or less). Conversely there was no death in the subgroup of 31 infants aged more than 2.5 months without major pulmonary hypoplasia (diameter of the right pulmonary artery over 3.5 mm). One-stage complete repair give the best short and medium-term surgical results in treatment of uncomplicated Tetralogy of Fallot in infants, irrespective of age and weight providing they have no diminutive pulmonary arteries.(ABSTRACT TRUNCATED AT 250 WORDS)

[Treatment of critical pulmonary valvular stenoses in newborn infants using percutaneous valvuloplasty. Study of 28 cases]. / Traitement des sténoses valvulaires pulmonaires critiques du nouveau-né par valvuloplastie percutanée. Etude de 28 cas.

Twenty-eight neonates aged 1 to 21 days with critical pulmonic stenosis were treated by percutaneous valvotomy consisting of balloon dilatation of the stenotic valve by percutaneous catheterization. The right ventricular systolic pressure (RVP) was either the same or higher than the systemic pressure in all cases but 3. In those it was somewhat lower (80%) than the left ventricular systolic pressure (LVP) so that, on the whole, the RVP/LVP ratio, here called rP ranged from 0.8 to 2 (m = 1.41 +/- 0.36). The procedure failed 5 times because of failure to introduce the balloon through the valve (4 cases) or because of an accident (1 case). All failures occurred during the initial phase of our experience with this procedure as the last 20 trials were successful. The procedure was inefficacious in 3 cases (13%) but immediately successful in the other 20 (87%) with a drop in the mean rP to 0.59 +/- 0.17. During the follow-up period, one neonate continued to present with an important degree of cyanosis, due to a poor right ventricular compliance. He had to be operated on. Pulmonic stenosis reappeared in 6 cases who underwent for a second time a percutaneous valvotomy. The procedure was successful in 3 and failed in 3 who eventually required surgery. Overall, the satisfactory results observed after the procedure persisted over time in 13 cases. In conclusion, interventional catheterization has become almost always possible in neonates with critical pulmonic stenosis. If performed under vigorously controlled conditions including an infusion of prostaglandin E1 in cases with severe cyanosis and the use of large diameter balloons, this method is relatively safe and generally efficacious since in 70% of cases it circumvents the need for surgery.

[Atresia of the left coronary ostium. Repair in a 2-month-old infant]. / Atrésie de l'ostium coronaire gauche. Réparation chez un nourrisson de deux mois.

A case of atresia of the left coronary ostium revealed by neonatal heart failure is reported. The initial diagnosis was anomalous origin of the left coronary artery from the pulmonary artery. At surgery performed in this 6-week old infant the diagnosis was amended and the malformation was repaired. Soon after the operation the child rapidly developed hypertrophic "myocardiopathy" of the left ventricle. Seven and a half months later, he is asymptomatic and the echocardiographic parameters of left ventricular systolic function are gradually returning to normality. Atresia of the left coronary ostium is an exceptional anomaly which must be considered, together with the other anomalous origins of the left coronary artery, when confronted with a case of severe heart failure caused by coronary ischaemia during the first months of life. The diagnosis rests on opacification of the coronary network during cardiac catheterization. Coronary "revascularization" may be performed either by aortocoronary bypass or by anatomical repair of the malformation.

[Cardiac transplantation in the infant and young child. Preliminary results]. / Transplantation cardiaque chez le nourrisson et le jeune enfant. Résultats préliminaires.

Between January and December, 1987, a programme of heart transplantation in paediatrics was designed and carried out in 9 children by the medical and surgical teams of the Necker/Enfants Malades-Laënnec hospitals group, Paris. Six of the patients were infants of less than 2 years (4 were under one year), and the oldest child was 10 years old. All patients seemed to be condemned to an early death either because their congenital heart disease was beyond the resources of conventional surgery (6 cases) or because their dilated cardiomyopathy was refractory to all medical treatments. Three children died at the end of the operation or a few days afterwards, due to poor quality graft (1 case), fulminating bacterial superinfection (1 case) or intractable pulmonary hypertension (1 case). The remaining 6 children are now living as normally as possible in their respective families. The long-term immunosuppressive treatment consists of cyclosporine and azathrioprine; corticosteroids are only used at the very beginning of treatment or in case of graft rejection. Only two episodes of rejection, confirmed by endomyocardial biopsy, were observed in the same patient during the first postoperative month. Biopsy was never performed systematically in order to spare the patient's vein, and the diagnosis of rejection was suspected on clinical grounds.(ABSTRACT TRUNCATED AT 250 WORDS)

[Practical aspects in the surveillance of heart and heart-lung grafts in children]. / Aspect pratique de la surveillance des greffes cardiaques et cardiopulmonaires chez l'enfant.

The short and middle term survivors of the 19 transplanted children (heart and heart-lungs) of our program are treated with an association of cyclosporine and azathioprine. The detection of rejection is the main worry and relies on endomyocardial biopsy, but this investigation may be dangerous and lowers the available veins of smaller children. Thus, we use it only for the situations of clinical suspicion (18 times in 20 months in 8 children, which proved rejection 6 times in 3 patients). Renal function supervision is careful and comprises systematic renal biopsy; tubulo-interstitial lesions were constant: minimal 3 times, moderate 3 times. This prompts to prescribe the lowest possible cyclosporine dosages which ensure an effective residual blood level (100-300 ng/ml). In case of heart-lung transplantation (3 cases), pulmonary rejection is difficult to prove, opportunistic infections more frequent and severe, and the tracheal suture is responsible for complications (stenosis in 2 cases). By and large, the constraints of supervision are reasonable and allow an almost normal life.

Optimism and locus of control for health and affiliation among elderly adults.

Ninety-two elderly adults ranging in age from 69 to 100 and residing in a retirement community were administered the Life Orientation Test (LOT) (a self-report measure of optimism), the Multidimensional-Multiattributional Causality Affiliation Scale (MMC-AF), and the Multidimensional Health Locus of Control Scale (MHLC). It was hypothesized that optimism would be correlated positively with internal locus of control measures and correlated negatively with external locus of control measures in the areas of affiliation and health. Five of nine comparisons between optimism and locus of control measures yielded significant relationships, all in the expected direction.