Pedicled pericardium for repair of right ventricular outflow tract and pulmonary arterial stenoses in tetralogy of Fallot: a six-year experience.

From June 1988 through June 1994, 20 children with symptomatic tetralogy of Fallot, associated with hypoplastic or stenotic pulmonary arteries in 19 cases, were operated on in our institution. Mean age at operation was 49.5 +/- 43 months (ranging from 10 months to 12.5 years). Mean weight was 13.5 +/- 6.5 kg (range 6.2 to 30 kg) and mean body surface area was 0.6 +/- 0.2 m2. Mean preoperative hematocrit value was 47.6% +/- 11.1%, and mean preoperative arterial oxygen saturation ratio was 75.7 +/- 9.5. Six patients (30%) had prior systemic-pulmonary arterial shunts. Pulmonary arterial stenoses were congenital or iatrogenic (or both) and were located principally on the left pulmonary artery (63%), on the pulmonary bifurcation (15%), or separately on the left and right pulmonary arteries (21%). In all children the pulmonary arterial tree was repaired with the patient's own pedicled pericardium. Mean follow-up is 36 +/- 25.2 months (range 1 to 71 months). Hospital mortality rate was 0%. There was one late death, and two children were lost to long-term follow-up. No child required reoperation. Seventeen children returned for evaluation, consisting of physical examination, electrocardiogram, chest roentgenogram, and Doppler echocardiogram, and 11 underwent late catheterization or magnetic resonance imaging of the chest to evaluate the pulmonary arterial repair. All children were in New York Heart Association class I or II. Right ventricular function was normal by echocardiography in 100% with a mean right ventricular systolic pressure of 39.37 +/- 8.4 mm Hg. Mild to moderate pulmonary regurgitation was present in the majority of patients. The results of pulmonary arterial repair were good in 100%. There was no residual stenosis, and we observed uniform enlargement of the repaired pulmonary arteries over a 5-month to 6-year follow-up period. These results are of particular interest inasmuch as other materials previously used for repair of pulmonary arteries do not grow and may even shrink, resulting in residual stenosis.

Surgical treatment of multiple ventricular septal defects using a biologic glue.

The closure of multiple ventricular septal defects remains a surgical challenge. Mortality and morbidity are high. Left ventricular incision and multiple patches or stitches impair septal motion and function. We searched for a method that would cause minimal left ventricular and septal dysfunction. The use of fibrin seal for closing ventricular septal defects was considered. The method was first tested in animals so as to assess the internal resistance of the fibrin seal to stretching and fragmentation in addition to its adhesiveness and hence the absence of left-to-right embolization of the fibrin seal clot and the long-term success of the ventricular septal defect closure further to complete resorption of the ventricular septal defect clot. This experimental work was very satisfactory. Between April 1986 and September 1991, 15 children were operated on with the use of this technique. The overall hospital mortality rate was 6%. There were no reoperations for residual ventricular septal defects. All the long-term survivors (n = 13) were in excellent clinical condition with no or trivial residual shunt attested by color flow mapping investigation. This experimental and clinical experience suggests that satisfactory results can be achieved with the use of fibrin seal for the closure of multiple muscular ventricular septal defects.

[Mitral valve replacement in atrioventricular septal defect]. / Remplacement valvulaire mitral dans le canal atrio-ventriculaire.

This study reports the cases of 14 patients aged 5 months to 13 years (average: 3.5 years) at the time of the initial repair of an atrioventricular septal defect (AVSD). The AVSD was partial in 5 and complete in the other 9 cases. After failure of the initial mitral valvuloplasty, the patients underwent mitral valve replacement with a prosthesis at the age of 1 to 29 years, after an interval of 0 days to 15 years. A second valvuloplasty had been attempted in 3 cases beforehand. The aim of this paper was to analyse the causes of failure of mitral valvuloplasty and to determine the conditions of mitral valve replacement and the specific complications of this type of surgery. Failure of mitral valvuloplasty was related to complex valvular malformations (5 cases), technically inadequate valvuloplasty procedures (4 cases), deterioration of an initially satisfactory valvuloplasty (5 cases). Four bioprostheses were implanted (repeat valvular replacement was necessary in 3 patients). Early mortality was 28% (4 patients). The postoperative complications specific to this condition were: immediate atrioventricular block (8 cases) but only 1 persistant complete atrioventricular block; traumatic fistula between the left ventricle and right atrium (4 cases); moderate stenosis of the left ventricular outflow tract (4 cases). There was one late death (due to isolated cardiac arrhythmia).(ABSTRACT TRUNCATED AT 250 WORDS)

Extrathoracic heart (ectopia cordis). Report of two cases and review of the literature.

We report 2 cases of extrathoracic heart (ectopia cordis) operated on a few hours after birth at Laennec Hospital, Paris. The first patient had an associated diastasis of the rectus muscles. The second one, in whom diagnosis was made by ultrasound during fetal life, had a normal abdominal wall. In both cases it was possible to accommodate the heart in the left pleural cavity after a wide dissection of the posterior mediastinum. Both babies, however, died soon after the operation. Double outlet right ventricle was found in both. We have reviewed those cases previously described and discuss the importance of prenatal echocardiographic diagnosis in this rare condition.

Oral acetylsalicylic acid improves patency rates in small-vessel anastomoses.

Because platelet aggregation is the critical step in vascular thrombosis, this study investigated the possibility that acetylsalicylic acid given orally would improve patency of anastomoses in small arteries. Under clean conditions, in male Sprague-Dawley rats, the superficial femoral arteries were divided and reanastomosed using the sleeve technique with 10-0 nylon suture. Fifty-one rats received a full laboratory diet and acetylsalicylic acid in their drinking water (1.08 mg/ml), before and after operation. A control group of 54 rats was matched for weight, water intake and duration of vessel occlusion. Vessel patency was assessed on postoperative day 7, at sacrifice, by dye angiography. In the experimental group 7 (13.7%) anastomoses became occluded, compared with 20 (37.0%) in the control group (chi 2 = 5.99, df = 1, p less than 0.025). Serum thromboxane B2 levels in five rats given acetylsalicylic acid orally (33.1 +/- 6.1 ng/ml) were significantly (p less than 0.001) lower than in five control rats (86.6 +/- 49.1 ng/ml). The authors concluded that acetylsalicylic acid administered in the drinking water to Sprague-Dawley rats improved the patency rate of femoral artery anastomoses probably because of a reduction in platelet aggregation. Orally administered acetylsalicylic acid may be of clinical benefit to patients who undergo small-vessel anastomoses.

Resting lung function in children after repair of tetralogy of Fallot.

Children with tetralogy of Fallot had pulmonary function tests (PFT) after intracardiac repair (ICR). According to the age at time of ICR and to the existence or not of palliative surgery preceding ICR, they were divided into three groups. Group 1 had ICR at a mean of one year one month of age (range four months to one year seven months), that is, during the active period of postnatal lung growth. Groups 2 and 3 had ICR later in childhood, that is, respectively, at four years seven months (range two years nine months to 11 years 9 months) and at five years four months (range two years seven months to 11 years five months). In group 3, ICR was preceded by palliative surgery. The PFT at rest included measurement of lung volumes (functional residual capacity), vital capacity (VC), dynamic or static compliance (CL), total pulmonary resistance, lung transfer factor for CO (TLCO) and blood gases. Group 1 had normal lung function suggesting that early repair of TOF saves lung development. In groups 2 and 3, significant decrease in VC(p less than 0.01) and CL (p less than 0.01) were found suggesting impaired alveolar growth. Additional defect in TLCO (p less than 0.01) in group 3 suggested that palliative surgery induces abnormal vascular growth. Thus, the present functional results suggest repair of TOF during the two first years of life, ie, the active period of postnatal lung growth.

Short-term variations of the right ventricular/left ventricular pressure ratio following repair of tetralogy of fallot.

In 50 consecutive patients undergoing repair of tetralogy of Fallot, the peak systolic right ventricular/left ventricular pressure ratio (pRV/LV) was measured prospectively in the operating room and in the third postoperative week in order to assess its eventual short-term variations. Postoperatively, the ratio fell in 64% of the patients, remained unchanged in 6%, and increased in 30%. The mean postoperative pRV/LV ratio for the group as a whole showed a small but statistically significant fall with respect to the mean operating room pRV/LV ratio (0.47 [standard deviation] +/- 0.16 and 0.52 +/- 0.12, respectively; mean difference, -0.05; p value for the significance level of difference, 0.004). A separate analysis of patients in whom the right ventricular outflow tract was reconstructed with (N = 12) or without (N = 38) a transannular patch showed essentially the same results. Nevertheless, the difference between the pRV/LV ratio measured in the operating room and three weeks postoperatively is not significant in the group with a transannular patch because of the small number of patients. These data corroborate that the pRV/LV ratio measured in the operating room immediately after repair of tetralogy of Fallot reflects closely the postoperative ratio, being slightly higher by an average of 10%, is expected to fall shortly after operation in almost two-thirds of the patients, and is a useful variable in intraoperative decision making and in predicting the surgical result.

[Medium- and long-term results of palliative surgery in complex cyanotic cardiopathies. A propos of 228 cases and 292 operations]. / Résultats à moyen et long terme de la chirurgie palliative dans les cardiopathies cyanogènes complexes. A propos de 228 observations et de 292 interventions.

The results of palliative surgery are assessed in a retrospective study of 228 children operated between 1968 and 1977 for complex cyanotic congenital cardiac malformations, the longest follow-up period being 10 years. The surgery consisted in revascularising the lung by systemo-pulmonary anastomoses: Blalock-Taussing, Potts, Waterston, cavo-pulmonary shunts or, on the contrary, of avoiding pulmonary hypertension by limiting pulmonary flow with banding. In addition to these two techniques, in some cases atrial septal defects were created to improve mixing of the two circulations; equalise the pressures between the two atria and relieve the left ventricle (Blalock-Hanlon procedure) or during catheterisation (Rashkind manoeuvre). The results of pulmonary revascularisation by systemo-pulmonary anastomoses were generally satisfactory with respect to the complexity of the malformations. There were 56% good results, 82 out of 146 cases. It often provided the time for curative surgery to be performed under better conditions for the children. The results were worse in children with pulmonary hypertension, with only 33% of good results, 25 out of 82 children, and there were many complications of banding which additional problems at the time of definitive repair. Despite the tendency to widen the indications of so-called curative surgery even in very small infants, palliative surgery retains a role in complex cardiac malformations either by allowing the children to reach an optimal age for repair or by being the only possible therapeutic attitude.

[Fistula between right pulmonary artery and left atrium. A case report and review of the literature (author's transl)]. / Fistule artério-veineuse entre artère pulmonaire droite et oreillette gauche. Revue de la littérature à propos d'une observation.

The communication between right pulmonary artery and left atrium is a very uncommon cause of right to left shunt. The 18th case in the world literature and the first case in France is reported here. Normal pressure in the right cavities and in pulmonary artery is evocative. Angiography assesses the diagnosis and precises the place and connections of the fistula. Surgical closing of the fistula must be promptly achieved in order to obviate embolic complications.

[2 cases of prosthetic tubes in left pathway obstructions]. / Deux cas de tubes prothétiques dans les obstructions de la voie gauche.

Two cases of severe left ventricular outflow tract obstruction were managed surgically by the insertion of prosthetic tube grafts. The first case was a 6 year old child with diffuse hypoplasia of the descending aorta. A prosthetic tube graft, from the aortic arch to the distal limit of the descending aorta, was inserted through a double left thoracotomy. The second child, aged 3 1/2 years, had hypoplasia of the aortic ring. In this case, a prosthetic tube with a prosthetic aortic valve was inserted from the apex of the left ventricle to the abdominal aorta. This is a simple operation for a complex lesion. In both cases, as in other reported series, the post operative clinical and angiographic results one year later, were good. Some technical points are discussed, and the features which, in the case of aortic ring hypoplasia, led to the choice of the above method instead of that proposed by Konno, are explained. The extra cardiac tube, in fact, does not involve the cardiac structures. Its insertion is simple and non-traumatic, and particularly indicated for children.

[Right pulmonary artery-left atrial fistula. Review of the literature apropos of a case]. / Communication entre artère pulmonaire droite et oreillette gauche revue de la littérature à propos d'une observation.

This is a rare congenital abnormality with only 17 other reported cases in the world literature. The most frequent sign is cyanosis with peripheral arterial desaturation. Angiography with selective injection in the main pulmonary shows early opacification of the left atrium, so confirming the diagnosis. Occasionally, a catheter can be passed through the fistula. Without surgical treatment there is a danger of systemic arterial embolism and two fatal cases were found in our review of the literature. The fistulous connection is often aneurysmal. It arises either from the right main pulmonary artery or from its branch to the inferior lobe. Associated vascular and pulmonary malformations are not rate. Surgical closure of the fistual is curative with immediate regression of the cyanosis and suppression of possible embolic complications.

[2 cases of solitary pulmonary arteriovenous fistula presenting in the neonatal period]. / Deux cas de fistule artério-veineuse pulmonaire solitaire à révélation néonatale.

Two case reports of pulmonary arterio-venous fistulae presenting in the neonatal period are described. One child had severe respiratory distress with cardiac failure and the other presented with cyanosis and a continuous murmur. In both, chest X-ray demonstrated a localised lesion and the presence of an arteriovenous fistula was confirmed by pulmonary angiography. Lobectomy cured both children.

[Anantomical aspects and surgical treatment of pulmonary stenoses and atresias with intact interventricular septum in the newborn and the infant under 6 months]. / Aspects anatomiques et traitement chirurgical des sténoses et atrésies pulmonaires á septum interventriculaire intact du nouveau-né et du nourrisson de moins de 6 mois

The series presented consists of 25 babies (18 of which were neonates), seen between 1972 and 1974. The anatomical and angiographic study revealed 5 anatomical types, according to whether the pulmonary orifice was patent or atretic, and according to the size of cavity of the right ventricle, which may be normal or reduced (perhaps almost totally). The diagnostic clinical features and findings on angiography are recalled. The operative procedure in all 25 cases was pulmonary valvotomy carried out with a brief normothermic period of cirulatory arrest. The technique is described briefly. The results, which vary widely with the anatomical type, were as follows: out of the 25 operated cases there were 12 deaths during or immediately after surgery. The figures are none-the-less encouraging, given the grave natural history of this condition in the baby. The best results were seen in cases in which there was a right ventricular cavity of normal or at least acceptable size. The maximum postoperative follow-up period was 3 years. Most of these children have a persistant pulmonary stenosis and hypertrophic right ventricle, and require a second operation in early infancy.

[Results of Mustard's operation for transposition of the great vessels. Apropos of 81 cases of children who survived the intervention with a follow-up of from 1 to 6 years]. / Les resultats de l'intervention de Mustard pour la transposition des gros vaisseaux. A propos de 81 observations d'enfants ayant survecu a l'intervention et comportant un recul de 1 à 6 ans

In a series of 81 children who survived to Mustard's operation, with a follow-up varying from 6 months to 6 years, there were 19% of secondary deaths, 19% of bad results, 29% of questionable results and only 33% of good results. The most frequent complications were: disturbances of cardiac rhythm and obstructions to pulmonary or caval venous return.

[Catheterization of the left heart by retrograde arterial pathway in children. Apropos of 100 explorations by femoral arteriotomy]. / Cathétérisme des cavités gauches par voie artérielle rétrograde chez l'enfant. A propos de 100 explorations par artériotomie fémorale

A study has been made of 100 cases in which a surgical technique has been used to carry out aortography in the child. The patients were aged between 12 days and 14 years. 20 per cent of the subjects were infants. The technique which was used was that of arteriotomy of the common femoral artery; the artery was repaired by interrupted sutures. The advantage of this technique is that it allows prolonged arterial catheterisation, and therefore seems to be indicated in the complex cardiopathies and also in cases of stenoses about the aortic outflow. The complications and local problems were 6 in number. They occurred either in the youngest subjects, or in those with aortic stenosis. Taken as a whole, the results were very satisfactory. It would appear that in a paediatric cardiology unit, this technique has a place alongside that of Seldinger, with its slightly different indications.

[Congenital mitral abnormalities in children. Apropos of 44 cases]. / Les malformations mitrales congenitales de l'enfant. A propos de 44 cas

Forty-four cases of mitral malformation were studied. Thirty times it was a mitral regurgitation, 10 times, a mitral stenosis. High frequency of associated malformations was observed. Tolerance was almost always very bad, leading to surgery in spite of the young age. Operative mortality was high (30%). To a certain extent, surgery should be palliative: commissurotomy in case of stenosis, with almost always bad results; annuloplasty in case of regurgitation, with sometimes good results. Good surgical results were observed in one third of the cases.

[Long-term results of surgery in total abnormal pulmonary venous return in the infant. Apropos of 32 cases]. / Résultats éloignés de la chirurgie du retour veineux pulmonaire anormal total chez le nourrisson. A propos de 32 cas

Report of 32 cases of infants with total anomalous pulmonary venous return, operated upon, below the age of 9 months. There were three immediate deaths, and one which occured six months after operation. All surviving children were followed-up regularly, and the post-operative follow-up period exceeded 18 months in all cases. 11 children went through control haemodynamic exploration. These were ten excellent post-operative results, 6 were considered as fairly good and two bad ones. For some of them who were markedly improved by the first operation a second intervention consisting in closing the atrial septal defect left patent, should be foreseen in later childhood.