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Biomedical subjects

M de Leval

Publications and source records attributed to M de Leval.

At least 55 records · Page 3Linked to original sources

Surgical treatment of absent pulmonary valve syndrome.

The absent pulmonary valve syndrome includes agenesis of the pulmonary valve, annular stenosis, and pulmonary insufficiency. The pulmonary arteries are aneurysmal and usually compress the tracheobronchial tree. These features are associated with a ventricular septal defect and right ventricular hypertrophy. Nineteen children aged 5 days to 11 years were treated between 1976 and 1983. Nine were younger than 1 year. Intractable respiratory symptoms and heart failure were the main features in the infant group; five required preoperative assisted ventilation. Older children had decreased exercise tolerance and repeated respiratory infections. Several surgical techniques were used in this series. The best results were achieved when the size of the main, right, and left pulmonary arteries was reduced by extensive anterior resection; the ventricular septal defect was closed; and an aortic homograft was interposed between the right ventricle and the pulmonary artery. Four infants and four children treated in this way survived the operation. Five of the nine operated infants died (56%); all older children survived the operation. At follow-up, six children were asymptomatic and one was in New York Heart Association Class II. All four infants were progressing satisfactorily although all had some residual pulmonary symptoms. Closure of the ventricular septal defect, reduction of the size of the pulmonary arteries, and insertion of an aortic homograft was the optimal treatment in our series.

Aneurysm↗

Anatomically corrected malposition of the great arteries: diagnostic difficulties and surgical repair of associated lesions.

Four patients, aged 5 to 14 years, had repair of abnormalities associated with anatomically "corrected malposition," a condition associated with mitral/aortic discontinuity with atrioventricular and ventriculoarterial concordance, and with the aorta lying anterior and to the left of the pulmonary artery. All 4 patients had ventricular septal defect; in addition, 1 patient had coarctation, 2 patients had pulmonary infundibular stenosis, 1 patient had acquired pulmonary valve atresia, 1 patient had acquired fixed subaortic stenosis and 2 patients had left juxtaposition of the atrial appendages. The position of the great arteries suggest corrected transposition, but the true diagnosis is made from finding atrioventricular and ventriculoarterial concordance with wide mitral/aortic separation on the cross-sectional echocardiogram. The electrocardiogram shows normal ventricular activation. Three of the 4 patients had anticlockwise rotation of the heart, which displaced the apex. This made echocardiography difficult and caused a problem in visualizing the right-sided valved conduit and closing the VSD in conventional ways. One patient died at reoperation for an obstructed conduit. Mitral regurgitation developed after resection of subaortic stenosis in 1 patient. No arrhythmias occurred in the 3 surviving patients, followed for 1 to 5 years, but the risk of subaortic stenosis remains.

Adolescent↗

The diagnosis of airway obstruction in children.

Airway obstruction in children has many causes. Although vascular rings may have early onset of symptoms there was considerable delay in establishing the correct diagnosis. Barium swallow is diagnostic. Other tests, such as angiocardiography, bronchoscopy, bronchography, and lung scan are usually unnecessary. Pulmonary sling is a less common cause of vascular obstruction. Barium swallow showing anterior indentation at the level of the pulmonary hilum is diagnostic. Cysts and tumors are other causes of severe airway obstruction. The diagnostic and operative problems in three patients are discussed as examples. It is concluded that barium swallow is the most important single investigation in the evaluation of airway obstruction.

Airway Obstruction↗

Pulmonary sequestration complicated by anomalies of pulmonary venous return.

Five anomalies of pulmonary venous drainage were seen among 12 children operated for lung sequestration. In two children, venous drainage from the sequestrated lobe and the rest of the right lung was via a single channel into the inferior vena cava ("scimitar syndrome"). In one of these children, the sequestrated lobe was resected and repair of the scimitar syndrome was delayed; in the second patient, the anomalous pulmonary venous drainage was not recognized preoperatively and the vein was ligated, resulting in acute hemorrhagic infarction of the right lung and death of the patient. Three patients had less severe anomalies of pulmonary venous drainage. We recommend very careful evaluation of patients with lung sequestration with special reference to pulmonary venous drainage.

Abnormalities, Multiple↗

Long-term results of the "palliative" Mustard operation.

A long-term follow-up study after 41 palliative Mustard operations for transposition of the great arteries and pulmonary vascular obstructive disease is presented. The operations were performed from 1973 to 1980. Mean pulmonary arteriolar resistance was 13.96 +/- 6.7 Um2. A ventricular septal defect was not closed in 34 patients; in 7 it was created at operation. There were three hospital and two late deaths. Survivors were followed up for 3 to 10 years (mean 76.7 +/- 22.8 months). Before operation 4 children were in New York Heart Association functional class IV and 33 were in class III. Mean hemoglobin concentration was 19.43 +/- 3.14 g/dl and arterial oxygen saturation was 63.44 +/- 11.29%. After operation 18 patients were in functional class I, 17 in class II and only 1 in class III. Hemoglobin level decreased to 14.19 +/- 2.3 g/dl and arterial oxygen saturation improved to 89.12 +/- 7.25%. Cardiac catheterization in 21 survivors confirmed no change in pulmonary artery pressure and resistance. Effective pulmonary blood flow improved from 1.39 +/- 0.39 before to 2.6 +/- 0.78 liters/min per m2 after operation. The palliative Mustard operation carries a low risk and provides excellent symptomatic improvement up to 10 years in patients with transposition of the great arteries and pulmonary vascular disease.

Adolescent↗

Mustard's operation for patients with ventriculoarterial concordance. Report of two cases and a cautionary tale.

Three patients with ventriculoarterial concordance and an abnormal atrioventricular connexion were investigated. One patient had total anomalous pulmonary venous connexion to a left vertical vein. In 1972 this was repaired and a ventricular septal defect was closed, but the presence of atrioventricular discordance was missed and the patient died. In the other two patients, Mustard's operation resulted in survival. One patient had situs solitus and atrioventricular discordance with an associated ventricular septal defect, which was closed. The other had left isomerism (quasi solitus) with an ambiguous atrioventricular connexion (quasi discordant). A previously noted ventricular septal defect had spontaneously closed.

Angiocardiography↗

Decision making in the definitive repair of the heart with a small right ventricle.

Data from 51 patients with small right ventricle who underwent a definitive repair were analyzed retrospectively. The repair was considered complete (29 patients) when it resulted in relief of the right ventricular outflow tract obstruction by closure of intracardiac (atrial septal defect) and extracardiac (when present) shunts. The repair was considered a definitive palliation when the intracardiac and the extracardiac shunts were left open (11 patients). A complete separation of the systemic and pulmonary circulations was established with the use of the Fontan procedure in another 11 patients. The outcome in these patients correlated with the preoperative size of the right ventricular cavity as assessed by measurement of the tricuspid valve diameter and by consideration of the right ventricular morphology (based on the tripartite right ventricular concept). If the tricuspid valve of a tripartite right ventricle has a diameter above the lower 99% confidence limit of the normal mean it can be safely incorporated in a complete repair (p less than .01). This also applies for a right ventricle without a trabecular portion, provided the criteria of the Fontan operation are fulfilled. Right ventricles with tricuspid valve diameters smaller than the lower 99% confidence limit of the normal mean cannot sustain total pulmonary blood flow. Definitive palliation should be reserved for use in patients whose right ventricles are too small for complete repair and who are not suitable candidates for Fontan's procedure.

Adolescent↗

The landmarks to the atrioventricular conduction system in hearts with absence or unroofing of the coronary sinus.

Histologic studies were made of the conduction system of two hearts, one with absence and the other unroofing of the coronary sinus. The findings show that, when present, the triangle of Koch and the orifice of the coronary sinus are usually good guides to the atrioventricular conduction axis. However, when these landmarks are malformed, or there is malalignment of the atrial and ventricular septal structures, a better guide is the fusion point of the ventricular septum with the atrioventricular junction.

Autopsy↗

Experience with fibrin seal (Tisseel) in operations for congenital heart defects.

Tisseel (human fibrin seal) was used in 38 children with complex congenital heart defects (2.8% of all operations for congenital heart defects) between July, 1980, and August, 1983. Control of bleeding was excellent in 31 patients, good in 6 patients, and unsatisfactory in 1 patient. Best results were achieved when Tisseel was applied on collagen substrate. If bleeding was particularly difficult to control, a combination of Tisseel with pharmacological hypotension (sodium nitroprusside) was used. We recommend the use of Tisseel to preseal Dacron conduits and to control bleeding from multiple suture lines (conduits, arterial switch operations, complex reconstructions of the pulmonary arteries), near major coronary arteries, from Gore-Tex patches in high-pressure systems, and from the raw areas of the heart at reoperation.

Aprotinin↗

Two-dimensional echocardiographic assessment of surgically closed ventricular septal defect.

One hundred twenty patients operated on for closure of a ventricular septal defect (VSD) had postoperative assessment by 2-dimensional echocardiography. The presence of persistent "drop-out" between ventricular septum (VS) and the periphery of the "patch" was considered a sign of detachment and residual shunt. Echocardiographic findings of intact VS or residual VSD were correlated with angiographic findings in 30 patients and with clinical signs in 90 patients. Minute shunts with increased flow ratios less than 1.5:1 were occasionally missed. Persistent small drop-out was sometimes wrongly diagnosed as residual shunt when the patch was in an angled position and in the early postoperative weeks when peripatch edema of the VS suggested drop-out. Thus, 2-dimensional echocardiography is a reliable method for assessing significant persistent ventricular shunts and intact VS after surgical closure of a VSD.

Echocardiography↗

Aortopulmonary septal defect coexisting with ventricular septal defect and pulmonary atresia.

Three patients are described in whom an aortopulmonary septal defect (aortopulmonary window) coexisted with a ventricular septal defect and pulmonary atresia. One patient had mild and another, moderate aortic regurgitation. In addition, one patient had a sinus of Valsalva aortic aneurysm, while another had a single coronary artery arising from the pulmonary trunk. One patient underwent surgical correction in infancy; the other two, in early adult life. In all three patients, surgical correction involved closure of the aortopulmonary window, closure of the ventricular septal defect, and placement of a valved conduit between the right ventricle and the distal pulmonary trunk. One patient died 3 weeks postoperatively due to secondary hemorrhage where the conduit had been sutured to the right ventricle. The other two patients are alive and well 3 1/2 years and 6 months after surgery, respectively. The presence of aortopulmonary window permits normal development of the pulmonary arteries in the presence of the coexisting pulmonary atresia; in the three patients described, the absence of pulmonary vascular disease made total surgical repair feasible for this combination of defects.

Adult↗

Congenital heart disease in the neonate: results of surgical treatment.

All 212 neonates undergoing cardiac surgery at this hospital during the 5-year period from 1976 to 1980 inclusive were reviewed. Forty required open heart surgery with 23 (57%) deaths. One hundred and seventy-four neonates underwent non-bypass procedures and could be divided into three groups: group 1 (82 patients) had inadequate pulmonary blood flow, group 2 (33 patients) had increased pulmonary blood flow or inadequate mixing, and group 3 (59 patients) had coarctation of the aorta, alone or with associated lesions. Forty-four (25%) of the neonates undergoing non-bypass procedures died. Two required bypass surgery later in the first month of life. Metabolic acidosis and the need for preoperative respiratory support were appreciably greater in non-surviving patients. The spectrum of diagnoses encountered and types of operative procedures performed are analysed.

Cardiac Surgical Procedures↗