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Biomedical subjects

G Stellin

Publications and source records attributed to G Stellin.

At least 73 records · Page 4Linked to original sources

The surgical anatomy of the Taussig-Bing malformation.

There is no consensus as to the most appropriate definition of the use of the term Taussig-Bing malformation. In this study, we endorse the suggestion that the lesion be considered a spectrum of anomalies, unified by a juxtapulmonary ventricular septal defect with malalignment of the infundibular septum. The ends of the spectrum are hearts with, on the one hand, double-outlet right ventricle and, on the other hand, a discordant ventriculoarterial connection. We studied 10 hearts within this spectrum, eight with a double outlet and two with discordant ventriculoarterial connection. We found that the same basic morphology could exist with a bilaterally complete infundibulum or in the presence of fibrous continuity between the mitral and the pulmonary valves. Features of most significance for surgical correction were the arrangement of the outlet septum (particularly the presence or absence of chordal attachments from the atrioventricular valves), the morphology of the coronary arteries, and the state of the subaortic infundibulum. The options for surgical correction are determined by the internal morphology of the lesions rather than by the precise ventriculoarterial connection.

Child↗

[Double outlet right ventricle. Angiographic study].

Cineangiographic study in 41 patients with double outlet right ventricle was performed. Segmental approach was utilized to describe the anatomical features. Atrial situs was inversus in 5 cases, ambiguous in 3 and solitus in the other cases. Atrio-ventricular (a-v) connections were biventricular in 34 and univentricular in 7. A common a-v valve was found in 2 cases. Mitral valve was imperforated in 1 and straddling in 1. The most frequent relationship between aorta and pulmonary artery was side by side (41.1%). Ventricular septal defect was subaortic in 14, subpulmonary in 9, double committed in 5 and non committed in 6. In 27 cases a double infundibulum was present; in 7 patients we described a subpulmonary conus only. Some nosological aspects of this congenital heart disease are discussed particularly in conformity with the surgical needs.

Adolescent↗

Description of a continent jejunal gastrostomy.

A new technique of permanent gastrostomy interposes a vascularized jejunal conduit with an intussuscepted valve between the stomach and abdominal wall, creating a "continent jejunal gastrostomy". In a series of dogs undergoing gastrostomy, the continent jejunal gastrostomy functioned well, with no leakage, and was superior to gastric tube gastrostomy in this regard. Furthermore, there was no evidence of marginal ulceration. A patient who underwent continent jejunal gastrostomy has an excellent one-month result.

Animals↗

Repair of ventricular septal defect and aortic regurgitation associated with severe hemophilia B.

A child with severe factor IX deficiency who underwent an open-heart operation using extracorporeal circulation is described. The factor IX level was normalized immediately before operation and at the end of cardiopulmonary bypass by infusing prothrombin complex concentrate and fresh frozen plasma. Partial thromboplastin time and factor IX serum levels were monitored for 20 days postoperatively and showed factor IX activity higher than 50%.

Acute Disease↗

The risk of surgical treatment of tetralogy of Fallot: an appraisal.

The risk of surgical treatment of tetralogy of Fallot was analyzed with multivariate statistical techniques in a recent surgical series (1975-1983) of 159 corrected and 59 palliated patients. The overall mortality of correction was 12.6% and that of palliation was 15.3%. The significant and independent risk factors that increased the mortality of palliation were the use of a shunt other than the Blalock or its modifications (mostly Waterston) (P = 0.01), and very young age (P = 0.02). Risk factors that increased the mortality of correction were high left over right ventricular pressure ratio (P-RV/LV) (P = 0.02), persistent patency of the arterial duct (P = 0.02), other major associated lesions (P = 0.02), and the use of a transannular patch (P = 0.04). Cardioplegia significantly decreased the risk (P = 0.03). With present techniques, correction can be performed in favorable cases with a mortality approaching zero. Staged procedures neutralized the age related risk of correction, but Blalock shunts carried a mortality rate ranging from 9.5% at 1 month to 1.7% at 12 months of age. This was due to the lack of prostaglandins along with technical faults. Predicted two-stage mortality rates ranged in infants (less than 12 months) from 6.2 to 16% and compared favorably with our observed 27% mortality of primary correction. Our results show that the transannular patch becomes a fully expressed risk only in those patients with restrictive pulmonary arteries or isolated branch stenosis. This is because it decreases the force the right ventricle can generate in maintaining the cardiac output. The chances of survival, when compared with those of patients without transannular patch, are significantly less when the P-RV/LV is between 0.6 and 1.0. We therefore discuss the measures to decrease or neutralize this risk related to insertion of a transannular patch. Among the associated lesions, we discuss briefly the potential for residual right outflow tract stenosis, anomalous origin of the left anterior descending coronary artery and anomalous muscle bundle of the right ventricle.

Adolescent↗

Non-invasive assessment of pulmonary blood supply after staged repair of pulmonary atresia.

Radionuclide studies were performed to determine pulmonary blood flow in six children who had undergone surgery for pulmonary atresia, ventricular septal defect, and hypoplastic pulmonary arteries with or without major aortopulmonary collateral arteries. Lung blood flow was assessed from both particle perfusion lung scans and the pulmonary and systemic phase of a radionuclide dynamic flow study. Five patients had perfusion defects identified on the particle perfusion lung scan. In three of these, abnormal areas were perfused only during the systemic phase of the flow study, a combination of findings that indicate the presence of perfusion by collateral arteries. In one patient no systemic perfusion was noted and in one an initial particle perfusion study indicated the presence of a lung segment perfused by a collateral artery. In this last patient the particle perfusion scan after total correction showed a reduction in the size of the lung perfusion defect and no evidence of lung perfusion during the systemic phase of the flow study. The particle perfusion lung scan in the sixth patient showed pronounced asymmetry in blood flow to the lungs with no segmental perfusion defect on the particle perfusion scan and no abnormalities on the systemic flow study. It is concluded that radionuclide lung perfusion and flow studies provide useful information on lung perfusion and merit further evaluation to define their role in the management of these patients.

Abnormalities, Multiple↗

Surgical management of double-outlet right ventricle.

From 1977 to 1983, 32 consecutive patients, ranging in age from 15 days to 24 years, underwent operations for double-outlet right ventricle. Twenty patients had a palliative operation either to increase (12 cases) or to reduce (eight cases) pulmonary blood flow: Ten of them have subsequently undergone total repair, and in another six correction was delayed because of possible incremental operative risk factors, such as multiple ventricular septal defects or the need for an extracardiac conduit. Four patients with multiple, complex associated intracardiac anomalies are currently considered to have uncorrectable defects. A total of 22 patients underwent correction either primarily (12) or after palliation (10). Intraventricular tunneling was performed in 16 patients with a subaortic ventricular septal defect and in one with a doubly committed ventricular septal defect. Seven of these had pulmonary stenosis and five had reconstruction of the right ventricular outflow by means of a patch (three) or a conduit (two); among this group, five also had enlargement of the ventricular septal defect. In three patients with a subpulmonary defect and in one with a remote ventricular septal defect, all of them without pulmonary stenosis, total repair was achieved by a Senning, a Mustard, or an arterial switch operation. Finally, the only patient with atrioventricular discordance and pulmonary stenosis had insertion of a left ventricle-pulmonary artery conduit. No operative deaths were observed after palliation, but one patient died of intrapulmonary hemorrhage after total repair (4.5%). Major postoperative complications included detachment of the ventricular septal defect patch in one patient and late progression of pulmonary vascular obstructive disease in another. No late deaths have been recorded. Surgical repair of double-outlet right ventricle can be accomplished with gratifying early and late results, the risk of operative death being below 5%. The outcome in patients with subaortic ventricular septal defect appears particularly favorable, despite the extensive intracardiac procedures required for total correction. An early intervention is recommended to prevent development of pulmonary vascular obstructive disease and to avoid massive cardiac hypertrophy and fibrosis, which may cause late rhythm disturbances and impede the intracardiac repair.

Adolescent↗

The multiple coagulopathies of biliary atresia.

Detailed coagulation studies were done prospectively on 43 patients with biliary atresia who had undergone Kasai operation (hepatic portoenterostomy). Patients were divided into three groups based on levels of factor V, factor II, and Echis II and/or response to vitamin K: no coagulopathy (46.5% of patients); coagulopathy of liver disease (30.2% of patients); and coagulopathy of vitamin K deficiency (23.3% of patients). Patients with the coagulopathy of liver disease had significantly lower levels of factors XII, V, and antithrombin III as well as longer thrombin times than patients with no coagulopathy or vitamin K deficiency. Factor V levels were decreased only in patients with more advanced liver disease; normal levels of factor V were not usually helpful in differentiating liver disease and vitamin K deficiency. The prothrombin time, factor VII-X levels, and factor II levels were significantly different for all three groups; the most abnormal values occurred in the vitamin K-deficient group. Comparison of the Echis II level to factor II coagulant activity was helpful in deciding whether a coagulopathy was due to liver disease, vitamin K deficiency, or both. Factor VIII levels were elevated in all groups. Factor VIII coagulant activity was significantly higher by the two-stage (TGT) method than by the one-stage (PTT) method. Hypersplenism causing neutropenia and thrombocytopenia was commonly seen after the age of 5 years. Vitamin E deficiency was more common than vitamin K deficiency; however, all vitamin K-deficient patients were vitamin E deficient. Coagulation status correlated well with hepatobiliary scan data, but not serum bilirubin levels. Recommendations for treatment of patients with vitamin K deficiency and/or liver disease are discussed.

Bile Duct Diseases↗

Variceal hemorrhage in biliary atresia.

Eleven of 47 patients (23%) with sustained bile drainage after Kasai's operation for biliary atresia had major variceal hemorrhage. Hemorrhage was esophageal in origin in five children and from the exteriorized conduit in eight. Two patients bled from both sites. The first major hemorrhage occurred at an average patient age of 40.4 months. Clinical manifestations of portal hypertension were poorly correlated with liver functions, however, markedly decreased calibre of the intrahepatic venous radicles was noted by ultrasonography in all patients examined. Primary treatment was by esophageal endosclerosis or by disconnection of portosystemic shunts coincident with stomal closure. Adjunctive splenic embolization was employed in six patients. Recurrent hemorrhage has not been encountered in limited follow-up.

Abdominal Muscles↗

Surgical treatment of absent pulmonary valve syndrome in infants: relief of bronchial obstruction.

Congenital absence of the pulmonary valve is usually associated with a malalignment type of ventricular septal defect as well as right ventricular outflow obstruction and aneurysmal dilatation of the pulmonary arteries. Symptomatic infants primarily have severe tracheobronchial obstruction caused by aneurysmal dilatation of the proximal pulmonary arteries; pulmonic and systemic blood flow are usually balanced. Surgical intervention to relieve the obstruction by plicating the pulmonary artery and its branches under deep hypothermia and circulatory arrest, together with patch closure of the ventricular septal defect, is advocated.

Airway Obstruction↗

Experience with operation for total anomalous pulmonary venous connection in infancy.

Twenty patients presenting with total anomalous pulmonary venous connection (TAPVC) in infancy underwent cardiac repair. Four had associated major intracardiac anomalies (complex TAPVC) and 16 had isolated TAPVC. All patients with complex lesions died during or shortly after the operation; they all had pulmonary venous obstruction (PVO). The associated malformations were critical pulmonary stenosis (one case), multiple ventricular septal defects (VSDs) (one case), mitral atresia (one case), and asplenia syndrome with common atrioventricular valve, double-outlet right ventricle, and pulmonary stenosis (one case). Among the patients with isolated lesions there were two surgical deaths, both in infants less than 1 month of age, with severe PVO and subdiaphragmatic drainage. Statistical analysis of these data shows a strongly incremental risk of surgical death due to the presence of associated malformations. Analysis of just isolated TAPVC shows a possible incremental effect due to neonatal age (less than 1 month) and PVO, these two factors being not clearly separated. There have been no late deaths and no late complications among the 14 survivors followed from 7 months to 10 years (mean 44 months). An aggressive surgical approach regardless of the age, degree of pulmonary hypertension, and type of anatomic connection is advised for isolated TAPVC. Some caution is recommended for complex TAPVC, in which a much higher risk is anticipated, particularly when a palliative pulmonary vein-to-left atrium anastomosis is performed.

Evaluation Studies as Topic↗

Esophageal endosclerosis in children with portal vein thrombosis.

During the past 3 2/3 yr, 6 children with portal vein thrombosis were treated for esophageal hemorrhage by direct injection of esophageal varices with sodium morrhuate (endosclerosis). Four children were actively hemorrhaging at the time of initial endosclerosis; 2 patients were treated electively. Endosclerosis was performed by (1) employment of an especially slotted rigid esophagoscope, (2) direct intravariceal injection, (3) injection of varices at the gastroesophageal junction only. In patients actively hemorrhaging, endosclerosis was repeated every 3 to 4 days until the bleeding ceased. Once bleeding was controlled the procedure was done at 6-wk intervals until esophageal varices were obliterated. In the four patients actively hemorrhaging, bleeding was controlled by 2 to 3 separate injection sessions. Four patients have completed treatment and varices are absent radiographically and endoscopically. No patient has rebled during or after treatment in follow-up from 1/6 to 3 2/3 yr. Esophageal endosclerosis is advocated as a legitimate alternative to portosystemic shunt operations and nonoperative management of esophageal variceal hemorrhage in children with portal vein thrombosis.

Adolescent↗

Catheter decompression of hepatic portocholecystostomy.

Temporary catheter decompression of the gallbladder was employed in three infants having hepatic portocholecystostomy for biliary atresia. Postoperative biliary obstructive complications were avoided. A progressive increase in the calibre of the distal bile ducts was observed.

Bile↗