[Unusual natural history of a case of congenital aneurysm of the sinus of Valsalva].
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Aged↗
Biomedical subjects
M Villani
Publications and source records attributed to M Villani.
Waterston anastomosis for initial palliation of tetralogy of Fallot.
Two hundred twenty-seven patients (median age 5.4 months) in whom a Waterston anastomosis was done for initial palliation of tetralogy of Fallot between 1966 and 1979 were studied. Twelve patients died in the hospital (5.3%; 70% confidence limits, 3.8% to 7.3%). Young age, low weight, and poor clinical condition did not appear to be incremental risk factors, whereas a too large or a too small shunt was largely responsible for the hospital mortality and morbidity. Follow-up information was available in all the 215 patients discharged from the hospital. At the last follow-up visit, before any further surgical procedure, 74% of the patients were clinically in good condition. By actuarial methods, 97.7% of hospital survivors were alive and 95.8% were event-free at and beyond 3 years postoperatively. Eighty-six patients have been catheterized in preparation for secondary repair (mean interval between Waterston shunt and catheterization, 2.9 +/- 1.38 years). One patient developed pulmonary vascular disease, four acquired pulmonary atresia, and 14 had a severe kinking of the right pulmonary artery at the site of the anastomosis.
Angiocardiography↗
[Symptomatic vascular rings in infancy. Surgical treatment in 19 patients (author's transl)].
The Authors report their experience in the surgical treatment of 19 patients under two years of age (75% under six months), with severe symptoms for the presence of a complete or incomplete vascular ring. Twelve patients had double aortic arch, two had a right aortic arch with a left ligamentum arteriosum, four a symptomatic retroesophageal right subclavian artery and one an abnormally placed innominate artery. Symptoms, methods of diagnosis, indications to the surgical treatment, surgical techniques and late results are considered. The Authors remark that even if correction of these rare congenital malformations may be simple and with a low operative mortality, many problems exist in the early diagnosis and in the post-operative treatment of these patients.
Aorta, Thoracic↗
Surgical repair of persistent truncus arteriosus in infancy.
Fourteen patients younger than two years of age with persistent truncus arteriosus underwent primary repair. Twelve of them were less than 1 year and 4 less than 3 months of age. Intractable heart failure was the indication for surgery in all patients but one who had increased pulmonary vascular resistance. There were 5 hospital and 2 late deaths. Six out of the 7 survivors (median follow-up: 29 months) were symptom-free. The remaining infant who preoperatively had significant truncal valve regurgitation was doing fairly well 2 1/2 years after repair. Our experience suggests that, although the mortality remains high, primary repair for infants with persistent truncus arteriosus is feasible and offers better overall results than does pulmonary artery banding followed by later intracardiac repair. We advise primary repair for all infants with intractable heart failure or increasing pulmonary vascular resistance with or without truncal valve regurgitation. Elective repair is recommended before the age of 2 years to minimize the risk of pulmonary vascular disease.
Follow-Up Studies↗
Protein-losing enteropathy after Fontan operation for tricuspid atresia (imperforate tricuspid valve).
Protein-losing enteropathy occurred in a 7-year-old girl with tricuspid atresia, concordant ventriculo-arterial connexions and a relatively large hypoplastic right ventricle, one year after an atrio-ventricular type of Fontan operation by means of a valveless woven Dacron conduit. Severe conduit regurgitation and a marked enlargement of the hypoplastic right ventricle were demonstrated at recatheterization. Insertion of a bioprosthetic valve at the base of the right atrial appendage led to a dramatic clinical recovery of the patient. The use of a valved conduit is recommended when an atrioventricular type of Fontan repair is planned in patients with tricuspid atresia, concordant ventriculo-arterial connexions and relatively large hypoplastic right ventricle.
Aortic Valve Insufficiency↗
Left ventricular outflow tract obstruction due to an aneurysm of the membranous septum in a patient with pulmonary atresia and ventricular septum defect - case report.
Although the occurrence of subpulmonary stenosis resulting from displacement of an aneurysm of the membranous septum is well known, resultant subaortic stenosis has not been well documented. We present an unusual case of acquired pulmonary atresia with restrictive ventricular septal defect and associated severe subaortic resulting from an aneurysm of the membranous septum.
Abnormalities, Multiple↗
Aortico-left ventricular tunnel in infancy. Two surgical cases.
Two infants with aortico-left ventricular tunnel and associated aortic valvular disease were operated upon. The first patient, age 28 months, had successful surgical closure of the anomalous communication. Four years postoperatively he is asymptomatic, but mild aortic regurgitation persists. The second infant, aged 3 months (the youngest reported surgical case), also had severe aortic stenosis (dysplastic valve) and mitral regurgitation. She died, twelve hours after the operation, from intractable left ventricular failure. On the basis of the previously reported cases (22), the clinical profile of the malformation is outlined with special reference to associated aortic valve lesions. Early operation is recommended because of the poor natural history of the condition.
Abnormalities, Multiple↗
Complete atrioventricular canal associated with tetralogy of Fallot. Successful repair using a new surgical technique. A case report.
A modified Mayo technique was successfully employed to correct a case of complete atrioventricular (a-v) canal associated with tetralogy of Fallot. The technique included: 1) separated patch closure of the atrial and ventricular septal defects, 2) attachment of the a-v valves between the two prosthetic septa without suturing the clefts, 3) mitral valve commissuroplasty, 4) relief of the right ventricular outflow obstruction. Postoperative catheterisation revealed normal haemodynamic findings. The child is asymptomatic two years after the operation.
Abnormalities, Multiple↗
[Surgical treatment of truncus arteriosus in the 1st year of life].
80% of patients with Truncus arteriosus died in the first year of life by cardiac insufficiency from left to right shunt, high level of pulmonary vascular disease, truncal valve insufficiency. The palliative procedure, banding of the pulmonary arteries, is technically difficult, with high hospital mortality (50%) and increases the risk of the second stage repair (70%). Eight infants (1 to 9 months) have been corrected with 3 operative deaths and two late deaths. Three patients are doing well 12, 12 and 36 months post-operatively. Two technical problems are of concern: 1: The size of the valved extracardiac conduit comparing with the size of the infants thorax and the long term follow-up of such conduit. 2: The management of the truncal valve insufficiency: no plasty or replacement have be done, but in three cases of truncal insufficiency one pericardial patch has been used to double the VSD's dacron patch to avoid hemolysis. The one-stage repair of truncus arteriosus in infancy is one acceptable approach.
Humans↗
[Fibroangioma of the small intestine. Description of a case].
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Child↗
Atrio-ventricular canal malformations. Recent surgical techniques.
Twenty patients with atrio-ventricular canal malformations (5 complete and 15 partial forms) were operated upon during the last two years, using new surgical techniques. The repair of the complete form based on a three-leaflet three-commissure mitral valve concept, was accomplished by: a) Two separate partitioning patches (one ventricular, one atrial), b) mitral and tricuspid valve attachment in between, c) no cleft sutures, d) correction of subvalvular apparatus abnormalities, e) commissuroplasty. The repair of the partial form was obtained by: a) Valvular and subvalvular repair of the lesions when present, b) patch closure of the ostium primum defect suturing on the tricuspid side. All patients survived operation and exhibited normal sinus rhythm. Residual mitral incompetence proved to be less frequent as compared to previous techniques.
Adolescent↗
Transposition of the great arteries. Successful Senning's operation in 35 consecutive patients.
Mustard's operation for TGA (transposition of the great arteries) has been obstructive complications. In order to try to avoid these complications, we used Senning's operation for TGA. Our experience in 35 consecutive cases is reported. There were no operative or late deaths. Four patients had a PDA; three had a significant VSD with subpulmonary stenosis in two. Previous atrial septectomy and persistent LSVC did not represent contraindications to this procedure. The postoperative course has been smooth and uneventful in all patients. Follow-up periods of one to twenty months demonstrated sinus rhythm in all patients and there were no significant gradients between the venae cavae and the new systemic atrium in 12 reinvestigated patients. On the basis of these results, Senning's operation is recommended as a valid alternative to Mustard's operation.
Cardiac Catheterization↗
Results of systemic-to-pulmonary artery anastomosis for tricuspid atresia with reduced pulmonary blood flow.
Fifty-six patients with tricuspid atresia and decreased pulmonary blood flow received a systemic-to-pulmonary artery anastomosis as a preliminary operation. Thirty-five had a Waterston shunt, 12 a Blalock-Taussig anastomosis, and nine various other procedures. The age at operation ranged from 2 days to 10 years (median 4.5 months). Pulmonary atresia was present in eight newborn infants. Four patients (7%), all less than 3 months old, died in hospital from persistent servere hypoxia despite the palliative procedure. By actuarial methods, 93% of the survivors were alive at and after one year (longest follow-up:9.8 years), while 83% had not required further palliation at and after four years from the first operation. None of the patients subsequently restudied had raised pulmonary pressure or resistances, and in most of them the pulmonary arterial tree was deemed adequate for the Fontan operation.
Age Factors↗
[Chylorthorax following heart surgery].
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Child↗
[Ventricular septal defect associated with aortic regurgitation. Results and surgical considerations in pediatric age group (author's transl)].
The Authors report their experience on the surgical treatment of ventricular septal defect and aortic regurgitation. Out of 13 patients operated upon, four required aortic valve replacement; five patients were treated with plasty of the aortic leaflets and four with the only patch closure of the V.S.D. There have not been reported hospital or late deaths. In one case, after the aortic plasty, the aortic regurgitation became severe; this patient had shown clinical signs of aortic regurgitation for more than five years before the operation. The Authors emphasize the surgical aggressiveness in this lesion when the aortic regurgitation has recently showed, whereas it is preferable to delay the operation when the aortic regurgitation has been present for more than five years.
Aortic Valve Insufficiency↗
[Autoptic findings and anatomo-clinical correlations in subjects who died after repair of tetralogy of Fallot (author's transl)].
The postoperative course and the post-mortem findings of a group of 30 patients who died after repair of tetralogy of Fallot were reviewed. A residual ventricular septal defect was the most common finding at the autopsy (13 patients = 43%) while a severe residual obstruction to the right ventricular outflow was found in 5 patients (17%), miscellaneous lesions in 3 (10%) and no intracardiac defects in 9 (30%). Since a residual anatomic defect is frequently responsible for the hospital mortality, prompts hemodynamic evaluation and reoperation is recommended when the postoperative course is deteriorating.
Autopsy↗
Experience in palliative treatment of univentricular heart including tricuspid atresia.
Over a period of ten years 110 patients with univentricular heart, including cases with tricuspid atresia, received palliation. The overall hospital mortality was 14.5%. Late mortality during a mean follow-up period of two years, was 6%. A group of patients with univentricular heart ("complex" group: 21 cases) had numerous and significant associated anomalies which affected the surgical results (16 survivors). Eight patients were discovered to have univentricular heart only at open heart surgery (5 survivors). In the remaining patients hospital mortality was 7.4% (6/81). As compared with the natural history these results indicate that palliative surgery is still an effective type of treatment for these two severe cardiac malformations.
Heart Defects, Congenital↗