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Biomedical subjects

R Tiraboschi

Publications and source records attributed to R Tiraboschi.

At least 37 records · Page 2Linked to original sources
PubMed1983

Tetralogy of Fallot with absent pulmonary valve: definitive diagnosis by two-dimensional echocardiography.

A case is presented of a 27-day-old boy with Tetralogy of Fallot and absent pulmonary valve. The two-dimensional echocardiographic examination demonstrated: subaortic ventricular septal defect, overriding aorta, absence of the pulmonary valve with restrictive anulus, and massive dilatation of the pulmonary artery. These anatomic details were then confirmed by angiography and surgery. The echocardiographic features of Tetralogy of Fallot with absent pulmonary valve are typical, and allow the correct diagnosis to be made noninvasively. The haemodynamic investigation becomes obviously easier, and angiocardiography may be devoted to the study of possible additional defects, that are difficult to detect by echocardiography, like multiple ventricular septal defects, or peripheral pulmonary branch anomalies.

Echocardiography↗
PubMed1983

Surgical treatment of non-critical right ventricular outflow tract obstruction with intact ventricular septum in patients over one year of age.

Between March 1966 and May 1982, 257 patients with clinically noncritical right ventricular outflow tract obstruction with intact ventricular septum (RVOTO/IVS) underwent elective open-heart correction, with an overall early mortality of 3.9%. Age ranged from 13 months to 48 years (mean 6.6 +/- 6.5 - median 5). We describe three different anatomical types, for which a standardized surgical approach is suggested. When one or more of the following features--small right ventricular cavity; rigid infundibular stenosis; hypoplastic pulmonary annulus and trunk; dysplastic cusps--were present, the mortality (10.6%) was significantly higher than in isolated valvular stenosis (2.2%) or in valvular plus dynamic infundibular stenosis or isolated low-lying stenosis (2.5%). In a series of 159 consecutive patients operated upon since September 1974 the cumulative early mortality was 1.3%. Late results on 202 patients (mean follow-up period of 25.6 +/- 22.6 months) are good in 90.6% and fair in 8.9% of the cases. One patient died of encephalitis 2 years after the operation. We believe that a more precise characterization of the underlying lesions and a more liberal use of different surgical techniques to minimize residual gradients, iatrogenic pulmonary incompetence and impairment of right ventricular contractility, may decrease early mortality and improve overall results.

Heart Defects, Congenital↗
PubMed1982

Surgical treatment of critical right ventricular outflow tract obstruction with intact ventricular septum in infancy.

Between December 1965 and August 1981, a total of 104 operations were performed on 101 infants for treatment of critical right ventricular outflow tract obstruction with intact ventricular septum (RVOTO/IVS). Twenty-three patients had pulmonary atresia (PA/IVS) and 78 critical pulmonary stenosis (CPS/IVS). One of those had an emergency reoperation within the first year of life because of acquired atresia after valvotomy. The cumulative early 30 days mortality was 35.6% (58.3% for PA/IVS and 28.7% for CPS/IVS). Analysis of this series indicates that the surgical risk does not significantly differ in PA/IVS and CPS/IVS within the same age group. Patients with small right ventricular cavity, dictating surgery within the first 30 days of life, present significantly higher surgical risk in both groups. The higher overall mortality in PA/IVS may be influenced by the lower median age at operation. Our present surgical indications are the following. Patients with normal sized right ventricle are treated with pulmonary valvotomy at any age. Patients with small sized right ventricle undergo early total correction over 6 months of age. Under 30 days of life balloon atrial septostomy, valvotomy and systemic-to-pulmonary artery shunt are performed. Between one and 6 months the surgical technique depends upon the clinical presentation and the right ventricular anatomy in the single patient. Since this policy of treatment has been pursued, in the last 12 consecutive cases the 30 days mortality was 8.3%.

Follow-Up Studies↗
PubMed1981

Systemic-pulmonary artery shunt using PTFE prosthesis (Gore-Tex). Early results and long-term follow-up on 105 consecutive cases.

One hundred-five patients (median age 14 months) in whom a PTFE prosthesis was used to create a systemic-pulmonary artery shunt were studied between 1978 and 1980. The prosthesis was mainly used to create a modified Blalock-Taussig anastomosis. Nine patients died in hospital (8.5%, 70% confidence limits: 5.7% to 12.3%). The clinical condition did not appear to be an incremental risk factor, while the young age and the underlying type of disease accounted for the hospital mortality to a large extent. All 96 survivals were available for follow-up information. One graft, which was damaged intraoperatively, occluded 3 months later. The remaining patients have a patent graft 3 months to 36 months (mean 24 months) postoperatively. In 6 patients the patency was assessed angiographically and no damages of the pulmonary artery were observed, therefore this type of palliation is advisable for any patient, particularly in the first year of life.

Adolescent↗
PubMed1981

Mitral insufficiency and aortic valve stenosis in infancy associated with endocardial fibroelastosis, surgical treatment and long-term results.

Surgical treatment of mitral insufficiency and aortic valve stenosis associated with endocardial fibroelastosis has not been well documented. We treated 2 infants with this complex lesion: in one case it was possible to repair the mitral valve. The long-term results are quite encouraging and emphasize that this lesion should be treated early to avoid the risk of compromising the left ventricular function, although the chance of a valve replacement in an infant can be high.

Aortic Valve Stenosis↗
PubMed1981

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↗
PubMed1981

[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↗
PubMed1980

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↗
PubMed1980

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↗
PubMed1980

Cervical aortic arch with aortic obstruction: report of two cases.

The occurrence of aortic obstruction in patients with cervical arch is very rare. The clinical and angiocardiographic findings of two patients with this combination of defects are presented. One of these patients, in whom tricuspid atresia with reduced pulmonary blood flow was also present, had a successful resection of the obstruction together with construction of a Waterston shunt.

Aorta, Thoracic↗
PubMed1980

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↗
PubMed1980

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↗
PubMed1980

[Situs inversus and dextrocardia. Analysis of the associated congenital heart diseases (author's transl)].

13 cases of congenital heart diseases in patients having atrial situs inversus and dextrocardia, all studied by means of angiography, are described following an up to date segmental approach. The type of atrioventricular connection was concordant in the 11 biventricular hearts; double inlet in the 2 univentricular hearts. The mode was always via two atrioventricular valves. In two cases the atrioventricular flows realized a criss-cross condition, being horizontal the interventricular septum, superior the morphologically right ventricle and inferior the morphologically left ventricle. In none of the univentricular hearts was an accessory chamber identified; they were both considered to be of indeterminate type. Ventriculo-arterial connections were: concordant in 3 cases (23%), including one case of Tetralogy with pulmonary atresia; discordant in 3 cases (23%); double outlet in 7 cases *54%). The aortic valve was in the assumed position (left and posterior to the pulmonary valve) only in the 3 cases with concordant as a rule. A ventricular septal defect was actually found in all cases; in one it was associated with pulmonary vascular disease. Obstruction to the pulmonary flow (subvalvular, valvular or vascular) was ascertained in 85% of cases, whereas aortic obstruction was never found. Overall, important congenital heart malformations appear to be frequently associated with situs inversus and dextrocardia. The ventriculo-arterial junction is identified as the most affected point.

Adolescent↗
PubMed1979

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↗
PubMed1979

[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↗
PubMed1979

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↗