[Long-term results of a deliberate conservative approach in the radical correction of tetralogy of Fallot].
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Biomedical subjects
Publications and source records attributed to G P Piccoli.
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Between January 1965 and December 1979, in our Unit 53 patients underwent Mustard's operation for TGA, intact ventricular septum and LVOTO. There were 35 males and 18 females, ranging in age from 27 days to 12 years (mean = 26 months) and in weight from 3.7 to 26 kg (mean = 9.6 Kg). Twenty-one infants had undergone previous palliative procedures. The degree of LVOTO was mild in 26 cases, moderate in 10 and severe in 17 patients. In 28 cases no distinct anatomical obstructions were identified. In the other 25 patients the obstruction occurred at various level and it was determined by several anatomical structures. Subvalvular fibromuscular narrowing, redundant mitral valve, valvular stenosis and subvalvular fibrous shelf were the commonest forms encountered. The LVOTO was managed in several different ways. In 28 patients in whom the gradient was judged to be functional, no surgical intervention at the level of the LVOT was attempted. In 8 cases, all operated on at the beginning of this experience, an anatomic obstruction was present but was considered too difficult to be resected and, therefore, was left untreated. In 9 patients the LVOT was inspected either through the pulmonary valve or from below, through a left ventriculotomy. Pulmonary valvotomy was carried out in 5 of these cases, resection of the subvalvular fibrous shelf in 3 and subvalvular fibromuscular tunnel resection in 1. In 6 patients a left ventricle to pulmonary artery conduit was used to bypass the obstruction. A fibromuscular tunnel type of obstruction was present in 5 of these cases, while in one the obstruction was mainly due to a redundant mitral valve.(ABSTRACT TRUNCATED AT 250 WORDS)
At the Hospital for Sick Children, London, between Jan. 1965 and Jan. 1980, 98 patients, ranging in age from 22 days to 20.8 years (mean = 29.6 months), with a clinical diagnosis of transposition of the great arteries (TGA) associated with a ventricular septal defect (VSD) found at cardiac catheterization, underwent Mustard procedure at the atrial level, whether or not associated with the VSD closure. Twenty-nine other patients, with irreversible obstructive pulmonary vascular disease in whom a "palliative" Mustard was performed, have been excluded from this report. At operation, in 8 cases, the VSD was found to have closed spontaneously during the interval between the cardiac catheterization and the surgical repair, while, in 13 patients, the VSD was judged to be too small to influence the hemodynamic results and, accordingly, was left open. In 35 cases the VSD was closed with mattress stitches buttressed with dacron pledgets and in 42 patients a dacron patch was used. The defects were accessible through the tricuspid valve in all but 10 cases. There were 24 early deaths (24%) and these did not correlate either with the year of operation or with the age at the time of operation. The mortality rate was higher in the cases who had required previous pulmonary artery banding (37%), than in those who had not (21.5%), but this difference was not statistically significant. The early results were related to the size of the VSD. In the 21 patients in whom the VSD was partially or completely closed, the early mortality was 9.5%.(ABSTRACT TRUNCATED AT 250 WORDS)
Between February, 1969, and March, 1980, 235 consecutive patients underwent repair of tetralogy of Fallot. Of these, 94 patients were less that 4 years of age, including 40 less than 2 years of age. Previous palliative procedures had been performed in 46 patients. Conservative technique to relieve the right ventricular outflow tract (RVOT) obstruction was employed in 194 patients; transannular approach was performed in the remaining 41. There were 18 hospital deaths (7.6%) and 3 late deaths. The early and late results were significantly related to the ratio of the right ventricular to left ventricular systolic peak pressure after repair. Until 1976, the age of patients of operation constituted an operative risk factor. Since 1977, none of the 17 infants who underwent operation died after the repair. In the overall series, the transannular approach to relieve the RVOT obstruction affected the early mortality and the late results. During the last 3 years, only 1 of the 12 patients who received an aortic homograft monocusp transannular gusset died in the hospital, and the 11 survivors were completely symptom free at follow-up.
Forty-four consecutive infants aged from 3 days to 10 months underwent repair of total anomalous pulmonary venous connection using deep hypothermia with circulatory arrest. There were eight (18%) early hospital deaths. Using multivariate analysis no significant association could be shown between early mortality and age or weight at operation, preoperative pulmonary or systemic pressure, and preoperative condition for patients undergoing operation during the most recent five year period. Late pulmonary venous obstruction developed in four (11%) of the survivors and all of these patients died.
Actual and potential factors that create an obstruction to the flow within the systemic side of the heart have been analyzed in 114 specimens with atrioventricular septal defects. Hearts with separate right and left atrioventricular orifices and with common atrioventricular orifice were included. Potential inlet obstruction was due to tethering of the "cleft" of the left atrioventricular valve to the septum in 17.5%, incomplete "cleft" in 8%, and deformed valve leaflets in 70% of cases. Potential factors predisposing to left ventricular outflow tract obstruction were seen in 70% of cases, 21% being due to abnormal position of the papillary muscle and the remainder due to intrinsic hypoplasia of the outflow tract. Anatomic factors producing unequivocal obstruction were seen in 29% of cases. Twenty-two percent had obstructions at the inlet level, of which 13% were due to malformations of the left atrioventricular valve or the left component of a common valve. The other 9% had overall inlet obstruction due to severe hypoplasia of the left ventricle itself. Unequivocal outlet obstruction was observed in 7% of cases, 5% due to abnormal disposition of papillary muscles in the outflow tract and 2% to aneurysm of the interventricular septal remnant. Although anatomic obstruction occurs in the minority of cases (29%), this report shows that potential obstruction must be taken into account when considering surgical repairs.
The early and late results of intracardiac repair of 175 cases of tetralogy of Fallot during the period 1969 to 76 are reviewed. Overall hospital mortality was 8%, with a 5% mortality in patients over 2 years of age. Late mortality was 1%. Both early and late mortality and morbidity were related to the right ventricular to left ventricular peak systolic pressure ratio measured at the end of the operation. During the eight year period, a fall in mortality from 37 to 15% was recorded in patients less than 2 years of age. As a result of the experience gained during this period, we have now adopted a more active policy towards reconstruction of the right ventricular outflow tract while remaining aware of the potential problems of pulmonary regurgitation.
Between January 1974 and November 1980, 13 symptomatic infants under 1 year of age with persistent truncus arteriosus type I or II underwent surgery. Pulmonary artery banding was performed in 10 cases, with five deaths. Among the survivors, one developed severe pulmonary vascular disease and only two underwent late intracardiac repair. Primary total correction was performed in three infants and all are well, though one required conduit replacement five and a half years after the initial procedure. Recently, antibiotic sterilised homograft conduits, rather than heterografts, have been preferred as extracardiac conduits in infancy. Early intracardiac repair followed, if necessary, by later conduit replacement appears to have significant advantages over "conventional" pulmonary artery banding and late total correction. Concern remains on the treatment of choice in infants under 3 months of age.
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Anatomic correction of transposition of the great arteries and ventricular septal defect has been performed successfully in two patients. The technique employed differed in several aspects from the so-called Jatene procedure: (1) Both great arteries were transected at low level; (2) the switch of the coronary arteries was carried out after transection of both great arteries; (3) a modified aortic homograft conduit was used to bridge the gap between the proximal aortic root and the distal main pulmonary artery; (4) the "new" main pulmonary arterial trunk was positioned on the right side of the ascending aorta; and (5) the ascending aorta was enlarged in order to match the dilated proximal pulmonary stump. The advantages of these modifications over other techniques reported in the literature are discussed.
Eighty successive patients underwent the Mustard operation for "simple" transposition of the great arteries (TGA) between January, 1970, and January, 1980. Ages ranged from 42 days to 12.4 years (mean 14.7 months). Balloon atrial septostomy (BAS) with balloon volumes of more than 2.5 ml produced significant increases of arterial oxygen saturation, although the initial benefit of BAS was greater than that found at follow-up catheterization. In these patients the left ventricular (LV) pressure tended to decrease during the first months of life, but this drop was less marked at the end of the first year. Deep hypothermia and cardiocirculatory arrest were employed in all cases. Several modifications were adapted to the original surgical technique in order to reduce last sequelae. There were two early and three late deaths. At follow-up (mean 4.5 years) 74% of patients were symptom free. Sinoatrial and atrioventricular (AV) conduction disturbances were present in five and six patients, respectively. Caval or pulmonary venous obstruction occurred in 13 patients, seven of whom required reoperation. Isolated tricuspid valve incompetence was present in one patient only, but in four it was associated with other defects. Primary right ventricular (RV) failure occurred in one patient. These findings, in relation to mortality, complications, and the excellent clinical results in the majority of survivors, compare favorably with results published for patients with "simple" TGA who have been subjected to other types of physiological or anatomic correction. At the present time, there seems to be no good reason to alter our surgical policy in this group of patients.
Between January 1965 and December 1979, 302 patients underwent Mustard's operation for "simple" transposition of the great arteries (TGA). 31 cases who underwent Senning operation for "simple" TGA and 7 patients with "simple" TGA and severe pulmonary vascular disease, in whom Mustard's operation was performed and a ventricular septal defect created, have not been included in the present series. The patients analysed ranged in age from 3 days to 17 years (mean = 27,6 months) and in weight from 2.7 to 40 Kg (mean = 9.9 Kg). The technique originally described by Mustard was used, with some technical modifications. There were 26 early deaths (early mortality rate = 9%). The hospital mortality was 16% during the period 1965-1969, 75% between 1970 and 1974, while during the last 5 years it was 6%. The hospital mortality was lower among the infants between 6 and 12 months of age (6%), compared with an early mortality rate of 11% among those younger than 6 months and 9% in the age group over 1 year. There were 34 late deaths (12% of the survivors). Reoperation was performed in 42 cases. Forty patients required revision of the inter-atrial baffle for pulmonary and/or caval venous obstruction and 10 of them subsequently died. Post-operative venous obstructions were observed more frequently in patients with dumb-bell shaped dacron patch, which has been since 6 years abandoned. Since the introduction of Brom's trouser shape pericardial patch, venous obstructions are extremely rare. Both patients who underwent reoperation respectively for severe tricuspid valve incompetence and for occlusion of the left pulmonary artery, died early after reoperation. In our Unit, the actual management program for patients with "simple" TGA is the following. We keep to a minimum the degree of invasive investigations, 2 D ECHO diagnoses TGA with great accuracy. At the initial cardiac catheterization, a balloon atrial septostomy (BAS) is performed. A good inter-atrial mixing is usually obtained with balloons sized more than 2.5 ml. Angiocardiography is undertaken only when associated lesions, such as patent ductus arteriosus or aortic coarctation are suspected. If the child improves, complete investigation is performed at 3-4 months of age and the inter-atrial redirection of the venous inflow is scheduled for the age of 8-12 months. If the child fails to improve after BAS or deteriorates during the waiting period, restudy is performed immediately to ensure that additional lesions are not present and that the inter-atrial shunt is adequate. Rather than a surgical atrial septectomy, in this group of cases we prefer an early intracardial total repair, irrespective of age and weight.
Anatomical studies were made on 114 necropsy specimens of atrioventricular defects with atrioventricular concordance. The malformation is characterised by disproportion between the ventricular inlet and outlet dimensions and a malorientation of the aortic valve relative to the atrioventricular valve or valves. Associated with this there is a characteristic 'scopped-out' appearance of the muscular ventricular septum, gross abnormalities of the membranous components of the septum as compared with the normal heart, and narrowing of the aortic outflow tract. Hearts with these anatomical features can be divided into partial and complete forms depending on the morphology of the atrioventricular annuli. In the partial form the septal leaflets are conjoined to give separate mitral and tricuspid orifices, the conjoined leaflets being displaced into the ventricles and usually attached to the crest of the septum. In the complete form, anterior and posterior components of the 'septal' leaflets are separate, so that a single valve orifice connects the atrial to the ventricular chambers. Further subdivision of the complete form, apart from the morphology of the anterior leaflet, is dependent upon the presence or absence of an ostium primum atrial septal defect.
Anatomical studies were made on 70 necropsied hearts with atrioventricular defects from patients with situs solitus and atrioventricular concordance, all having a common atrioventricular orifice. The arterial connections were concordant in 68 and were double outlet right ventricle in two; cases with arterial discordance (transposition) or single outlet of the heart were excluded. It proved possible to subdivide the hearts, depending on the morphology of the valve leaflets. Five leaflets were distinguished by the commissural pattern and their insertion to major papillary muscles. They were a posterior bridging leaflet, right and left lateral leaflets, and right and left anterior leaflets. Subdivision was made on the basis of the disposition of the anterior leaflets. In six hearts the left anterior leaflet was committed to the left ventricle and the right anterior leaflet to the right ventricle, the commissure between them being on the crest of the ventricular septum. In 39 hearts there was minimal bridging of the left anterior leaflet so that it extended between the anterior papillary muscle of the left ventricle and the medial papillary complex of the right ventricle. In eight hearts the right margin of the left anterior leaflet was attached to an apical papillary muscle, while in 17 hearts it was attached to the anterolateral papillary muscle of the right ventricle. As the bridging of the left anterior leaflet increased, so the size of the right anterior leaflet decreased, but in all hearts both leaflets were identified. These findings were compared with previous classifications of complete atrioventricular defects.
Preoperative distinction between common atrioventricular orifice and ostium primum atrial septal defect may be difficult. To improve diagnostic accuracy, the right and left ventricle angiocardiograms were reviewed 'blind' in 92 patients with atrioventricular defects. The true diagnosis was known from necropsy or surgery in 60. Angiocardiograms had been obtained in various projections with or without craniocaudal tilt. Those features thought to distinguish between common orifice and ostium primum were coded, together with the ventricular systolic pressures. Computerised disciminant function analysis identified the following distinguishing features: (1) right ventricular systolic pressure; (2) immediate right ventricular outflow tract opacification from the left ventricle; (3) identification of the anterior attachment of the mitral component; (4) recognition of a single straddling atrioventricular orifice; (5) passage of contrast medium above or below the anterior or posterior bridging leaflets. Feature (3) indicates that in contrast to classic teaching the direct septal attachment of the mitral component does not contribute to the 'gooseneck' in complete atrioventricular defects. The significance of (4) and (5) is that they may be identified from right as well as left ventriculography, and are more likely to be identified in oblique than standard projections. Computerisation produced a correct diagnosis in 92 per cent of known cases, and determined precise probabilities of diagnosis in the remainder.
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A follow-up study of 31 patients, 1 1/2 years after they underwent a mitral valve replacement with a Beall prosthesis, is reported. Eighty-four per cent of the patients experienced marked improvement of symptoms and are fully active. Late cardiac recatheterization has showed a decrease of pulmonary wedge pressure, both at rest and under exercise. The prosthesis area has been found to be smaller than the original size. Hemolysis related to the prosthesis is of moderate degree and the incidence of systemic embolism was 3.1%.