Results of the use of a graft with a monocusp in radical correction of Fallot's tetrad

The article deals with the results of complex study of the effect of pulmonary insufficiency on intracardiac hemodynamics and function of the heart in the immediate and late-term periods after radical correction of Fallot's tetralogy. Answers are also given to questions concerning the expediency of the use and functional adequacy of a xeno-pericardial monocusp in the closure mechanism of the pulmonary artery valve. The study showed that massive pulmonary regurgitation has a negative effect on the functional condition of the right ventricle in late-term postoperative periods. Convincing data were obtained which allow a graft with a monocusp of a bull's pericardium to be recommended for further clinical use to prevent insufficiency of the pulmonary artery valve after radical correction of Fallot's tetralogy.     

Surgical aspects of radical correction of tetralogy of Fallot]

The article deals with the analysis of variant anatomy of Fallot's tetralogy due to hypoplasia of the pulmonary trunk on basis of morphometric and intraoperative examination of 141 patients 4 to 43 years of age (15.3 +/- 3.26) who underwent radical correction of the anomaly; 48 (34%) of these patients were operated on after various palliative operations. On grounds of detailed study of the variants of the relations of anomalies of the cardiac anatomical structures in Fallot's tetralogy the authors elaborated operative proceedings by means of which mortality after radical correction fell to 6.5%.     

Complications of the Waterston-Cooley-Edwards anastomosis and their correction in radical surgery of the tetralogy of Fallot

The work is based on the analysis of complications of Waterston-Cooley-Edwards anastomosis which create tactical and technical difficulties in correction of Fallot's tetralogy. Rational methods for their correction are suggested. From January, 1982 to April, 1989 a total of 133 radical operations were carried out for Fallot's tetralogy in patients with Waterston-Cooley-Edwards anastomosis formed earlier; mortality was 6%. Negative sequelae of the anastomosis were found in 62 (46.6%) patients. These were: pathology of the right pulmonary artery in 33 (27%) patients, functional atresia of the outflow paths from the right ventricle in 11 (8.3%), acquired atresia of the valve of the pulmonary artery in 2 (1.5%), pulmonary hypertension in 12 (9%), and acquired cavopulmonary fistula in 1 (0.8%) patient. Despite the rather large number of complications, most of them were successfully removed in radical correction of the anomaly. The postoperative mortality in the group of patients without complications (5.6%) hardly differed from that among patients with unfavourable sequelae of the anastomosis (6.5%).     

Initial experience in the correction of the tetralogy of Fallot in infants using an approach through the right atrium and the pulmonary artery

Radical correction of Fallot's tetralogy by means of an approach through the right atrium and pulmonary artery without ventriculotomy (10 patients) or with minimal ventriculotomy (23 patients) was carried out in children whose ages ranged from 13 to 50 months and body weight from 7.9 to 15 kg. Lethality was 12%. The death of 3 patients was not connected with the peculiarities of the surgical tactics, the death of the fourth patient was due to insufficient experience in development of the surgical techniques of the first operations. The operative techniques applied are described in detail and the possibility of adequate correction of Fallot's tetralogy by it is proved principally. The late-term operative results are good in 10 patients. The first results of the suggested method of surgical correction of the anomaly are claimed by the authors to be promising, which allows them to continue its further elaboration.     

Ascending aorta-right pulmonary artery anastomosis in children with complex cardiac malformations. Long-term results in 30 cases.

In this report, we shall analyze the results obtained with palliative treatment in 30 patients with congenital heart disease who were operated upon at the University Hospital of Caracas during the period 1968 to 1972. In all cases, an ascending aorta-right pulmonary artery anastomosis was performed. Although we believe that the Blalock-Taussig and Potts shunt are satisfactory operations in some cases, at the present time we prefer the aorta-right pulmonary branch anastomosis in Fallots tetralogy and other congenital heart diseases such as single ventricle. In Fallot's tetralogy, particularly, this procedure provides better results, because the anastomosis can be closed through the aorta when these patients are later subjected to total correction with extracorporeal circulation.     

The diameter of the descending thoracic aorta in patients with Fallot's tetralogy (angiographic study)

The diameter of the descending thoracic aorta was measured at the level of the diaphragm on angiograms in 91 patients with Fallot's tetralogy. The existence of an exponential dependence of the diameter on the body surface area of patients as well as of healthy individuals was shown. The course of the regression curve in patients with Fallot's tetralogy repeated exactly the course of that in healthy individuals, but the former ran 0.5 mm higher. Thus, to determine the diameter of the descending thoracic aorta in patients with Fallot's tetralogy one may use the nomogram of the dependence of this diameter on the body surface area of healthy individuals or an equation suggested by the authors. It is practically impossible to locate the descending thoracic aorta by means of echography, whereas the size of its diameter is included in equations for calculating the important hemodynamic parameters. The data obtained by the authors allow wider use of noninvasive methods for the diagnosis (echo-morphometry) and prognostication of the results of radical correction of Fallot's tetralogy in a concrete patient.     

Pulmonary valve insertion late after repair of Fallot's tetralogy.

OBJECTIVES: To analyze the results of pulmonary valve insertion late after initial repair of Fallot's tetralogy. Pulmonary insufficiency (PI) after correction of Fallot's tetralogy is usually well tolerated in the short term, but is associated with symptomatic right ventricular dilatation and an increased risk of ventricular arrhythmias over longer periods of time. METHODS: From 1993 to July 2000, 51 patients were reoperated for PI at a mean age of 25.7+/-11.9 years. The mean age at initial repair was 6.4+/-7.2 years. Patients with a conduit inserted at initial operation, with absent pulmonary valve syndrome or with a more than moderate ventricular septal defect at reoperation were excluded from the study. A cryopreserved pulmonary (96%) or aortic (4%) homograft was implanted in the orthotopic position with the use of cardiopulmonary bypass 19.3+/-9.1 years (2.7-40.3 years) after initial correction. Preoperative symptoms (New York Heart Association, NYHA class), degree of PI (echo-Doppler, MRI), right ventricular dimensions (MRI) and QRS duration were compared to findings at last follow-up. RESULTS: Follow-up is complete and had a mean duration of 1.7+/-1.4 years. Hospital mortality was 2%. No serious morbidity occurred. Severe PI was present preoperatively in all patients. At last follow-up echo-Doppler studies showed PI to be absent or trivial in 96% and mild in 4% of patients. In 13 patients MRI studies were performed both pre- and postoperatively: in this group PI was reduced from a mean of 48 to 4%. After 6 months NYHA capacity class had improved significantly from 2.3+/-0.6 to 1.4+/-0.5. After 1 year end-diastolic and end-systolic right ventricular volumes were reduced significantly. Right ventricular ejection fraction and QRS duration remained unchanged. CONCLUSIONS: PI late after correction of Fallot's tetralogy may lead to serious symptomatic right ventricle dilatation. After pulmonary homograft insertion right ventricular dimensions decrease rapidly and functional improvement is observed in almost all patients.     

Correction of Tetralogy of Fallot with Combined Transatrial and Pulmonary Approach: Experience with 184 Consecutive Cases

The authors present their technique and results for complete correction of Fallot's tetralogy by closure of the ventricular septal defect by the transatrial approach, and resection of the infundibular hypertrophy by a combined approach through the tricuspid and pulmonary valve orifices. Right ventricular pressures were often high at the end of operation due to a residual muscular gradient but the outcome of these postoperative gradients was similar to that of valvular pulmonary stenosis with an intact ventricular septum.     

An unusual post-operative complication following modified left Blalock-Taussig shunt

A left modified Blalock-Taussig shunt was performed in a 3-year old child with Fallot's tetralogy. Intraoperative course was uneventful. Persistent hypoxia was noticed in the post-operative period in spite of a clinically functioning shunt. Angiography revealed a functioning shunt with totally blocked left pulmonary artery. A thrombus was removed from the left pulmonary artery which dramatically improved the clinical course of the patient.     

Correction of tetralogy of Fallot in an adult using a stented bioprosthetic valved conduit

A 55-year-old man with tetralogy of Fallot successfully underwent correction using a valved conduit. He was diagnosed as having congenital heart disease during childhood, but no surgical intervention was performed. Cyanosis and dyspnea on effort had progressed gradually. Catheterization showed a left ventricular end diastolic volume of 126 ml, and the pulmonary arteries had sufficient diameters. To prevent postoperative pulmonary regurgitation, we planned to use a bioprosthetic valved conduit for right ventricular outflow tract reconstruction. At 4.5 years after the operation he is in New York Heart Association functional class I. The catheterization performed 1.5 years after the surgery showed no pressure gradient between the right ventricle and the pulmonary artery. Thus, total correction of tetralogy of Fallot in an adult can be achieved safely, and the use of a bioprosthetic stented valved conduit can be beneficial.     

Generalized pulmonary hyperinflation and Fallot''s tetralogy in a neonate investigated by pulmonary physiological and radioisotopic methods.

An infant is described who presented a complex cardiopulmonary problem which was evaluated with the help of new physiological techniques. the infant was born at term after an emergency Caesarian section for fetal distress and was found to have meconium aspiration. He remained persistently tachypnoeic and hypoxic despite high ambient oxygen. Chest radiography suggested cystic lesions at the lung bases, and lung function tests confirmed hyperinflation with delayed nitrogen washout. In addition the child had signs of Fallot's tetralogy, and this diagnosis was confirmed by cardiac catheterization. Because of persistent hypoxia and tachypnoea disproportionate to the cardiac condition, the possibility of localized lung disease was considered. Regional lung function tests were carried out in the neonatal period and again at six months of age useing radioisotopic 13N given by both inhalation and injection. These studies showed gross ventilation/perfusion imbalance in the lungs, particularly marked at the bases, but with enough generalized abnormality to preclude the possibility of surgical intervention. The principles of the measurement of lung mechanics in the newborn by whole-body plethysmography, nitrogen washout, and regional radioisotopic spirometry are outlined. The particular value of these techniques in the evaluation of complex disorders is discussed, especially where both cardiac and pulmonary abnormalities are present.     

Tetralogy of Fallot: the centenary of the name A new translation of the first of Fallot's papers

1988 marks the centenary of the cognomen “tetralogy of Fallot”. Fallot's works are often quoted and translated without mentioning that it was he who applied the noun “tetralogy” to the malformation. This new translation shows that his contribution to the morbid anatomy was not great, but he was unknowingly responsible for providing a unifying concept and a name for the commonest of all cyanotic congenital heart defects.     

Reconstruction of right ventricular outflow with a valved homograft conduit

Wheatley, D. J., Prusty, S., and Ross, D. N. (1974).Thorax, 29, 617-623. Reconstruction of right ventricular outflow with a valved homograft conduit. Since 1966 a conduit of homograft ascending aorta with its valve has been used for reconstruction of the right ventricular outflow. This technique has been applied to 123 operations for pulmonary autograft replacement of the aortic valve, 48 operations for correction of severe Fallot's tetralogy or pulmonary atresia, five operations for truncus arteriosus, two operations for transposition of the great arteries with ventricular septal defect, and six operations for tricuspid atresia or common ventricle. Ease of insertion and satisfactory function have encouraged us in the use of this form of conduit.     

Characteristics of the radical correction of Fallot's tetralogy in anomalous distribution of the coronary arteries

The variants of distribution of the coronary arteries were studied on 41 specimens of the heart with Fallot's tetralogy. The typical topography of the coronary arteries in this cardiac defect is characterized by counterclockwise rotation of the coronary sinuses, dominant system of the right coronary artery with a large infundibular branch, and right type of heart blood supply. There were 6 variants of anomalies of the coronary arteries. The most characteristic of Fallot's tetralogy were: a single coronary artery, origin of the large infundibular artery by an independent orifice, and anomalous origin of the anterior interventricular branch from the right coronary artery. The peculiarities of radical correction of the tetralogy in atypical distribution of the coronary arteries were discussed on the basis of analysis of 27 operations conducted with the use of non-traditional methods of correction.     

The use of a cusp-bearing homograft patch to the outflow tract and pulmonary artery in Fallot's tetralogy and pulmonary valvular stenosis

A technique is described for maintaining pulmonary valve competence whilst widening the hypoplastic pulmonary artery ring found invariably in Fallot's tetralogy and pulmonary valve stenosis. A homograft composed of the aortic cusps, a portion of ascending aorta, and the anterior mitral leaflet is used. One, two, or three cusps may be replaced or added by variations of technique. The right ventricular outflow tract and the pulmonary trunk are also widened. The operation is applicable to children, and adult homografts can be used. The ring should be widened to a circumference of from 6 to 8 cm. so as to prevent residual pressure gradients. One or two cusp replacements are favoured in the hope that growth will proceed because a continuous portion of natural pulmonary artery and ring remains untouched. An advantage of the technique is the small ventriculotomy required to excise the infundibular obstruction and close the ventricular septal defect. Four case histories are presented. Three patients have survived operation and are doing well. Up to six months post-operatively no serious pulmonary incompetence has developed.     

Infundibular spasm in Fallot's tetralogy - an account and its management in anaesthesia

The value of propranolol in relieving infundibular spasm is illustrated. The pathophysiology of infundibular spasm in Fallot's tetralogy and its management in anaesthesia is reviewed.     

Hypothermic cardiopulmonary bypass in a patient with sickle-cell trait

A male patient, aged 7.5 years, with known sickle cell trait, presented for correction of Fallot's tetralogy. The successful management under hypothermic cardiopulmonary bypass is described and the behaviour of sickle haemoglobin under hypothermic conditions is discussed.     

Indications for the Brock operation in current treatment of tetralogy of Fallot

It is now generally accepted that a certain proportion of children with severe tetralogy of Fallot are anatomically unsuitable for one-stage total correction of the anomaly. The choice of the best preliminary operation for these is still in some doubt, however. Following Brock's original hypothesis that relief of the outflow obstruction will encourage enlargement of the main pulmonary artery and annulus (and therefore favour subsequent successful total correction) we have preferred this procedure in all cases not suitable for immediate total correction. In a consecutive series of 36 cases the results have been found to be very acceptable and to compare favourably with those obtained with anastomotic procedures such as the Blalock or Waterston shunt. The operation has been accomplished with an 11% mortality, and in 72% of cases cyanosis has been abolished under conditions of normal exercise. Sixteen cases have subsequently come to total correction with a 25% mortality and a 75% `cure' rate. In the light of this experience we find that closed pulmonary valvotomy with or without infundibular resection has a definite and valuable place in the current treatment of Fallot's tetralogy. General and specific indications for its use are presented.     

Surgical treatment of double-outlet left ventricle in 2 patients with D-position and L-position of the aorta.

Two cases of double-outlet left ventricle (DOLV) are described, 1 with D-position and 1 with L-position of the aorta. Both patients had situs solitus, atrioventricular concordance with D-relationship of the two ventricles, a ventricular septal defect, and subpulmonary stenosis. The patient with D-position of the aorta was operated on using a technique similar to that for repair of Fallot's tetralogy but including emergency implantation of a bioprosthesis into the pulmonary artery position on the eighth postoperative day. Surgical correction in the patient with L-position of the aorta required the implantation of a composite valved conduit between the right ventricle and the pulmonary artery. The various surgical techniques for correction of DOLV are described, and the literature is reviewed. Postoperative right heart failure in both patients is explained by the impaired function of the right ventricle being exposed to gross pulmonary incompetence or by the persistence of elevated right ventricular pressure.     

Left ventricular diverticulum associated with Cantrell's syndrome and tetralogy of Fallot in an adult

In this report we describe the surgical treatment of a 27-year-old patient with complete Cantrell's syndrome, i.e. multiple ventricular septal defect, left ventricular diverticulum, dextrorotation of the heart, an anterior diaphragmatic defect, and a midline supraumbilical abdominal wall defect with tetralogy of Fallot. Resection of the diverticulum was combined with correction of the tetralogy of Fallot and thoracoabdominal defects. The postoperative period was uncomplicated. We have have found only one previous report describing resection of the diverticulum combined with correction of Fallot's tetralogy and thoracoabdominal defects in an adult. One-stage repair of these complex anomalies is technically feasible and should be the treatment of choice.