[Preoperative care of children with Fallot tetralogy].
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[Surgical correction of tetralogy of Fallot with an iatrogenic obstruction on 1 branch of the pulmonary artery].
The au;hors report 12 cases of Fallot' tetralogy with stenosis or obstruction of one of the two branches of the pulmonary artery following palliative anterior anastomosis. The complications of correction by open heart surgery include the frequent incidence of pulmonary hypertension, which was responsible for 4 deaths. No satisfactory explanation could be found for these poor results. It is desirable to avoid the creation of asymetry in pulmonary blood supply in Fallot's tetralogy and, if such should be found, suggestions are made for its correction by open heart surgery in order to improve the prognosis.
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[Radical correction of the tetralogy of Fallot: 122 cases].
In 122 patients with Fallot tetralogy total correction was performed. 18 were previously operated with palliative surgery. The results were the following: hospital mortality: 8,5%; very good or good improvement: 78%; satisfactory in 13,5%.
Adolescent↗
Ectopic aortic origin of the right pulmonary artery in tetralogy of Fallot.
A case is described wherein Fallot's tetralogy was associated with aortic origin of the right pulmonary artery. The left pulmonary artery was the continuation of the pulmonary trunk. Although a number of examples of Fallot's tetralogy associated with aortic origin of the left pulmonary artery are known, combination of Fallot's tetralogy and aortic origin of the right pulmonary artery is rare and has been described in only two cases previously.
Angiography↗
'Bradycardia-tachycardia' syndrome 8 yr after correction of Fallot's tetralogy.
A 13-yr-old boy presented with atrial flutter 8 yr after surgical correction of tetralogy of Fallot; antiarrhythmic therapy caused depression of the sinoatrial node, with syncope. Disordered sinoatrial function and intraventricular conduction were demonstrated by intracardiac electrography, and appear to have resulted from the operation. Sinoatrial disease may be responsible for supraventricular arrhythmias or syncope long after operative correction of Fallot's tetralogy and may be one of the explanations for the tendency of such patients to die suddenly.
Adolescent↗
Recent results in the surgical treatment of Fallot's tetralogy.
Tetralogy of Fallot consists essentially of two abnormalities: a large, unrestrictive ventricular septal defect and severe obstruction to the outflow of the right ventricle. A clear understanding of the principles described in this article should result in a mortality of under 5% after complete correction of tetralogy of Fallot.
Aorta↗
The surgical correction of Fallot's tetralogy.
The success of total repair of tetralogy of Fallot depends on a clear understanding of the morbid anatomy and deranged hemodynamics of this anomaly, which consists essentially of two abnormalities, namely, a large unrestrictive ventricular septal defect and a severe obstruction to the outflow of the right ventricle. The ventricular septal defect acts as a safety valve and, therefore, cannot be closed unless the obstruction to the outflow of the right ventricle is adequately relieved. In severe cases, this often necessitates reconstruction of the outflow. With an adequate bypass technique and a clear understanding of the principles described in this article, complete correction of tetralogy of Fallot can be accomplished with a low mortality. In a series of 100 consecutive patients presented, the mortality was only 3%.
Adolescent↗
[The current role of palliative operations in the surgical treatment of tetralogy of Fallot. A report on 172 operated cases (author's transl)].
172 consecutive palliative operations in patients with Fallot's tetralogy are presented. 19% of the patients were aged less than 6 and 45% less than 12 months. The overall mortality was 5.8%, but has been recently reduced with the improvement of anaesthesiologic and technical experience. In fact, during the last two years, 69 Waterston shunts (33 under 12, and 17 under 6 months of life) were performed with no deaths. On the grounds of this experience, indications of the different types of systemic to-pulmonary artery shunts are discussed, as well as problems related to the choice between one-stage and two-stages surgical correction. The authors' opinion is that palliative procedures still play an important role in the treatment of Fallot's tetralogy, although the future approach may well be early total correction. In their experience, the last approach is restricted to children over 18-24 months of life with favorable anatomy, and to all patients over 4 years of age.
Heart Arrest↗
[Complete correction of Fallot's tetralogy in infants. Preliminary results].
Complete repair was undertaken in 28 infants with severe forms of tetralogy of Fallot because severe cyanosis or anoxic spells. Age at operation ranged from 8 days to 11 months (mean: 4,4 months). Among the 24 cases without any other intracardiac defect, there were 6 early deaths related in 4 cases to a wrong indication or a technical error. Re-investigation was performed in 14 out of the 18 survivors from 3 weeks to 6 months post-operatively: the result is good in 10 cases, fair in 1 case, and poor in 3 cases requiring twice a reoperation with 1 further death and 1 good final result. More accuracy in selecting the surgical patients and improvement in operative techniques should result in a further decrease of our early mortality rate. Primary complete repair should therefore become a substantial alternative to the palliative shunts which results seem to be hazardous in the more severe types of the defect.
Age Factors↗
[Acquired pulmonary atresia complicating tetralogy of Fallot].
Pf 51 cases of Fallot's tetralogy who underwent preoperative angiography before a palliative anastomosis and then a second angiography before complete correction, 10 had developed pulmonary atresia (infundibular in 8 cases, valvular in 2 cases). The type of anastomosis (Blalock-Taussig of Waterston), and the interval between the two operations do not seem to be determinant factors. The initial severity of the stenosis seems to favour the development of atresia but this is not invariable. The authors discuss the aggravating role of the anastomosis and the consequences of this type of complication.
Adolescent↗
Correction of tetralogy of Fallot after Waterston shunt.
The authors have reviewed their experience concerning twenty-seven patients who underwent intracardiac repair of Fallot's Tetralogy after a previous Waterston shunt. They are divided into 4 groups according to the classification of Shinebourne, Anderson and Bowyer. The risk factors are analyzed in group 1 patients in whom primary total correction is contra-indicated and in whom the mortality at repair is high in presence of kinking of right pulmonary artery. The type of surgery is still debatable. For the symptomatic patients of the other groups, primary total correction at any age seems, at this time, to be the treatment of choice. The contra-indications are an anterior descending coronary artery arising from the right coronary artery or associated complex anomalies which would make total correction difficult.
Aorta↗
[Long-term results of palliative operations and complete cure of tetralogy of Fallot].
714 cases of Fallot's tetralogy have been encountered over the last thirty years. A closed heart operation was undertaken in 472 patients. The mean age at operation was 5,1 years. In 87% of cases, a Blalock-Taussig anastomosis was carried out. The operative mortality was of the order of 3%. The operative risk was almost three times as large before the age of two years as after. The patients benefit by a demonstrable improvement of function after this operation, but it does not protect them from the complications of the condition (cervica abscess, Osler's disease, cerebro-vascular accidents) or from death. Open heart surgery was carried out in 403 patients. 161 as a primary procedure, and 242 after an initial palliative operation. The mean age at operation was 13.3 years. The operative mortality was 15%. The functional improvement was striking, and the patients appeared to be protected from the usual sequelae of the condition. On the other hand they cannot always be considered as cured: 16 died some time after the operation; 37% had pulmonary incompetence; 15% had a residual BSD; 8% had a block of both bundles, and 3% had a definitive atrio-ventricular block.
Age Factors↗
Echocardiographic diagnosis of tetralogy of Fallot.
The echocardiographic features of tetralogy of Fallot were defined in 25 patients with this malformation proved by cardiac catheterization. The echocardiographic characteristic that was present in all patients and that was most sensitive was the abrupt ending of the interventricular septal echoes with the aorta overriding the ventricular septal defect. The following additional echocardiographic features were frequently demonstrated in these 25 patients: right ventricular enlargement (20 patients), hypertrophy of the interventricular septum (20 patients), diminution of the right ventricular outflow tract (21 patients) and widening of the aorta (24 patients). The suggestion is made that the most specific echocardiographic pattern of tetralogy of Fallot is the finding of several echocardiographic abnormalities rather than the single feature of aortic overriding. Recognition of the altered anatomic relation coupled with a complete echocardiographic evaluation of all cardiac structures is a reliable means of diagnosing tetralogy of Fallot.
Adolescent↗
Repair of divided anomalous anterior descending coronary artery in tetralogy of Fallot.
Coronary artery anomalies in tetralogy of Fallot have often compromised surgical repair and imposed an increased mortality rate. Thus accidental division of the anterior descending coronary artery crossing the right outflow tract has previously usually resulted in death. The case is presented of a 13-year-old boy with tetralogy of Fallot in whom the anomalous left anterior descending coronary artery was divided. The blood supply to the left anterior descending artery was successfully restored using a reversed saphenous vein graft.
Adolescent↗
Transcatheter embolization of bronchial collateral arteries prior to intracardiac operation for tetralogy of Fallot.
In a case of tetralogy of Fallot with numerous bronchial collateral arteries (BCA), the collateral arteries were successfully occluded by transcatheter embolization of Gelfoam and use of the Gianturco spring as a preliminary to intracardiac repair. Pulmonary embolism would not result from this procedure in the absence of large direct communications between collateral and pulmonary arteries. The femoral artery and vein were prepared for cannulation before the procedure, so that it would be possible to start assisted perfusion immediately if arterial PO2 fell after transcatheter embolization. The procedure is performed under fluoroscopic control.
Adult↗
Preoperative prediction from cineangiograms of postrepair right ventricular pressure in tetralogy of Fallot.
To aid preoperative decision-making, we have related the ratio of postrepair peak pressure in the right and left ventricles (PRV/LV) to preoperative cineangiographic measurements in a retrospective study of 135 patients undergoing complete repair of tetralogy of Fallot or tetralogy of Fallot with pulmonary atresia. Postrepair PRV/LV was related to the preoperative diameter of right (DRPA) and left (DLPA) pulmonary arteries normalized to the descending thoracic aorta (DescThAo) in patients undergoing repair with transannular patching or a valved external conduit by the dquation: PRV/LV = 0.4840/(DRPA/DescThAO + DLPA/DesThAo) + 0.2007. Stenosis of the right pulmonary artery orifice and pulmonary artery arborization abnormalities incrementally increased postrepair PRV/LV. When a transannular patch was not used in classical tetralogy of Fallot, an increment of postrepair PRV/LV usually resulted, depending upon the size of the "anulus" measured intraoperatively: Incremental PRV/LV = 0.09437 . exp(-0.6344 . Z) where Z is a normalized expression in circumference terms of the diameter of the pulmonary arterial outflow tract (DPAOT) measured intraoperatively after infundibular dissection and valvotomy. DPAOT is itself related to the cineangiographically measured pulmonary valve anulus diameter (DPVA): DPAOT = 3.357 . DPVA0.5789 . BSA0.1551. In toto, these relations allow postrepair PRV/LV without transannular patching to be estimated from preoperative cineangiographic measurements. This allows preoperative predictiom in classical tetralogy of Fallot of the need for transannular patching, and in infants this can determine the choice between primary one-stage repair and two-stage repair. Prediction of postrepair PRV/LV when transannular patching or an external conduit is planned allows identification of patients in whom right and left pulmonary arteries are too small for safe complete repair, and in them an initial palliative operation should be done to enlarge the arteries.
Adolescent↗
Identification of mitochondrial energy metabolism-related candidate genes UQCR10 and NDUFA6 in pediatric tetralogy of fallot: an exploratory bioinformatics study.
BACKGROUND: Tetralogy of Fallot (TOF) is one of the most common cyanotic congenital heart diseases in infants and young children. Its molecular basis remains incompletely understood. This study aimed to identify mitochondrial energy metabolism-related candidate genes associated with pediatric TOF using public heart tissue transcriptomic datasets from the GEO database. METHODS: Datasets GSE146218 and GSE217772 were downloaded and merged, followed by batch-effect correction. Differential expression analysis was performed to identify differentially expressed genes (DEGs). Functional enrichment analysis, weighted gene co-expression network analysis (WGCNA), and protein-protein interaction (PPI) network analysis were used to prioritize candidate genes. The Comparative Toxicogenomics Database (CTD) was used as an exploratory literature-based tool to summarize gene-disease associations. RESULTS: A total of 960 DEGs were identified. Functional enrichment analyses showed that these genes were mainly enriched in mitochondrial energy metabolism-related pathways, including oxidative phosphorylation and the mitochondrial respiratory chain. WGCNA and PPI network analyses further prioritized UQCR10 and NDUFA6 as candidate genes, and both genes showed increased expression in TOF heart tissue samples. CTD analysis suggested literature-based associations between these genes and cardiovascular or developmental disease-related terms. CONCLUSION: This exploratory bioinformatics study identified UQCR10 and NDUFA6 as mitochondrial energy metabolism-related candidate genes upregulated in pediatric TOF heart tissue. These findings suggest that mitochondrial respiratory chain-related transcriptional alterations may be involved in TOF-associated myocardial remodeling or stress responses. Further experimental and clinical validation is required to confirm their biological relevance.
Humans↗
Routine primary repair vs two-stage repair of tetralogy of Fallot.
Fifteen of 194 patients (7.7%) with tetralogy of Fallot operated upon since January 1, 1972 under a protocol of routine primary repair despite young age died in-hospital. Most deaths were from low cardiac output. Young age and smallness of size increased the risk of operation. No deaths occurred among patients older than 4 years. High hematocrit was also a risk factor. Transannular patching has an independent effect in increasing risk. The post-repair ratio of peak pressure in the right ventricle to that in the left did not exert an independent effect. To project current risks of a two-stage approach, we determined that five of 158 patients (3.2%) died in-hospital after secondary intracardiac repair after a previous Blalock-Taussig or Waterston anastomosis between 1967--1978. Using these data and those we have published on the risk of shunting, we project that except in very small babies, the risks of hospital death of a two-stage approach are not less than those of primary repair done without a transannular patch, except when body surface area is less than about 0.35 m2. When a transannular patch is used in the primary repair, the two-stage approach is projected to be safer when the child has a body surface area of about 0.48 m2 or smaller.
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