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PubMed2001

[Tetralogy of Fallot complicated by severe hemoptysis in the adult].

Fallot tetralogy with pulmonary artery atresia is a special situation in which the patient can survive up to middle-age. In these cases some complications such as hemoptysis can appear with difficult treatment that endangers the life of the patient. When the cause of bleeding is the arteriovenous shunts, which are frequent in the multiple vascular malformations originated in this congenital cardiopathy, the selective embolization of these malformations can be an effective option of treatment.

Adult↗
PubMed2003

[Thoracic ectopia cordis with tetralogy of fallot].

Ectopia cordis; very rare congenital malformation, characterized by an evisceration of the heart through a parietal defect. The thoracic localization is most frequent. We report the case of a full term baby girl without follow-up of the pregnancy, presenting a beating mass in thoracic position, expansive to the effort, covered by a translucent membrane corresponding to an ectopique position of the heart. Transthoracic echocardiography shows cardiac malformation: Fallot tetralogy. The precocious diagnosis is possible by prenatal ultrasound examination after 12th week of pregnancy. The surgical treatment remain the only hope for these neonates. It's results depends on the associated malformations and the neonatal complications especially the infections. The prognosis remains poor in spite of the progress of surgical techniques.

Female↗
PubMed1992

[Tetralogy of Fallot. Physical work capacity after its total correction].

Results of exercise tests in 21 children after total correction of Fallot tetralogy (mean 5 years after surgery) are reviewed. Maximal exercise capacity recalculated for body mass and for total body area was approximately 60% of normal values. Maximal heart rate was lower than among healthy children. There was no statistical correlation between exercise capacity nad age while operated, nor time after surgery.

Adolescent↗
PubMed1979

[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↗
PubMed2005

Use of cutting balloon for palliative treatment in tetralogy of Fallot.

In tetralogy of Fallot, severe cyanosis due to insufficient pulmonary blood flow necessitates early intervention. The Cutting Balloon, developed for percutaneous coronary angioplasty, was used successfully for transcatheter incision and dilation of infundibular and valvar pulmonary stenosis in four patients with tetralogy of Fallot.

Angiography↗
PubMed1987

Transatrial-transpulmonary repair of tetralogy of Fallot.

Classical tetralogy of Fallot was repaired by a transatrial-transpulmonary approach in 61 of 70 patients treated surgically on one surgical service between July 1981 and December 1985. Their ages ranged from 2.5 months to 57 years (mean 52.9 +/- 104.2 months). The ventricular septal defect was closed through the right atrium and tricuspid valve in 60 patients and via the pulmonary artery in one patient with a subpulmonary defect. A pulmonary arteriotomy was made in 38 patients to expose the stenotic pulmonary valve for valvotomy and to begin the infundibular mobilization and resection. It was extended through a small pulmonary anulus primarily in 15 patients and secondarily in one, a minimal distance upon the right ventricular infundibulum to enlarge the outflow tract to 2 standard deviations larger than the mean normal pulmonary valve anulus diameter. There were no hospital or late deaths or reoperations among the entire group. The mean postrepair peak right ventricular/left ventricular pressure measured in the operating room was 0.52 +/- 0.165 for the entire group of 61 patients which indicates similar relief of pulmonary stenosis to that obtained by the classical transventricular repair. At follow-up between 2.3 and 53.8 months (mean 22.6) each patient was in New York Heart Association Class I and without cardiac medication. Postoperative cardiac catheterization studies performed in nine patients between 4 and 28 months after operation showed no residual ventricular septal defects and a mean peak right/left ventricular pressure of 0.42 +/- 0.13. This series indicates that successful repair of tetralogy of Fallot can be accomplished in most patients by a transatrial-transpulmonary approach, which may provide improved right ventricular function over the long term.

Child, Preschool↗
PubMed2004

[Long-term outcome of surgically treated teratology of Fallot].

Tetralogy of Fallot is the most common cyanotic congenital heart disease. The surgical treatment that is palliative or complete repair has allowed to transform the preview of this heart disorder. We suggests to study the long term outcome in patients undergoing surgical repair of tetralogy of Fallot, by emphasizing the quality of their lives, the complications, as well as the mortality. Ventricular arrhythmia and sudden cardiac death after repair of tetralogy of Fallot are devastating complications in adults survivors and their prediction remains difficult.

Adolescent↗
PubMed2004

Timing and type of surgery for severe pulmonary regurgitation after repair of tetralogy of Fallot.

Repaired tetralogy of Fallot (rTOF) has an excellent long-term prognosis; however, survival is somewhat less than normal. Of all the residual lesions and sequellae after rTOF, pulmonary regurgitation (PR) is the most important, correlating with right ventricular (RV) size, exercise intolerance and serious ventricular arrhythmias. Pulmonary valve replacement (PVR) has beneficial effects on RV size and function, provided it is performed early, before irreversible RV dysfunction ensues. Moreover, PVR is associated with an improvement in patients' symptoms and exercise tolerance and combined with arrhythmia surgery (cryoablation) it leads to a dramatic decrease in the incidence of fatal ventricular arrhythmias. Associated lesions, especially branch pulmonary artery stenosis, which aggravates PR, and tricuspid regurgitation, which further impacts on RV size and function, need addressing. Large right ventricular outflow (RVOT) akinetic and aneurysmal regions are frequent and further compromise RV function; therefore, resection during PVR should be attempted. Despite excellent mid-term results, homografts and xenografts, usually used for RVOT reconstruction, suffer late dysfunction and failure, committing patients and surgeons to further operations. Therefore, the decision to operate should be based on the balance between progressive RV dilatation, exercise intolerance, symptoms, arrhythmias and the fact that further reoperations will be needed. Research on the ideal valve for RVOT reconstruction is ongoing. Prospective follow-up of patients with rTOF with exercise testing and assessment of RV size and function, preferably with magnetic resonance, will define better the natural history of the disease and will probably provide firm guidelines for PVR timing especially in asymptomatic patients.

Arrhythmias, Cardiac↗
PubMed2000

Long-term survival after repair of tetralogy of Fallot.

BACKGROUND: Tetralogy of Fallot (TOF) is the most common cause of cyanotic congenital heart disease. Long-term survival has improved because of lower operative mortality and surgical repair in infancy or early childhood prior to the development of cardiac compromise from chronic hypoxia. Thus, more people with repaired TOF will survive to adulthood and will be interested in insurance coverage. RESULTS: This paper reported 36-year follow-up of 490 patients observed for a mean duration of 25 years after surgical repair of TOF; 42 deaths occurred, 62% of which were due to cardiac causes, principally sudden cardiac death and congestive cardiac failure. Mortality ratios were generally highest for the first 10 years of follow-up, ranging from 309% (ages < 10 years) to 714% (ages 20-39 years). By age, mortality ratios were stable and relatively similar for ages to 19 years and variable at older ages due to the small exposure. Most patients were actively at work (70.8%), homemakers (20.1%), or retired (6.1%); 3% were unable to work. CONCLUSION: For patients < 10 years of age and 10-19 years of age at the time of surgery who were alive after 1 year after the repair of the TOF, excess mortality persisted at least 30 years after surgery and did not vary a great deal with duration of the follow-up. Based on limited data, excess mortality for age group 20-39 years was higher compared with those in a younger age group during the first 10 years after surgery and decreased thereafter. Survival rates similar to those of the general population were attained in subjects who did not require treatment with a right ventricular outflow patch and who had normal hematocrit levels prior to surgery. Elevated morbidity risk was manifest by cardiac symptoms, arrhythmias, subsequent heart surgery, and disability in 3% of patients.

Adolescent↗
PubMed2000

Influence of ventricular septal defect type on surgical results in children with tetralogy of Fallot.

BACKGROUND: Tetralogy of Fallot (TOF) with subpulmonary ventricular septal defects (VSD) is relatively more common among Orientals than among Occidentals. This study assessed the influence of type of VSD on surgical results in children with TOF. METHODS: Fifty-one patients with TOF (age range, 14 months to 11 years, mean 40.7 months, median 30 months, 29 boys, 22 girls) who underwent total correction by a single surgeon between January 1992 and December 1995 were retrospectively studied. Of the 51 patients, 17 patients with subpulmonary VSD were classified as group I. The remaining 34 patients with perimembranous VSD were classified as group II. All patients underwent a combined transatrial and transpulmonary surgical approach. Early postoperative results were evaluated 7 to 10 days after surgery and late postoperative results were evaluated 1 year after surgery. RESULTS: There were no statistically significant differences between the two groups in age, sex, body weight, pulmonary to systemic flow ratio, pulmonary to aortic annulus diameter ratio or preceding palliative shunt or balloon pulmonary valvuloplasty. Nevertheless, group I patients had better oxygen saturation preoperatively (84.2 +/- 4.4% vs 80.3 +/- 7.6%, p = 0.037). During surgery, the transannular patch rate was higher in group I (100% vs 73.5%, p = 0.003). The cardiopulmonary bypass time, postoperative stay in the intensive care unit, duration of chest tube placement, early postoperative residual VSD rate, and early and late postoperative residual pulmonary stenosis rate were not significantly different between the groups. However, late postoperative residual VSD rate was 33.3% in group I and 6.45% in group II, which was statistically significant (p = 0.029). There were no significant differences in arrhythmia between the two groups. CONCLUSIONS: Patients with TOF with subpulmonary VSD had a better preoperative oxygenation, higher transannular patch rate during surgery and a higher incidence of late residual VSD than those with TOF with perimembranous VSD.

Cardiac Surgical Procedures↗
PubMed2003

Baroreflex resetting after successful surgical repair of tetralogy of Fallot.

BACKGROUND: Tetralogy of Fallot (TOF) and its surgical repair are associated with alterations in right ventricular systolic and diastolic functions. The cardiopulmonary baroreflex describes the peripheral vasoconstriction response to the volume-unloading deactivation of left and possibly right ventricular receptors. Alterations in cardiac geometry or distensibility and pulmonary vasculature of operated TOF may affect the mechanical stimulation of sensitive cardiopulmonary receptors leading to an impaired baroreflex function. There has to date been no report on the integrity of baroreflex function in postoperative TOF. OBJECTIVE: To characterize the combined cardiopulmonary and baroreflex response of patients successfully operated for TOF in early childhood to central volume unloading using graded lower body negative pressure (LBNP) application. METHODS: Fifteen patients operated on for TOF (mean+/-SEM) 15.7+/-1.4 years previously and 13 healthy age-matched control subjects were submitted to four consecutive 5 min LBNP applications at -10, -20, -30 and -40 mmHg. Forearm blood flow and vascular resistance, left ventricle end-diastolic diameter (LVEDD), arterial blood pressure and heart rate were obtained. RESULTS: TOF exhibited a lower LVEDD (42.7+/-1.5 mm) than control subjects (51.9+/-1.6). The forearm vascular resistance to LVEDD relationship was shifted left and upward in TOF compared with that of control subjects, but the slope of the relationship was not different between groups. LBNP -40 mmHg induced a lesser change in heart rate in TOF (+10.6+/-1.5%) than in control subjects (+14.7 +/-2.4%) and an increase (P<0.05) in diastolic blood pressure in TOF (-2.4+/-2.5%), which was not seen in control subjects (+4.3+/-2.9%). CONCLUSIONS: Young adults successfully operated on for TOF in early childhood exhibit a resetting of the cardiopulmonary baroreflex to operate at smaller LVEDD and at a higher level of forearm vascular resistance. The blunted heart rate response to LBNP -40 mmHg is consistent with previous observations pointing to disturbances in the efferent arm of the baroreflex.

Adolescent↗
PubMed2005

Risk factors of prolonged postoperative pleural effusion after repair of tetralogy of Fallot.

BACKGROUND: Tetralogy of Fallot (TOF) is the most common cyanotic congenital heart disease, and total correction is the definitive treatment. Chest tube drainage of pleural effusion (PE) is essential after surgery. Prolonged PE (> 7 days) is one of the complications; it may increase hospital stay and the risks of morbidity and mortality. The aim of this study was to investigate and analyze the possible risk factors for prolonged PE after total correction of TOF. METHODS: Thirty-seven patients who received total correction of TOF between July 1999 and April 2001 were included in this study. They were divided into 2 groups according to the duration of chest tube drainage for postoperative PE: Group I had postoperative PE < or = 7 days; Group II had postoperative PE > 7 days. Detailed records were taken on patients' demographic characteristics, blood parameters, surgery, electrocardiographic and radiologic data, and angiographic and echocardiographic findings. The data of the 2 groups were compared using the Wilcoxon rank-sum test and Fisher's exact test. Risk factors were analyzed by logistic regression and model selection. RESULTS: Of the 37 patients, 16 were male and 21 were female. There were 32 patients (86.5%) in Group I and 5 (13.5%) in Group II. Mean patient age at repair was 1.82 +/- 1.29 years (range, 0.53-3.11 years). Significant differences (p < 0.05) between the 2 groups were noted for gender, age at repair, body weight, presence of wound infection, duration on heart-lung machine (bypass time), oxygen saturation before surgery, duration of endotracheal intubation, length of hospital stay, and Nakata index. These risk factors were analyzed by logistic regression and model selection. Two models were set up: Model 1--oxygen saturation before surgery, presence of wound infection, age at repair; Model 2--oxygen saturation before surgery, presence of wound infection. CONCLUSION: Prolonged PE is a significant morbidity after TOF repair. The risk factors for prolonged PE are gender, age at repair, body weight, bypass time, low oxygen saturation before surgery, wound infection after surgery, duration of endotracheal intubation, length of hospital stay, and Nakata index. Oxygen saturation before surgery and wound infection were major risk factors while age at repair was a confounder.

Age Factors↗
PubMed2005

Arrhythmias after tetralogy of fallot repair.

Tetralogy of Fallot is the most common cyanotic congenital heart disease, with a good outcome after total surgical correction. In spite of a low perioperative mortality and a good quality of life, late sudden death remains a significant clinical problem, mainly related to episodes of sustained ventricular tachycardia and ventricular fibrillation. Fibro-fatty substitution around infundibular resection, intraventricular septal scar, and patchy myocardial fibrosis, may provide anatomical substrates of abnormal depolarization and repolarization causing reentrant ventricular arrhythmias. Several non-invasive indices based on classical examination such as ECG, signal-averaging ECG, and echocardiography have been proposed to identify patients at high risk of sudden death, with hopeful results. In the last years other more sophisticated invasive and non-invasive tools, such as heart rate variability, electroanatomic mapping and cardiac magnetic resonance added a relevant contribution to risk stratification. Even if each method per se is affected by some limitations, a comprehensive multifactorial clinical and investigative examination can provide an accurate risk evaluation for every patient.

Journal Article↗
PubMed1983

[Cardiovascular malformations associated with tetralogy of Fallot. Apropos of a series of 250 cases of tetralogy of Fallot].

This study was undertaken in 39 patients with Fallot's tetralogy associated with one or more cardiovascular malformations. Some common anomalies, simple anatomical variations were excluded (right sided aortic arch, patent foramen ovale). Out of a total of 48 anomalies (6 children had 2, 3 or 4 associated malformations) there were 5 anomalous systemic venous drainages (including 4 supernumerary left superior vena cava), 8 secundum atrial septal defects (including one multiperforated defect), 2 muscular ventricular septal defects, 7 atrioventricular canals (5 in mongol children); 1 tricuspid hypoplasia, 1 absent left pulmonary artery, 1 mitral valve prolapse, 1 obstructive cardiomyopathy, 10 coronary anomalies (including 5 ectopic left anterior descending arteries arising from the right coronary artery), 8 patent ductus arteriosus (arising from the left subclavian in 4 cases of right aortic arch), 3 retro oesophageal subclavian arteries and one congenital subclavian steal syndrome. The incidence of associated malformations was 15 p. 100, but values of up to 30 p. 100 have been reported mainly in anatomical studies. The authors emphasise the diagnostic and therapeutic implications of these associated malformations. In most cases, especially Fallot's tetralogy with an endocardial cushion defect, 2D echocardiography proved to be an essential diagnostic tool. Catheter studies, however, remain necessary, especially for the detection of aortic and coronary anomalies: selective coronary angiography is sometimes required. Difficult surgical problems are mainly encountered when the associated anomaly has not been diagnosed preoperatively. Although the variations of systemic venous drainage are not of great importance, those of the aortic arch can influence the technique of palliative procedures. Muscular VSD must not be missed. Tricuspid hypoplasia may pose a complex surgical problem. Malformations of the left heart which are sometimes unrecognised, aggravate the situation considerably. The association of Fallot's tetralogy and atrioventricular canal can now be corrected under good conditions. Finally, variations in the coronary anatomy must be documented meticulously as they may contraindicate early complete repair.

Angiocardiography↗
PubMed1985

[Malformations of the tricuspid valve associated with Fallot's tetralogy. Apropos of a series of 224 surgically treated Fallot's tetralogies].

This study was based on 7 children aged 20 months to 13 years with tetralogy of fallot (TOF) and tricuspid valve defects. Cases of endocardial cushion defects (8 cases of atrioventricular) were excluded. Three types of tricuspid valve defect were observed: 4 tricuspid valve prolapse (with one associated mitral valve prolapse); 2 accessory tricuspid valves passing through a ventricular septal defect to prolapse in diastole under the aortic valve; 1 Ebstein anomaly. The prevalence of tricuspid valve defects associated with TOF 3 p. 100 in this series. The diagnosis can: usually be made by 2D echocardiography. Tricuspid valve prolapse (usually the septal leaflet) is visualised in the apical 4 chamber view. The passage of accessory tricuspid tissue across the VSD into the left ventricular outflow tract is visible in the parasternal long axis or subcostal long axis views. Ebstein anomaly can be demonstrated in apical 4-chamber views showing the abnormally apical site of insertion of the septal leaflet of the tricuspid valve and "atrialisation" of part of the right ventricle. Tricuspid valve defects did not pose any special surgical problems during complete repair of TOF but this series did not include any cases of tricuspid hypoplasia or parachute tricuspid valve which have been reported in the literature and which do complicate surgery. Tricuspid valve and subendocardial cushion defects should be looked for systematically in patients with TOF undergoing 2D echocardiography.

Adolescent↗
PubMed1981

[Tetralogy of Fallot and aortic insufficiency].

Seven out of 348 cases of Fallot's tetralogy (2 %) referred over an eight year period were complicated by aortic incompetence (AI). The incidence of AI was much lower than in VSD (7.6 %), probably because of the prevailing haemodynamics: in high VSD, the left-to-right shunt may damage the aortic cusps whilst in Fallot's tetralogy the output of both ventricles is ejected directly into the aorta with no alteration of aortic valve function. In this series, infective endocarditis was the predominant cause of AI (3 cases); surgery had to be undertaken in the infectious phase in 2 cases. AI was coincidental in two other cases (rheumatic valvular disease and calcified bicuspid valve). In the remaining two cases, the role of increased aortic flow in late distension of the aortic valve ring is discussed. AI should be corrected in the same operative time as the Fallot's tetralogy. Late onset AI occurring after repair of Fallot's tetralogy should also be corrected; in this situation, it is often difficult to diagnose the origin of the diastolic murmur. Without surgery the prognosis is poor as the diastolic regurgitation occurs in a ventricle whose function is often already compromised.

Adolescent↗
PubMed1979

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↗