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PubMed1975

Variations in clinical presentation of Fallot's tetralogy in infancy. Angiographic and pathogenetic implications.

Striking variability has been observed in the presenting features in infancy of patients subsequently shown to have tetralogy of Fallot. Some patients presented with severe cyanosis in the neonatal period while others had a systolic murmur and cyanosis only on crying. In these latter patients cyanosis became present at rest over the subsequent months. Yet others presented with episodic attacks of unconsciousness, and a final group presented with dyspnoea and heart failure, accompanying a left-to-right shunt. Angiography demonstrates corresponding variations in the anatomy of the ventricular outflow tracts. In the severely cyanosed patients, the conus septum was deviated so as to obstruct the pulmonary outflow tract, and was best visualized in the lateral projection. In the patients with increasing cyanosis or episodic attacks of unconsciousness, the conus septum again obstructed the pulmonary infundibulum, but was obliquely orientated, not being seen clearly on either lateral or frontal projections. The episodic attacks were considered to be related to infundibular spasm, as previously shown to occur in Fallot's tetralogy. In contrast, increasing cyanosis was believed to be related to hypertrophy of infundibular musculature. In the patients with an initial left-to-right shunt, the arteries were orientated side-by-side so that the conus septum was observed in the frontal projection. Again, subsequent cyanosis was related to ventricular hypertrophy producing outflow tract obstruction. These findings are interpreted in the setting of a recent study, indicating that Fallot's tetralogy is produced by rotation of the embryonic conus together with sinistro-anterior deviation of the conus septum.

Angiocardiography↗

PubMedDate not supplied

The influence of pulmonary insufficiency on ventricular function following total correction of Fallot's tetralogy: evaluation using radionuclide ventriculography and clinical findings.

Long-standing pulmonary insufficiency after repair of tetralogy of Fallot may adversely affect ventricular function. We evaluated 20 patients postoperatively by radionuclide ventriculography and clinical findings after total correction of tetralogy of Fallot. Patients were divided into two groups as follows: Group I patients (10) had no pulmonary insufficiency; Group II patients (10) had moderate or severe pulmonary insufficiency. Preoperatively, there was no difference between groups in terms of age, functional capacity according to the New York Heart Association criteria, hemoglobin and hematocrit level, cardiothoracic ratio, McGoon ratio, left and right ventricular ejection fraction, cardiac output or cardiac index. Postoperatively, right ventricular ejection fraction was 40.10 +/- 2.28 in Group I and 29.5 +/- 2.86 in Group II, p < 0.01. Left ventricular ejection fraction was 59.3 +/- 2.90 in Group I and 50.9 +/- 4.19 in Group II, p < 0.01. Radionuclide ventriculography is a useful means of identifying right ventricular dysfunction following repair of tetralogy of Fallot. The dysfunction appears significantly worse in patients with pulmonary insufficiency.

Child↗

PubMed1992

[Diagnosis of congenital heart defects today. Part 2: Aortic stenosis, aortic isthmus stenosis, tetralogy of Fallot, transposition of great vessels].

In this second part of our review, the diagnosis of the following congenital heart disease is discussed: aortic stenosis, aortic isthmus stenosis (coarctation of the aorta), Fallot's tetralogy and transposition of the great vessels. Aortic stenosis and coarctation of the aorta each represents a spectrum of cardiac diseases of varying severity. Cases that are clinical less severe may escape diagnosis until late childhood or adolescence. Fallot's tetralogy and transposition of the great vessels in contrast, lead to cyanosis, and are therefore usually diagnosed already in the young infant. In all four conditions, the suspected diagnosis can be established on the basis of clinical or auscultatory findings. Further diagnostic clarification is achieved with the aid of non-invasive procedures such as CT scan, chest X-ray, echocardiography and, where indicated, NMR imaging. Additional cardiac catheterization is required only in the case of the tetralogy of Fallot.

Aortic Coarctation↗

PubMed1985

[Ebstein's disease associated with Fallot's tetralogy. Apropos of a familial case, review of the literature, embryologic and genetic implications].

Ebstein anomaly is a rare congenital cardiac malformation, representing 0.3 p. 100 of all congenital heart disease. The authors report a case of a child with Ebstein anomaly associated with tetralogy of Fallot, whose father had an apparently simple Ebstein anomaly. The association of Ebstein-Fallot is extremely rare with only 5 previously reported cases in the literature. The association Ebstein-VSD-pulmonary stenosis is more frequent (13 cases), whilst the association Ebstein-isolated VSD (33 cases) and Ebstein-pulmonary stenosis/atresia (38 cases) are relatively common. Other very varied associations have also been described. Atrial septal defect is practically an integral part of Ebstein anomaly with an ostium secundum or patent foramen ovale in 51 to 80 p. 100 of cases. The possibility of 2 subjects in the same family with Ebstein anomaly is far from negligible. A review of the literature revealed 7 other reported "pairs". A certain number of other cases also demonstrate the association of Ebstein anomaly with another congenital cardiac malformation in the same family, usually a VSD or tetralogy of Fallot. Our familial case raises the question of a possible embryological link between Ebstein anomaly and tetralogy of Fallot. Our experimental embryological studies also suggest the presence of a common denominator between these two conditions.

Adult↗

PubMed2005

Aortic root dilatation in tetralogy of Fallot long-term after repair--histology of the aorta in tetralogy of Fallot: evidence of intrinsic aortopathy.

The ascending aorta or pulmonary trunk in congenital heart disease may dilate out of proportion to hemodynamic or morphogenetic expectations, may become aneurysmal, and may rupture. A bicuspid aortic valve and/or coarctation of the aorta are consistently associated with ascending aortic and para-coarctation medial abnormalities. Congenital heart diseases such as single ventricle, truncus arteriosus, transposition of the great arteries and tetralogy of Fallot are also associated with aortic medial abnormalities. Aortic regurgitation in unrepaired tetralogy of Fallot imposes volume overload on both ventricles. A significant subset of adults late after repair of tetralogy of Fallot exhibits progressive aortic root dilatation which may lead to regurgitation and predispose to dissection and rupture which can be fatal, and necessitating aortic valve and aortic root surgery. The aortic dilatation relates medial abnormalities coupled with previous long-standing volume overload of the ascending aorta. Risk factors for aortic dilatation and regurgitation in tetralogy of Fallot relate to specific hemodynamic abnormalities such as pulmonary atresia, right aortic arch and a history of an aorto-pulmonary shunt, and patient demographics such as male sex and the association of chromosome 22q11 deletion. There is no current consensus on beta-blocker administration for limiting progressive dilatation of the aortic root in patients with congenital heart disease and repaired tetralogy of Fallot. Aortic root surgery should be considered for these patients and address aortic regurgitation and or prevent the risk of aortic dissection. Meticulous follow-up of the aortic root after repair, tetralogy of Fallot are thus recommended.

Adrenergic beta-Antagonists↗

PubMed1983

Atypical Fallot's tetralogy with doubly committed subarterial ventricular septal defect. Diagnostic value of 2-Dimensional echocardiography.

Five patients with clinical features of tetralogy of Fallot had subarterial doubly committed ventricular septal defect (VSD) with absence or deficiency of the infundibular septum. Two-dimensional (2-D) echocardiography differentiated this special group from those with the usual Fallot's anatomy. In addition to the long-axis view of the left ventricle which showed the subaortic VSD and the aortic and mitral fibrous continuity, the short-axis view at the cardiac base clearly demonstrated the subpulmonary extension of the VSD in the atypical group. Two-dimensional echocardiography was found superior to biplane angiography, which in 3 patients suggested the erroneous diagnosis of double-outlet right ventricle with pulmonic valve stenosis. This variant form of Fallot's tetralogy poses special surgical problems.

Adolescent↗

PubMed1979

'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↗

PubMed2001

Open surgical repair of tetralogy of Fallot in dogs.

Tetralogy of Fallot is a congenital heart defect that causes cyanosis, severe progressive weakness and activity intolerance, polycythemia, and shortened life span in dogs. Open surgical repair of tetralogy of Fallot consisting of closure of the ventricular septal defect and reconstruction of the right ventricular outflow tract via a right ventriculotomy during cardiopulmonary bypass is feasible in severely affected dogs. Successful surgical repair can completely resolve clinical signs associated with the defect.

Animals↗

PubMed1990

[Fallot's tetralogy in a calf--a case report].

A report is given on a calf with tetralogy of Fallot. This is the most common cyanotic congenital heart lesion in calves. Diagnosis was made by two-dimensional echocardiography and heart catheterisation. Tetralogy of Fallot was confirmed by necropsy.

Animals↗

PubMed1986

[Hypertrophic myocardiopathy and tetralogy of Fallot. Study of 2 cases].

The authors report two observations of concentric hypertrophic cardiomyopathy associated with Fallot's tetralogy in one case and with pulmonary atresia complicated by atrial septal defect in the other. The association of Fallot's tetralogy with hypertrophic cardiomyopathy is rare, as only 5 cases have been reported in the literature. In this connection, the mechanism of myocardial hypertrophy and the therapeutic problems posed by this association are discussed.

Adolescent↗

PubMed1990

[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%).

Adolescent↗

PubMed1997

The modified Blalock-Taussig shunt: clinical impact and morbidity in Fallot's tetralogy in the current era.

BACKGROUND: The Blalock-Taussig shunt is considered a low-risk management option for palliation in tetralogy of Fallot, but the morbidity associated with a Blalock-Taussig shunt can have a significant impact on patient care. We reviewed the outcome for this operation in the current era. METHODS: Between 1990 and 1994, 65 children with tetralogy of Fallot received a modified Blalock-Taussig shunt. Sixty patients who had follow-up angiography were assessed for clinical outcomes and shunt-related morbidity and mortality. From the same study period, 68 of 247 pediatric patients who underwent angiography and tetralogy repair, but did not receive palliation, were randomly selected to comprise a comparison group. RESULTS: Palliation was more likely in the presence of a complicated tetralogy malformation or if there was an associated medical condition. Median age at palliation was 58 days (range: 1 to 535 days). Ninety-five percent of shunts were right-sided. Self-limited morbidity complicated 11% of shunt operations. Significantly smaller distal right pulmonary arteries were observed in the palliated group before total repair compared with findings in the group without palliation and 33% of patients who underwent palliation had angiographic evidence of pulmonary artery distortion. Shunt stenosis was common and correlated with younger age at palliation. Shunt occlusion resulted in one death. Excluding noncardiac causes of death, overall survival was 90% in the palliated group versus 97% in the nonpalliated group (p = 0.09). CONCLUSIONS: Pulmonary artery hypoplasia and angiographic evidence of pulmonary artery distortion are common after initial palliation by a modified Blalock-Taussig shunt. Neonatal palliation was associated with significantly smaller pulmonary arteries before repair, which necessitated additional interventions.

Anastomosis, Surgical↗

PubMed1991

[Inter-arterial anastomosis in dextral position of the aortic arch in patients with tetralogy of Fallot].

The article discusses the results of formation of inter-arterial anastomoses in 58 (12.7%) patients with a severe form of Fallot's tetralogy in dextra-position of the arch of the aorta. The specific features of the course and diagnosis of dextra-position of the aortic arch in Fallot's tetralogy are analysed, and the significance of radiologic and echocardiographic examination in the choice of the position and type of interarterial anastomoses is determined. It is proved that Blalock-Taussig's anastomoses must be formed on the side opposite to the aortic arch, while shunting operations must be carried out on the side on which the arch of the aorta is located.

Abnormalities, Multiple↗

PubMed1996

Primary repair of Fallot's tetralogy in an older adult: a 20-year follow-up.

A patient with tetralogy of Fallot who presented at 58 years of age is described. Following surgical intracardiac repair he had 20 years of improved functional capacity. Though such surgery is now usually performed in infancy, the literature indicates that surgical correction of tetralogy of Fallot in appropriate adults is associated with low mortality and good long-term results.

Age Factors↗

PubMed1975

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↗

PubMed1980

Right ventricular cineangiography in Tetralogy of Fallot.

Accurate delineation of the anatomy of the cardiac chambers and the pulmonary vessels is a necessary component of the preoperative workup in Fallot's tetralogy. Selective right ventriculograms in shallow right anterior oblique and steep left anterior oblique views were used in the evaluation of 65 cases. Our observations indicate that these views display the anatomy of Fallot's tetralogy to a better advantage than do the conventional anteroposterior and lateral views.

Adolescent↗

PubMedDate not supplied

[The morphology of the peripheral blood erythrocytes in patients with tetralogy of Fallot before and after its surgical correction].

Quantitative changes in red cells were studied by dark field scanning electron microscopy of native preparations obtained from patients with Fallot's tetralogy of varying severity and duration. Mainly echinocytic transformation of red cells was revealed that directly depended on the disease duration. Morphologic findings have confirmed the efficacy of surgical correction and drug therapy of Fallot's tetralogy.

Adolescent↗

PubMed1989

[Incidence and significance of ventricular arrhythmia after surgical correction of Fallot's tetralogy].

Patients may die suddenly after surgical repair of tetralogy of Fallot. Ventricular arrhythmias (VA) have been considered the possible cause of sudden death in such patients by many Authors. We evaluated the incidence and severity of VA in a group of 65 patients (25 females and 40 males) after surgical repair of tetralogy of Fallot. The age at the time of follow-up varied between 8 and 47 years. We divided our population into 4 groups (A, B, C, D) according to the surgical results (poor and good) and to the age of total correction (less than 12, greater than 12 years). We tested the presence and severity of VA in the different groups of patients and we found a significant difference in the incidence of VA between patients who underwent surgery before and after the age of 12 years (p = 0.0083). The surgical result did not show a significant relation with VA (p = 0.47). Among the patients submitted to surgical repair in the adult age, however, more serious VA are present in those with poor surgical result, and both our cases of sudden death occurred in this kind of patient. We speculate that postoperative ventricular arrhythmias are related to right ventricular function, which in turn appears to be worse in patients who underwent surgical correction at a later age. At last only perspective trials will be able to assess the significance of VA and their relationship with sudden death in this kind of patient.

Adolescent↗