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Results for “Tetralogy of Fallot”

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At least 19 recordsLinked to original sources
PubMed1998

[Recurrent false infundibulo-pulmonary aneurysm after complete correction of Fallot tetralogy].

Infundibulo-pulmonary aneurysm is a rare complication of complete correction of Tetralogy of Fallot and its recurrence has not been previously reported. A girl with Tetralogy of Fallot with two small pulmonary branches underwent complete correction at 3 years of age with widening of the infundibulum, the pulmonary annulus and artery with a pediculated pericordial path. Five years later, the left parasternal systolic murmur increased in intensity due to an infundibulo-pulmonary aneurysm and severe stenosis of the bifurcation of the pulmonary artery confirmed by echocardiography and catheterisation. The child was reoperated with resection of the aneurysm and widening of the pulmonary tract and its two branches with a Dacron patch. Three years later, the aneurysm and pulmonary stenoses recurred and required percutaneous angioplasty and stenting. The inadequacy of the result led to a further surgical procedure.

Aneurysm, False↗
PubMed2005

[First diagnosis of Fallot tetralogy in a 74-year-old man].

Tetralogy of Fallot is the most common cyanotic cardiac malformation in late childhood and adult, occurring in approximately 0.25 of 1000 live births. Most patients undergo early surgical correction. Therefore, the natural history of this disease has been evaluated in only a few cases. We report a complex case of a tetralogy of Fallot, who reached the age of 74 years without surgical or medical treatment and who was transferred to our clinic after syncope due to ventricular tachycardia.

Aged↗
PubMed2004

Hypertrichosis, Fallot tetralogy, growth and developmental delay.

We report a female patient with a unique pattern of pre- and postnatal growth deficiency, tetralogy of Fallot, very long eyelashes (trichomegaly), progressive and generalized hypertrichosis lanuginosa, brain atrophy with epilepsy, and puffy hands and feet. This appears to be a "new" entity within the group of syndromal hypertrichoses, possibly pathogenetically related to, but clinically distinct from Ambras or Oliver-McFarlane syndromes.

Child, Preschool↗
PubMed2005

Peeping through heart's window: a case report of Fallots tetralogy.

Tetralogy of Fallot is a congenital heart defect. A combination of four abnormalities in heart allow oxygen rich and oxygen-depleted blood to mix. The resulting low-oxygen blood then circulates through the body. A child with the condition tends to develop slowly, eat poorly and grow slowly and may experience "blue" spells on crying and dyspnea on exertion. A 12 year old child with this congenital heart defect and cleft palate with cleft lip is discussed. Details of the latest diagnostic and treatment procedures are also presented.

Child↗
PubMed2002

Lung perfusion scintigraphy in the diagnosis of peripheral pulmonary stenosis in patients after repair of Fallot tetralogy.

BACKGROUND: The frequency of peripheral pulmonary artery stenosis in patients after surgical repair of tetralogy of Fallot (TOF) ranges from 20 to 40%. This can be either primary or secondary to the surgical intervention. The influence of resulting lung perfusion alterations on the life quality of patients is difficult to predict. The aim of this study was to compare the utility of the diagnostic procedures in this group of patients, with particular focus on lung perfusion scintigraphy. MATERIAL AND METHODS: This study comprised 104 patients who underwent repair of TOF at ages from 5 months to 25 years. The patients have been followed up for from 4.2 to 25 years. On the basis of chest x-ray peripheral pulmonary artery stenosis was suspected in 11 patients, in 12 on the basis of echocardiography examination. RESULTS: Lung perfusion scintigraphy has been performed on 87 patients. The disturbances in lung perfusion (mostly in the left lung) were show by means of lung perfusion scintigraphy in 43 (49%) of patients. In 27 of them heart catheterisation has been performed. Angiography revealed stenosis of the lung artery branch in 15/43 (34.9%) patients with abnormal perfusion lung scan and in 4/44 (9%) in patients with normal perfusion lung scan. Intervention procedures were carried out on 10 patients. CONCLUSIONS: Lung perfusion scintigraphy may prove a valuable, non-invasive screening tool in the assessment of patients after TOF repair, although both false-negative and false-positive results may happen. Therefore, it should play an auxiliary role together with other diagnostic modalities.

Journal Article↗
PubMed1988

Fetal echocardiography. VIII. Aortic root dilatation--a marker for tetralogy of Fallot.

Tetralogy of Fallot has increased in incidence during the past decade. Although abnormalities of the four-chamber screening examination of the fetal heart identifies structural anomalies associated with semilunar and atrioventricular valve hypoplasias, the anomalies associated with tetralogy of Fallot are not as readily identifiable from the four-chamber view alone. This study was designed to determine whether aortic root dilatation, commonly observed in the newborn with tetralogy of Fallot, is a marker for tetralogy of Fallot diagnosed in utero. In 45 normal (control) fetuses the biparietal diameter, head circumference, abdominal circumference, and femur length were measured and M-mode measurements were made of the biventricular outer dimension and aortic root dimension. The aortic root dimension from seven fetuses (18, 19, 20, 23, 32, 33, and 34 weeks' gestation) with tetralogy of Fallot was increased in dimension when compared with cardiac (biventricular outer dimension) and noncardiac biparietal diameter, head and abdomen circumferences, and femur length parameters. In conclusion, incorporation of aortic root imaging with the four-chamber view of the fetal heart during a routine screening examination allows for identification of fetuses with tetralogy of Fallot.

Adult↗
PubMed2000

Early onset of progressive subaortic stenosis after complete repair of tetralogy of Fallot.

Tetralogy of Fallot is often found in association with a wide variety of other cardiac lesions, but is rarely found in association with lesions causing obstruction to the left ventricular inflow or outflow. Subaortic stenosis has only rarely been reported in association with tetralogy of Fallot. We report a patient with Marfan syndrome who underwent repair of tetralogy of Fallot at five years of age. Discrete and progressive subaortic stenosis developed two years after the surgical correction, in a previously normal and unobstructed left ventricular outflow tract. Surgical removal of the acquired fibrous subaortic shelf was successful. Clinical signs of obstruction within the left ventricular outflow tract after surgical repair of tetralogy of Fallot should prompt further investigation to exclude the onset of acquired subaortic stenosis.

Adolescent↗
PubMed2006

Genetic heterogeneity and phenotypic anomalies in children with atrioventricular canal defect and tetralogy of Fallot.

Tetralogy of Fallot associated with the atrioventricular canal defect has been usually reported in association with Down syndrome. The aim of the present study was to describe the cardiac aspects and the genetic anomalies in children with this association of heart defects. We identified 64 patients with atrioventricular canal defect tetralogy of Fallot. All children underwent complete cardiovascular, clinical phenotypic and genetic evaluation. A genetic syndrome or extracardiac anomalies were found in 56 patients (87.5%). Down syndrome (43 patients, 67.2%) was the most frequent genetic diagnosis. Other syndromes were 8p deletion, trisomy 13, duplication 5p, cranio-cerebello-cardiac syndrome, Cantrell syndrome, CHARGE association, VACTERL association, and DiGeorge syndrome related to maternal diabetes. No patients in our series had 22q11 deletion. Tetralogy of Fallot with extreme dextroposition of the aorta was found in seven patients (only one with Down syndrome). Additional cardiac malformations were present in 23 patients (only 11 with Down syndrome). The association between atrioventricular canal defect and tetralogy of Fallot represents a cardiac phenotype with strong genetic characteristics. For this reason, a careful genetic examination is required. Our study confirms the high prevalence of Down syndrome, but also reveals a significant genetic heterogeneity. Additional cardiac defects are prevalent in patients without Down syndrome.

Adolescent↗
PubMedDate not supplied

Association of Fallot Tetralogy with Carney's complex.

The Carney complex is an inherited, autosomal disease of multicentric tumors in many organs. Some components of Carney's complex are cardiac myxoma, spotty pigmentation, and endocrine overactivity. Primary pigmented nodular adrenocortical dysplasia (PPNAD) is an exceedingly rare cause of Cushing's syndrome in infants, children, and young adults. PPNAD occurs sporadically or as part of a familial syndrome called Carney's complex. Up to our knowledge, the association of Fallot Tetralogy with Carney's complex has not been previously reported. We presented, a 20-year-old woman, who had been operated for Fallot Tetralogy at the age of 3 years, had Carney's complex, i.e. left atrial myxoma, two facial spotty pigmented areas, and PPNAD.

Adrenal Cortex Neoplasms↗
PubMedDate not supplied

Exercise capacity in young patients after total repair of Tetralogy of Fallot.

Tetralogy of Fallot is the most common form of cyanotic congenital heart disease. Measurement of physical activity is usually performed as a routine part of the patient's cardiac evaluation. The aim of this study was to examine the exercise performance of young patients operated on for tetralogy of Fallot, assessing the possible influence of known negative prognostic factors related to the surgical repair. The study group comprised 41 consecutive patients (29 male and 12 female, ages 11.2 +/- 3.9 years, range 6-16 years) operated on for tetralogy of Fallot. Patients in the study group were divided in subgroups in relation to the age of surgical intervention (before or after 2 years of life), the surgical approach (combined transatrial/transpulmonary approach or right ventriculotomy), and the presence of aortopulmonary shunts prior to performing total correction. Their data were compared with those of 33 aged-matched asymptomatic control subjects (19 male and 14 female, ages 11.9 +/- 1.3 years, range 11-16 years). Blood pressure and heart rate measured at rest were similar between control and Fallot groups. A normal increase in systolic blood pressure was observed in response to exercise intensity for all subgroups. No significant difference between control and Fallot groups was found under conditions of mild or moderate exercise or for diastolic blood pressure at rest and in response to exercise. Lower maximal heart rate and systolic blood pressure values were recorded in all patients when compared with the control subjects. Significant differences in peak workload were detected between control and Fallot groups and between the control and each subgroup; however, no difference was found between subgroups. In conclusion, despite their very satisfactory clinical status, all patients showed a reduced peak workload, irrespective of the surgical approach, age at surgery, and aortopulmonary shunts prior to performing total correction.

Adolescent↗
PubMed2003

Aortic stenosis after uncomplicated surgical repair of tetralogy of Fallot.

Tetralogy of Fallot is only rarely associated with aortic valvar disease. We present a child who had uncomplicated repair of tetralogy of Fallot at 16 months of age, and who developed mild aortic stenosis three years later, with mild to moderate aortic incompetence being seen two years subsequent to that event. No aortic valvar disease had been noted prior to surgery. No intervention is planned at this stage.

Aortic Valve Insufficiency↗
PubMed2004

Survival to the age of 52 years in a man with unrepaired tetralogy of Fallot.

Tetralogy of Fallot is the most common cyanotic congenital heart defect during infancy. It is composed of a ventricular septal defect, an overriding aorta, obstruction of right ventricular outflow, and right ventricular hypertrophy. Most patients experience cyanosis at birth and die in childhood without surgical intervention. The rate of survival at 40 years without surgical correction is only 3%. We present the case of a man with tetralogy of Fallot who survived until the age of 52 years without surgical intervention.

Aortography↗
PubMed1990

Tetralogy of Fallot.

Tetralogy of Fallot is the most common malformation of children born with cyanotic heart disease, with an incidence of approximately 10 per cent of congenital heart disease. There can be a wide spectrum as to the severity of the anatomic defects, which include ventricular septal defect, aortic override, right ventricular outflow tract obstruction, and right ventricular hypertrophy. Cyanosis may vary from mild to severe, and patients may present as newborns or, more commonly, young infants. Infants with classic tetralogy of Fallot and stable anatomy should undergo primary complete intracardiac repair. The overall hospital mortality is approximately 3 to 5 per cent, with most patients who survive having an excellent clinical and hemodynamic result.

Cardiac Catheterization↗
PubMed1994

[Myocardium protection using warm blood cardioplegia in corrective operation of tetralogy Fallot].

In this paper, the children, who had undergone the operation with the approach similar to that of Tetralogy Fallot, were selected and divided into 4 groups, according to different kinds of cardioplegia were infused during corrective operation. St. Thomas group (CC group) 24 cases, cold blood cardioplegia group (CBC group) 21 cases, terminal warm blood cardioplegia group (TWBC group) 12 cases, and continuous warm blood cardioplegia group (CWBC group) 12 cases. Through the measurement of left ventricular contraction pressure and CK-Mb release in the postoperative period, the effect of myocardial preservation was better in TWBC and CWBC groups than in CC and CBC groups (P < 0.05), ATP volume of myocardium in TWBC and CWBC groups were higher (17.1 and 18.2 mumol/g) than that in CBC group (2.48 mumol/g) (P < 0.01) CP values were respectively 8.74 mumol/g, 7.63 mumol/g and 5.5 mumol/g (P < 0.05). 9 cases (37%) in CC group needed inotropic drug to assist circulation postoperation, 4 cases (16%) in CBC group, 2 cases (12%) in both TWBC and CWBC group. The results demonstrated that WBC had a better myocardial protection effect.

Bicarbonates↗
PubMed1999

A case of lateral facial clefts with Fallot tetralogy, duodenal stenosis and intestinal malrotation: a new multiple congenital anomaly syndrome?

Multiple congenital malformations in a Caucasian female infant are described which include lateral facial clefts, malformed external ears, cleft palate, Fallot tetralogy, duodenal stenosis and intestinal malrotation. There were no associated limb or spinal anomalies. This case appears to be an example of a new multiple congenital anomaly syndrome.

Abnormalities, Multiple↗
PubMedDate not supplied

[Ventricular arrhythmias and conduction disturbances in patients with corrected tetralogy of Fallot].

Tetralogy of Fallot is the most common cyanotic heart disease. Corrective intracardiac repair has been performed for >40 years with excellent results. Nevertheless ventricular arrhythmias have consistently been reported, and sudden cardiac death is the most common cause of mortality late after repair. Programmed ventricular stimulation has been used in selected patients at many centers; however, attempts to clarify its predictive value have been limited by small patient number and relatively low event rates. The emergence of successful methods for both the prevention of arrhythmias, (including electrophysiological ablation and implantable defibrillators), has meant the identification of those at risk of even greater importance.

Arrhythmias, Cardiac↗
PubMed2004

Predicting late sudden death from ventricular arrhythmia in adults following surgical repair of tetralogy of Fallot.

Tetralogy of Fallot was the first complex congenital cardiac defect to undergo open repair. The life expectancy and quality of life of those surviving surgery is now good, although late survival is compromised by the occurrence of sudden death. The emergence of successful methods for both the prevention of arrhythmias (including valve replacements and electrophysiological ablation) and the treatment of arrhythmias when they occur (including implantable defibrillators), has meant the identification of those at risk is of even greater importance. This paper reviews the predictive methods currently available to the practising physician caring for these increasingly common patients.

Adult↗