Se expone el caso de un niño de catorce meses de edad, con tetralogía de Fallot asociada a síndrome de cimitarra, cuyo diagnóstico se realizó mediante. Version Info. Last reviewed on 12/8/; Steven Kang, MD, Director, Cardiac Electrophysiology, Alta Bates Summit Medical Center, Stanford Healthcare. Download Citation on ResearchGate | Tetralogía de Fallot | La tetralogía de Fallot es la cardiopatía congénita cianógena más frecuente. Desde el punto de vista.

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Mortality rate depends on the severity of the tetralogy of Fallot. This malformation is infrequently described as sub-pulmonary stenosis or subpulmonary obstruction. The first total repair of tetralogy of Fallot was done by a team led by C.

This feature is now generally agreed to be a secondary anomaly, as the level of hypertrophy tends to increase with age. This redirected a large portion of the partially oxygenated blood leaving the heart for the body into the lungs, increasing flow through the pulmonary circuit, and greatly relieving symptoms in patients. Transposition of the great arteriesEisenmenger syndromeEbstein anomaly [7].

Tetralogy of Fallot TOF is a type of heart defect present at birth.

There is anatomic variation between the hearts of individuals with tetralogy of Fallot. The aorta is too large, thus “overriding,” and this “steals” from the pulmonary artery, which is therefore stenosed. Children with tetralogy of Fallot may develop “tet spells”. The first Blalock-Thomas-Taussig shunt surgery was performed on month-old Eileen Saxon on November 29, with dramatic results.

Taussigand lab assistant Vivien Thomas at Johns Hopkins University developed a palliative surgical procedure, which involved forming a side to end anastomosis between the subclavian artery and the pulmonary artery.

Tetralogía de Fallot

The material is in no way intended to replace professional medical care by a qualified specialist and should not be used as a basis for diagnosis or treatment.

Tetralogy of Fallot results in low oxygenation of blood due to the mixing of oxygenated and deoxygenated blood in the left ventricle via the ventricular septal defect VSD and preferential flow of the mixed blood from both ventricles through the aorta because of the obstruction to flow through the pulmonary valve.


Episodes of bluish color to the skindifficulty breathing, heart murmurfinger clubbing [2]. Journal of the American College of Cardiology. The documents contained in this web site are presented for information purposes only. It can occur at the pulmonary valve valvular stenosis or just below the pulmonary valve infundibular stenosis.

Archived from the original on 3 October The baby may turn blue with breast feeding or crying. In some cases thickening of the septum septal hypertrophy can narrow the margins of the defect. Presumably, this arises from an unequal growth of the aorticopulmonary septum.

The defect is centered around the most superior aspect of the ventricular septum the outlet septumand in the majority of cases is single and large. Based on symptoms, echocardiogram [6]. This is not to be confused with the similarly named teratologya field of medicine concerned with abnormal development and congenital malformations including tetralogy of Fallot.

Diagnostic methods Useful diagnostic tests are the chest radiograph, electrocardiogram, and echocardiogram. Chronic issues thatnow face such adults include pulmonary regurgitation, recurrence of pulmonary stenosis, and ventricular dee.

Due to the misarrangement of the external ventricular septum, the right ventricular falllt increases in size to deal with the increased obstruction to the right outflow tract.

Older children will often squat during a tet spell. Health care resources for this disease Expert centres Diagnostic tests 29 Patient organisations 29 Orphan drug s 1. Summary and related texts. Other search option s Alphabetical list. Tex Heart Inst J.

Tetralogía de Fallot asociada a síndrome de cimitarra – ScienceDirect

Patients nowadays usually present as neonates, falot cyanosis of varying intensity based on the degree of obstruction to flow of blood to the lungs. Patients who have undergone total surgical repair of tetralogy of Fallot have improved hemodynamics and often have good to excellent cardiac function after the operation with some to no exercise intolerance New York Heart Association Class I-II.


Right pulmonary valves stenosis insufficiency absence tricuspid valves stenosis atresia Ebstein’s anomaly Hypoplastic right heart syndrome Uhl anomaly. The aetiology is multifactorial, but reported associations include untreated maternal diabetes, phenylketonuria, and intake of retinoic acid. TOF occurs in about 1 in 2, newborns. The abnormal ” coeur-en-sabot ” boot-like appearance of a heart with tetralogy of Fallot is classically visible via chest x-ray, although most infants with tetralogy may not show this finding.

Only comments seeking to improve the quality and accuracy of information on the Orphanet website are accepted. Before more sophisticated techniques became available, chest x-ray was the definitive method of diagnosis. This then prevents ventricular wall closure, therefore VSD, and this increases the pressures on the right side, and so the R ventricle becomes bigger to handle the work.

Primarily, the degree of right ventricular outflow tract obstruction varies between patients and generally determines clinical symptoms and disease progression. The pulmonic stenosis is the major cause of the malformations, with the other associated malformations acting as compensatory mechanisms to the pulmonic stenosis.

As the strategies for surgical and medical management have progressed, the morbidity and mortality of those born with tetralogy of Fallot in the current era is expected to be significantly improved. The echocardiogram establishes the definitive diagnosis, and usually provides sufficient information for planning of treatment, which is surgical. Views Read Edit View history.

This page was last edited on 29 Octoberat Untreated TOF also causes delayed growth and development, including delayed puberty.