Professional Documents
Culture Documents
Tetralogy of Fallo2
Tetralogy of Fallo2
The tetralogy of Fallot is a congenital heart defect which classically has four anatomical components. It is the most common cyanotic heart defect and the most common cause of blue baby syndrome. It was described in 1672 by Niels Stensen and in 1888 by the French physician Etienne Fallot, for whom it is named.[1]
Contents
[hide]
1 Anatomic morphology o 1.1 Primary four malformations o 1.2 Other variations 2 Epidemiology and etiology
3 Pathophysiology 4 Symptoms 5 Diagnosis 6 Treatment o 6.1 Emergency management of tet spells o 6.2 Palliative surgery o 6.3 Total surgical repair 7 Prognosis 8 See also 9 References 10 External links
There is anatomic variation between the hearts of individuals with tetralogy of Fallot. The degree of right ventricular outflow tract obstruction varies between patients and is generally determines clinical symptoms and disease progression. Tetralogy of Fallot may present with other anatomical anomalies, including: 1. 2. 3. 4. 5. stenosis of the left pulmonary artery, in 40% of patients a bicuspid pulmonary valve, in 40% of patients right-sided aortic arch, in 25% of patients coronary artery anomalies, in 10% of patients an atrial septal defect, in which case the syndrome is sometimes called a pentalogy of Fallot 6. an atrioventricular septal defect 7. partially or totally anomalous pulmonary venous return 8. forked ribs and scoliosis Tetralogy of fallot with pulmonary atresia or pseudotruncus arteriosus is a severe variant in which there is complete obstruction of the right ventricular outflow tract and absence of the pulmonary trunk. In these individuals, there is complete right to left shunting of blood. The lungs are perfused via extensive collaterals from the systemic arteries.
[edit] Pathophysiology
Tetralogy of Fallot results in low oxygenation of blood due to mixing of oxygenated and deoxygenated blood in the left ventricle through the VSD and preferential flow of both oxygenated and deoxygenated blood from the ventricles through the aorta because of obstruction to flow through the pulmonary valve. This is known as a right-to-left shunt. Children with tetralogy of Fallot may develop acute severe cyanosis or hypoxic "tet spells". The precise mechanism of these episodes is in doubt, but presumably results from an increase in resistance to blood flow to the lungs with increased preferential flow of desaturated blood to the body.
[edit] Symptoms
The primary symptom is low blood oxygen saturation with or without cyanosis from birth or developing in the first year of life. Without cyanosis, the baby is referred to as a "pink tet". Other symptoms include a heart murmur which may range from almost imperceptible to very loud, difficulty in feeding, failure to gain weight, retarded growth and physical development, dyspnea on exertion, clubbing of the fingers and toes, and polycythemia. Tet spells are characterized by a sudden, marked increase in cyanosis, syncope, and may result in hypoxic brain injury and death.
[edit] Diagnosis
Often a simple chest x-ray is enough to determine the presence of this condition. The heart may present with a "boot-like" appearance, rather than the symmetric appearance of a normal heart.
[edit] Treatment
Tetralogy of Fallot is treated on two levels: with immediate emergency care for hypoxic or "tet" spells and with corrective surgery.
Currently, Blalock-Thomas-Taussig shunts are not normally performed on infants with TOF except for severe variants such as TOF with pulmonary atresia.
[edit] Prognosis
Untreated, tetralogy of Fallot results in progressive right ventricular hypertrophy and dilatation due to the increased resistance on the right ventricle. The dilated cardiomyopathy progresses to right heart failure, usually with accompanying left heart failure. Actuarial survival for untreated tetralogy of Fallot is approximately 75% after the first year of life, 60% by four years, 30% by ten years, and 5% by forty years. Patients with repaired tetralogy of Fallot have the potential to lead normal lives with continued excellent cardiac function, with some considerations: Current techniques for total surgical repair greatly improve the hemodynamic function of the heart with tetralogy of Fallot but do not provide a lifetime correction of the defect. Ninety percent of patients with total repair as infants develop a progressively leaky pulmonary valve as the heart grows to its adult size. Patients also may have some degree of residual right outflow stenosis and damage to the electrical system of the heart from surgical incisions, causing abnormalities as detected by EKG and/or arrhythmias. Long-term follow up studies show that this patient population is at risk for sudden cardiac death and for heart failure. Therefore, lifetime follow-up care by an adult congenital cardiologist is recommended to monitor these risks and to recommend treatment, such as interventional procedures or re-operation, if it becomes necessary.
Antibiotic prophylaxis is indicated during dental treatment in order to prevent infective endocarditis.
Trilogy of Fallot
[edit] References
1. ^ synd/2281 at Who Named It 2. ^ a b c Gatzoulis MA, Webb GD, Daubeney PE. (2005) Diagnosis and Management of 3. 4. 5. 6.
Adult Congenital Heart Disease. Churchill Livingstone, Philadelphia. ISBN 0443071039. ^ Bartelings M, Gittenberger-de Groot A (1991). "Morphogenetic considerations on congenital malformations of the outflow tract. Part 1: Common arterial trunk and tetralogy of Fallot". Int. J. Cardiol. 32 (2): 213-30. PMID 1917172. ^ Anderson RH, Weinberg. The clinical anatomy of tetralogy of Fallot. Cardiol Young. 2005 15;38-47. PMID 15934690. ^ Anderson RH, Tynan M. Tetralogy of Fallot a centennial review. Int J Cardiol. 1988 21; 219-232. PMID 3068155. ^ Murakami T (2002). "Squatting: the hemodynamic change is induced by enhanced aortic wave reflection". Am. J. Hypertens. 15 (11): 986-8. PMID 12441219.