The tricuspid atresia is the absence of the tricuspid valve, connected to a hypoplastic right ventricle. There are many accompanying malformations such as atrial septal defect, patent ductus arteriosus, pulmonary valve stenosis and transposition of the great vessels. Symptoms include cyanosis and heart failure. The second heart sound (S2) is single and the cardiac murmurs depend on the accompanying malformations. The diagnosis is made by echocardiography. A cardiac catheterization may be required. The final treatment is surgical.

The tricuspid atresia accounts for 1-3% of congenital heart defects. The most common type (sometimes called classic tricuspid atresia called) surrounds a ventricular septal defect (VSD) and pulmonary stenosis, leading to a decreased pulmonary blood flow to an elevated pressure in the right atrium and an obligatory right-left shunt at the atrial level through a lead stretched patent foramen ovale or atrial septal defect (ASD), whereby a cyanosis caused (tricuspid atresia). In 12-25% of cases, a transposition of the great vessels with a VSD and a normal pulmonary valve is present. The lung receives blood directly from the left ventricle, as a result, produces a heart failure and pulmonary hypertension. Thus, the pulmonary blood flow can be increased or decreased.

The tricuspid atresia is the absence of the tricuspid valve, connected to a hypoplastic right ventricle. There are many accompanying malformations such as atrial septal defect, patent ductus arteriosus, pulmonary valve stenosis and transposition of the great vessels. Symptoms include cyanosis and heart failure. The second heart sound (S2) is single and the cardiac murmurs depend on the accompanying malformations. The diagnosis is made by echocardiography. A cardiac catheterization may be required. The final treatment is surgical. The tricuspid atresia accounts for 1-3% of congenital heart defects. The most common type (sometimes called classic tricuspid atresia called) surrounds a ventricular septal defect (VSD) and pulmonary stenosis, leading to a decreased pulmonary blood flow to an elevated pressure in the right atrium and an obligatory right-left shunt at the atrial level through a lead stretched patent foramen ovale or atrial septal defect (ASD), whereby a cyanosis caused (tricuspid atresia). In 12-25% of cases, a transposition of the great vessels with a VSD and a normal pulmonary valve is present. The lung receives blood directly from the left ventricle, as a result, produces a heart failure and pulmonary hypertension. Thus, the pulmonary blood flow can be increased or decreased. Tricuspid atresia, the tricuspid valve is absent and the right ventricle is hypoplastic. An antechamber connection must exist. AO = aorta; IVC = inferior vena cava; LA = left atrium; LV = Left ventricle; PA = pulmonary artery; PV = pulmonary veins, RA = right atrium; RV = right ventricle; SVC = superior vena cava. Symptoms and complaints infants with decreased pulmonary blood flow have at birth is usually mild to moderate cyanosis, sometimes dramatically worsened in the first months of life. Infants with increased pulmonary blood flow are at an age of 4-6 Wochenin usually signs of heart failure (eg. As tachypnea, dyspnea with feeding, poor weight gain, diaphoresis). On clinical examination, you hear a single second heart sound (S2) and a holosystolic or frühsystolisches sound of grade 2-3 / 6 of a VSD on the lower left sternal. The systolic outflow sound of a pulmonary stenosis or the continuous sound of a patent ductus arteriosus is to be heard also at the upper left sternal. A systolic shock is rare to palpate. With strongly increased pulmonary blood flow can sometimes stethoscope apical rumble. Cyanosis may cause drumstick fingers when it lasts> 6 months. Diagnostic chest X-ray and ECG echocardiography Normally cardiac catheterization Diagnosis is suspected clinically, supported by a chest x-ray absorption and an EKG and by two-dimensional Farbechokardiographie and Doppler sonography confirmed. In most cases, the X-ray image is inconspicuous or shows a slightly enlarged heart, an enlargement of the right atrium and increased pulmonary vascular markings. Sometimes the heart silhouette of tetralogy of Fallot (shoe-shaped heart and a concave pulmonary segment) is similar. In children with an associated transposition of the great vessels, the pulmonary vascular markings can be reinforced and be cardiomegaly. The ECG typically shows a deviation of the left heart axis (between 0 and 90 °) and left ventricular hypertrophy. A left deviation of the axis of the heart is not present in general, if an associated transposition of the great vessels present. Frequently, a right ventricular hypertrophy occurs with an atrial hypertrophy in combination. A cardiac catheterization may even before the first palliative measures be necessary (especially with older children), to determine the hemodynamics and the anatomy of the pulmonary arteries, unless an echocardiogram or other methods clearly show the pulmonary vascular anatomy and confidently predict a normal pulmonary artery pressure , Treatment For strongly cyanotic neonates: infusion of prostaglandin E1 Sometimes atrial Ballonseptostomie (Rashkind process) Multi-stage operation Most newborns with tricuspid atresia are compensated in the first weeks of life, although they are cyanotic. In critically ill neonates Prostaglandin E1 infusion (first 0.01-0.1 mcg / kg / min iv) to prevent the closure of the ductus arteriosus before the cardiac catheterization or planned surgical correction or to open it again. Although not always necessary, atrial Ballonseptostomie may be part of a first catheterization to depressurizing the right atrium and improve the atrial right-left shunt when the interatrial connection is not sufficient (Rashkind method). Some children with transposition of the great vessels and heart failure must be treated with medication (diuretics, digoxin, ACH inhibitor; Congenital heart defects include: treatment). A final correction requires multi-stage operations. If the intervention for hypoxemia within the first 4-8 weeks of life is necessary, a modified Blalock-Taussig shunt (compound of systemic to pulmonary artery through a Gore-Tex tube) is performed. Otherwise, would be if the child remains stable with good growth, the first method is a bidirectional Glenn- or hemi-Fontan procedure (anastomosis between the superior vena cava and right pulmonary artery) at an age of 3-6 months. A modified Fontan procedure is performed later, usually between 1 and 2 years. The Fontan procedure is the rerouting of the vena cava blood flow directly to the pulmonary artery by a baffle disposed within the right atrium (lateral tunnel) is created, or an extracardiac conduit which completely bypasses the right atrium, is either used. The proximal pulmonary root is ligated, preventing the anterograde blood flow through the pulmonary output. An appropriate interatrial opening is created if it does not exist in order to allow a compensation of the left and right arteries pressures and a free connection between the chambers. A fenestration (small aperture) is often created between the Fontan pathway and the right atrium. A right-left shunt from Fontan way to the courts and to the left ventricle allows decompression of the systemic venous pressure and an improvement in the cardiac outflow, albeit at the expense of a weak arterial desaturation. This procedure resulted in an increase of early survival rates of> 90%. The survival rate at 5 years is> 80% and with 10 years at> 70%. Procedimiento de Fontan var model = {thumbnailUrl: ‘/-/media/manual/professional/images/fontan_procedure_high_blausen_de.jpg?la=de&thn=0&mw=350’ imageUrl: ‘/ – / media / manual / professional / images / fontan_procedure_high_blausen_de. ? jpg lang = en & thn = 0 ‘, title:’ Procedimiento de Fontan ‘description:’ u003Ca id = “v37897566 ” class = “”anchor “” u003e u003c / a u003e u003cdiv class = “” para “” u003e u003cp u003eLa primera fase del procedimiento de Fontan es un procedimiento de Glenn bidireccional. Se crea una anastomosis entre la vena cava y la arteria pulmonar derecha. Esto redirige el flujo sanguíneo salteando la derecha AURICULA para que la sangre fluya desde la vena cava superior a la arteria pulmonar y luego a los pulmones para oxigenarse (imagen central). La segunda fase del procedimiento de Fontan consiste en derivar el flujo de la vena cava directamente a la arteria pulmonar

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