The persistent truncus arteriosus (ductus arteriosus) is produced during fetal development when the original trunk does not share in the pulmonary artery and aorta. It results from a large, single arterial truncus riding on a large, perimembranous ventricular septal defect. As a result, flows a mixture of oxygenated and nichtoxygeniertem blood into the general circulation, the pulmonary artery or coronary circulation. The symptoms include cyanosis and heart failure with poor eating habits, diaphoresis and tachypnea. 1. A normal heart sound (S1) and a pure, single 2. heart sound (S2), and are often, the murmur can vary. The diagnosis is made by echocardiography or cardiac catheterization. Heart failure is only with medication, then treated surgically.

The persistent truncus arteriosus (truncus arteriosus.) Accounts for 1-2% of congenital heart defects. Approximately 35% of patients have the deletion syndrome 22q11, which includes a DiGeorge syndrome and a velokardiofaziales syndrome.

The persistent truncus arteriosus (ductus arteriosus) is produced during fetal development when the original trunk does not share in the pulmonary artery and aorta. It results from a large, single arterial truncus riding on a large, perimembranous ventricular septal defect. As a result, flows a mixture of oxygenated and nichtoxygeniertem blood into the general circulation, the pulmonary artery or coronary circulation. The symptoms include cyanosis and heart failure with poor eating habits, diaphoresis and tachypnea. 1. A normal heart sound (S1) and a pure, single 2. heart sound (S2), and are often, the murmur can vary. The diagnosis is made by echocardiography or cardiac catheterization. Heart failure is only with medication, then treated surgically. The persistent truncus arteriosus (truncus arteriosus.) Accounts for 1-2% of congenital heart defects. Approximately 35% of patients have the deletion syndrome 22q11, which includes a DiGeorge syndrome and a velokardiofaziales syndrome. Truncus arteriosus. The original trunk is not divided into the pulmonary artery and the aorta. This results in a large, single arterial truncus riding on a large ventricular septal defect. 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. Classification Various classification systems will used. The first classification (Collett and Edwards) is Type I: The Hauptpulmonalarterie arises from the trunk, and then splits into a right and left pulmonary artery. Type II: The right and left pulmonary artery originate separately (but next to each other) from the rear part of the trunk. Type III: The right and left pulmonary arteries arise from the sides of the truncal artery and are relatively far apart. Type IV: Both pulmonary arteries are supplied by collaterals of the descending aorta. (Type IV is now reclassified as tetralogy of Fallot with pulmonary atresia.) An updated classification of van Praagh consists of type A (truncus arteriosus with ventricular septal defect [VSD]) and the very rare Type B (truncus arteriosus without VSD). Type A is divided into 4 types: Type A1: The Hauptpulmonalarterie arises from the trunk, and then splits into a right and left pulmonary artery. Type A2: The right and left pulmonary artery originate separately from the rear part of the trunk. Type A3: A lung is powered by a pulmonary artery branch that emerges from the trunk, and the other lung (usually the left) is supplied by a duct-like collateral. Type A4: The trunk is a large pulmonary artery and the aortic arch is interrupted or coarctation of the aorta. The trunk-lid can be quite abnormal and manifest with stenosis, insufficiency or both. Other malformations (z. B. right aortic arch, interrupted aortic arch, malformations of the coronary arteries, atrial septal defect) may also be present and also contribute to the high mortality. The physiological consequences of truncus arteriosus are slight cyanosis, severe pulmonary circulatory stress and heart failure. Symptoms and complaints newborn with a type I have a mild cyanosis and signs of heart failure (tachypnea, poor eating habits, diaphoresis) in the first weeks of life. On clinical examination, one finds a precordial impulse, well palpable pulses, a loud, single second heart sound (S2) and a Auswurfton. A holosystolic sound of Grade 2-4 / 6 along the left sternum can be heard. A mittsystolisches murmur over the apex can be heard at an elevated pulmonary blood flow. In valve insufficiency a high-frequency diastolic decrescendo over the center of the left sternum can be heard. Diagnostic chest X-ray and ECG echocardiography Occasionally, cardiac catheterization, cardiac MRI or CT angiography Diagnosis is suspected clinically, supported confirmed by a chest x-ray absorption and an EKG and by two-dimensional Farbechokardiographie and Doppler sonography. A cardiac catheterization is occasionally necessary to detect associated abnormalities before surgery. However, the cardiac MRI or CT angiography can minimize the need for catheterization. Radiographs show various dimensions of a cardiomegaly with increased lung vascular pattern, a right aortic arch (about 30%) and a relatively high outlet of the pulmonary arteries. The ECG can be found a combined ventricular hypertrophy. The pronounced pulmonary circulatory stress can cause signs of left atrial enlargement. Treatment Surgical correction Medical treatment of heart failure (eg. As with diuretics, digoxin, ACE inhibitors) before surgery Heart failure is first aggressively with diuretics, digoxin and ACE inhibitors, later treated with surgical correction. The infusion of prostaglandins does not help. The surgical procedure consists of a complete correction. The VSD is closed, so that the blood from the left ventricle into the artery trunkale flows. A conduit with or without a lid is placed between the right ventricle and the confluence of the two pulmonary arteries. The perioperative mortality was reduced in recent years to 10%. Since the conduit is laid in early childhood, its size will be too slow with age and growth of the child and must be adjusted in the course of childhood. Zweigpulmonalarterienstenose is a common consequence. Endocarditis prophylaxis preoperatively recommended but is only required for the first 6 months after surgery, unless there is a residual defect in addition to a surgical patch or prosthetic material before. Important Points In persistent truncus arteriosus, the original trunk does not share on into the pulmonary artery and the aorta. This results in a large, single arterial truncus riding on a large ventricular septal defect (VSD). Various types are distinguished based on the origin of the pulmonary artery and the associated defects. The patients present with mild cyanosis, significant pulmonary About circulation and heart failure (HF) before; a degree of 2 to 4/6 systolic murmur can be heard along the left sternal border and a mittdiastolisches mitral flow noise may be heard at the top. Heart failure is treated with diuretics, digoxin and ACE inhibitors; a prostaglandin infusion does not help. Surgical repair is made early; one or more revisions are needed usually when the children grow.

Comments

Leave a Reply

Sign In

Register

Reset Password

Please enter your username or email address, you will receive a link to create a new password via email.