विवरण
Transposition of the Great Arteries (TGA) is a congenital heart defect where the two main arteries that carry blood away from the heart, the aorta and the pulmonary artery, are switched in position. In a normal heart, the aorta arises from the left ventricle and carries oxygen-rich blood to the body, while the pulmonary artery arises from the right ventricle and carries oxygen-poor blood to the lungs. However, in TGA, the positions of these two arteries are reversed. TGA occurs during fetal development when the arteries do not form properly. The exact cause is unknown, but genetic and environmental factors may play a role. Babies with TGA may exhibit symptoms shortly after birth, such as cyanosis (bluish skin due to lack of oxygen), rapid breathing, and poor feeding. TGA is typically diagnosed using prenatal ultrasound or shortly after birth through physical examination and tests like echocardiography and cardiac catheterization.
सारांश
- Transposition of the Great Arteries (TGA) is a cyanotic congenital heart defect where the aorta arises from the right ventricle and the pulmonary artery from the left ventricle, leading to parallel rather than serial circulation. This condition is incompatible with life unless there is mixing of oxygenated and deoxygenated blood via an atrial septal defect (ASD), ventricular septal defect (VSD), or patent ductus arteriosus (PDA). The severity of cyanosis depends on the degree of mixing.
- Without intervention, the natural history of TGA involves hypoxia, acidosis, and potentially congestive cardiac failure (CCF). Most infants do not survive beyond six months without treatment. The presence and size of mixing defects such as ASD, VSD, and PDA significantly impact the clinical course.
- Diagnosis involves a thorough history, physical examination, arterial blood gas (ABG) analysis, and echocardiography, the gold standard for visualizing the transposed vessels and associated defects. ECG can show right axis deviation and right ventricular hypertrophy. Chest X-rays may exhibit a characteristic "egg on a string" appearance.
- Management aims to establish safe oxygen levels and stable cardiac/pulmonary function. Prostaglandin E1 infusion is used to maintain ductal patency if the PDA is the primary source of mixing. Balloon atrial septostomy (BAS) can improve mixing and stabilize the infant before definitive surgery.
- Surgical management involves the arterial switch operation (ASO), ideally performed within the first one to three weeks of life. This procedure involves transecting the aorta and pulmonary artery, transferring the coronary arteries to the pulmonary artery root (which becomes the neo-aorta), and re-anastomosing the great vessels. The presence of VSD or pulmonary stenosis may alter the surgical approach.
- In cases of delayed presentation or significant left ventricular regression, staged procedures or atrial switch operations (Senning or Mustard procedures) may be considered. These procedures reroute blood flow at the atrial level, although they are associated with long-term complications such as right ventricular dysfunction, arrhythmias, and baffle obstructions. Post-operative complications of ASO include coronary artery occlusion, great vessel stenosis, and neoaortic insufficiency. Extracorporeal membrane oxygenation (ECMO) may be needed for post-operative support if the left ventricle is not prepared.
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