Chẩn đoán được thực hiện trong giai đoạn trứng với các triệu chứng tím tái, không phát triển mạnh và khó thở exertional. Siêu âm tim, MRI và đặt ống thông tim xác nhận chẩn đoán. Tiên lượng tổng thể là người nghèo, nhưng thay đổi, tùy thuộc vào sự ổn định và đầy đủ của lưu lượng máu phổi. Kéo dài sự tồn tại mà không cần phẫu thuật là khó xảy ra. | 134 Chapter 16 Presentation and course in childhood Diagnosis is made in infancy with symptoms of cyanosis failure to thrive and exertional dyspnea. Echocardiography MRI and cardiac catheterization confirm the diagnosis. The overall prognosis is poor but variable depending upon the stability and adequacy of pulmonary blood flow. Prolonged survival without surgery is unlikely. If pulmonary flow is duct-dependent cyanosis and symptoms worsen as the duct closes. Prostaglandin E1 infusion keeps the duct open until cardiac catheterization and surgery can be performed. Those with adequate but not excessive pulmonary blood flow can survive into adulthood without surgery. This well-balanced circulation occurs infrequently. Most commonly individuals are stable but lack adequate pulmonary blood flow. Long-term survival is guarded unless surgical intervention to improve pulmonary blood flow takes place. Examination Unrepaired patients - Cyanosis. - No murmur or a continuous murmur from a patent ductus or systemic collateral flow to the lungs. - Single S2. Repaired patients - With a valved conduit a pulmonic ejection systolic with or without a regurgitant murmur. - Signs of right heart failure suggesting conduit or pulmonary artery obstruction. - Aortic regurgitation secondary to aortic root dilatation . Useful investigations EKG EKG showing RAD and RVH. Chest radiography boot-shaped cardiac silhouette and usually decreased pulmonary vascularity. Echocardiography similar to tetralogy of Fallot plus absence of direct flow between the right ventricle RV and pulmonary artery PA . Determine conduit function in repaired patients RV function aortic root dilatation and the presence of aortic regurgitation. Cardiac catheterization or MRI or CT to determine size and confluence of pulmonary arteries sources of pulmonary blood flow and pulmonary vascular resistance. Pulmonary Atresia with Ventricular Septal Defect 135 Surgical management Individuals with palliative procedures or .
