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THE PEDIATRICS CLERKSHIP - PART 6

Sa van hai lá sinh lý bệnh gây ra bởi tờ rơi dày và dư thừa lồi vào Vòng tròn hai lá van. DỊCH Thường xảy ra ở trẻ lớn và thanh thiếu niên. Có một thành phần gia đình (tính trạng trội). Gần như tất cả các bệnh nhân với hội chứng Marfan có nó. DẤU HIỆU VÀ TRIỆU CHỨNG nghe tim thai: | Mitral Valve Prolapse Pathophysiology Caused by thick and redundant valve leaflets that bulge into the mitral annulus. Epidemiology Usually occurs in older children and adolescents. Has a familial component autosomal dominant . Nearly all patients with Marfan s syndrome have it. Signs and Symptoms Auscultation midsystolic click and late systolic murmur Often asymptomatic with some history of palpitations and chest pain Treatment Management is symptomatic e.g. p blocker for chest pain . I OTHER CONGENITAL CARDIOVASCULAR DEFECTS Cardiovascular Disease Coarctation of the aorta is associated with Turner s syndrome. Coarctation of the Aorta Pathophysiology Most commonly found in the juxtaductal position where the ductus arteriosus joins the aorta . Development of symptoms may correspond to the closure of the ductus arteriosus the patent ductus provides additional room for blood to reach the postductal aorta . Epidemiology More common in males than females male-to-female ratio 2 1 Seen in one third of patients with Turner s syndrome Signs and Symptoms Clinical Presentation of Symptomatic Infants Failure to thrive respiratory distress and CHF develop in the first 2 to 3 months of life. Lower extremity changes decreased pulses in the lower extremities. Acidosis may develop as the lower body receives insufficient blood. Usually a murmur is heard over the left back. Clinical Presentation of Asymptomatic Infants or Children Normal growth and development Occasional complaint of leg weakness or pain after exertion Decreased pulses in the lower extremities Upper extremity HTN or at least greater than in the lower extremities Diagnosis CXR 3 sign dilated ascending aorta that displaces the superior vena cava to the right see Figure 13-9 . Treatment Resection of the coarctation segment with end-to-end anastomosis is the intervention of choice for initial treatment. Allograft patch augmentation can also be used. 212 FIGURE 13-9. Coarctation of the aorta. Artwork by Dr. John .