![]() ![]() Currently, there are two major modalities, primary cardiac transplantation or a series of staged functionally univentricular palliations. Children with the syndrome require surgery as neonates, as they have duct-dependent systemic circulation. ![]() Differential diagnosis includes other left-sided obstructive lesions where the systemic circulation is dependent on ductal flow (critical aortic stenosis, coarctation of the aorta, interrupted aortic arch). ![]() The syndrome can be diagnosed by fetal echocardiography between 18 and 22 weeks of gestation. The most useful diagnostic modality is the echocardiogram. The embryologic cause of the disease, as in the case of most congenital cardiac defects, is not fully known. Often the liver is enlarged secondary to congestive heart failure. The second heart sound is loud and single because of aortic atresia. Usually, no heart murmur, or a non-specific heart murmur, may be detected. As the arterial duct closes, the systemic perfusion becomes decreased, resulting in hypoxemia, acidosis, and shock. #BABIES WITH INTERRUPTED AORTIC ARCH SYNDROME FULL#Newborn infants with the condition generally are born at full term and initially appear healthy. HLHS has been reported to occur in approximately 0.016 to 0.036% of all live births. In addition, the syndrome includes underdevelopment of the left ventricle, aorta, and aortic arch, as well as mitral atresia or stenosis. Hypoplastic left heart syndrome(HLHS) refers to the abnormal development of the left-sided cardiac structures, resulting in obstruction to blood flow from the left ventricular outflow tract. ![]()
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