One of the rarest of congenital cardiac defects is Hypoplastic Left Heart Syndrome, HLHS. This syndrome is described as a collection of anomalies affecting the left heart and connecting structures. Due to advancements in surgical intervention mortality rates in neonates with Hypoplastic Left Heart Syndrome have decreased. Management of these patients requires a symphony of multidisciplinary teams including fetal and pediatric cardiologist, cardiac surgeons, neonatologist, pulmonologist, nutritionist and family counselors. Options currently comprise of heart transplant, three-staged palliative surgery and comfort care. There may be co-morbidities present which further complicate treatment, but with surgical intervention the prognosis of this once deemed fatal condition is improved. This paper will examine the anatomy and abnormal function of the hypoplastic left heart, three staged surgical intervention and management of patient with HLHS.
Hypoplastic left heart syndrome is a congenital heart defect where the structures in the left heart are severely underdeveloped. The left ventricle, aorta and aortic arch are unable to adequately pump and deliver blood to the body due to their size. Aortic and mitral valve stenosis or atresia is present and in some cases atrial septal defect accompanies. (CDC) Normal fetal circulation involves the use of three shunts that act as liver, heart and lung bypass in order to supply the systemic circulation of oxygen rich blood. Fetal blood is oxygenated by the placenta and travels to the fetus via the umbilical vein. The ductus venous the first shunt which encountered and channels oxygenated blood pass the liver. The shunted blood is then carried via the inferior...
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...on is as follows; oxygen-poor blood returns directly to the lungs via the new connection between the pulmonary arteries and the superior and inferior vena cava. Gas exchange occurs in the lungs which then return oxygen-rich blood via the pulmonary arteries
Superior and inferior vena cava carry deoxygenated blood directly to the lungs by dumping into the pulmonary artery. Blood is then returned to the heart by the pulmonary veins to the left and right atria which are now connected then to the right ventricle which functions as the main pumping chamber. Blood exits the heart via the reconstructed aorta which has been attached to the pulmonary artery trunk.
The circulatory pathway is not restored to normal but redirected to ensure adequate gas exchange in the lungs as well as creating a sufficient pump in the right ventricle to support adequate systemic circulation.
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