Management of the Neonate With Critical Congenital Heart Disease

Posted by e-Medical PPT Friday, June 25, 2010
Hypoplastic left heart syndrome , is a rare congenital heart defect in which the left side of the heart is severely underdeveloped.It is usually assosiated with aortic atresia/severe stenosis, mitral atresia/severe stenosis, hypoplastic left ventricle and aortic arch.It accounts for 1.5% of congenital heart defects and Most common cause of cardiac related neonatal mortality.It May be diagnosed by fetal ultrasound.The Classic presentation of Hypoplastic left heart syndrome is cardiogenic shock, poor perfusion, decreased pulses, profound metabolic acidosis. May have systolic murmur.
Without life-prolonging interventions,Hypoplastic left heart syndrome is fatal, but with intervention, an infant may survive. A pediatric cardiothoracic surgeon may perform a series of operations or a full heart transplant. In the meantime, the ductus may be kept open to allow blood-flow using prostaglandin. Currently, infants who undergo either the staged reconstructive surgery (Norwood procedure in infancy, Glenn procedure at 3 to 6 months of age, and the Fontan procedure at 3 to 5 years of age) or who undergo cardiac transplantation have a 5-year survival of approximately 60%. The traditional three-stage procedure is a palliative procedure , as the child's circulation is made to work with only two of the heart's four chambers.

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