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Congenital Cardiac Lesions Overview Three Shunts of Fetal Circulation Ductus Arteriosus Ductus Venosus Protects lungs against circulatory overload Allows RV to strengthen High pulmonary vascular resistance, low pulmonary blood flow Carries moderately saturated blood Connects umbilical vein to IVC Flow regulated via sphincter Conducts highly oxygenated blood Foramen Ovale Shunts highly oxygenated blood from RA to LA Circulatory Changes at Birth Aeration of Lungs at Birth Decreased pulmonary vascular resistance secondary to lung expansion Increase in pulmonary blood flow- raising LA pressure to higher than that of the IVC Thinning of walls of PA secondary to stretch as lungs increase in size with first few breaths Changes Associated with First Breath Alveoli open Pressure in pulmonary tissues decrease Pressure in R. heart decreases Pressure in the L. heart increases as blood returns from highly vascularized pulmonary tissue to the LA Fate of the shunts… Foramen Ovale: Ductus Arteriosus: Closes at birth due to decreased flow from placenta and IVC Pulmonary venous return causes pressure in LA to be higher than that in RA Due to decreased pulmonary vascular resistance, PA pressure falls below systemic pressure and blood flow through DA is diminished Closure mediated by bradykinin Prostaglandin E2 may reopen DA Umbilical Vessels Constrict at birth and are then tied and cut The Normal Heart Right-Sided Heart Lesions Other right-sided cardiac abnormalities that may present with or without cyanosis include: Pulmonary Valve and Infundibular Stenosis Pulmonary Regurgitation Absence of the pulmonary valve Pulmonary Artery Stenosis Tricuspid Stenosis Double-chambered right ventricle Ebstiens anomaly Ebsteins Anomaly Defect that mainly affects the tricuspid valve. Tricuspid valve may be malformed. The tricuspid valve is located lower than normal The upper part of the right ventricle is part of the right atrium, making the right ventricle is too small and the right atrium is too large. Abnormal leaflets may let blood leak back into the atrium after it has flowed into the ventricle. The backward flow of blood makes the atrium even larger and the ventricle even smaller. Often Associated with other heart lesions ASD Pulmonary Stenosis Pulmonary Atresia Treatment The mainstays of treatment for cyanotic heart lesions are oxygen and prostaglandin. PGE1 serves to reopen the ductus arteriosus or prevent it from closing, which allows partially desaturated blood to enter PA and be oxygenated. Initial dose of PGE1 is 0.1 mg/kg/min; can be reduced to 0.02-0.05 mg/kg/min when patient is stable. Adverse Effects of PGE1 are rare, including apnea, hypotension, edema, and fever. Treatment- continued General procedure for cyanotic heart lesions involves a systemic to PA shunt. Procedure known as the Blalock-Taussig shunt. Uses a small Gore-Tex® shunt to connect either left or right subclavian to left or right branch PA. Allows partially desaturated blood to enter PA, increasing pulmonary blood flow and oxygenation