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Transcript
Congenital Heart Disease • congenital heart disease can be organized into three major categories: Malformations causing a left-to-right shunt Malformations causing a right-to-left shunt Malformations causing an obstruction A shunt is an abnormal communication between chambers or blood vessels • right-to-left shunt: pulmonary/venous ---- Systemic/arterial Deoxygenated ------ Oxygenated --- cyanosis Hypoxia ----- clubbing (hypertrophic osteoarthropathy) paradoxical embolism • left-to-right shunt: Systemic/arterial ---- pulmonary/venous Oxygenated ----------- deoxygenated--- no cyanosis right ventricular hypertrophy Pulmonary congestion ------ hypertension Rt-to-left shunt (Eisenmenger syndrome)--- cyanosis • left-to-right shunt: - m.C - ASD, VSD (m.c) , PDA, AVSD • right-to-left shunt: Tetralogy of Fallot ------- m.c Transposition of the Great Arteries persistent truncus arteriosus Tricuspid atresia total anomalous pulmonary venous connection • Obstructive Lesions: aortic or pulmonary valve stenosis or atresia coarctation of the aorta subpulmonary stenosis in TOF ASD • Defect in septum primum or secundum Mortality is low, and long-term survival is comparable to that of the normal population. Asymptomatic until adulthood Patent Foramen Ovale • foramen ovale/ostium secundum permits continued right-to-left shunting of blood during intrauterine development. • the unsealed flap can open if right-sided pressures become elevated (coughing, /sneezing) can produce brief periods of rightto-left shunting Ventricular Septal Defect • Membranous (m.c), muscular, infundibular • The functional consequences of a VSD depend on the size Patent Ductus Arteriosus • ductus arteriosus---pulmonary artery---aorta • During intrauterine life, it permits blood flow from the pulmonary artery to the aorta, thereby bypassing the unoxygenated lungs. • + prostaglandin, immuture • Used to save life in pulmonary or oartic valve obstruction or atresia atrioventricular (AV) septal defect • Endocardial cushion defect • Down syndrome Tetralogy of Fallot (1) VSD (2) subpulmonary stenosis/infundibulum (3) an aorta that overrides the VSD (4) Right ventricular hypertrophy Transposition of the Great Arteries • Aorta---anterior---right ventricle • pulmonary artery---posterior---left ventricle • Parallel instead of series • incompatible with postnatal life unless a shunt exists for adequate mixing of blood. persistent truncus arteriosus • failure of separation of truncus into aorta and pulmonic trunck . • The truncus overrides both ventricles. • Always accompanied by a membranous VSD. total anomalous pulmonary venous return (TAPVR) • pulmonary veins----innominate vein/coronary sinus • Patent foramen ovale/ or ASD always present Coarctation of the Aorta • Narrowing • 2 types 1- infantile: + PDA---- early after birth--- cyanosis in lower ½ of body 2- adult: - PDA---- late presentation---- HTN in upper extremities Aortic Stenosis and Atresia • Valvular--- hypoplastic left heart syndrome • Supravalvular • Subvalvular----- sudden death with exertion