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Congenital Heart Disease
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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
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pulmonary/venous ---- Systemic/arterial
right-to-left shunt: pulmonary/venous ---- Systemic/arterial Deoxygenated Oxygenated --- cyanosis Hypoxia clubbing (hypertrophic osteoarthropathy) paradoxical embolism
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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
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left-to-right shunt: - m.C - ASD, VSD (m.c) , PDA, AVSD
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right-to-left shunt: Tetralogy of Fallot m.c Transposition of the Great Arteries persistent truncus arteriosus Tricuspid atresia total anomalous pulmonary venous connection
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Obstructive Lesions: aortic or pulmonary valve stenosis or atresia coarctation of the aorta subpulmonary stenosis in TOF
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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
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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 right-to-left shunting
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Ventricular Septal Defect
Membranous (m.c), muscular, infundibular The functional consequences of a VSD depend on the size
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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
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atrioventricular (AV) septal defect
Endocardial cushion defect Down syndrome
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Tetralogy of Fallot VSD (2) subpulmonary stenosis/infundibulum
(3) an aorta that overrides the VSD (4) Right ventricular hypertrophy
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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.
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persistent truncus arteriosus
failure of separation of truncus into aorta and pulmonic trunck . The truncus overrides both ventricles. Always accompanied by a membranous VSD.
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total anomalous pulmonary venous return (TAPVR)
pulmonary veins----innominate vein/coronary sinus Patent foramen ovale/ or ASD always present
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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
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Aortic Stenosis and Atresia
Valvular--- hypoplastic left heart syndrome Supravalvular Subvalvular----- sudden death with exertion
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