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

pulmonary/venous ---- Systemic/arterial 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 right-to-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 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