Left-to-right-shunts.ppt

Slide Title,Body Text,Second Level,Third Level,Fourth Level,Fifth Level,Slide Title,Body Text,Second Level,Third Level,Fourth Level,Fifth Level,7 yo acyanotic female,Whats the diagnosis?,Atrial Septal Defect,Atrial Septal Defect,Four Major Types,Ostium secundum,Ostium primum,Sinus venosus,Posteroinferior,Atrial Septal Defect,Ostium Secundum Type,Most common is ostium secundum(60%)located at fossa ovalis,High association with prolapse of mitral valve,Right atrium open looking into left atrium through ASD,Normal,Frank Netter,MD Novartis,Atrial Septal Defect,Ostium Primum Type,Ostium primum type usually part of endocardial cushion defect,Frequently associated with cleft mitral and tricuspid valves,Tends to act like VSD physiologically,Looking through ostium primum defect at cleft mitral valve,Proximity of ostium primum defect to tricuspid valve,Frank Netter,MD Novartis,Atrial Septal Defect,Sinus Venosus Type,Sinus venosus type located high in inter-atrial septum,90%association of anomalous drainage of R upper pulmonary vein with SVC or right atrium,Partial anomalous pulmonary venous return,Right atrium open looking into left atrium through ASD,Frank Netter,MD Novartis,Atrial Septal Defect,Posteroinferior Type,Most rare type,Associated with absence of coronary sinus and left SVC emptying into LA,Atrial Septal Defect,Pulmonary Hypertension,Rare in ostium secundum variety(R shunt on atrial level,Right atrium is mildly dilated;RV,LV and LA size are normal,SCMR,Auckland MRI,1 yo acyanotic female,Whats the diagnosis?,Ventricular Septal Defect,Ventricular Septal Defect,General,Most common L,R shunt,Shunt is actually from left ventricle into pulmonary artery more than into right ventricle,Ventricular Septal Defect,Types,Membranous,Supracristal,Muscular,AV canal,Ventricular Septal Defect,Membranous,Membranous=perimembranous VSD(75-80%most common),Location:Posterior and inferior to crista supraventricularis near right and posterior(=non-coronary)aortic valve cusps,Associated with:small aneurysms of membranous septum,Right ventricle opened,Cristasupraventricularis,Membranous VSD,Normal,Frank Netter,MD Novartis,Aneurysm of membranous septum,Normal,Frank Netter,MD Novartis,Ventricular Septal Defect,Supracristal,Supracristal=conal VSD(5%least common),Crista supraventricularis=inverted U-shaped muscular ridge posterior and inferior to the pulmonic valve high in interventricular septum,Right aortic valve cusp may herniate,aortic insufficiency,Ventricular Septal Defect,Muscular,Muscular VSD(510%),Low and anterior within trabeculations of muscular septum,May consist of multiple VSDs=“Swiss-cheese septum”,Swiss cheese,Frank Netter,MD Novartis,Ventricular Septal Defect,AV Canal,Atrioventricular canal=endocardial cushion type=posterior VSD(510%),Location:adjacent to septal and anterior leaflet of mitral valve,Large VSD,pulmonary hypertension,eventually shunt reversal,Eisenmengers physiology,Very large VSD,CHF soon after birth,Large posterior VSD(AV canal),Frank Netter,MD Novartis,Ventricular Septal Defect,Natural History,Natural history of VSD is affected by two factors:,Location of defect,Muscular and perimembranous have high incidence of spontaneous closure,Endocardial cushion defects have low rate of closure,Ventricular Septal Defect,Natural History,Size of the defect,Larger the defect,more likely to,CHF,Smaller the defect,more likely to be asymptomatic,Ventricular Septal Defect,Eisenmenger Physiology,Progressive increase in pulmonary vascular resistance,Intimal and medial hyperplasia,Reversal of L,R shunt to R,L shunt,Cyanosis,Serial chest x-rays may show decrease in size of pulmonary vessels,Ventricular Septal Defect,Clinical Course,Neonates usually asymptomatic because of high pulmonary vascular resistance from birth to 6 weeks,Common cause of CHF in infancy,Bacterial endocarditis may develop,Severe pulmonary hypertension,Eisenmengers physiology/cyanosis,Ventricular Septal Defect,X-ray Findings,Prominent main pulmonary artery,Adult,Shunt vasculature(increased flow to the lungs),LA enlargement(80%),Aorta normal in size,LA,RA,RV,LV,Ventricular Septal Defect,Why Left Atrium Is Enlarged,VSD,Ventricular Septal Defect,Prognosis,Spontaneous closure occurs in 40%during first 2 years of life,60%by 5 years,Ventricular Septal Defect,Indications For Surgery,Greater than 2:1 shunt,surgery required before pulmonary arterial hypertension develops,CHF unresponsive to medical management,Failure to grow,Supracristal defects because of their high incidence of AI,Amersham,Membranous VSD,Auckland MRI,8 mos old acyanotic female,Whats the diagnosis?,Patent Ductus Arteriosus,Patent Ductus Arteriosus,General,Higher incidence in,Trisomy 21,Trisomy 18,Rubella,Preemies,Predominance in females 4:1,Patent Ductus Arteriosus,Anatomy,Ductus connects pulmonary artery to descending aorta just distal to left subclavian artery,Ductus Arteriosus,Frank Netter,MD Novartis,Ductus Arteriosus,Physiology,In fetal life,shunts blood from pulmonary artery to aorta,At birth,increase in arterial oxygen concentration,constriction of ductus,Ductus Arteriosus,Normal ClosureFunctional closure,By 24 hrs of life,Normal anatomic closure,Complete by 2 months in 90%,Closure at 1 year in 99%,Patent Ductus Arteriosus,Pathophysiology,Ductus may persist,Because of defect in muscular wall of ductus,or,Chemical defect in response to oxygen,Anatomic persistence of ductus beyond 4 months is abnormal,Blood is shunted from aorta to pulmonary arteries,Patent Ductus Arteriosus,Clinical,Common cause of CHF in premature infants,Usually at age 1 week(after HMD subsides and pulmonary arterial pressure falls),Wide pulse pressure,Continuous murmur,Patent Ductus Arteriosus,X-ray Findings,Cardiomegaly,Enlarged left atrium,Prominent main pulmonary artery(adult),Prominent peripheral pulmonary vessels,Prominence of ascending aorta,PDA,Patent Ductus Arteriosus,Why Left Atrium Is Enlarged,LA,RA,RV,LV,Patent Ductus Arteriosus,Calcifications,Punctate calcification at site of closed ductus is normal finding,Linear or railroad track calcification at site of ductus may be seen in adults with PDA,Patent Ductus Arteriosus,Prognosis,Spontaneous closure may occur,Patent Ductus Arteriosus,Complications,CHF,Failure to grow,Pulmonary infections,Bacterial endocarditis,Eisenmengers physiology with advanced lesions,Jet of signal loss showing continuous flow from the aorta to the MPA consistent with sizeable PDA,MPA is severely dilated at level of PDA,SCMR,Auckland MRI,Auckland MRI,2 yo old cyanotic female,Whats the diagnosis?,Partial or TotalAnomalous Pulmonary Venous Return,Cyanosis With Increased Vascularity,Truncus types I,II,III,TAPVR,Tricuspid atresia*,Transposition*,Single ventricle,*Also appears on DDx of Cyanosis with,Inc,Vascularity,CHF In Newborn,Impede Return of Flow to Left Heart,Infantile coarctation,Congenital aortic stenosis,Hypoplastic left heart syndrome,Congenital mitral stenosis,Cor triatriatum,Obstruction to venous return from lungs,TAPVR from below diaphragm,Two Types,Partial(PAPVR),Mild physiologic abnormality,Usually asymptomatic,Total(TAPVR),Serious physiologic abnormalities,Normal heart,Return of blood from lungs is by four pulmonary veins to LA,RA,LA,RV,LV,PA,Ao,PAPVR,General,One or two of four pulmonary veins may drain into right atrium,Mild or no physiologic consequence,Associated with ASD,Sinus venosus or ostium secundum types,Partial Anomalous Pulmonary Return,Return of blood from lungs is mostly to LA,One vein abnormally connected to right heart,Frequently associated with sinus venosus or secundum ASD,RA,LA,RV,LV,PA,Ao,PAPVR,Auckland MRI,Korean Journal of Radiology,TAPVR,General,All have shunt through lungs,R heart,All must also have R,L shunt for survival,Obligatory ASD to return blood to the systemic side,All are cyanotic,Identical oxygenation in all four chambers,TAPVR,Types,Supracardiac,Cardiac,Infracardiac,Mixed,TAPVR,Supracardiac TypeType I,Most common(52%),Pulmonary veins drain into vertical vein(behind left pulmonary artery),left brachiocephalic vein,SVC,DDx:VSD with large thymus,Left superior vena cava,Right superior vena cava,Left Brachiocephalic vein,Vertical vein,TAPVR-Supracardiac Type 1,Pulmonary veins,Right atrium,Frank Netter,MD Novartis,TAPVR-Supracardiac Type 1,Frank Netter,MD Novartis,TAPVR,Supracardiac Type 1X-ray Findings,Snowman heart=dilated SVC+left vertical vein,Shunt vasculature 2 increased return to right heart,Enlargement of right heart 2 volume overload,TAPVR-Supracardiac Type 1,RA,LA,RV,LV,PA,Ao,TAPVRType ISupracardiac type,Blood moves through L brachiocephalic v to R SVC,Blood from lungs drains into left vertical veinto L SVC,I,ncreased return to right heart overloads lungs,shunt vessels,ASD provides R,L shunt to allow oxygenated blood to reach body(moderate cyanosis),TAPVR,Cardiac TypeType II,Second most common:30%,Blood from lungs,coronary sinus or RA,Coronary sinus more common,Overload of RV,CHF after birth,Increased pulmonary vasculature,20%of Is and IIs survive to adulthood,Remainder expire in first year,TAPVR-Coronary Sinus-Type II,Coronary sinus,Frank Netter,MD Novartis,TAPVR,Frank Netter,MD Novartis,Pulmonary veins,TAPVRType IICardiac Type,Blood returns from lung to RA or coronary sinus,ASD provides R,L shunt to allow oxygenated blood to reach body(moderate cyanosis)I,ncreased return to right heart overloads lungs,shunt vessels,RA,LA,RV,LV,PA,Ao,TAPVR,Infracardiac TypeType III,Percent of total:12%,Long pulmonary veins course down along esophagus,Empty into portal vein(more common)or IVC,Vein constricted by diaphragm as it passes through esophageal hiatus,TAPVRType III-Infradiaphragmatic,Portal vein,Pulmonary veins,Frank Netter,MD Novartis,TAPVR,Infracardiac TypeContinued,Severe CHF(90%)2 obstruction to venous return,Cyanotic 2 right,left shunt through ASD,Associated with asplenia(80%),or polysplenia,Prognosis=death within a few days,TAPVRType IIIInfracardiac type,ASD provides R,L shunt to allow oxygenated blood to reach body(cyanotic),CHF vasculature,RA,LA,RV,LV,PA,Ao,Blood returning from lungs,pulmonary veins which are constricted by diaphragm,CHF,To portal v,IVC,RA,TAPVR,Mixed TypeType IV,Percent of total:6%,Mixtures of types I III,TAPVR,Frank Netter,MD Novartis,University of Minnesota,The End,