Congenital Heart Dz BEFORE BREAK.pdf

Congenital Heart Disease CHD with L to R shunts: LateR Cyanosis • Acyanosis (pink babies) results in L to R shunt (oxygenated blood shunted back to pulmonary circulation) • Less severe than cyanotic defects • Premed not needed • Acyanotic defects result in volume or pressure overload • increase pulm...

Congenital Heart Disease CHD with L to R shunts: LateR Cyanosis • Acyanosis (pink babies) results in L to R shunt (oxygenated blood shunted back to pulmonary circulation) • Less severe than cyanotic defects • Premed not needed • Acyanotic defects result in volume or pressure overload • increase pulmonary vascular resistance • Leads to Cyanosis (Eisenmenger syndrome) • Atrial septal defects (ASD): cyanotic volume overload • Ventricular septal defects (VSD) cyanotic volume overload • Hypertrophic Cardiomyopathy (HCM) • Patent ductus arteriosus (PDA): cyanotic volume overload • Coarctation of the aorta: pressure overload Atrial Septal Defects (ASD) • L to R atrium • Enlargement of RA, RV, Pulmonary Artery • Types of ASD o Ostium Secundum (most common): At fossae ovale between upper chambers o Ostium Primum: Lower septum o Sinus Venosus: Upper septum near SVC • ASD Associated Diseases o Down syndrome o Holt-Oram o Familial ASD • Pathophysiology of ASD o Pulmonary HTN o Shunt can reverse pushing deoxygenated blood into circulation causing hypoxemia, cyanosis • Treatment of ASD: Need repair Ventricular Septal Defect (VSD) (Most common CHD) • L to R shunting • L side enlargement unlike ASD (R side enlarged) • Increased blood return to L side of heart as result of shunt • Enlargement of LA, LV • Treatment of VSD: Large VSD needs to be repaired • VSD Classification: Membranous is 70% • Large VSDs cause significant L to R shunting increasing chance of irreversible pulmonary HTN Three Groups of VSDs • First Group (small, asymptomatic) • Second Group (moderate defect) o Pulmonary HTN o L sides volume overload • Third Group o Uncorrected large VSDs with Eisenmenger’s complex o Cyonosis present o Pulmonary HTN o Shunt reverses in a R-to-L direction, with deoxygenated blood flowing into the systemic circulation with resultant cyanosis Eisenmenger’s complex: - VSD - Pulmonary HTN - Cyanosis Eisenmenger Syndrome (R to L Shunt)- deoxygenated blood is going into circulation Acyonosis o Uncorrected L-to-R shunt (VSD, ASD, PDA) o Pulmonary HTN o RV hypertrophy o clubbing, and polycythemia. Patient Ductus Arteriosus (PDA) L to R shunt • connects the pulmonary artery to the descending aorta during fetal life • PDA Is normal in utero, closes only after birth due to á O2, â prostaglandin • Increased blood return to L side of heart as result of shunt • Enlargement of LA, LV and Ao • PDA Treatment o In infants, normally PG allow the duct to remain open o Thus, a prostaglandin inhibitor, can close the duct § Indomethacin (Indocin) § Ibuprofen (Motrin) Coarctation of Aorta • Congenital narrowing or constriction of aorta • Assc with Turner’s syndrome (45XO karyotype) • Complications of Coarctation: HTN • Pathophysiology of Aortic Coarctation o Lower extremities is diminished o If the coarctation is not corrected,: § LV hypertrophy § dilatation of collateral blood vessels § Reduced BP in lower extremities CHD with R to L shunts: eaRLy cyanosis • Cyanosis (blue babies) • R to L shunt: deoxygenated blood is shunted into systemic circulation • Cyanotic congenital heart dz (5 T’s) o Truncus Arteriosus (1 vessel) o Transposition (2 vessels) o Tricuspid atresia (3 Tri) o Tetralogy of fallot (4 Tetra) o TAPVR (5 letters) Truncus Arteriosus • Fails to divide pulmonary trunk and aorta o Failure of aorticopulmonary septum formation o Most have accompanying VSD Transposition of Great Arteries (Great vessels are switched) • Aorta arises from RV • Pulmonary artery arises from LV • Great vessels are switched • Prognosis and Tx o Without treatment isolated transposition has a high mortality o better if infants have VSD or large PDA or ASD Congenitally Corrected Transposition of the Great Arteries (Acyanotic) • RV and LV are switched • Vessels are in correct position but RV and LV are switched • Ao and pulmonary artery are normal (providing blood to systemic and pulmonary circulation respectively) Teratology of Fallot (TOF) • 4 COMPONENTS: o Pulmonary infundibular stenosis (most important determinant for prognosis) o RV hypertrophy (RVH)— boot shaped heart o Overriding aorta o VSD • Associated with DiGeorge syndrome – chromosome 22q11.2 microdeletion Total Anomalous Pulmonary Venous Return (TAPVR) • Pulmonary veins drain into R circulation Practice Which congenital heart lesion is associated with Turner syndrome (45, XO)? a. Atrial septal defect b. Ventricular septal defect c. Coarctation of the aorta d. Patent ductus arteriosus Children with which congenital cardiac lesion learn to alleviate their symptoms by squatting? a. Coarctation of the aorta b. Tetralogy of Fallot c. Patent ductus arteriosus d. Transposition of the great arteries