Congenital Heart Defects

Acyanotic- ASD,VSD, PDA, CoA

Cyanotic- truncus arteriosus, TGA, tricuspid atresia, TOF, TAPVR, HLHS, DORV, DILV

Truncus Arteriosus- blood from left ventricle, and right ventricle out in one vessel, treatment is anti hypertensive if CHF, calcium, associated with digeorge syndrome

HLHS- hypoplasia LV, severe aortic stenosis, and or mitral valve, hypoplasia of ascending aorta and arch, ductal dependent oxygenated blood to the body. LOW CO!

s/s of HLHS: mild cyanosis, tachycardia, tachyons, crackers, metabolic acidosis

Surgeries for HLHS

#1: BT SHUNT OR RV-PA CONDUIT / Norwood sano-shunt: creates a new aorta, BT OR RV-PA provides blood flow to lungs

#2: Bidirectional Glenn: Blood flow is based on pathway to least resistance, blood goes to lungs and heart, less blood flow to the lungs = low spo2; less blood flow to the body=hypotension; SVC attached to pulmonary artery =blood from upper body to the lungs

#3: Fontan procedure (~age 1.5-3yo): IVC disconnected from heart and attached to pulmonary artery (all deoxygenated blood bypass the heart)

Tricuspid atresia: tricuspid valve missing (r lower chamber/ ventricle is small): blood must flow through holes like ASD VSD so blood can go to the lungs and body)

Pulmonary atresia with IVS: pulmonary valve fails to form, blocking blood flow from right ventricle to the lungs; baby relies on opening of ASD or PFO

TOF: 4 different defects: VSD, PULM STENOSIS(narrowing Pulm valve) OVERRIDING AORta, R VENTRICLE HYPERTROPY. s/s cyanosis, SOB, FTT, irritable, TET spells (sudden cyanosis with eating or crying)

TAPVR: ALL 4 pulmonary veins do not connect normally to the left atrium, instead 4 pulmonary veins drain abnormally to the right atrium thru an abnormal anomalous connections = mixing with deoxygenated Blood; SNOWMAN ON CXR; SATS lower than normal with no obstruction and really low sats if there’s a PA obstruction; treatment lasix, O2

TGA: aorta arising from the right ventricle, pulmonary artery arising from left ventricle; cyanosis CHF in newborn, poor feeding, tachypnea, murmur of VSD. Hypoxia and acidosis; egg shaped heart; prostin and O2 (lowers PVR; expect lower sats) treat acidosis, hypoglycemia and hypocalcemia( treatment is arterial switch

ASD: hole in septum wall in the two upper chambers allowing oxygen rich blood from left atrium to right atrium = increase blood flow to the lungs

PDA: ductus arteriosus a vessel that allows blood to bypass lungs in a fetus fails to close after birth = o2 rich blood from aorta mixing with oxygen poor blood from PA

CoA: narrowing of the aorta (normal sats); lethargy poor feeding; FTT