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Congenital Heart Dz


Percentage of babies born with heart defect 1%
Most common congenital heart malformation VSD (30-60% of all CHDs; 2-6/1000 live births)
second most common congenital heart malformation atrial septal defect (30-40% of all CHDs)
name the two anatomic right-to-left intracardiac / great vessel shunts in utero foramen ovale, ductus arteriosus
Location of the ductus arteriosus small projection connecting the pulmonary artery to the aorta, bypassing the pulmonary circulation
Location of the ductus venosus shunt from the umbilical vein to the inferior vena cava, bypassing the liver
Major signs and symptoms of congenital heart disease cyanosis, dyspnea/tachypnea, syncope, failure to thrive, edema/ascites
lab finding consistent with cyanosis low oxygen sat, elevated hem
Initial imaging modality of choice in suspected congenital heart disease echo
Eisenmenger's syndrome phenomenon occurring with long standing L to R shunts in which increasing pulmonary arterial pressures and flow rate result in shunt reversal to R to L
Location of ASD hole between the right and left atria
3 types of ASD sinus venosus, ostium secundum (most common type: 6-10% of all CHDs), ostium primum
Most commonly diagnosed congenital cardiac defect VSD
Location of VSD hole between the right and left ventricles
Most common of the mixed shunts transposition of the great arteries
Anatomical outcome in transposition of the great arteries two separate closed systems
The two anatomical abnormalities that must be present in order for a patient to survive transposition of the great arteries: ASD, PDA
Components of truncus arteriosus single trunk from the heart, VSD
Tetralogy of Fallot consists of: pulmonic stenosis, VSD, overriding aorta, RV stenosis & obstruction, RV hypertrophy
Anatomical abnormality that must be present for survival in tetralogy of Fallot patent ductus arteriosus
Anatomical abnormality in tricuspid atresia hypoplasia of the R ventricle
What keeps a PDA open? prostaglandin E2 (inhibitors will close it)
Post common congenital obstructing lesion pulmonary stenosis
Prominent physical findings in coarctation of the aorta Differential BP (lower in legs), delayed femoral pulse compared with brachial; FTT; bilateral claudication; HTN; HA; S4; AS & AR murmurs
Anatomical cause of coarctation of the aorta complication due to closure of the ductus arteriosus; usually located distal to left subclavian artery
Most rare congenital heart disease mitral valve disease
Most common cyanotic heart defect (R to L shunt) Tetralogy of Fallot
Shunting in ASD & VSD is typically: left to right (TEE is TOC to determine)
ASD clinical features often asx until adult; possible failure to thrive; DOE & fatigue (possible pHTN); RV lift; murmur; clubbing; cyanosis
VSD clinical features FTT in infancy, dyspnea, JVD, LLSB thrill, S3, clubbing, cyanosis
Coarctation of aorta on CXR: rib notching
PDA may result from: maternal rubella
PDA clinical features FTT; recurrent resp infxns; fatigue; DOE; angina & syncope in adults. Bounding pulse; wide pulse pressure and loud S2
Small boot-shaped heart in pediatric patient = tetralogy of Fallot
tetralogy of Fallot: CBC finding = polycythemia
Types of acyanotic CHD (6) ASD, VSD, PDA, coarctation of aorta, aortic stenosis (bicuspid valve), pulmonic stenosis
Cyanotic ductal-independent CHD truncus, transposition, TAPVR
Cyanotic ductal-dependent pulmonary-flow CHD Tricuspid atresia, tetralogy, Ebstein
Cyanotic ductal-dependent systemic-flow CHD HLHS
How many patients with ASD develop symptoms? 70% have sxs by their 40s (DOE, fatigue, palps, syncope)
In a teen/young adult with HTN, need to rule out: coarctation of aorta
In 50% of cases of coarctation of aorta, one also finds: bicuspid valve (also increased incidence of cerebral berry aneurysm)
Coarctation of aorta mgmt: Prostaglandin E1; usually surgery (balloon dilation of segment)
What closes a PDA? Prostaglandin inhibitors (eg, indomethacin; thus, NSAIDs are contraindicated in PG)
Imaging test for PDA: MRI, CT, or CTA >> TTE
Tet spells = sudden, marked increase in cyanosis followed by syncope (may result in hypoxic brain injury & death); older kids often squat during spell to improve cerebral blood flow
Created by: Adam Barnard Adam Barnard