Mod13 CHD Flashcards by Amanda Haluk

Congenital Heart Disease (CHD)

defect in embryogenesis of the heart that may be severe enough to alter cardiac function

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Anatomical defect usually results from:

altered embryonic development of normal structure

failure of such a structure to progress beyond early stage of embryonic or fetal development

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Exposure of these during critical period are RFs for CDH

anticoagulant Warfarin
Anticonvulsive trimethadione
chronic alcohol abuse
infection with rubella

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Congenital defects allow survival into adult life if:

lesion is mild
multiple lesions counterbalance each other
compensatory mechs take place
lesions have had intervetions (surgery/catherterization)

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Cyanotic CDH

defect that allows blood low in O2 to be shunted from R-L side of heart, bypassing lungs for re-oxygenation; results in baby’s skin having blue tint

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Common Cyanotic Lesions

tetralogy of Fallot
transposition of the great arteries
Ebstein’s anomaly (intermittent cyanosis)
Eisenmenger’s syndrome

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Common Acyanotic lesions

atrial septal defect (ASD)
ventricular septal defect (VSD)
Atrioventricular septal defect 
Patent Ductus arteriosus 
congential aortic stenosis 
pulmonic stenosis 
coarctation of aorta (dissection)

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Atrial Septal Defect (ASD)

opening in the interatrial septum that remains after birth; causing predominantly L-R shunting

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3 main types of ASD

Ostium Secundum (70%) 
Ostium Primum (20%)
Sinus Venosus (10%)

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Ostium Secundum (type of ASD)

occurs where there is inadequate dev of septum primum and/or septum secundum

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Ostium Primum (type of ASD)

occurs when the septum primum in unable to fuse with endocardial cushions

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Sinus Venosus (type of ASD)

occurs when sinus venosus is not completely absorbed in RA; usually found along with anomalous drainage of pulmonary veins

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Patent Foramen Ovale (PFO)

when fusion of atrial septa fails; extremely common type of ASD (25%); 40% of patients with ischemic stroke also have PFO

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Signs and Symptoms of ASD

heart murmur
exertional dyspnea 
fatigue 
atrial arrhythmias 
lower respiratory tract infections 
***may spontaneously close in first 5 years of life

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Diagnosis of ASD

Colour doppler interrogation with Echo or TEE; can see saline bubbles crossing interatrial septum

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Treatment of ASD

Surgical treatment with pericardial or Dacron patch; transcatheter closure

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Ventricular Septal Defect (VSD)

common opening in interventricular septal wall (divides LV and RV) which results in L-R shunting of blood

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5 types of ventricular septal defects

Perimembranous (most common)
Trabecular (2nd most common)
Inlet: associated with atrioventricular septal defects
Outlet: associated with aortic regurgitation
Malalignment- tetralogy of Fallot

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Formation of VSD

deficiency of growth/failure of alignment or fusion of the ventricular muscle septum

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signs/symptoms of VSD

large VSD show early signs of HF, tachypnea, poor feeding, frequent respiratory tract infections and cyanotic

Diagnosis of VSD

harsh holosytolic murmur; colour doppler interrogation with TEE or TTE

Treatment of VSD

surgical treatment with pericardial or Dacron patch or transcatheter closure

Atrioventricular septal defect

spectrum of defects that occur in the embryonic endocardial cushions which can affect the atrial and ventricular septa and parts of atrioventricular valves

3 types of atrioventricular septal defects

partial, complete and incomplete

partial atrioventricular septal defect

ostium primum ASD and cleft mitral valve

Complete atrioventricular septal defect

ostium primum, inlet type VSD, common AV valve

Incomplete atrioventricular septal defect

two AV valve orifices, LV and RA shunt, trileaflet cleft mitral valve

Signs and Symptoms of Atrioventricular septal defect

associated with HF, cyanosis, and Eisenmenger's syndrome

Treatment of Atrioventricular septal defect

closure of ASD or VSD | Repair of AV valve

Patent Ductus Arteriosus (PDA)

persistence of fetal connection of the aorta to the pulmonary artery which is supposed to begin to close within few hours after birth; common in infants whose mothers contacted rubella, who are premature and who are born at high altitudes; will see blood shunting from aorta into pulmonary vascular beds

Formation of Patent ductus arteriosus

normally, due to sudden increase in O2 tension and decrease in protaglandins at birth the ductus will close; when it fails it results in abnormal L-R shunting of blood btw systemic and pulmonary vascular beds

Role of Ductus arteriosus

diverts blood flow away from high resistance pulmonary circuit and into descending aorta

Amount of O2 blood shunted from aorta into pulmonary artery determined by:

cross-sectional area and length of ductus | relative resistance of pulmonary and systemic vasculatures

signs and symptoms of patent ductus arteriosus (PDAs)

HF, Tachycardia, poor feeding, slow growth; may be cyanotic in presence of pulmonary vascular disease will feel fatigue, dyspnea, respiratory tract infections and palpitations

Diagnosis of PDA

continous murmur; doppler colour interrogation with echo

Treatment of PDA

ligration division or placement of clip on ductus | transcatheter closure with an occlude, coil or plug

Congenital Aortic Stenosis

narrowing of aortic valve orifice from abnormal development of valve

Signs and symptoms of Congenital aortic stenosis

dependant on severity; HF can cause tachycardia/pnea, and poor feeding as it worsens: exertional dyspnea, angina, syncope, soft A2, systolic ejection murmur

Diagnosis of congenital aortic stenosis

harsh crescendo-decresendo systolic ejection murmur; but echo is always the best tool

Treatment of congenital aortic stenosis

valvuloplasty | valve replacement

Congenital pulmonic stenosis

obstruction of blood flow from RV to main pulmonary artery during systole; at level of valve, surpravalvular or subvalvular

Diagnosis of congenital pulmonic stenosis

pulmonic ejection click; echo is most useful tool to determine severity, morphology of valve and extent of compensatory mechs

Treatment of congenital pulmonic stenosis

valvuloplasty valve replacement ***same as aortic stenosis

Coarctation of Aorta

constricted segment in aortic lumen; associated with Turner's Syndrome; majority of patients also have Bicuspid Aortic valve (50-80%)

Three types of Coarctations (based on location of narrowing in relation to ductus arteriosus)

Preductal: proximal of ductus Postductal: distal to ductus Juxtaductal: adjacent to ductus

Preductal Coarctation formation:

occurs when there is decrease in blood flow through left side of heart during fetal development (causing underdeveloped aorta); will see cyanosis if they have preexisitng PDA

Postductal coarctation formation:

caused my muscular tissue extending into aorta during fetal development which later constricts

Diagnosis of Coarctation

diagnosed by difference in 15-20mmHg or greater in systolic BP in right arm vs right leg; echo is still best tool

treatment of aortic coarctation

bypass tube graft dacron graft/patch repair surgical resection